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Sample records for vertebral sarcoidosis demonstration

  1. Vertebral sarcoidosis: long-term follow-up with MRI

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    Lefere, M. [University Hospitals Gasthuisberg, Department of Radiology, Leuven (Belgium); Larbi, A.; Malghem, J.; Vande Berg, B.; Dallaudiere, B. [University Hospitals St Luc, Department of Radiology, Brussels (Belgium)

    2014-08-15

    Vertebral involvement in sarcoidosis is rare and its clinical and imaging features are non-specific. Indeed, because the lesions are hard to differentiate from metastatic disease based on imaging alone, a histological confirmation is advised. Fatty replacement is a well-known finding indicating stabilization and healing in both benign and malignant conditions. It can be used as an indicator of a favorable disease course and response to treatment. We report the case of a 43-year-old woman with multifocal vertebral sarcoidosis lesions and long-term follow-up showing progressive and gradual fatty involution on magnetic resonance imaging (MRI) during 4 years of steroid treatment with a final favorable outcome. (orig.)

  2. Sarcoidosis

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    Sarcoidosis is a disease that leads to inflammation, usually in your lungs, skin, or lymph nodes. It starts as tiny, grain-like lumps, called granulomas. Sarcoidosis can affect any organ in your body. No ...

  3. Sarcoidosis

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    Nilgün Solak Tekin

    2012-09-01

    Full Text Available Sarcoidosis is a multisystemic, inflammatory disease of unknown etiology that is characterized by noncaseating granulomas. Approximately, one-third of the patients with systemic sarcoidosis are observed cutaneous manifestations of different morphologies. Skin sarcoidosis have extremely heterogeneous clinical picture, so it is defined as “The Great Imitator” or “clinical chameleon”. (Turk J Dermatol 2012; 6: 80-6

  4. Sarcoidosis

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    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  5. Sarcoidosis.

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    Valeyre, Dominique; Prasse, Antje; Nunes, Hilario; Uzunhan, Yurdagul; Brillet, Pierre-Yves; Müller-Quernheim, Joachim

    2014-03-29

    Sarcoidosis is a systemic disease of unknown cause that is characterised by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system. Studies show that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in individuals who are genetically susceptible. Several new insights have been made, particularly with regards to the diagnosis and care of some important manifestations of sarcoidosis. The indications for endobronchial ultrasound in diagnosis and for PET in the assessment of inflammatory activity are now better specified. Recognition of unexplained persistent disabling symptoms, fatigue, small-fibre neurological impairment, cognitive failure, and changes to health state and quality of life, has improved. Mortality in patients with sarcoidosis is higher than that of the general population, mainly due to pulmonary fibrosis. Predicted advances for the future are finding the cause of sarcoidosis, and the elucidation of relevant biomarkers, reliable endpoints, and new efficient treatments, particularly in patients with refractory sarcoidosis, lung fibrosis, and those with persistent disabling symptoms. Copyright © 2014 Elsevier Ltd. All rights reserved.

  6. Sarcoidosis

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    Valeyre Dominique

    2007-11-01

    Full Text Available Abstract Sarcoidosis is a multisystemic disorder of unknown cause characterized by the formation of immune granulomas in involved organs. It is an ubiquitous disease with incidence (varying according to age, sex, race and geographic origin estimated at around 16.5/100,000 in men and 19/100,000 in women. The lung and the lymphatic system are predominantly affected but virtually every organ may be involved. Other severe manifestations result from cardiac, neurological, ocular, kidney or laryngeal localizations. In most cases, sarcoidosis is revealed by persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum. Abnormal metabolism of vitamin D3 within granulomatous lesions and hypercalcemia are possible. Chest radiography is abnormal in about 90% of cases and shows lymphadenopathy and/or pulmonary infiltrates (without or with fibrosis, defining sarcoidosis stages from I to IV. The etiology remains unknown but the prevailing hypothesis is that various unidentified, likely poorly degradable antigens of either infectious or environmental origin could trigger an exaggerated immune reaction in genetically susceptible hosts. Diagnosis relies on compatible clinical and radiographic manifestations, evidence of non-caseating granulomas obtained by biopsy through tracheobronchial endoscopy or at other sites, and exclusion of all other granulomatous diseases. The evolution and severity of sarcoidosis are highly variable. Mortality is estimated at between 0.5–5%. In most benign cases (spontaneous resolution within 24–36 months, no treatment is required but a regular follow-up until recovery is necessary. In more serious cases, a medical treatment has to be prescribed either initially or at some point during follow-up according to clinical manifestations and their evolution. Systemic corticosteroids are the mainstay of treatment of sarcoidosis. The minimal duration of

  7. Sarcoidosis Th17 Cells are ESAT-6 Antigen Specific but Demonstrate Reduced IFN-γ Expression

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    Richmond, Bradley W.; Ploetze, Kristen; Isom, Joan; Chambers-Harris, Isfahan; Braun, Nicole A.; Taylor, Thyneice; Abraham, Susamma; Mageto, Yolanda; Culver, Dan A.; Oswald-Richter, Kyra A.; Drake, Wonder P.

    2013-01-01

    Rationale Sarcoidosis is a granulomatous disease of unknown etiology. Many patients with sarcoidosis demonstrate antigen-specific immunity to mycobacterial virulence factors. Th-17 cells are crucial to the immune response in granulomatous inflammation, and have recently been shown to be present in greater numbers in the peripheral blood and bronchoalveolar lavage (BAL) fluid (BALF) of sarcoidosis patients than healthy controls. It is unclear whether Th-17 cells in sarcoidosis are specific for mycobacterial antigens, or whether they have similar functionality to control Th-17 cells. Methods Flow cytometry was used to determine the numbers of Th-17 cells present in the peripheral blood and BALF of patients with sarcoidosis, the percentage of Th-17 cells that were specific to the mycobacterial virulence factor ESAT-6, and as well as to assess IFN-γ expression in Th-17 cells following polyclonal stimulation. Results Patients with sarcoidosis had greater numbers of Th-17 cells in the peripheral blood and BALF than controls and produced significantly more extracellular IL-17A (p=0.03 and p=0.02, respectively). ESAT-6 specific Th-17 cells were present in both peripheral blood and BALF of sarcoidosis patients (psarcoidosis patients produced less IFN-γ than healthy controls. Conclusions Patients with sarcoidosis have mycobacterial antigen-specific Th-17 cells peripherally and in sites of active sarcoidosis involvement. Despite the Th1 immunophenotype of sarcoidosis immunology, the Th-17 cells have reduced IFN-γ expression, compared to healthy controls. This reduction in immunity may contribute to sarcoidosis pathogenesis. PMID:23073617

  8. Sarcoidosis.

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    Wu, Jennifer J; Schiff, Karin Rashcovsky

    2004-07-15

    Sarcoidosis is a systemic granulomatous disease of unknown cause affecting young and middle-aged adults. Patients commonly present with bilateral hilar lymphadenopathy, pulmonary infiltrates, and ocular and skin lesions. The heart, liver,spleen, salivary glands, muscles,bones, kidneys, and central nervous system also may be involved. Diagnosis is based on clinicoradiologic findings plus histologic evidence of noncaseating epithelioid granulomas, and exclusion of other granulomatous diseases. Prognosis correlates with mode of onset, host characteristics, initial clinical course, and extent of disease. The optimal management of sarcoidosis has not been well defined. Although corticosteroids remain the mainstay of treatment, there is little evidence for the optimal initiation, dosage, or duration of therapy. Topical steroids maybe considered for treatment of anterior uveitis and skin lesions. Systemic steroids are indicated for treatment of cardiac, nervous system, severe ocular, and symptomatic or progressive pulmonary involvement. There is little evidence for the efficacy of inhaled steroids. Cytotoxic agents and immunomodulators usually are reserved for treatment of complex or refractory disease. Of these agents, methotrexate is used more frequently because of its safety profile and possible steroid-sparing effects. Antimalarial agents are used frequently for skin lesions, and they have limited success in the treatment of pulmonary disease. Lung and cardiac transplantation is reserved for end-stage disease. Monitoring for symptoms of drug toxicity is essential, and prevention of osteoporosis must be addressed in patients taking long-term oral corticosteroids. It is not known if current therapy alters disease progression.

  9. Right ventricular involvement in cardiac sarcoidosis demonstrated with cardiac magnetic resonance.

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    Smedema, Jan-Peter; van Geuns, Robert-Jan; Ainslie, Gillian; Ector, Joris; Heidbuchel, Hein; Crijns, Harry J G M

    2017-11-01

    Cardiac involvement in sarcoidosis is reported in up to 30% of patients. Left ventricular involvement demonstrated by contrast-enhanced cardiac magnetic resonance has been well validated. We sought to determine the prevalence and distribution of right ventricular late gadolinium enhancement in patients diagnosed with pulmonary sarcoidosis. We prospectively evaluated 87 patients diagnosed with pulmonary sarcoidosis with contrast-enhanced cardiac magnetic resonance for right ventricular involvement. Pulmonary artery pressures were non-invasively evaluated with Doppler echocardiography. Patient characteristics were compared between the groups with and without right ventricular involvement, and right ventricular enhancement was correlated with pulmonary hypertension, ventricular mass, volume, and systolic function. Left ventricular late gadolinium enhancement was demonstrated in 30 patients (34%). Fourteen patients (16%) had right ventricular late gadolinium enhancement, with sole right ventricular enhancement in only two patients. The pattern of right ventricular enhancement consisted of right ventricular outflow tract enhancement in 1 patient, free wall enhancement in 8 patients, ventricular insertion point enhancement in 10 patients, and enhancement of the right side of the interventricular septum in 11 patients. Pulmonary arterial hypertension correlated with the presence of right ventricular enhancement (P Right ventricular enhancement correlated with systolic ventricular dysfunction (P Right ventricular enhancement was present in 16% of patients diagnosed with pulmonary sarcoidosis and in 48% of patients with left ventricular enhancement. The presence of right ventricular enhancement correlated with pulmonary arterial hypertension, right ventricular systolic dysfunction, hypertrophy, and dilation. © 2017 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

  10. Sarcoidosis Quiz

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    ... Share this page from the NHLBI on Twitter. Sarcoidosis Quiz Sarcoidosis is a disease of unknown cause that leads ... various organs in the body. The outcome of sarcoidosis varies. The disease leads to organ damage in ...

  11. Learn About Sarcoidosis

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    ... Lung Health and Diseases > Lung Disease Lookup > Sarcoidosis Learn About Sarcoidosis Sarcoidosis is a disease of unknown ... file."); } }); } } --> Blank Section Header Lung Disease Lookup Sarcoidosis Learn About Sarcoidosis Sarcoidosis Symptoms, Causes and Risk Factors ...

  12. [Musculoskeletal manifestations of sarcoidosis].

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    Korsten, P; Chehab, G

    2017-06-01

    Musculoskeletal manifestations in the context of sarcoidosis are frequently observed. The rheumatologist regularly encounters this disease in clinical practice. In the present review, we aim to give a current overview of the manifestations and treatments relevant to the practicing rheumatologist. The most frequently encountered manifestation is Lofgren's syndrome, which is characterized by bilateral ankle periarthritis, bilateral hilar lymphadenopathy, and erythema nodosum and has an excellent prognosis. Chronic arthropathy most commonly manifests as oligoarthritis, which sometimes hampers its differentiation from spondylarthropathies, especially when sacroiliitis, enthesitis or dactylitis are simultaneously present. Isolated vertebral granulomas are rare and require infectious and malignant disorders to be excluded, since there are no specific imaging findings that are exclusively found in vertebral sarcoidosis. The presence of granulomas in skeletal muscle is common in muscle biopsies, whereas clinically overt myopathy is present in only around 1-2% of patients. Therapeutic responses vary among the different clinical phenotypes. Non-steroidal anti-inflammatory drugs and low to medium dose glucocorticoids are the first-line therapy for musculoskeletal manifestations and often lead to adequate disease control in acute sarcoidosis. When these are ineffective or not tolerated, steroid-sparing agents are increasingly used in chronic sarcoidosis. Evidence for all medications used in sarcoid-related arthritis is comparatively scant. When supplementing vitamin D, the possible development of hypercalcemia, even at standard doses, needs to be considered; the optimal therapeutic levels for the prevention of medication-induced osteoporosis in sarcoidosis have not been firmly established.

  13. Cardiac Sarcoidosis or Giant Cell Myocarditis? On Treatment Improvement of Fulminant Myocarditis as Demonstrated by Cardiovascular Magnetic Resonance Imaging

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    Hari Bogabathina

    2012-01-01

    Full Text Available Giant cell myocarditis, but not cardiac sarcoidosis, is known to cause fulminant myocarditis resulting in severe heart failure. However, giant cell myocarditis and cardiac sarcoidosis are pathologically similar, and attempts at pathological differentiation between the two remain difficult. We are presenting a case of fulminant myocarditis that has pathological features suggestive of cardiac sarcoidosis, but clinically mimicking giant cell myocarditis. This patient was treated with cyclosporine and prednisone and recovered well. This case we believe challenges our current understanding of these intertwined conditions. By obtaining a sense of severity of cardiac involvement via delayed hyperenhancement of cardiac magnetic resonance imaging, we were more inclined to treat this patient as giant cell myocarditis with cyclosporine. This resulted in excellent improvement of patient’s cardiac function as shown by delayed hyperenhancement images, early perfusion images, and SSFP videos.

  14. Ocular Sarcoidosis

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    Pasadhika, Sirichai; Rosenbaum, James T

    2015-01-01

    Sarcoidosis is one of the leading causes of inflammatory eye disease. Ocular sarcoidosis can involve any part of the eye and its adnexal tissues, and may cause uveitis, episcleritis/scleritis, eyelid abnormalities, conjunctival granuloma, optic neuropathy, lacrimal gland enlargement and orbital inflammation. Glaucoma and cataract can be complications from inflammation itself or adverse effects from therapy. Ophthalmic manifestations can be isolated, or associated with other organ involvement. Patients with ocular sarcoidosis can present with a wide range of clinical presentations and severity. Multi-disciplinary approaches are required to achieve the best treatment outcomes for both ocular and systemic manifestations. PMID:26593141

  15. Foundation for Sarcoidosis Research

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    ... a Clinical Trial Our mission is to stop sarcoidosis — join us. The sarcoidosis community needs your help ... receive periodic emails from the Foundation. Foundation For Sarcoidosis Research 1820 W. Webster Ave., Ste 304 Chicago, ...

  16. Cardiac sarcoidosis

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    Costello BT

    2016-04-01

    Full Text Available Benedict T Costello,1,2 James Nadel,3 Andrew J Taylor,1,21Department of Cardiovascular Medicine, The Alfred Hospital, 2Baker IDI Heart and Diabetes Research Institute, Melbourne, VIC, 3School of Medicine, University of Notre Dame, Sydney, NSW, Australia Abstract: Cardiac sarcoidosis is a rare but life-threatening condition, requiring a high degree of clinical suspicion and low threshold for investigation to make the diagnosis. The cardiac manifestations include heart failure, conducting system disease, and arrhythmias predisposing to sudden cardiac death. A number of investigations are available to assist in making the diagnosis. The diagnosis may be made from the clinical history and evidence of inflammation on imaging modalities in the active phase and evidence of myocardial scarring in the chronic phase. Keywords: cardiac magnetic resonance, positron emission tomography, sarcoidosis, sudden cardiac death

  17. Papular sarcoidosis of the knees. A frequent form of presentation of systemic sarcoidosis.

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    Marcoval, Joaquim; Mañá, Juan

    2016-03-29

    In recent years we have observed with increasing frequency granulomatous papular lesions involving the knees, for which we proposed the term papular sarcoidosis of knees. To evaluate the clinicopathological features of papular sarcoidosis of the knees. Patients with papular lesions of the knees and histopathologically sarcoid granulomas were included in the study. Systemic sarcoidosis was investigated in all cases. Clinical charts were retrospectively retrieved. Biopsy specimens were evaluated under polarized light to detect foreign bodies. Fifty-three patients fulfilled inclusion criteria. In 36 cases systemic sarcoidosis was diagnosed and these cases were considered as papular sarcoidosis of the knees. Foreign particles were observed in 21 of these 36 patients. In only 9/36 patients did the activity of systemic disease persist over two years. In 17 cases sarcoidosis could not be demonstrated during follow-up. Papular sarcoidosis of the knees can be considered a relatively frequent form of cutaneous sarcoidosis usually present at the beginning of the disease that can be useful for the diagnosis of sarcoidosis. It is mainly observed in acute forms of sarcoidosis and can be considered a sign of good prognosis.

  18. Sarcoidosis: An unusual presentation.

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    Madureira, Pedro; Pimenta, Sofia; Cardoso, Hélder; Guimarães Cunha, Rui; Costa, Lúcia

    A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis. Lab tests revealed increased liver enzymes. Liver MRI evidenced an enlarged liver and retroperitoneal lymphadenopathy. Histological analysis of the finger skin, lymph nodes and liver demonstrated the presence of granulomas, confirming the diagnosis of sarcoidosis. The patient started prednisolone with rapid improvement of the symptoms. Skin lesions are divided into two groups: specific for sarcoidosis (with granulomas, lupus pernio-like) and nonspecific (without granulomas, erythema nodosum-like). Specific cutaneous lesions usually cause no other symptoms beyond cosmetic changes. Lupus pernio stands out for having distinctive features but, to the best of our knowledge, the simultaneous involvement of both hands and feet has never been reported. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  19. Systemic Sarcoidosis with Thyroid Involvement

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    Okuma, Hideyuki; Hashimoto, Koshi; Wang, Xin; Ohkiba, Noriaki; Murooka, Nozomi; Akizuki, Norikazu; Inazawa, Takeshi; Ogawa, Yoshihiro

    2017-01-01

    A 66-year-old woman, who was diagnosed with iritis, visited our hospital due to general malaise. A blood analysis revealed hypercalcemia. Computed tomography revealed mediastinal and hilar lymph node hyperplasia. Moreover, 67Gallium scintigraphy demonstrated strong accumulation in the lesions, suggesting sarcoidosis. A core needle biopsy (CNB) of the hypoechoic areas of the thyroid was performed because the patient refused to undergo a bronchoscopic examination. The scattering of slightly acidophilic epithelioid cell granulomas was observed in the pathological examination of the biopsy specimen. Based on this finding, the patient was diagnosed with sarcoidosis. Although sarcoidosis rarely involves the thyroid gland, in the present case, thyroid CNB was an alternative diagnostic method that allowed a pathological diagnosis to be obtained. PMID:28781308

  20. Sarcoidosis - Main Topic

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    Nilgün Solak Tekin

    2012-09-01

    Full Text Available Sarcoidosis is a multisystemic, inflammatory disease of unknown etiology that is characterized by noncaseating granulomas. Approximately, one-third of the patients with systemic sarcoidosis are observed cutaneous manifestations of different morphologies. Skin sarcoidosis have extremely heterogeneous clinical picture, so it is defined as “The Great Imitator” or “clinical chameleon”.

  1. gli-1 Oncogene is highly expressed in granulomatous skin disorders, including sarcoidosis, granuloma annulare, and necrobiosis lipoidica diabeticorum.

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    Macaron, Nada C; Cohen, Cynthia; Chen, Suephy C; Arbiser, Jack L

    2005-02-01

    Sarcoidosis, which occurs most commonly in African American women, is a granulomatous multisystem disorder affecting the skin, lungs, and central nervous system. In a previous immunohistochemistry study of keloids, a scar granuloma stained highly positive for glioma-associated oncogene homologue (gli)-1. This observation led us to study whether gli-1, one of the vertebrate zinc finger transcription factor genes of the gli superfamily, is expressed in granulomatous skin disorders such as cutaneous sarcoidosis, granuloma annulare (GA), and necrobiosis lipoidica diabeticorum (NLD). Immunohistochemistry studies for gli-1 were performed on biopsy specimens from patients with cutaneous sarcoidosis, GA, and NLD. All sarcoid lesions were highly positive for gli-1 expression, and 75% of the cells demonstrated positivity with a stain intensity of 3 on a scale of 1 to 3. The gli-1 expression was confined to cutaneous granulomas. CD68 staining was highly positive in the sarcoid lesions as well. Similarly, GA and NLD lesions were uniformly positive for gli-1 expression. We found that gli-1 is inappropriately expressed in granulomatous lesions of the skin such as cutaneous sarcoidosis, GA, and NLD. These findings provide a rationale for clinical trials of inhibitors of gli-1 signaling, including tacrolimus and sizolimus, for the treatment of cutaneous sarcoidosis and other granulomatous disorders of the skin.

  2. Gallium scan in intracerebral sarcoidosis

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    Makhija, M.C.; Anayiotos, C.P.

    1981-07-01

    Sarcoidosis involving the nervous system probably occurs in about 4% of patients. The usefulness of brain scintigraphy in these cases has been suggested. In this case of cerebral sarcoid granuloma, gallium imaging demonstrated the lesion before treatment and showed disappearance of the lesion after corticosteroid treatment, which correlated with the patient's clinical improvement.

  3. Sarcoidosis Occurring After Lymphoma

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    London, Jonathan; Grados, Aurélie; Fermé, Christophe; Charmillon, Alexandre; Maurier, François; Deau, Bénédicte; Crickx, Etienne; Brice, Pauline; Chapelon-Abric, Catherine; Haioun, Corinne; Burroni, Barbara; Alifano, Marco; Le Jeunne, Claire; Guillevin, Loïc; Costedoat-Chalumeau, Nathalie; Schleinitz, Nicolas; Mouthon, Luc; Terrier, Benjamin

    2014-01-01

    Abstract Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy. An association of sarcoidosis and lymphoproliferative disease has previously been reported as the sarcoidosis-lymphoma syndrome. Although this syndrome is characterized by sarcoidosis preceding lymphoma, very few cases of sarcoidosis following lymphoma have been reported. We describe the clinical, biological, and radiological characteristics and outcome of 39 patients presenting with sarcoidosis following lymphoproliferative disease, including 14 previously unreported cases and 25 additional patients, after performing a literature review. Hodgkin lymphoma and non-Hodgkin lymphoma were equally represented. The median delay between lymphoma and sarcoidosis was 18 months. Only 16 patients (41%) required treatment. Sarcoidosis was of mild intensity or self-healing in most cases, and overall clinical response to sarcoidosis was excellent with complete clinical response in 91% of patients. Sarcoidosis was identified after a follow-up computerized tomography scan (CT-scan) or 18fluorodeoxyglucose-positron emission tomography/computerized tomography (18FDG-PET/CT) evaluation in 18/34 patients (53%). Sarcoidosis is therefore a differential diagnosis to consider when lymphoma relapse is suspected on a CT-scan or 18FDG-PET/CT, emphasizing the necessity to rely on histological confirmation of lymphoma relapse. PMID:25380084

  4. [Optic disc granuloma secondary to sarcoidosis].

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    Qu-Knafo, L; Auregan-Giocanti, A

    2017-02-01

    We report a case of optic disc granuloma due to sarcoidosis. A 64-year-old, caucasian female with a history of pulmonary sarcoidosis presented with a vision loss on her left eye. The ophthalmologic examination revealed a discrete optic disc infiltrate compatible with the diagnosis of optic disc granuloma. Fluorescein angiography showed diffusion and impregnation of the granuloma without vascularitis. The optical coherence tomography demonstrated a homogenous and isoreflective lesion at the optic disc. The patient recovered her visual acuity after systemic corticosteroid treatment. Isolated optic disc granuloma is a rare condition of ocular sarcoidosis. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  5. [Chronic meningitis associated with lymph node sarcoidosis].

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    Thielemans, P; Jann, E

    1989-01-01

    A 59-year-old woman with maturity-onset diabetes presented with symmetrical transient polyarthralgia and acido-cetosis. Bilateral hilar adenopathy and erythematous rash on lower limbs were demonstrated. While low-grade chronic meningeal irritation supervened, lymph node biopsy showed typical sarcoidosis. Administration of corticosteroids resulted in reduction of cerebrospinal fluid albumin content and of lymphocytosis in bronchoalveolar lavage. In this patient, sarcoidosis was therefore associated with Löfgren's syndrome and meningitis.

  6. Biomarkers in sarcoidosis.

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    Chopra, Amit; Kalkanis, Alexandros; Judson, Marc A

    2016-11-01

    Numerous biomarkers have been evaluated for the diagnosis, assessment of disease activity, prognosis, and response to treatment in sarcoidosis. In this report, we discuss the clinical and research utility of several biomarkers used to evaluate sarcoidosis. Areas covered: The sarcoidosis biomarkers discussed include serologic tests, imaging studies, identification of inflammatory cells and genetic analyses. Literature was obtained from medical databases including PubMed and Web of Science. Expert commentary: Most of the biomarkers examined in sarcoidosis are not adequately specific or sensitive to be used in isolation to make clinical decisions. However, several sarcoidosis biomarkers have an important role in the clinical management of sarcoidosis when they are coupled with clinical data including the results of other biomarkers.

  7. Rheumatologic Manifestations of Sarcoidosis

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    Sweiss, Nadera J.; Patterson, Karen; Sawaqed, Ray; Jabbar, Umair; Korsten, Peter; Hogarth, Kyle; Wollman, Robert; Garcia, Joe G.N.; Niewold, Timothy B.; Baughman, Robert P.

    2012-01-01

    Sarcoidosis is a systemic, clinically heterogeneous disease characterized by the development of granulomas. Any organ system can be involved, and patients may present with any number of rheumatologic symptoms. There are no U.S. Food and Drug Administration–approved therapies for the treatment of sarcoidosis. Diagnosing sarcoidosis becomes challenging, particularly when its complications cause patients’ symptoms to mimic other conditions, including polymyositis, Sjögren syndrome, or vasculitis. This review presents an overview of the etiology of and biomarkers associated with sarcoidosis. We then provide a detailed description of the rheumatologic manifestations of sarcoidosis and present a treatment algorithm based on current clinical evidence for patients with sarcoid arthritis. The discussion will focus on characteristic findings in patients with sarcoid arthritis, osseous involvement in sarcoidosis, and sarcoid myopathy. Arthritic conditions that sometimes coexist with sarcoidosis are described as well. We present two cases of sarcoidosis with rheumatologic manifestations. Our intent is to encourage a multidisciplinary, translational approach to meet the challenges and difficulties in understanding and treating sarcoidosis. PMID:20665396

  8. Childhood sarcoidosis: A rare but fascinating disorder

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    Gedalia Abraham

    2008-09-01

    Full Text Available Abstract Childhood sarcoidosis is a rare multisystemic granulomatous disorder of unknown etiology. In the pediatric series reported from the southeastern United States, sarcoidosis had a higher incidence among African Americans. Most reported childhood cases have occurred in patients aged 13–15 years. Macrophages bearing an increased expression of major histocompatibility class (MHC II molecules most likely initiate the inflammatory response of sarcoidosis by presenting an unidentified antigen to CD4+ Th (helper-inducer lymphocytes. A persistent, poorly degradable antigen driven cell-mediated immune response leads to a cytokine cascade, to granuloma formation, and eventually to fibrosis. Frequently observed immunologic features include depression of cutaneous delayed-type hypersensitivity and a heightened helper T cell type 1 (Th1 immune response at sites of disease. Circulating immune complexes, along with signs of B cell hyperactivity, may also be found. The clinical presentation can vary greatly depending upon the organs involved and age of the patient. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestations, with frequent hilar lymphadenopathy and pulmonary infiltrations. Early-onset sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in children presenting before four years of age. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. Other granulmatous diseases should be reasonably excluded. The current therapy of choice for sarcoidosis in children with multisystem involvement is oral corticosteroids. Methotrexate given orally in low doses has been effective, safe and steroid sparing in some patients. Alternative immunosuppressive agents, such as azathioprine, cyclophosphamide, chlorambucil, and cyclosporine, have been tried in adult cases

  9. Childhood sarcoidosis: A rare but fascinating disorder.

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    Shetty, Avinash K; Gedalia, Abraham

    2008-09-23

    Childhood sarcoidosis is a rare multisystemic granulomatous disorder of unknown etiology. In the pediatric series reported from the southeastern United States, sarcoidosis had a higher incidence among African Americans. Most reported childhood cases have occurred in patients aged 13-15 years. Macrophages bearing an increased expression of major histocompatibility class (MHC) II molecules most likely initiate the inflammatory response of sarcoidosis by presenting an unidentified antigen to CD4+ Th (helper-inducer) lymphocytes. A persistent, poorly degradable antigen driven cell-mediated immune response leads to a cytokine cascade, to granuloma formation, and eventually to fibrosis. Frequently observed immunologic features include depression of cutaneous delayed-type hypersensitivity and a heightened helper T cell type 1 (Th1) immune response at sites of disease. Circulating immune complexes, along with signs of B cell hyperactivity, may also be found. The clinical presentation can vary greatly depending upon the organs involved and age of the patient. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestations, with frequent hilar lymphadenopathy and pulmonary infiltrations. Early-onset sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in children presenting before four years of age. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. Other granulmatous diseases should be reasonably excluded. The current therapy of choice for sarcoidosis in children with multisystem involvement is oral corticosteroids. Methotrexate given orally in low doses has been effective, safe and steroid sparing in some patients. Alternative immunosuppressive agents, such as azathioprine, cyclophosphamide, chlorambucil, and cyclosporine, have been tried in adult cases of sarcoidosis with

  10. Asymptomatic ocular sarcoidosis

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    Luiz Guilherme Azevedo de Freitas

    2013-04-01

    Full Text Available Sarcoidosis is an idiopathic systemic granulomatous disease. It commonly affects the skin, lungs, kidneys, and central nervous system. In the eyes it primarily affects the uveal tract, conjunctiva, lacrimal glands and optic nerve. Here in we describe the case of a patient with systemic sarcoidosis and asymptomatic eye inflammation.

  11. Takayasu Arteritis Associated with Sarcoidosis: A Case Report

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    Im, Mi Hye; Woo, Jeong Joo; An, Jin Kyung; Choi, Yun Sun [Dept. of Radiology, Eulji Hospital, Eulji University School of Medicine, Seoul (Korea, Republic of); Lee, Byung Hoon [Dept. of Internal Medicine, Eulji Hospital, Eulji University School of Medicine, Seoul (Korea, Republic of)

    2011-11-15

    Takayasu arteritis associated with sarcoidosis is very rare with only a few cases reported. We report on a case of a 55-year-old woman who was incidentally diagnosed with sarcoidosis and demonstrated numbness and weakness of the left upper limb and lower extremities associated with Takayasu arteritis.

  12. Comorbid autoimmune diseases in patients with sarcoidosis: A nationwide case-control study in Taiwan.

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    Wu, Chi-Hung; Chung, Pei-I; Wu, Chen-Yi; Chen, Yen-Ta; Chiu, Yun-Wen; Chang, Yun-Ting; Liu, Han-Nan

    2017-04-01

    The association between sarcoidosis and autoimmune comorbidities has been reported, however, it has seldom been confirmed by a large nationwide study. Our study aimed to clarify the association between sarcoidosis and autoimmune comorbidities in the Taiwanese. A total of 1237 patients with sarcoidosis and 4948 age- and sex-matched control subjects were selected from the National Health Insurance Research Database of Taiwan from 1997 to 2010. Multiple logistic regressions were performed to calculate the odds of comorbidities between the two groups. The prevalence of sarcoidosis was 2.17/100 000 individuals in Taiwan. Sarcoidosis patients tended to run a higher risk of autoimmune comorbidities than the control group (17.6% vs 9.4%, P sarcoidosis. The sex-stratified analyses were carried out to demonstrate a significant association of sarcoidosis with ankylosing spondylitis in both sexes, but with autoimmune thyroid disease in male patients and with Sjögren's syndrome female patients, respectively. Besides, the diagnosis of the autoimmune comorbidities strongly associated with sarcoidosis tended to be established after that of sarcoidosis. This study demonstrated that patients with sarcoidosis tended to have autoimmune thyroid disease, Sjögren's syndrome and ankylosing spondylitis, and the diagnosis of sarcoidosis usually preceded that of associated comorbidities. Clinicians should be alert to autoimmune comorbidities in patients with sarcoidosis. © 2016 Japanese Dermatological Association.

  13. Musculoskeletal involvement in sarcoidosis*, **

    Science.gov (United States)

    Nessrine, Akasbi; Zahra, Abourazzak Fatima; Taoufik, Harzy

    2014-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown cause. It most commonly affects the pulmonary system but can also affect the musculoskeletal system, albeit less frequently. In patients with sarcoidosis, rheumatic involvement is polymorphic. It can be the presenting symptom of the disease or can appear during its progression. Articular involvement is dominated by nonspecific arthralgia, polyarthritis, and Löfgren's syndrome, which is defined as the presence of lung adenopathy, arthralgia (or arthritis), and erythema nodosum. Skeletal manifestations, especially dactylitis, appear mainly as complications of chronic, multiorgan sarcoidosis. Muscle involvement in sarcoidosis is rare and usually asymptomatic. The diagnosis of rheumatic sarcoidosis is based on X-ray findings and magnetic resonance imaging findings, although the definitive diagnosis is made by anatomopathological study of biopsy samples. Musculoskeletal involvement in sarcoidosis is generally relieved with nonsteroidal anti-inflammatory drugs or corticosteroids. In corticosteroid-resistant or -dependent forms of the disease, immunosuppressive therapy, such as treatment with methotrexate or anti-TNF-α, is employed. The aim of this review was to present an overview of the various types of osteoarticular and muscle involvement in sarcoidosis, focusing on their diagnosis and management. PMID:24831403

  14. [Opportunistic infections and sarcoidosis].

    Science.gov (United States)

    Jamilloux, Y; Bernard, C; Lortholary, O; Kerever, S; Lelièvre, L; Gerfaud-Valentin, M; Broussolle, C; Valeyre, D; Sève, P

    2017-05-01

    Opportunistic infections (OI) are uncommon in sarcoidosis (1 to 10%) and mostly occur in patients with previously diagnosed disease or can rarely be the presenting manifestation. The most common OIs are, in descending order: aspergillosis, cryptococcosis, and mycobacterial infections. Treatment with corticosteroids is the most frequent risk factor for OI occurrence during sarcoidosis but immunosuppressive drugs and therapy with anti-TNFα are also risk factors. Overall, clinical presentation, treatment, and outcome are identical to that occur in other conditions complicated with the occurrence of OIs. However, some atypical presentations of OIs can mimic sarcoidosis exacerbation and misdiagnosis may lead clinicians to increase immunosuppression, causing worsening of the OI. The meticulous collection of patient's history along with factors differentiating OI from sarcoidosis exacerbation is key factor to optimally manage these patients. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  15. Living with Sarcoidosis

    Science.gov (United States)

    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  16. How Is Sarcoidosis Treated?

    Science.gov (United States)

    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  17. How Is Sarcoidosis Diagnosed?

    Science.gov (United States)

    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  18. What Causes Sarcoidosis?

    Science.gov (United States)

    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  19. Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association

    Science.gov (United States)

    Grados, Aurélie; Ebbo, Mikael; Bernit, Emmanuelle; Veit, Véronique; Mazodier, Karin; Jean, Rodolphe; Coso, Diane; Aurran-Schleinitz, Thérèse; Broussais, Florence; Bouabdallah, Reda; Gravis, Gwenaelle; Goncalves, Anthony; Giovaninni, Marc; Sève, Pascal; Chetaille, Bruno; Gavet-Bongo, Florence; Weitten, Thierry; Pavic, Michel; Harlé, Jean-Robert; Schleinitz, Nicolas

    2015-01-01

    Abstract The association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported. We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients. Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series. This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse. PMID:26181571

  20. From granuloma to fibrosis: sarcoidosis associated pulmonary fibrosis.

    Science.gov (United States)

    Bonham, Catherine A; Strek, Mary E; Patterson, Karen C

    2016-09-01

    Up to twenty percent of patients with sarcoidosis develop pulmonary fibrosis, transforming an often benign disease into a highly morbid and potentially fatal one. We highlight the fibrotic pulmonary sarcoidosis phenotype as an area of intense clinical and translational investigation, review recent developments in treatment, and provide a roadmap for future research in sarcoidosis associated pulmonary fibrosis. Granulomatous inflammation in a lymphatic distribution is the hallmark finding of pulmonary sarcoidosis and the nidus for fibrosis. Recent research demonstrates that fibrotic sarcoidosis begins in the setting of persistent, uncontrolled inflammation, and is aided by pro-fibrotic genetic features and immune responses. Comparison to other fibrotic lung diseases also reveals key features that inform our understanding of common pathways in fibrosis. Understanding the mechanisms of fibrotic transformation in sarcoidosis enhances clinical care and facilitates development of novel therapeutic options. The impact of these findings in fibrotic sarcoidosis may be amplified through application to other interstitial lung diseases marked by inflammatory to fibrotic transformation. Important aspects of clinical management of fibrotic sarcoidosis include surveillance for co-morbidities, such as pulmonary hypertension, airway disease, and infection, and assessment for pulmonary disease activity that may benefit from immunosuppression.

  1. Unusual cause of generalized osteolytic vertebral lesions: a case report

    Directory of Open Access Journals (Sweden)

    Nanda Sudip

    2007-06-01

    Full Text Available Abstract Background Vertebral sarcoidosis is an extremely rare form of osseous sarcoidosis. Although osseous sarcoidosis is almost always an incidental finding of sarcoidosis elsewhere in the body, vertebrae may be the primary disease site. Involvement of vertebrae is usually localized and sclerotic or lytic. Case presentation We describe a case of extensive asymptomatic vertebral involvement by sarcoid with osteolytic lesions. Making the diagnosis requires biopsy and ruling out other commoner causes of osteolytic vertebral lesions. Conclusion We report this case in the hope of expanding the knowledge of osseous sarcoidosis. Our patient was unique in that all involvement was axial with sparing of the peripheral skeleton, near absence of any other organ involvement, diffuse involvement of the whole spine and osteolytic bone lesions.

  2. Molecular detection of vertebrates in stream water: A demonstration using rocky mountain tailed frogs and Idaho giant salamanders

    Science.gov (United States)

    Goldberg, C.S.; Pilliod, D.S.; Arkle, R.S.; Waits, L.P.

    2011-01-01

    Stream ecosystems harbor many secretive and imperiled species, and studies of vertebrates in these systems face the challenges of relatively low detection rates and high costs. Environmental DNA (eDNA) has recently been confirmed as a sensitive and efficient tool for documenting aquatic vertebrates in wetlands and in a large river and canal system. However, it was unclear whether this tool could be used to detect low-density vertebrates in fast-moving streams where shed cells may travel rapidly away from their source. To evaluate the potential utility of eDNA techniques in stream systems, we designed targeted primers to amplify a short, species-specific DNA fragment for two secretive stream amphibian species in the northwestern region of the United States (Rocky Mountain tailed frogs, Ascaphus montanus, and Idaho giant salamanders, Dicamptodon aterrimus). We tested three DNA extraction and five PCR protocols to determine whether we could detect eDNA of these species in filtered water samples from five streams with varying densities of these species in central Idaho, USA. We successfully amplified and sequenced the targeted DNA regions for both species from stream water filter samples. We detected Idaho giant salamanders in all samples and Rocky Mountain tailed frogs in four of five streams and found some indication that these species are more difficult to detect using eDNA in early spring than in early fall. While the sensitivity of this method across taxa remains to be determined, the use of eDNA could revolutionize surveys for rare and invasive stream species. With this study, the utility of eDNA techniques for detecting aquatic vertebrates has been demonstrated across the majority of freshwater systems, setting the stage for an innovative transformation in approaches for aquatic research.

  3. Nonsteroidal therapy of sarcoidosis

    Science.gov (United States)

    Korsten, Peter; Mirsaeidi, Mehdi; Sweiss, Nadera J.

    2014-01-01

    Purpose of review None of the medications used in clinical practice to treat sarcoidosis have been approved by the regulatory authorities. Understanding how to use disease-modifying antisarcoid drugs, however, is essential for physicians treating patients with sarcoidosis. This review summarizes the recent studies of medications used for sarcoidosis with a focus on nonsteroidal therapies. Studies from 2006 to 2013 were considered for review to update clinicians on the most relevant literature published over the last few years. Recent findings Several recently published pieces of evidence have helped expand our ability to more appropriately sequence second-line and third-line therapies for sarcoidosis. For instance, methotrexate and azathioprine may be useful and well tolerated medications as second-line treatment. Mycophenolate mofetil might have a role in neurosarcoidosis. TNF-α blockers and other biologics seem to be well tolerated medications for the most severely affected patients. Summary Corticosteroids remain the first-line therapy for sarcoidosis as many patients never require treatment or only necessitate a short treatment duration. Second-line and third-line therapies described in this article should be used in patients with progressive or refractory disease or when life-threatening complications are evident at the time of presentation. PMID:23884295

  4. Fatigue syndrome in sarcoidosis.

    Science.gov (United States)

    Górski, Witold; Piotrowski, Wojciech J

    2016-01-01

    Sarcoidosis is an inflammatory disease of unknown etiology. Most commonly it results in the formation of non-caseating granulomas in intrathoracic lymph nodes and lung parenchyma, but the clinical course and picture may be complicated by extrapulmonary involvement and many non-respiratory signs and symptoms which are directly related to the disease. In addition, sarcoidosis patients may suffer from a plethora of symptoms of uncertain or unknown origin. Fatigue is one of these symptoms, and according to some authors it is reported by the majority of patients with active sarcoidosis, but also by a smaller proportion of patients with inactive sarcoidosis, or even with complete clinical and radiological remission. Therefore the term fatigue syndrome is frequently used to name this clinical problem. The definition of fatigue syndrome in sarcoidosis is imprecise and the syndrome is usually recognized by use of validated questionnaires. In this review the uptodate knowledge in this field was presented and different challenges connected with this syndrome were described.

  5. Cardiac sarcoidosis. A case with unusual manifestation.

    Science.gov (United States)

    Cepin, D; McDonough, M; James, F

    1983-01-01

    A 26-year-old woman was hospitalized with new-onset congestive heart failure. M-mode and two-dimensional echocardiography revealed abnormal motion involving the apex and posterolateral wall of the left ventricle. Cardiac catheterization was performed. The left ventriculogram demonstrated apical akinesis and an apical filling defect. The diagnosis of cardiac sarcoidosis was made from an open myocardial biopsy.

  6. Sarcoidosis appearing in a tattoo.

    Science.gov (United States)

    Ali, Saba M; Gilliam, Anita C; Brodell, Robert T

    2008-01-01

    Sarcoidosis is a systemic disease that may present as tattoo granulomas. A patient with systemic sarcoidosis who developed a granulomatous reaction within a tattoo is presented to stimulate interest in this unusual phenomenon. A patient with a 6-year history of pulmonary sarcoidosis developed sarcoidal granulomas restricted to one pigment of a tattoo. Previous reports of sarcoidal granulomas within tattoos are reviewed, and information about the pathogenesis of this process is explored. Sarcoid granulomas may develop in tattoos as an isolated local reaction or as the presenting sign of systemic sarcoidosis. The reaction itself may provide insight into further understanding the pathogenesis of sarcoidosis.

  7. Pulmonary sarcoidosis: management.

    Directory of Open Access Journals (Sweden)

    Sharma O

    2002-04-01

    Full Text Available During the last two decades many advances have been made in the field of sarcoidosis. The disease is now recognised as a multisystem disorder occurring in patients with a genetic predisposition and an exposure to yet unknown transmissible environmental agent/s. The diagnosis is based on a compatible clinical and/or radiological picture, histological evidence of non-caseating granuloma and exclusion of other diseases capable of producing a similar clinical or histological picture. Treatment primarily consists of administration of corticosteroids, although there are valuable alternative drugs. Treatment should be considered in symptomatic patients with evidence of radiologic or lung function deterioration. The patients with extra-pulmonary involvement particularly with ocular, myocardial, and neuro-sarcoidosis almost always need treatment. For asymptomatic pulmonary sarcoidosis patients no therapy is needed.

  8. Sarcoidosis-lymphoma syndrome.

    Science.gov (United States)

    Brandy-García, Anahy M; Caminal-Montero, Luis; Fernández-García, María Soledad; Saiz Ayala, Angel; Cabezas-Rodríguez, Ivan; Morante-Bolado, Isla

    A 65 year-old female with a history of sarcoidosis with pulmonary and joint involvement, who after 5 years of diagnosis begins with central nervous system involvement manifesting as diplopia. She presents normal analysis results. In imaging results, a mass is identified in the right intraconal space; it depends of right optic nerve, and shows multiple lymph node involvement. Biopsy was performed diagnosed with large B-cell lymphoma, an atypical form of tumor associated with sarcoidosis. Copyright © 2015 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  9. Case study: sarcoidosis

    African Journals Online (AJOL)

    Introduction. Sarcoidosis is a multisystem granulomatous disease of unknown cause. It occurs worldwide but there are higher incidences in certain racial groups, being three to four times more common in African-Americans [1]. It can also aggregate in families. Most patients do not need treatment and the disease often ...

  10. Animal models of sarcoidosis.

    Science.gov (United States)

    Hu, Yijie; Yibrehu, Betel; Zabini, Diana; Kuebler, Wolfgang M

    2017-03-01

    Sarcoidosis is a debilitating, inflammatory, multiorgan, granulomatous disease of unknown cause, commonly affecting the lung. In contrast to other chronic lung diseases such as interstitial pulmonary fibrosis or pulmonary arterial hypertension, there is so far no widely accepted or implemented animal model for this disease. This has hampered our insights into the etiology of sarcoidosis, the mechanisms of its pathogenesis, the identification of new biomarkers and diagnostic tools and, last not least, the development and implementation of novel treatment strategies. Over past years, however, a number of new animal models have been described that may provide useful tools to fill these critical knowledge gaps. In this review, we therefore outline the present status quo for animal models of sarcoidosis, comparing their pros and cons with respect to their ability to mimic the etiological, clinical and histological hallmarks of human disease and discuss their applicability for future research. Overall, the recent surge in animal models has markedly expanded our options for translational research; however, given the relative early stage of most animal models for sarcoidosis, appropriate replication of etiological and histological features of clinical disease, reproducibility and usefulness in terms of identification of new therapeutic targets and biomarkers, and testing of new treatments should be prioritized when considering the refinement of existing or the development of new models.

  11. Cryptococcal pyarthrosis and sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Geller, David S.; Pope, John B. [Albert Einstein College of Medicine, Montefiore Medical Center, Department of Orthopaedic Surgery, Bronx, NY (United States); Thornhill, Beverly A. [Albert Einstein College of Medicine, Montefiore Medical Center, Department of Radiology, Musculoskeletal Radiology, Bronx, NY (United States); Dorfman, Howard D. [Albert Einstein College of Medicine, Montefiore Medical Center, Section of Orthopaedic Pathology, Bronx, NY (United States)

    2009-07-15

    Cryptococcus neoformans is an infrequent cause of septic arthritis. Cryptococcal infections have been linked to sarcoidosis because of both inherent immunologic consequences of the disease and its typical immune modulating treatments. Cryptococcal infections should be suspected in patients with underlying immune deficiencies, and a high degree of vigilance should be exercised to avoid misdiagnosis, dissemination of infection, and meningitis. (orig.)

  12. [Roentgenological semiotics of sarcoidosis].

    Science.gov (United States)

    Terpigorev, S A; Stashuk, G A; Dubrova, S E

    2008-01-01

    The aim of this review was to summarize semiotics of X-ray and CT-observable manifestations of intrathoracic sarcoidosis and clarify the role of conventional X-ray examination and CT (including high resolution CT) in the diagnosis of this disease and its complications. Also analysed are changes in pulmonary parenchyma compared with those detected in morphological studies.

  13. Sarcoidosis: assessment of disease severity using HRCT

    Energy Technology Data Exchange (ETDEWEB)

    Drent, Marjolein [Department of Respiratory Medicine, University Hospital of Maastricht, P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Nutrition and Toxicology Research Institute Maastricht (NUTRIM), P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Sarcoidosis Management Center, University Hospital of Maastricht, P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Vries, Jolanda De [Department of Clinical Health Psychology, Tilburg University, P.O. Box 90153, 5000, LE Tilburg (Netherlands); Lenters, Merinke; Wouters, Emiel F.M. [Department of Respiratory Medicine, University Hospital of Maastricht, P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Nutrition and Toxicology Research Institute Maastricht (NUTRIM), P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Lamers, Rob J.S. [Department of Radiology, University Hospital of Maastricht, P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Rothkranz-Kos, Snjezana; Dieijen-Visser, Marja P. van [Nutrition and Toxicology Research Institute Maastricht (NUTRIM), P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Department of Clinical Chemistry, University Hospital of Maastricht, P.O. Box 5800, 6202, AZ Maastricht (Netherlands); Verschakelen, Johny A. [Department of Radiology, Catholic University, 3000, Leuven (Belgium)

    2003-11-01

    The value of high-resolution computed tomography (HRCT) in diagnosing and assessing inflammatory activity in sarcoidosis is well established. The aim of the present study was to address the intra- and inter-observer agreements of the HRCT score by Oberstein et al. [8], and to evaluate the relationship between HRCT findings and disease severity expressed in respiratory functional impairment in sarcoidosis. The clinical records of 80 known sarcoidosis patients visiting the outpatient clinic between January 2000 and August 2001, who underwent a HRCT as well as lung function tests (including exercise testing), were reviewed. Two readers scored the first 60 HRCT images twice. Weighted kappa and intra-class correlation coefficient were used to assess the reliability of the HRCT scoring system. Spearman's rank correlation coefficients and multiple regression analyses were performed to evaluate the relationship between HRCT findings (first reading, reader A) and respiratory functional impairment. Intra- and inter-reader reliability demonstrated good agreement. All HRCT subscores, except enlargement of lymph nodes, were correlated to the FEV{sub 1}, FVC, DLco, Pao{sub 2}max (all p<0.05) and A-aPo{sub 2}max (p <0.001). Furthermore, HRCT abnormalities, but not the chest radiographic stage, were strongly associated with functional parameters. Abnormal changes of lung parenchyma, established by HRCT features, were associated with respiratory functional impairment in sarcoidosis. Moreover, compared with the radiographic stages, HRCT findings appeared to be much more sensitive in depicting respiratory disability, especially abnormal gas exchange. (orig.)

  14. Characteristics of patients presenting with erythema nodosum and sarcoidosis.

    LENUS (Irish Health Repository)

    O'Connor, T M

    2012-01-31

    We explored the relationship between erythema nodosum (EN) and sex, age, serum angiotensin converting enzyme (ACE), bronchoalveolar lavage lymphocytosis (BAL-I), interstitial granulomas and radiological stage in patients presenting with pulmonary sarcoidosis in Ireland. Sixty-nine patients diagnosed with sarcoidosis between 2003 and 2006 were studied. Forty one patients (59%) were male. Sixteen patients (23%) presented with EN. Forty one patients of 65 (63%) had transbronchial biopsies demonstrating non-caseating granulomas. Patients with sarcoidosis presenting with EN were more likely to be female (p=0.042), younger (p=0.012) and have earlier stage pulmonary disease (p=0.02). There were no correlations between serum ACE, interstitial granulomas and disease stage. BAL-I did however predict increasing disease radiological stage (p=0.042). In this study, one quarter of patients with sarcoidosis presented with EN among their presenting features. These patients were more likely to be young females with early stage radiological disease.

  15. Infrequent cardiac manifestations of sarcoidosis.

    Science.gov (United States)

    Ashraf, Said; Briasoulis, Alexandros; Afonso, Luis

    Cardiac sarcoidosis (CS) is found in 2-7% of patients with systemic sarcoidosis (SS). Its diagnosis and treatment is challenging, notwithstanding the poor prognosis and treatment. Hereby, we present a case of systemic sarcoidosis with rare cardiac manifestations of severe mitral incompetence and large coronary aneurysm in a previously healthy woman. She underwent successful mitral valve replacement and coronary artery bypass surgery and was maintained on low dose glucocorticoid therapy. Copyright © 2016. Published by Elsevier Inc.

  16. Immune aspects of sarcoidosis.

    OpenAIRE

    Poulter, L W

    1988-01-01

    Although the initiating factor(s) is unknown, it is now accepted that pulmonary sarcoidosis develops as a result of an over-stimulated local cellular immune response. Starting as a lymphocytic alveolitis, there is a progression to granuloma formation within the interstitium as stimulated T lymphocytes release mediators capable of attracting and activating monocytes to differentiate into macrophages and epithelioid cells. We are also aware that macrophage-like cells must act as antigen present...

  17. Morbidity and Mortality in Sarcoidosis

    Science.gov (United States)

    Gerke, Alicia K.

    2015-01-01

    Purpose of Review Chronic sarcoidosis is a complex disease with numerous comorbid conditions and can be fatal in some cases. Recognizing causes of morbidity and mortality is important to effectively select treatments, manage symptoms, and improve outcomes. The purpose of this review is to examine emerging knowledge on morbidity and mortality in sarcoidosis. Recent Findings Approximately one to five percent of patients with sarcoidosis die from complications of sarcoidosis. Recent population studies indicate that mortality may be increasing over the past decade. The reasons behind these trends are unclear, but could include increasing incidence, detection rates, severity of disease, or age of the population. Morbidity of sarcoidosis is reflected by a trend of increased hospitalizations over recent years and increased use of healthcare resources. Morbidity can be caused by organ damage from granulomatous inflammation, treatment complications, and psychosocial effects of the disease. Recent studies are focused on morbidity related to cardiopulmonary complications, bone health, and aging within the sarcoidosis population. Last, sarcoidosis is associated with autoimmune diseases, pulmonary embolism, and malignancy; however, the underlying mechanisms linking diseases continue to be debated. Summary Morbidity in sarcoidosis is significant and multifactorial. Mortality is infrequent, but may be increasing over the years. PMID:25029298

  18. Pulmonary hypertension complicating pulmonary sarcoidosis

    NARCIS (Netherlands)

    Huitema, M P; Grutters, J C|info:eu-repo/dai/nl/258116129; Rensing, B J W M; Reesink, H J; Post, M C

    Pulmonary hypertension (PH) is a severe complication of sarcoidosis, with an unknown prevalence. The aetiology is multifactorial, and the exact mechanism of PH in the individual patient is often difficult to establish. The diagnostic work-up and treatment of PH in sarcoidosis is complex, and should

  19. Gene polymorphisms in fibrotic sarcoidosis

    NARCIS (Netherlands)

    Kruit, A.

    2006-01-01

    Sarcoidosis is a systemic disease of unknown cause, which is characterized by the presence of noncaseating granulomas in one or multiple organs. In approximately 90 percent of patients with sarcoidosis, the disease is manifested as pulmonary granulomas. Although parenchymal abnormalities often

  20. [Cardiac sarcoidosis - clinical manifestation and diagnosis].

    Science.gov (United States)

    Błaut-Jurkowska, Justyna; Podolec, Piotr; Olszowska, Maria

    2016-08-01

    Sarcoidosis is a multisystem inflammatory disease defined histologically by the formation of noncaseating granulomas. The etiology of sarcoidosis remains unknown. Heart involvement in the course of sarcoidosis concerns about 5% of patients. The most common manifestation of cardiac sarcoidosis are conduction abnormalities, arrhythmias and heart failure. The diagnostic algorithm includes performing a clinical history, a 12-lead electrocardiogram (ECG) and an echocardiogram. If any of the initial screening investigations yields an abnormality, diagnostics should be continue using advanced imaging techniques: cardiovascular magnetic resonance (CMR) or fluorodeoxyglucose positron emission tomography (FDG-PET). Nowadays endomyocardial biopsy is not performed routinely.The clinical picture of cardiac sarcoidosis is highly variable. Screening for cardiac sarcoidosis should be performed in all patients diagnosed with extracardiac sarcoidosis. Cardiac sarcoidosis should also be suspected in young patients without a diagnosis of sarcoidosis who present with conduction abnormalities of unknown etiology, because cardiac sarcoidosis may be the first or the only manifestation of the disease. © 2016 MEDPRESS.

  1. Sarcoidosis in Patients with Psoriasis

    DEFF Research Database (Denmark)

    Khalid, Usman; Gislason, Gunnar Hilmar; Hansen, Peter Riis

    2014-01-01

    PURPOSE: Psoriasis is a chronic inflammatory disease characterized by a systemic immunological response which is mainly driven by activated T helper (Th) 1 and Th17 lymphocytes. Like psoriasis, sarcoidosis is a chronic inflammatory disorder with Th1/Th17-driven inflammation. Therefore, we...... investigated the risk of sarcoidosis in patients with psoriasis compared to the background population in a nationwide cohort. METHODS: The study included the entire Danish population aged ≥10 years followed from 1st January 1997 until diagnosis of sarcoidosis, death or 31st December 2011. Patients...... with a history of psoriasis and/or sarcoidosis at baseline were excluded. Information on comorbidity and concomitant medication was identified by individual-level linkage of administrative registers. Incidence rates of sarcoidosis were calculated and adjusted hazard ratios (HRs) were estimated by multivariable...

  2. Evidence for Mycobacteria in Sarcoidosis

    Science.gov (United States)

    Brownell, Isaac; Ramírez-Valle, Francisco; Sanchez, Miguel

    2011-01-01

    Despite its recognition as a distinct granulomatous disease for over a century, the etiology of sarcoidosis remains to be defined. Since the early 1900s, infectious agents have been suspected in causing sarcoidosis. For much of this time, mycobacteria were considered a likely culprit, yet until recently, the supporting evidence has been tenuous at best. In this review, we evaluate the reported association between mycobacteria and sarcoidosis. Historically, mycobacterial infection has been investigated using histologic stains, cultures of lesional tissue or blood, and identification of bacterial nucleic acids or bacterial antigens. More recently, advances in biochemical, molecular, and immunological methods have produced a more rigorous analysis of the antigenic drivers of sarcoidosis. The result of these efforts indicates that mycobacterial products likely play a role in at least a subset of sarcoidosis cases. This information, coupled with a better understanding of genetic susceptibility to this complex disease, has therapeutic implications. PMID:21659662

  3. Sarcoidosis-associated hepatocellular carcinoma.

    Science.gov (United States)

    Ogata, Sho; Horio, Takuya; Sugiura, Yoshiaki; Shimazaki, Hideyuki; Saito, Hiroki; Aiko, Satoshi; Nakanishi, Kuniaki; Kawai, Toshiaki

    2010-12-01

    Sarcoidosis is a systemic granulomatous inflammation of unknown etiology, and seems to involve the liver parenchyma in most cases. However, sarcoidosis-associated hepatocellular carcinoma is rare. We report here a case in which a hepatocellular carcinoma occurred within the liver, which was probably involved as a result of systemic sarcoidosis. A 57-year-old Japanese man had been followed up for 2 years because of diabetic nephropathy and sarcoidosis. On admission for pneumonia, imaging studies revealed an unexpected hepatic tumor. Histology revealed a hepatocellular carcinoma accompanied by T-lymphocytic infiltration and marked granulomatous inflammation, which was surrounding some tumor nodules. The background liver parenchyma exhibited a moderate degree of fibrosis with granulomatous inflammation. The patient had no other apparent liver disease such as viral hepatitis, steatohepatitis, or primary biliary cirrhosis. Therefore, in the present case, sarcoidosis may be considered the probable background etiology for hepatocarcinogenesis.

  4. Granuloma formation in pulmonary sarcoidosis

    Directory of Open Access Journals (Sweden)

    Caroline E. Broos

    2013-12-01

    Full Text Available Sarcoidosis is a granulomatous disorder of unknown cause, affecting multiple organs, but mainly the lungs. The exact order of immunological events remains obscure. Reviewing current literature, combined with careful clinical observations, we propose a model for granuloma formation in pulmonary sarcoidosis. A tight collaboration between macrophages, dendritic cells and lymphocyte subsets, initiates the first steps towards granuloma formation, orchestrated by cytokines and chemokines. In a substantial part of pulmonary sarcoidosis patients, granuloma formation becomes an on-going process, leading to debilitating disease and sometimes death. The immunological response, determining granuloma sustainment is not well understood. An impaired immunosuppressive function of regulatory T cells has been suggested to contribute to the exaggerated response. Interestingly, therapeutical agents commonly used in sarcoidosis, such as glucocorticosteroids and anti-TNF agents, interfere with granuloma integrity and restore the immune homeostasis in autoimmune disorders. Increasing insight into their mechanisms of action may contribute to the search for new therapeutical targets in pulmonary sarcoidosis.

  5. Granulomatous lithiasic cholecystitis in sarcoidosis

    Directory of Open Access Journals (Sweden)

    Adriana Handra-Luca

    2016-03-01

    Full Text Available Gallbladder granulomas are exceedingly rare, reported in association with tuberculosis or sarcoidosis. Here we report a case of gallbladder granulomatous cholecystitis occurring in the context of sarcoidosis. A 70-years old man presented with abdominal pain, nausea and vomiting. The medical history revealed sarcoidosis diagnosed more than 20-years previously. 2-years previously the patient showed renal lithiasis, hypercalcemia and, increased angiotensin converting enzyme. The imaging features suggested thoraco-abdominal sarcoidosis. Prednisone was given at 1.2 mg/kg/day initially, than decreased, being at 2.5 mg/day at present. The ultrasound examination showed gallbladder lithiasis. A cholecystectomy was performed. Microscopy showed subacute and chronic cholecystitis with several epithelioid and giant cell granulomas some of them perineural. In conclusion, we report a case of granulomatous cholecystitis occurring in the course of treated sarcoidosis. The perineural location of granulomas may give further insights into the pathogenesis of gallbladder dysmotility.

  6. Acquired Functional Asplenia in Sarcoidosis

    Science.gov (United States)

    Stone, Richard W.; McDaniel, Willie R.; Armstrong, Earl M.; Young, Roscoe C.; Higginbotham-Ford, Edith A.

    1985-01-01

    Sarcoidosis is a recently identified cause of functional asplenia that can be diagnosed by radionuclide imaging. A 31-year-old woman with a five-year history of histologically compatible sarcoidosis was found to have nonvisualization of the spleen on technetium 99m sulfur colloid (radiopharmaceutical) liver-spleen scan. This scintigraphic finding was accompanied by poikilocytosis and Howell-Jolly bodies in the peripheral blood smear. A subsequent gallium 67 citrate scan reflected an abnormal increase in concentration of activity in the spleen, suggesting an active inflammatory process. Based upon this constellation of findings, it was concluded that acquired functional asplenia is the result of reticuloendothelial cell replacement via infiltration of the spleen by epithelioid cell granulomas of active sarcoidosis. This case also illustrates the reversibility of functional asplenia of sarcoidosis following adrenocorticosteroid therapy. Functional asplenia in sarcoidosis is now found to have a recognizable radionuclide imaging pattern. ImagesFigure 1Figure 2Figure 3 PMID:3908697

  7. Tomographic and pathological findings in pulmonary sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Melo, Alessandro Severo Alves de; Marchiori, Edson [Universidade Federal Fluminense (UFF), Niteroi, RJ (Brazil). Dept. of Radiology; Capone, Domenico [Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ (Brazil). Dept. of Pneumology

    2011-07-15

    Objective: To analyze radiological findings observed at high-resolution computed tomography in patients with sarcoidosis, and establishing their correlation with pathological findings. Materials and Methods: High-resolution computed tomography findings in ten patients with sarcoidosis were reviewed and correlated with findings in specimens obtained by surgical biopsy or at necropsy of four of such patients. Results: The most frequently observed finding was presence of nodules with perilymphatic distribution, predominating along bronchovascular sheaths and pleural surface, with subpleural nodules and nodular scissurae. Other less frequent findings were ground-glass attenuation and interlobular septa thickening. Conclusion: In general, all the mentioned findings demonstrated anatomopathological correlation with development of granulomas in these regions. (author)

  8. Multi-technique imaging of sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Balan, A. [Department of Clinical Radiology, Leeds Teaching Hospitals NHS Trust, Leeds (United Kingdom); Hoey, E.T.D. [Department of Clinical Radiology, Heartlands Hospital, Bordesley Green, Birmingham (United Kingdom); Sheerin, F. [Department of Neuroradiology, The John Radcliffe, Headington, Oxford (United Kingdom); Lakkaraju, A. [Department of Clinical Radiology, Leeds Teaching Hospitals NHS Trust, Leeds (United Kingdom); Chowdhury, F.U., E-mail: fahmid.chowdhury@leedsth.nhs.u [Department of Clinical Radiology, Leeds Teaching Hospitals NHS Trust, Leeds (United Kingdom)

    2010-09-15

    Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. The diagnosis is suggested on the basis of wide ranging clinical and radiological manifestations, and is supported by the histological demonstration of non-caseating granulomas in affected tissues. This review highlights the multisystem radiological features of the disease across a variety of imaging methods including multidetector computed tomography (CT), magnetic resonance imaging (MRI) as well as functional radionuclide techniques, particularly 2-[{sup 18}F]-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography/computed tomography (PET/CT). It is important for the radiologist to be aware of the varied radiological manifestations of sarcoidosis in order to recognize and suggest the diagnosis in the appropriate clinical setting.

  9. Clinically isolated laryngeal sarcoidosis

    DEFF Research Database (Denmark)

    Plaschke, Christina Caroline; Owen, Hanne Hoejris; Rasmussen, Niels

    2011-01-01

    to a combination of CO(2)-laser excision of supraglottic tissue and closure of the incision with sutures. All serological tests were negative or normal, including angiotensin 1 converting enzyme. The clinical expression was uniform with pale, smooth swellings of the supraglottic structures. Surgery proved...... successful to maintain normal breathing. None of the many parameters examined--some previously having been found to be abnormal in sarcoidosis--were abnormal in the present cohort. We are therefore unable to elucidate the pathogenesis. The combined surgical approach re-established normal airway function...

  10. [Maxillary bone sarcoidosis].

    Science.gov (United States)

    El Fares, N; El Bouihi, M; Zouhair, K; El Kabli, H; Benchikhi, H

    2011-04-01

    Sarcoidosis is an unexplained systemic granulomatosis. Bone localizations of the disease are rare. We report a maxillary localization. A 35-year-old patient consulted for facial dysmorphia first observed 2 years before. This swelling was associated to nodular panniculitis lesions and xerostomia. The facial asymmetry was due to maxillary gingival and alveolar swelling. It was associated with scarring in the legs and a purple nodular facial lesion. Lip and jaw biopsies revealed epithelioid and giant cell granulomas without caseous necrosis. The panoramic dental X-ray showed diffuse horizontal alveolar ridge lysis and CT scan revealed an osteolytic lesion of the right maxilla associated to a bone-condensing lesion of the left hemi-mandible. The diagnosis of sarcoidosis is made in case of epithelioid and giant cell granulomas without caseous necrosis and the ruling out of other possible diagnoses, including tuberculosis. Bone involvement is rare; face and maxillary localization are extremely rare. The recommended treatment is corticosteroids. Facial remodeling surgery is not recommended. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  11. [Pulmonary sarcoidosis imaging].

    Science.gov (United States)

    Brillet, P-Y; Nunes, H; Soussan, M; Brauner, M-W

    2011-04-01

    Sarcoidosis is a juvenile systemic granulomatosis. Its polymorphic clinical presentation depends on its different localisations, thoracic and extrathoracic. The role of imaging is very important for all localisations; but for mediastinopulmonary involvement, which is the most frequent (>90% of cases), it plays a major role in detecting the disease, diagnosing it, its prognosis, decision-making regarding treatment of it and in the monitoring of its development. Standard radiography, which sometimes detects the disease, forms the basis for its four-stage prognostic classification. CT scanning enables the study of mediastinal and hilar lymphadenopathy and the study of parenchyma, making it possible to identify micronodules of lymphatic distributions, alveolar opacities, septal lines, ground-glass hyperintensities, nodules surrounded by a ring of satellite micronodules, peribronchovascular thickening; all potentially reversible lesions. Elsewhere, it highlights irreversible fibrous lesions: hilar peripheral linear opacities; septal linear opacities; bronchial distortion, honeycomb destruction or even perihilar fibrotic masses. Less frequently we can visualise bronchiolar or cystic involvement. Benign in most cases, the sarcoidosis prognosis becomes bleaker in the event of hemoptysis, Aspergillus colonisation or before the onset of pulmonary hypertension. Copyright © 2010 Elsevier Masson SAS. All rights reserved.

  12. Sarcoidosis: A truly protean disease

    Energy Technology Data Exchange (ETDEWEB)

    Lopez-Majano, V.

    1984-07-01

    Sarcoidosis is a multisystem disease, the activity of the disease process can be ascertained by determining the angiotension levels in blood. This also can be achieved by /sup 67/Ga scintigraphy that will inform also different areas of localisation of the disease process. /sup 67/Ga-scintigraphy is also useful in the follow-up of patients with sarcoidosis and to evaluate the response to therapy.

  13. Sarcoidosis and Thyroid Autoimmunity

    Directory of Open Access Journals (Sweden)

    Piera Fazzi

    2017-08-01

    Full Text Available Most of the studies have shown a higher risk for subclinical and clinical hypothyroidism, antithyroid autoantibodies [overall antithyroid peroxidase antibodies (TPOAb], and in general, thyroid autoimmunity, overall in the female gender in patients with sarcoidosis (S. A significantly higher prevalence of clinical hypothyroidism and Graves’ disease was also described in female S patients with respect to controls. Gallium-67 (Ga-67 scyntigraphy in S patients, in the case of thyroid uptake, suggests the presence of aggressive autoimmune thyroiditis and hypothyroidism. For this reason, ultrasonography and thyroid function should be done in the case of Ga-67 thyroid uptake. In conclusion, thyroid function, TPOAb measurement, and ultrasonography should be done to assess the clinical profile in female S patients, and the ones at high risk (female individuals, with TPOAb positivity, and hypoechoic and small thyroid should have periodically thyroid function evaluations and suitable treatments.

  14. Rare coexistence of sarcoidosis and lung adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Amit Girish Kachalia

    2014-01-01

    Conclusion: While evidence is still lacking regarding association between sarcoidosis and lung adenocarcinoma, it is important for clinicians to exclude metastatic malignancy in patients exhibiting clinical and radiographic findings consistent with sarcoidosis.

  15. Intra-abdominal Complications of Sarcoidosis

    OpenAIRE

    MacArthur, Kristin Loening; Forouhar, Faripour; Wu, George Yung-Hsing

    2010-01-01

    Sarcoidosis is an inflammatory disease characterized by non-caseating granulomas in the absence of other autoimmune processes, infectious diseases, or foreign agents. The etiology of sarcoidosis is not completely understood. Several organ systems can be affected, of which the most frequently involved include the lungs and lymph nodes. Intra-abdominal sarcoidosis is less common, but can be found in the absence of pulmonary or lymphatic disease. Intra-abdominal sarcoidosis is most often asympto...

  16. [Livedo-like cutaneous sarcoidosis].

    Science.gov (United States)

    Duboys, S; Cante, V; Monégier du Sorbier, C; Guillet, G

    2016-05-01

    The cutaneous signs of sarcoidosis are extremely polymorphous and may be classified under several different headings. Herein, we report the case of a 51-year-old female patient presenting bilateral livedo of the knees accompanied by systemic signs with polyarthralgia, impaired general state of health, weight loss, and a sensation of dyspnoea. Skin biopsy revealed giant-cell granuloma around the dermal vessels, with no caseous necrosis. Histopathological examination of the patient's blood vessels revealed no abnormalities. Laboratory tests showed high levels of angiotensin converting enzyme (1.5×ULN), bilateral mediastinal adenomegaly and incipient pulmonary fibrosis. Systemic sarcoidosis was diagnosed on the basis of the overall clinical and laboratory picture. The patient subsequently presented hepatic involvement and hypercalcaemia associated with the sarcoidosis; systemic corticosteroid therapy (prednisone) was initiated, with slow dose-reduction. The outcome was favourable with a return to normal laboratory values, regression of the adenomegaly and subsidence of the livedo. The literature contains reports of a number of cases of livedo heralding sarcoidosis. The majority of such cases involved young or middle-aged female patients of Asian origin presenting systemic sarcoidosis, with a high frequency of ocular and neural involvement. Livedo could be accounted for by the specific disposition of granulomas around the arterioles, resulting in disturbance of local blood flow, which was probably associated with the mechanical compression of vessels and with the micro-thrombi noted at histology. Livedo may be considered a clinical sign of cutaneous sarcoidosis. Laboratory tests to screen for sarcoidosis may be proposed in the exploration of atypical livedo. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  17. The evidence for biologic immunotherapy in Sarcoidosis: A systematic review

    Directory of Open Access Journals (Sweden)

    Pooja Shah

    2017-09-01

    Full Text Available Background Sarcoidosis is a chronic inflammatory disease with a myriad of clinical manifestations. Treatment involves immunosuppression with corticosteroids or steroid-sparing agents. A proportion of patients does not respond to or are intolerant to therapy. Targeted immunotherapy with biologic agents has emerged as a novel approach with plausible mechanistic reasons to warrant study. Aims The aim of this review was to evaluate the evidence for the efficacy of biological therapy in sarcoidosis. Methods We conducted a systematic literature review and meta-analysis of all published randomised-controlled trials (RCT evaluating biological therapy in sarcoidosis, using MEDLINE and Embase databases, through to September 2017. The search terms included sarcoidosis, infliximab, adalimumab, etanercept, golimumab, certolizumab, rituximab, abatacept, tocilizumab, anakinra, ustekinumab, secukinumab. Only articles reporting RCTs were selected. Improvements in respiratory disease were assessed by changes in forced vital capacity (FVC by weighted mean difference (WMD. There were insufficient data on outcome measures in other organ systems to comparatively assess efficacy. Results The search identified 2,324 studies of which only 5 provided relevant and original data. This comprised a total of 364 patients, evaluating pulmonary, cutaneous and ocular sarcoidosis. One study in pulmonary disease and one study in cutaneous disease demonstrated improvements in the primary outcome. In pulmonary disease, meta-analysis of the treatment effect of anti-TNF therapy versus placebo on FVC revealed a WMD of 1.69 per cent (95 per cent confidence interval, 1.44–1.94. Conclusion There are insufficient data to suggest the long-term efficacy of anti-TNFα inhibitors in the treatment of sarcoidosis. This may be due to heterogeneity, small sample sizes and the lack of consistent reporting of outcome measures.

  18. Skeletal sarcoidosis; Skelettsarkoidose

    Energy Technology Data Exchange (ETDEWEB)

    Freyschmidt, J. [Klinikum Bremen-Mitte, Beratungsstelle und Referenzzentrum fuer Osteoradiologie, Bremen (Germany); Freyschmidt, P. [Dermatologische Gemeinschaftspraxis, Schwalmstadt (Germany)

    2016-10-15

    Presentation of the etiology, pathology, clinical course, radiology and differential diagnostics of skeletal sarcoidosis. Noncaseating epithelioid cell granulomas can trigger solitary, multiple or disseminated osteolysis, reactive osteosclerosis and/or granulomatous synovitis. The incidence of sarcoidosis is 10-12 per 100,000 inhabitants per year. Skeletal involvement is approximately 14 %. Skeletal involvement occurs almost exclusively in the stage of lymph node and pulmonary manifestation. Most cases of skeletal involvement are clinically asymptomatic. In the case of synovial involvement, unspecific joint complaints (arthralgia) or less commonly arthritis can occur. Typical skin alterations can be diagnostically significant. Punch out lesions osteolysis, coarse destruction and osteosclerosis can occur, which are best visualized with projection radiography and/or computed tomography. Pure bone marrow foci without interaction with the bone can only be detected with magnetic resonance imaging (MRI) and more recently with positron emission tomography (PET), mostly as incidental findings. There is a predeliction for the hand and trunk skeleton. Skeletal tuberculosis, metastases, multiple myeloma, Langerhans cell histiocytosis and sarcoid-like reactions in solid tumors must be differentiated. The key factors for correct diagnosis are thorax radiography, thorax CT and dermatological manifestations. (orig.) [German] Darstellung von Aetiologie, Pathologie, Klinik, Radiologie und Differenzialdiagnose der Skelettsarkoidose. Nichtverkaesende Epitheloidzellgranulome koennen solitaere, multiple oder disseminierte Osteolysen, reaktive Osteosklerosen und/oder eine granulomatoese Synovialitis ausloesen. Inzidenz der Sarkoidose: 10-12/100.000 Einwohner/Jahr. Skelettbeteiligung ca. 14 %. Skelettbeteiligungen kommen fast ausschliesslich im Stadium einer Lymphknoten- und pulmonalen Manifestation vor. Die meisten Skelettbeteiligungen verlaufen klinisch stumm. Bei synovialer

  19. Sarcoidosis Presenting with Cicatrizing Conjunctivitis.

    Science.gov (United States)

    O'Donnell, John J; Karakus, Sezen; Doroslovacki, Pavle; Akpek, Esen K

    2015-08-01

    To report two patients with sarcoidosis initially presenting with cicatrizing conjunctivitis. Both patients with chronic conjunctivitis were referred for further management. The first patient had conjunctival granulomas, subepithelial fibrosis, and forniceal foreshortening. The second patient had extensive upper and lower conjunctival scarring with forniceal foreshortening and symblepharon formation of both eyes. Conjunctival biopsy specimens revealed noncaseating granulomas. Immunofluorescein studies were negative for immunoreactant deposition in the basement membrane. Because of further diagnostic evaluations, sarcoidosis was determined to be the final cause of the cicatrizing conjunctivitis. Sarcoidosis should be included in the differential diagnosis of cicatrizing conjunctivitis. Recognition of the characteristic noncaseating granulomas in the conjunctival biopsy and initiation of the appropriate evaluations are essential in establishing the diagnosis and determining the extent of systemic involvement.

  20. Cutaneous sarcoidosis: A rare case report

    Directory of Open Access Journals (Sweden)

    Bindu Suparna M, Joshi Shivani

    2014-07-01

    Full Text Available Sarcoidosis is a Greek word (Sarco means flesh and Eido means type or like. Cutaneous sarcoidosis occurs in up to one third of patients with systemic sarcoidosis. This disease is characterised by the presence of non – caseating epitheloid cell granulomas in the skin. Cutaneous sarcoidosis presents as a diagnostic challenge to the dermatopathologists due to its varied presentations and almost identical histologic pictures. Hence, exclusion of infectious causes and compatibility with clinical and radiologic picture serve as significant criteria to come up to a diagnosis. Sometimes; skin lesions are the first manifestation of systemic sarcoidosis. This is not a contagious or allergic disease. There is a risk of development of systemic manifestations at a later date; for which a close follow up is a must. We are presenting a case of cutaneous sarcoidosis, which later on progress to sarcoidosis with systemic manifestations.

  1. Hepatic sarcoidosis: a case series.

    Science.gov (United States)

    Ennaifer, Rym; Ayadi, Shema; Romdhane, Hayfa; Cheikh, Myriam; Nejma, Houda Ben; Bougassas, Wassila; Hadj, Najet Bel

    2016-01-01

    Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.

  2. The role of serum amyloid A staining of granulomatous tissues for the diagnosis of sarcoidosis.

    Science.gov (United States)

    Huho, Albert; Foulke, Llewellyn; Jennings, Timothy; Koutroumpakis, Efstratios; Dalvi, Siddhartha; Chaudhry, Haroon; Chopra, Amit; Modi, Aakash; Rane, Neha; Prezant, David J; Sheehan, Christine; Yucel, Recai; Patel, Mehul; Judson, Marc A

    2017-05-01

    Previous studies demonstrated that SAA staining of sarcoidosis granulomas was qualitatively and quantitatively different from other granulomatous diseases. These data suggest that positive SAA staining of granulomatous tissue may have adequate specificity to establish a diagnosis of sarcoidosis. Our objective was to determine the diagnostic specificity of SAA staining for sarcoidosis relative to other granulomatous disorders. Pathological specimens demonstrating granulomatous inflammation were retrospectively identified at one institution, plus 4 specimens were obtained from New York City firefighters with biopsy-confirmed World Trade Center "sarcoidosis-like" pulmonary disease. Specimens were analyzed if specific diagnoses related to the granulomatous inflammation were confirmed through medical record review. SAA staining was performed using previously developed methods. Two pathologists, blinded to each other and the diagnoses, determined if the stained material was SAA positive or negative. Discordant results were adjudicated by the two pathologists. 106 specimens were analyzed from 100 patients, with 36 biopsies (34%) from sarcoidosis tissues and 70 (66%) from other granulomatous disorders. The Cohen Kappa correlation between the two pathologists for SAA staining positivity was excellent (0.85, 0.73-0.98). The overall specificity of SAA staining for the diagnosis of sarcoidosis was 84% (59/70). The sensitivity was 44% (16/36). Although SAA staining of various granulomatous tissues was fairly specific for the diagnosis of sarcoidosis, the specificity was inadequate for SAA staining to be used as a diagnostic test for sarcoidosis in isolation. These data suggest that SAA production may not be a universal mechanism in the development of sarcoidosis. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. Sarcoidosis presenting with severe hypocalcaemia.

    LENUS (Irish Health Repository)

    Saeed, A

    2012-01-31

    Disorders of calcium metabolism, especially hypercalcemia and hypercalceuria, are common in sarcoidosis. They are caused by extra renal unsuppressed production of 1,25 dihydroxy vitamin D at the level of the sarcoid granuloma. Hormonal changes during pregnancy have a physiological synergistic effect on this mechanism, which is primarily parathyroid hormone (PTH) dependant. However, the combination of primary hypoparathyroidism with hypocalcemia and sarcoidosis is rare. Di George syndrome, is a dysmorphic disorder characterised by aplasia\\/hypoplasia of thymus and parathyroid glands in addition to aortic arch anamolies and facial dysmorphia. After commencing appropriate treatment this lady made excellent recovary.

  4. [Current Therapeutic Approaches to Sarcoidosis].

    Science.gov (United States)

    Pizarro, Carmen; Skowasch, Dirk; Grohé, Christian

    2017-01-01

    Sarcoidosis represents a non-caseating, granulomatous disorder of unknown aetiology whose clinical manifestation is heterogeneous and frequently multisystemic. The portion of patients needing systemic treatment varies: though many patients may undergo spontaneous remission, organ-threatening courses demand systemic therapy. Corticosteroids are the first-line treatment option; however, disease´s progression and/or major corticosteroid side effects may require second- and third-line therapeutics. A current stepwise therapeutic algorithm to sarcoidosis that characterizes additive and alternative therapeutic agents is given in the following review. © Georg Thieme Verlag KG Stuttgart · New York.

  5. Immune response CC Chemokines, CCL2 and CCL5 are associated with Pulmonary Sarcoidosis

    LENUS (Irish Health Repository)

    Palchevskiy, Vyacheslav

    2011-04-04

    Abstract Background Pulmonary sarcoidosis involves an intense leukocyte infiltration of the lung with the formation of non-necrotizing granulomas. CC chemokines (chemokine (C-C motif) ligand 2 (CCL2)-CCL5) are chemoattractants of mononuclear cells and act through seven transmembrane G-coupled receptors. Previous studies have demonstrated conflicting results with regard to the associations of these chemokines with sarcoidosis. In an effort to clarify previous discrepancies, we performed the largest observational study to date of CC chemokines in bronchoalveolar lavage fluid (BALF) from patients with pulmonary sarcoidosis. Results BALF chemokine levels from 72 patients affected by pulmonary sarcoidosis were analyzed by enzyme-linked immunosorbent assay (ELISA) and compared to 8 healthy volunteers. BALF CCL3 and CCL4 levels from pulmonary sarcoidosis patients were not increased compared to controls. However, CCL2 and CCL5 levels were elevated, and subgroup analysis showed higher levels of both chemokines in all stages of pulmonary sarcoidosis. CCL2, CCL5, CC chemokine receptor type 1 (CCR1), CCR2 and CCR3 were expressed from mononuclear cells forming the lung granulomas, while CCR5 was only found on mast cells. Conclusions These data suggest that CCL2 and CCL5 are important mediators in recruiting CCR1, CCR2, and CCR3 expressing mononuclear cells as well as CCR5-expressing mast cells during all stages of pulmonary sarcoidosis.

  6. High resolution CT findings of pseudoalveolar sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Ji Eun; Park, Jun Gyun; Choe, Kyu Ok; Kim, Sang Jin [Yonsei University College of Medicine, Seoul (Korea, Republic of); Ryu, Young Hoon; Im, Jung Gi [Seoul National University College of Medicine, Seoul (Korea, Republic of); Lee, Kyoung Soo [Sungkunkwan University College of Medicine, Seoul (Korea, Republic of); Song, Koun Sik [University of Ulsan College of Medicine, Seoul (Korea, Republic of); Kim, Hyae Young [National Cancer Centar, Seoul (Korea, Republic of)

    2002-08-01

    To determine the specific high-resolution CT features of sarcoidosis in which the observed pattern is predominantly pseudoalveolar. We retrospectively reviewed the HRCT findings in 15 cases in which chest radiography demonstrated pseudoalveolar consolidation. In all 15, sarcoidosis was pathologically proven. The distribution and characterization of the following CT features was meticulously scrutinized: distribution and characterization of pseudoalveolar lesions, air-bronchograms, micronodules, thickening of bronchovascular bundles and interlobular septa, lung distortion, ground-glass opacities and combined hilar and mediastinal lymphadenopathy. Follow-up CT scans were available in three cases after corticosteroid administration. Between one and 12 (mean, 5.6) pseudoalveolar lesions appeared as dense homogeneous or inhomogeneous opacities 1-4.5 cm in diameter and with an irregular margin located either at the lung periphery adjacent to the pleural surface or along the bronchovascular bundles, with mainly bilateral distribution (n=14, 93%). An air-bronchogram was observed in ten cases. Micronodules were observed at the periphery of the lesion or surrounding lung, which along with a thickened bronchovascular bundle was a consistent feature in all cases. Additional CT features included hilar and mediastinal lymphadenopathy (n=14, 93%), thickened interlobular septa (n=12, 80%), and ground-glass opacity (n=10, 67%). Lung distortion was noted in only one case (7%). After steroid administration pseudoalveolar lesions decreased in number and size in all three cases in which follow-up CT was available. The consistent HRCT features of pseudoalveolar sarcoidosis are bilateral multifocal dense homogenous or inhomogenous opacity and an irregular margin located either at the lung periphery adjacent to the pleural surface or along the bronchovascular bundles. Micronodules are present at the periphery of the lesion or surrounding lung. The features are reversible administration.

  7. Teriparatide for osteoporosis in patients with sarcoidosis: Report on risk-benefit ratio in four cases.

    Science.gov (United States)

    Challal, Salima; Semerano, Luca; Nunes, Hilario; Valeyre, Dominique; Boissier, Marie-Christophe; Saidenberg-Kermanac'h, Nathalie

    2016-05-01

    For patients with sarcoidosis, no consensus exists about the management of osteoporosis, whether related or unrelated to glucocorticoid therapy. We report the first series of 4patients with histologically documented sarcoidosis who received teriparatide therapy for severe osteoporosis manifesting as a fracture cascade with multilevel vertebral fractures. When teriparatide was started, 1patient was receiving 10mg of prednisone equivalents per day, 1 was progressively tapering glucocorticoid dose, 2 had never received any glucocorticoid treatment. In all 4patients, teriparatide was effective in halting the fracture cascade, including in the 2patients who remained on long-term glucocorticoid therapy. None of the patients developed hypercalcemia. During teriparatide therapy, 3patients underwent a flare or a complication of sarcoidosis. Only the patient on stable glucocorticoid treatment did not present any adverse event. Before teriparatide initiation, sarcoidosis had been well controlled in all 3patients for several years. Even if the implication of teriparatide is unclear, the appearance of adverse events in 3 out of 4patients in this small series suggest caution in the use of teriparatide in patients with sarcoidosis. Copyright © 2015 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  8. Sarcoidosis | Allwood | Continuing Medical Education

    African Journals Online (AJOL)

    Continuing Medical Education. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 31, No 9 (2013) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. Sarcoidosis. B Allwood, C Ainslie. Abstract. No Abstract ...

  9. Granuloma formation in pulmonary sarcoidosis

    NARCIS (Netherlands)

    C.E. Broos (Caroline); M. van Nimwegen (Menno); H.C. Hoogsteden (Henk); R.W. Hendriks (Rudi); M. Kool (Mirjam); B. van den Blink (Bernt)

    2013-01-01

    textabstractSarcoidosis is a granulomatous disorder of unknown cause, affecting multiple organs, but mainly the lungs. The exact order of immunological events remains obscure. Reviewing current literature, combined with careful clinical observations, we propose a model for granuloma formation in

  10. Husband and wife with sarcoidosis: possible environmental factors involved.

    Science.gov (United States)

    Leli, Ilaria; Salimbene, Ivano; Varone, Francesco; Fuso, Leonello; Valente, Salvatore

    2013-01-25

    Sarcoidosis is a granulomatous multisystem disorder of unclear etiology that involves any organ, most commonly the lung and the lymph nodes. It is hypothesized that the disease derives from the interaction between single or multiple environmental factors and genetically determined host factors. Multiple potential etiologic agents for sarcoidosis have been proposed without any definitive demonstration of causality.We report the case of two patients, husband (57 years old) and wife (55 years old), both suffering from sarcoidosis. They underwent a lymph node biopsy by mediastinoscopy which showed a "granulomatous epithelioid giant cell non-necrotising chronic lymphadenitis". They had lived up to 3 years ago in the country in a farm, in contact with organic dusts, animals such as dogs, chickens, rabbits, pigeons; now they have lived since about 3 years in an urban area where there are numerous chemical industries and stone quarries. The aim of this case report was to focus on environmental factors that might be related to the pathogenesis of the sarcoidosis.

  11. Husband and wife with sarcoidosis: possible environmental factors involved

    Directory of Open Access Journals (Sweden)

    Leli Ilaria

    2013-01-01

    Full Text Available Abstract Sarcoidosis is a granulomatous multisystem disorder of unclear etiology that involves any organ, most commonly the lung and the lymph nodes. It is hypothesized that the disease derives from the interaction between single or multiple environmental factors and genetically determined host factors. Multiple potential etiologic agents for sarcoidosis have been proposed without any definitive demonstration of causality. We report the case of two patients, husband (57 years old and wife (55 years old, both suffering from sarcoidosis. They underwent a lymph node biopsy by mediastinoscopy which showed a “granulomatous epithelioid giant cell non-necrotising chronic lymphadenitis”. They had lived up to 3 years ago in the country in a farm, in contact with organic dusts, animals such as dogs, chickens, rabbits, pigeons; now they have lived since about 3 years in an urban area where there are numerous chemical industries and stone quarries. The aim of this case report was to focus on environmental factors that might be related to the pathogenesis of the sarcoidosis.

  12. Assessment of Cardiac Sarcoidosis with Advanced Imaging Modalities

    Science.gov (United States)

    Akasaka, Takashi

    2014-01-01

    Sarcoidosis is a chronic systemic disease of unknown etiology that is characterized by the presence of noncaseating epithelioid granulomas, usually in multiple organs. Several studies have shown that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in genetically susceptible individuals. Cardiac involvement may occur and lead to an adverse outcome: the heart mechanics will be affected and that causes ventricular failure, and the cardiac electrical system will be disrupted and lead to third degree atrioventricular block, malignant ventricular tachycardia, and sudden cardiac death. Thus, early diagnosis and treatment of this potentially devastating disease is critically important. However, sensitive and accurate imaging modalities have not been established. Recent studies have demonstrated the promising potential of cardiac magnetic resonance imaging (MRI) and 18F-fluoro-2-deoxyglucose positron emission tomography (18F-FDG PET) in the diagnosis and assessment of cardiac sarcoidosis (CS). In this review, we discuss the epidemiology, etiology, histological findings, and clinical features of sarcoidosis. We also introduce advanced imaging including 18F-FDG PET and cardiac MRI as more reliable diagnostic modalities for CS. PMID:25250336

  13. Exercise capacity, muscle strength and fatigue in sarcoidosis.

    Science.gov (United States)

    Marcellis, R G J; Lenssen, A F; Elfferich, M D P; De Vries, J; Kassim, S; Foerster, K; Drent, M

    2011-09-01

    The aim of this case-control study was to investigate the prevalence of exercise intolerance, muscle weakness and fatigue in sarcoidosis patients. Additionally, we evaluated whether fatigue can be explained by exercise capacity, muscle strength or other clinical characteristics (lung function tests, radiographic stages, prednisone usage and inflammatory markers). 124 sarcoidosis patients (80 males) referred to the Maastricht University Medical Centre (Maastricht, the Netherlands) were included (mean age 46.6±10.2 yrs). Patients performed a 6-min walk test (6MWT) and handgrip force (HGF), elbow flexor muscle strength (EFMS), quadriceps peak torque (QPT) and hamstring peak torque (HPT) tests. Maximal inspiratory pressure (P(I,max)) was recorded. All patients completed the Fatigue Assessment Scale (FAS) questionnaire. The 6MWT was reduced in 45% of the population, while HGF, EFMS, QPT and HPT muscle strength were reduced in 15, 12, 27 and 18%, respectively. P(I,max) was reduced in 43% of the population. The majority of the patients (81%) reported fatigue (FAS ≥22). Patients with reduced peripheral muscle strength of the upper and/or lower extremities were more fatigued and demonstrated impaired lung functions, fat-free mass, P(I,max), 6MWT and quality of life. Fatigue was neither predicted by exercise capacity, nor by muscle strength. Besides fatigue, exercise intolerance and muscle weakness are frequent problems in sarcoidosis. We therefore recommend physical tests in the multidisciplinary management of sarcoidosis patients, even in nonfatigued patients.

  14. Validation of the King's Sarcoidosis Questionnaire (KSQ) in a Dutch sarcoidosis population

    NARCIS (Netherlands)

    Van Manen, Mirjam J G; Wapenaar, Monique; Strookappe, Bert; Drent, Marjolein; Elfferich, Marjon; De Vries, J.; Gosker, Harry R; Birring, Surinder S; Patel, Amit S; Van den Toorn, Leon; Van den Blink, Bernt; Boomars, Karin; Hoitsma, Elske; Wijsenbeek, Marlies S

    2016-01-01

    Background The King's Sarcoidosis Questionnaire (KSQ) is a brief questionnaire assessing health status using five modules (General Health Status, Lung, Eyes, Skin, Medication) in patients with sarcoidosis. The KSQ was only validated in one English sarcoidosis cohort.Objective The aim of this study

  15. Abdominal Sarcoidosis May Mimic Peritoneal Carcinomatosis

    Science.gov (United States)

    Gorkem, Umit; Gungor, Tayfun; Bas, Yılmaz; Togrul, Cihan

    2015-01-01

    Sarcoidosis is a multisystem inflammatory disorder of unknown etiology. It shows a great variety of clinical presentation, organ involvement, and disease progression. Lungs and lymphoid system are the most common sites involved with a frequency of 90% and 30%, respectively. Extrapulmonary involvement of sarcoidosis is reported in 30% of patients and abdomen is the most frequent site. Furthermore, peritoneal involvement is extremely rare in sarcoidosis. The case presented here described peritoneal manifestations of sarcoidosis without involvement of lungs. A 78-year-old woman possessing signs of malignancy on blood test and abdominal magnetic resonance imaging underwent laparatomy with a suspicion of ovarian malignancy. The macroscopic interpretation during surgery was peritoneal carcinomatosis. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, peritoneal biopsies, total omentectomy, and appendectomy were performed. Final histopathological result revealed the diagnosis of sarcoidosis. Clinicians must keep in mind that peritoneal sarcoidosis can mimic intra-abdominal malignancies. PMID:26558122

  16. Dual diagnosis of sarcoidosis and lymphoma.

    LENUS (Irish Health Repository)

    Brady, B

    2013-06-01

    Sarcoidosis is a multisystem granulomatous disease of unknown origin with pulmonary and extrapulmonary manifestations. Worldwide it is most often diagnosed in the third and fourth decades and most often affects Swedish, Danish and black patients. The association between malignancy and sarcoidosis has not been conclusively proven. Cancer can eventually occur in patients who have an established diagnosis of sarcoidosis for example, in sarcoidosis-lymphoma syndrome. Sarcoidosis can also subsequently develop in an oncology patient. There are multiple obstacles to confirming epidemiologically the linkage between sarcoidosis and malignancy. Histological verification and clinical acumen are needed to avoid misdiagnosis. The 18 fluorodeoxyglucose (18-FDG) PET has failed to provide a non invasive diagnostic method to differentiate neoplasia from benign sarcoid lesions and tissue diagnosis is essential before commencing a new therapeutic intervention in patients with lymphoma.

  17. Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association: Report of 12 Cases and Review of the Literature.

    Science.gov (United States)

    Grados, Aurélie; Ebbo, Mikael; Bernit, Emmanuelle; Veit, Véronique; Mazodier, Karin; Jean, Rodolphe; Coso, Diane; Aurran-Schleinitz, Thérèse; Broussais, Florence; Bouabdallah, Reda; Gravis, Gwenaelle; Goncalves, Anthony; Giovaninni, Marc; Sève, Pascal; Chetaille, Bruno; Gavet-Bongo, Florence; Weitten, Thierry; Pavic, Michel; Harlé, Jean-Robert; Schleinitz, Nicolas

    2015-07-01

    The association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported. We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients. Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series. This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse.

  18. Sarcoidosis Presenting as Massive Splenic Infarction

    Directory of Open Access Journals (Sweden)

    Ishita Patel

    2012-01-01

    Full Text Available Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. Granulomatous inflammation involving the spleen is common and associated with splenomegaly. However, massive splenomegaly is a rare occurrence. Infrequently massive splenomegaly can result in splenic infarction. Massive splenic infarction in sarcoidosis has, to our knowledge, not been previously reported. We present a case of a woman presenting with massive splenic infarction and sarcoidosis confirmed by granulomatous inflammation of the liver.

  19. Sarcoidosis presenting as massive splenic infarction.

    Science.gov (United States)

    Patel, Ishita; Ismajli, Mediola; Steuer, Alan

    2012-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. Granulomatous inflammation involving the spleen is common and associated with splenomegaly. However, massive splenomegaly is a rare occurrence. Infrequently massive splenomegaly can result in splenic infarction. Massive splenic infarction in sarcoidosis has, to our knowledge, not been previously reported. We present a case of a woman presenting with massive splenic infarction and sarcoidosis confirmed by granulomatous inflammation of the liver.

  20. Sarcoidosis Presenting as Massive Splenic Infarction

    OpenAIRE

    Ishita Patel; Mediola Ismajli; Alan Steuer

    2012-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. Granulomatous inflammation involving the spleen is common and associated with splenomegaly. However, massive splenomegaly is a rare occurrence. Infrequently massive splenomegaly can result in splenic infarction. Massive splenic infarction in sarcoidosis has, to our knowledge, not been previously reported. We present a case of a woman presenting with massive splenic infarction and sarcoidosis confirmed by granulomatous in...

  1. Childhood sarcoidosis: A rare but fascinating disorder

    OpenAIRE

    Gedalia Abraham; Shetty Avinash K

    2008-01-01

    Abstract Childhood sarcoidosis is a rare multisystemic granulomatous disorder of unknown etiology. In the pediatric series reported from the southeastern United States, sarcoidosis had a higher incidence among African Americans. Most reported childhood cases have occurred in patients aged 13–15 years. Macrophages bearing an increased expression of major histocompatibility class (MHC) II molecules most likely initiate the inflammatory response of sarcoidosis by presenting an unidentified antig...

  2. Imaging of Sarcoidosis: A Contemporary Review.

    Science.gov (United States)

    Guidry, Carey; Fricke, Robert Gaines; Ram, Roopa; Pandey, Tarun; Jambhekar, Kedar

    2016-05-01

    Sarcoidosis is a systemic granulomatous disorder with a variety of clinical presentations and radiological appearances. Although it primarily affects the lungs and lymphatics, sarcoidosis potentially involves essentially every organ system. On imaging, sarcoidosis can mimic different disease entities, including primary and metastatic neoplasms, vasculitis, and other granulomatous infections. Definitive diagnosis often requires a combination of clinical, radiological, and histologic information. Imaging plays a crucial role in diagnosis and evaluating response to therapy. This review covers imaging findings in sarcoidosis within each organ system, with an emphasis on the use of imaging in the diagnosis and management of this condition. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Neuro-otologic manifestations of sarcoidosis.

    Science.gov (United States)

    Hybels, R L; Rice, D H

    1976-12-01

    Sarcoidosis is an idiopathic disease which presents in anatomic areas of concern to otorhinolaryngologists. It can cause dysfunction of both auditory and vestibular systems. In patients known previously to have sarcoidosis, this disease should be seriously considered. In patients presenting with otologic disorders and associated facial nerve paralysis or other neuropathies, uveitis, granulomatous meningitis or diabetes insipidus, sarcoidosis should be suspected. An examination of the eyes as well as a chest X-ray is imperative. Sudden and fluctuating neurosensory hearing loss has been reported. There is a great need for the study of temporal bones from sarcoidosis patients.

  4. A rare clinical presentation of sarcoidosis; gingivitis.

    Science.gov (United States)

    Güzel, Aygül; Köksal, Nurhan; Aydın, Davut; Aslan, Kerim; Gören, Fikret; Karagöz, Filiz

    2013-10-01

    Gingivitis due to sarcoidosis is a relatively rare condition. Gingivitis or isolated gingival involvement may be the first sign of systemic sarcoidosis. We report the case of a 37 year-old woman with isolated gingivitis due to sarcoidosis confirmed by biopsy. Following treatment with a systemic corticosteroid (prednisolone 40 mg/day), all clinical and radiologic findings were completely improved. In cases of chronic and intractable gingivitis, systemic sarcoidosis should be suspected. It should be confirmed with a biopsy, and the patient should be referred to a chest disease clinic to exclude other organ involvement. Copyright © 2013 Elsevier Inc. All rights reserved.

  5. Sarcoidosis presenting as prepatellar bursitis

    Energy Technology Data Exchange (ETDEWEB)

    Fujimoto, Hajime; Shimofusa, Ryota [Numazu City Hospital, Department of Radiology, Numazu, Shizuoka (Japan); Shimoyama, Katsuhito; Nagashima, Ryota [Numazu City Hospital, Department of Orthopaedic Surgery, Numazu, Shizuoka (Japan); Eguchi, Masanobu [Numazu City Hospital, Department of Pathology, Numazu, Shizuoka (Japan)

    2006-01-01

    A 61-year-old woman complained of a subcutaneous mass in her right knee. MR images revealed a well-defined subcutaneous mass in the prepatellar region, containing some fluid and foci of short T2. The lesion showed some marginal contrast uptake after an administration of meglumine gadopentetate (Gd-DTPA), consistent with prepatellar bursitis. The pathological specimen, however, revealed subcutaneous sarcoidosis involving the bursa. (orig.)

  6. Hepatic sarcoidosis mimicking liver cancer

    OpenAIRE

    Yu, Kang-Kang; Liu, Han-Qiu; Zhou, Zhong-Wen; Chen, Ming-Quan

    2015-01-01

    We present a case of a 50-year-old woman with multiple occupations in the liver. Liver cancer was strongly suspected initially according to the results of imaging examination. However, sarcoidosis was confirmed subsequently by liver biopsy, so methylprednisolone was then prescribed and the patient showed favorable therapeutic response. This case report suggests that hepatic mass in Chinese patients without any history of hepatitis virus infection should be carefully investigated before giving...

  7. Cardiac Sarcoidosis: The Impact of Age and Implanted Devices on Survival.

    Science.gov (United States)

    Zhou, Ying; Lower, Elyse E; Li, Hui-Ping; Costea, Alexandru; Attari, Mehran; Baughman, Robert P

    2017-01-01

    To assess the clinical characteristics, diagnosis, and outcome of cardiac sarcoidosis in a single institution sarcoidosis clinic. Patients with cardiac sarcoidosis were identified using refined World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG) criteria of highly probable and probable. Patient demographics, local and systemic treatments, and clinical outcome were collected. Of the 1,815 patients evaluated over a 6-year period, 73 patients met the WASOG criteria for cardiac sarcoidosis. The median age at diagnosis was 46 years, with a median follow-up of 8.8 years. Reduced left ventricular ejection fraction (LVEF) was the most common manifestation (54.8%). Patients with arrhythmias experienced ventricular tachycardia or severe heart block, (35.6% and 19.2%, respectively) with or without reduced LVEF. A total of 45 (61.6%) patients underwent cardiac PET scan and/or MRI, with 41 (91.1%) having a positive study. During follow-up, 10 patients (13.7%) either underwent transplant (n = 3) or died (n = 7) from sarcoidosis. Kaplan-Meier survival curves revealed 5- and 10-year survival rates of 95.5% and 93.4%, respectively. Univariate factors of age at diagnosis  40% were associated with improved survival. Cox regression analysis demonstrated that age ≥ 46 years and lack of an implanted pacemaker or defibrillator were the only independent predictors of mortality. The new WASOG criteria were able to characterize cardiac involvement in our sarcoidosis clinic. Age and lack of pacemaker or defibrillator were the significant predictors of mortality for cardiac sarcoidosis, and reduced LVEF < 40% was associated with worse prognosis. ClinicalTrials.gov; No.: NCT02356445; URL: www.clinicaltrials.gov. Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  8. Generalized sarcoidosis in two horses.

    Science.gov (United States)

    Reijerkerk, E P R; Veldhuis Kroeze, E J B; Sloet van Oldruitenborgh-Oosterbaan, M M

    2008-08-15

    Equine sarcoidosis is a rare disorder usually characterized by exfoliative dermatitis, moderate to severe wasting, and sarcoidal granulomatous inflammation of multiple organ systems. It has an unknown aetiopathogenesis. The condition is not related to equine sarcoid. This case report describes generalized cutaneous and systemic sarcoidosis in an 11-year-old Trakehner mare (case A) and in a 7-year-old Dutch Warmblood gelding (case B). Case A was presented with cutaneous sarcoidosis on the head and body and was diagnosed on the basis of histological examination of skin. Case B presented with multiple subcutaneous nodules (2-15 cm in diameter) and the diagnosis was established at postmortem examination. Both horses showed distinctive histology of the skin with extensive lymphohistiocytic infiltration and Langhans-type multinucleated giant cells. Haematology and biochemistry revealed a normal total white blood cell count with a right shift in both horses. Case B was anaemic and had a slightly elevated total protein concentration with hyperglobulinaemia. Both horses were unresponsive to corticosteroids and were euthanized.

  9. Anxiety, its relation to symptoms severity and anxiety sensitivity in sarcoidosis.

    Science.gov (United States)

    Holas, Pawel; Krejtz, Izabela; Urbankowski, Tomasz; Skowyra, Artur; Ludwiniak, Anna; Domagala-Kulawik, Joanna

    2013-12-17

    Sarcoidosis is a chronic systemic granulomatous disease of unknown etiology. Previous studies demonstrated that patients with sarcoidosis had high rates of depression and anxiety, and high magnitude of stressful life events. To date, however, studies have not examined the anxiety sensitivity in sarcoid patients and the relationship between psychopathology and symptom severity of sarcoidosis.The aims of this study were to evaluate prevalence of depression and anxiety in sarcoid patients, to assess their relationship with the disease symptom severity, and to investigate the relationship between sarcoidosis and anxiety sensitivity. Thirty three sarcoid patients and thirty three control subjects completed the following:Hospital Anxiety and Depression Scale, Anxiety Sensitivity Index-3. The prevalence of depression (29%) and anxiety (31%) was high among patients and comparable to results from other research groups. Anxiety was significantly correlated with symptom severity and was the main covariate of physical symptoms reported by sarcoid patients. Patients exhibited an increase of their total anxiety sensitivity index and had an increased number of physical concerns. These data confirmed earlier reports that anxiety and depression are common in patients with sarcoidosis and expanded on the previous results by showing that patients exhibited increased anxiety sensitivity and a fear of physical sensations. These results, together with the findings that anxiety was associated with sarcoidosis symptom severity, suggest that targeting anxiety and the physical health concerns may be important in the diagnosis and management of this disease.

  10. Polygenic risk assessment reveals pleiotropy between sarcoidosis and inflammatory disorders in the context of genetic ancestry.

    Science.gov (United States)

    Lareau, C A; DeWeese, C F; Adrianto, I; Lessard, C J; Gaffney, P M; Iannuzzi, M C; Rybicki, B A; Levin, A M; Montgomery, C G

    2017-03-01

    Sarcoidosis is a complex disease of unknown etiology characterized by the presence of granulomatous inflammation. Though various immune system pathways have been implicated in disease, the relationship between the genetic determinants of sarcoidosis and other inflammatory disorders has not been characterized. Herein, we examined the degree of genetic pleiotropy common to sarcoidosis and other inflammatory disorders to identify shared pathways and disease systems pertinent to sarcoidosis onset. To achieve this, we quantify the association of common variant polygenic risk scores from nine complex inflammatory disorders with sarcoidosis risk. Enrichment analyses of genes implicated in pleiotropic associations were further used to elucidate candidate pathways. In European-Americans, we identify significant pleiotropy between risk of sarcoidosis and risk of asthma (R2=2.03%; P=8.89 × 10-9), celiac disease (R2=2.03%; P=8.21 × 10-9), primary biliary cirrhosis (R2=2.43%; P=2.01 × 10-10) and rheumatoid arthritis (R2=4.32%; P=2.50 × 10-17). These associations validate in African Americans only after accounting for the proportion of genome-wide European ancestry, where we demonstrate similar effects of polygenic risk for African-Americans with the highest levels of European ancestry. Variants and genes implicated in European-American pleiotropic associations were enriched for pathways involving interleukin-12, interleukin-27 and cell adhesion molecules, corroborating the hypothesized immunopathogenesis of disease.

  11. Multimodality Imaging in Cardiac Sarcoidosis: Is There a Winner?

    Science.gov (United States)

    Perez, Irving E.; Garcia, Mario J.; Taub, Cynthia C.

    2016-01-01

    Sarcoidosis is a multisystem granulomatous disease of unknown cause that can affect the heart. Cardiac sarcoidosis may be present in as many as 25% of patients with systemic sarcoidosis, and it is frequently underdiagnosed. The early and accurate diagnosis of myocardial involvement is challenging. Advanced imaging techniques play important roles in the diagnosis and management of patients with cardiac sarcoidosis. PMID:25784137

  12. Laser therapy for cutaneous sarcoidosis: A review

    Directory of Open Access Journals (Sweden)

    Teo Soleymani

    2016-03-01

    Full Text Available Sarcoidosis is a systemic, multi-organ disease of unknown etiology characteristically defined by the development of non-caseating granulomas. The development of sarcoidosis has been associated with a number of environmental and microbacterial factors coupled with genetic susceptibility. Depending on the type, location and distribution of disease, sarcoidosis can cause functional impairment, symptomatic distress, scarring and disfigurement. The advent of lasers as precise, minimally destructive surgical tools has allowed for their development as promising alternatives that minimize the morbidity associated with current therapies.In this paper, we reviewed the role of laser therapy in the treatment of cutaneous sarcoidosis. A comprehensive search of the Cochrane Library, MEDLINE and PUBMED databases was performed to identify relevant literatures investigating the role of laser therapy in the treatment of cutaneous sarcoidosis. In our opinion, laser therapy, particularly PDL, appears to be an effective, safe and generally well-tolerated modality for the treatment of cutaneous sarcoidosis and should be considered in patients with localized cutaneous disease that is refractory to conventional treatments. Less is known about the efficacy and tolerability of ablative laser therapy for the treatment of cutaneous sarcoidosis, though the limited data appears promising as well. With that said, however, the data is limited and warrants a need for additional larger, randomized controlled studies to further investigate the utility and efficacy of laser therapy in the treatment of cutaneous sarcoidosis.

  13. FDG PET/CT in bone sarcoidosis.

    Science.gov (United States)

    Grozdic Milojevic, Isidora; Sobic-Saranovic, Dragana; Videnovic-Ivanov, Jelica; Saranovic, Djordjije; Odalovic, Strahinja; Artiko, Vera

    2016-03-29

    Bone sarcoidosis is rare manifestation of disease usually accompanied with pulmonary involvement. Until today, exact prevalence of bone sarcoidosis is not known, since reported prevalence varies widely depending on the studied population and the used diagnostic techniques. To determine the prevalence of bone involvement and distribution pattern in active chronic sarcoidosis by using FDG PET/CT. Between January 2010 and December 2011, 98 patients with chronic sarcoidosis and presence of prolonged symptoms or other findings suggestive of active disease were referred to FDG PET/CT examination. Active disease was found in 82 patients, and they all were screened for presence of bone sarcoidosis on FDG PET/CT. All patients also underwent MDCT and assessment of serum ACE level. Bone sarcoidosis was present in 18/82 patients with active sarcoidosis. FDG uptake in bones was focal in 8 (44.4%), diffuse in 6 (33.3%) and both diffuse and focal in 4 (22.2%) patients. CT indicated bone abnormalities only in 5% patients. Osseous involvement was present in: pelvis (61.1%), vertebrae (44.4%), ribs (27.8%) and bone marrow (16.7%). Some patients had two or more locations of disease. Follow-up FDG PET/CT showed normal findings in two patients, same localization of active disease in four patients and progression of disease in one. In patients with active chronic sarcoidosis 22% of patients had osseous abnormalities on FDG PET/CT that mostly were not detected on CT.

  14. Is sarcoidosis a rickettsiosis? An archival study

    DEFF Research Database (Denmark)

    Svendsen, Claus Bo; Milman, Nils; Andersen, Claus B

    2011-01-01

    Based on earlier research, Rickettsia helvetica could possibly be involved in the pathogenesis of sarcoidosis. Rickettsiae are transmitted to humans by a tick vector, Ixodes ricinus; this tick is highly prevalent in Northern Europe. We aimed to investigate the association between evidence...... of rickettsiae and sarcoidosis in histological samples....

  15. Does physical training reduce fatigue in sarcoidosis?

    NARCIS (Netherlands)

    Marcellis, R. G. J.; van der Veeke, M.A.F.; Mesters, I.; Drent, M.; Bie, R.A.; de Vries, Jolanda; Lenssen, A.F.

    2015-01-01

    Background: Sarcoidosis patients frequently experience fatigue, exercise intolerance and muscle weakness, resulting in reduced quality of life (QOL). Scientific studies on the benefits of physical training in sarcoidosis have been scarce, so the aim of this pilot study was to examine the impact of a

  16. Typical and Atypical Manifestations of Intrathoracic Sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hyun Jin; Jung, Jung Im; Chung, Myung Hee; Song, Sun Wha; Kim, Hyo Lim; Baik, Jun Hyun; Han, Dae Hee [St. Vincent' s Hospital, The Catholic University of Korea, Suwon (Korea, Republic of); Kim, Ki Jun [Incheon St. Mary' s Hospital, The Catholic University of Korea, Incheon (Korea, Republic of); Lee, Kyo Young [Seoul St. Mary' s Hospital, The Catholic University of Korea, Seoul (Korea, Republic of)

    2009-12-15

    Sarcoidosis is a systemic disorder of unknown cause that is characterized by the presence of noncaseating granulomas. The radiological findings associated with sarcoidosis have been well described. The findings include symmetric, bilateral hilar and paratracheal lymphadenopathy, with or without concomitant parenchymal abnormalities (multiple small nodules in a peribronchovascular distribution along with irregular thickening of the interstitium). However, in 25% to 30% of cases, the radiological findings are atypical and unfamiliar to most radiologists, which cause difficulty for making a correct diagnosis. Many atypical forms of intrathoracic sarcoidosis have been described sporadically. We have collected cases with unusual radiological findings associated with pulmonary sarcoidosis (unilateral or asymmetric lymphadenopathy, necrosis or cavitation, large opacity, ground glass opacity, an airway abnormality and pleural involvement) and describe the typical forms of the disorder as well. The understanding of a wide range of the radiological manifestations of sarcoidosis will be very helpful for making a proper diagnosis.

  17. Intra-abdominal complications of sarcoidosis.

    Science.gov (United States)

    MacArthur, Kristin Loening; Forouhar, Faripour; Wu, George Yung-Hsing

    2010-07-01

    Sarcoidosis is an inflammatory disease characterized by non-caseating granulomas in the absence of other autoimmune processes, infectious diseases, or foreign agents. The etiology of sarcoidosis is not completely understood. Several organ systems can be affected, of which the most frequently involved include the lungs and lymph nodes. Intra-abdominal sarcoidosis is less common, but can be found in the absence of pulmonary or lymphatic disease. Intra-abdominal sarcoidosis is most often asymptomatic. However, long-standing unrecognized disease can result in life-threatening complications. The identification, monitoring and prevention of these complications will be discussed, with emphasis on both clinical and histological presentations of intra-abdominal sarcoidosis. Copyright 2010 Formosan Medical Association & Elsevier. Published by Elsevier B.V. All rights reserved.

  18. Intra-abdominal Complications of Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Kristin Loening MacArthur

    2010-07-01

    Full Text Available Sarcoidosis is an inflammatory disease characterized by non-caseating granulomas in the absence of other autoimmune processes, infectious diseases, or foreign agents. The etiology of sarcoidosis is not completely understood. Several organ systems can be affected, of which the most frequently involved include the lungs and lymph nodes. Intra-abdominal sarcoidosis is less common, but can be found in the absence of pulmonary or lymphatic disease. Intra-abdominal sarcoidosis is most often asymptomatic. However, long-standing unrecognized disease can result in life-threatening complications. The identification, monitoring and prevention of these complications will be discussed, with emphasis on both clinical and histological presentations of intra-abdominal sarcoidosis.

  19. Sarcoidosis Nodules on the Lateral Nasal Osteotomy Lines.

    Science.gov (United States)

    Basat, Salih Onur; Ceran, Fatih; Aksan, Tolga; Ozturk, Muhammed Besir

    2016-09-01

    Sarcoidosis is a multisystem inflammatory disease that manifests as noncaseating granulomas, commonly in the lungs and intrathoracic lymph nodes. Subcutaneous manifestations of sarcoidosis that are caused by granulomas are referred to as specific for sarcoidosis, whereas other lesions are considered nonspecific. The authors present "sarcoidosis nodule formation on the lateral nasal osteotomy lines" in a sarcoidosis patient undergoing rhinoplasty surgery as a rare patient. V.

  20. CT findings in a case of laryngeal sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Ferretti, Gilbert R.; Calaque, O.; Coulomb, Max [Department of Radiology, Hopital Michallon, Grenoble (France); Reyt, E. [Department of Oto-Rhino-Laryngology, Hopital Michallon, Grenoble (France); Massot, C. [Department of Internal Medicine, Hopital Michallon, Grenoble (France)

    2002-04-01

    Laryngeal sarcoidosis is a rare manifestation of systemic sarcoidosis. It affects mainly the supraglottic larynx. Involvement of the glottic and subglottic levels are exceptional. We present the case of a 56-year-old man with a 2-year history of systemic sarcoidosis, involving the mucosa of paranasal sinuses, a joint, and mediastinal lymph nodes, who developed laryngeal sarcoidosis. We emphasize the CT appearance of laryngeal sarcoidosis. (orig.)

  1. Familial early onset sarcoidosis with bone cysts and erosions

    Energy Technology Data Exchange (ETDEWEB)

    Blank, Norbert; Max, Regina; Lorenz, Hanns-Martin [University of Heidelberg, Department of Internal Medicine V, Division of Rheumatology, Heidelberg (Germany); Autschbach, Frank [University of Heidelberg, Department of Pathology, Heidelberg (Germany); Libicher, Martin [University of Cologne, Department of Radiology, Cologne (Germany)

    2007-09-15

    Early onset sarcoidosis is a granulomatous disease which is characterized by synovitis, polyarthritis, skin and eye involvement. We report the skeletal features of one patient with a family history and clinical symptoms suggestive of early onset sarcoidosis (EOS) which was confirmed by skin biopsy. Radiographs reveal postarthritic deformities of the MCP joints, contractures, a coarsened trabecular pattern at the PIP joints and small bone cysts resembling osteitis cystoides multiplex. Similar lesions were described in radiographs of the older sister and an uncle of our patient. This is the first report demonstrating bone cysts and erosions which could be a diagnostic feature in this rare disease and may help to differentiate other rheumatoid disorders. (orig.)

  2. Dual-energy CT in vertebral compression fractures: performance of visual and quantitative analysis for bone marrow edema demonstration with comparison to MRI

    Energy Technology Data Exchange (ETDEWEB)

    Bierry, Guillaume; Venkatasamy, Aina; Kremer, Stephane; Dosch, Jean-Claude; Dietemann, Jean-Louis [University Hospital of Strasbourg, Department of Radiology, Strasbourg (France)

    2014-04-15

    To prospectively evaluate the performance of virtual non-calcium (VNC) dual-energy CT (DECT) images for the demonstration of trauma-related abnormal marrow attenuation in collapsed and non-collapsed vertebral compression fractures (VCF) with MRI as a reference standard. Twenty patients presenting with non-tumoral VCF were consecutively and prospectively included in this IRB-approved study, and underwent MRI and DECT of the spine. MR examination served as a reference standard. Two independent readers visually evaluated all vertebrae for abnormal marrow attenuation (''CT edema'') on VNC DECT images; specificity, sensitivity, predictive values, intra and inter-observer agreements were calculated. A last reader performed a quantitative evaluation of CT numbers; cut-off values were calculated using ROC analysis. In the visual analysis, VNC DECT images had an overall sensitivity of 84 %, specificity of 97 %, and accuracy of 95 %, intra- and inter-observer agreements ranged from k = 0.74 to k = 0.90. CT numbers were significantly different between vertebrae with edema on MR and those without (p < 0.0001). Cut-off values provided sensitivity of 85 % (77 %) and specificity of 82 % (74 %) for ''CT edema'' on thoracic (lumbar) vertebrae. VNC DECT images allowed an accurate demonstration of trauma-related abnormal attenuation in VCF, revealing the acute nature of the fracture, on both visual and quantitative evaluation. (orig.)

  3. Fibromatosis of breast mimicking sarcoidosis

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    Silonie Sachdeva

    2011-01-01

    Full Text Available Primary mammary fibromatosis is a rare skin condition which can arise after trauma or previous surgery. The exact etiology is unknown. Very few cases have been reported in literature and the main emphasis is to differentiate this condition from breast carcinoma. We report here an unusual case of a 60 year old female who presented with skin lesion which clinically looked sarcoid with history suggestive of sarcoidosis, but on histopathology fibromatosis of breast was revealed. Complete work up ruled out any carcinomatous changes. Surgical excision of the lesion was done with no recurrence seen in one year follow up period.

  4. Sarcoidosis extent relates to molecular variability.

    Science.gov (United States)

    Monast, C S; Li, K; Judson, M A; Baughman, R P; Wadman, E; Watt, R; Silkoff, P E; Barnathan, E S; Brodmerkel, C

    2017-06-01

    The molecular basis of sarcoidosis phenotype heterogeneity and its relationship to effective treatment of sarcoidosis have not been elucidated. Peripheral samples from sarcoidosis subjects who participated in a Phase II study of golimumab [anti-tumour necrosis factor (TNF)-α] and ustekinumab [anti-interleukin (IL)-12p40] were used to measure the whole blood transcriptome and levels of serum proteins. Differential gene and protein expression analyses were used to explore the molecular differences between sarcoidosis phenotypes as defined by extent of organ involvement. The same data were also used in conjunction with an enrichment algorithm to identify gene expression changes associated with treatment with study drugs compared to placebo. Our analyses revealed marked heterogeneity among the three sarcoidosis phenotypes included in the study cohort, including striking differences in enrichment of the interferon pathway. Conversely, enrichments of multiple pathways, including T cell receptor signalling, were similar among phenotypes. We also identify differences between treatment with golimumab and ustekinumab that may explain the differences in trends for clinical efficacy observed in the trial. We find that molecular heterogeneity is associated with sarcoidosis in a manner that may be related to the extent of organ involvement. These findings may help to explain the difficulty in identifying clinically efficacious sarcoidosis treatments and suggest hypotheses for improved therapeutic strategies. © 2017 British Society for Immunology.

  5. Characterization of chicken octamer-binding proteins demonstrates that POU domain-containing homeobox transcription factors have been highly conserved during vertebrate evolution

    Energy Technology Data Exchange (ETDEWEB)

    Petryniak, B.; Postema, C.E.; McCormack, W.T.; Thompson, C.B. (Univ. of Michigan Medical Center, Ann Arbor (USA)); Staudt, L.M. (National Cancer Institute, Bethesda, MD (USA))

    1990-02-01

    The DNA sequence motif ATTTGCAT (octamer) or its inverse complement has been identified as an evolutionarily conserved element in the promoter region of immunoglobulin genes. Two major DNA-binding proteins that bind in a sequence-specific manner to the octamer DNA sequence have been identified in mammalian species--a ubiquitously expressed protein (Oct-1) and a lymphoid-specific protein (Oct-2). During characterization of the promoter region of the chicken immunoglobulin light chain gene, the authors identified two homologous octamer-binding proteins in chicken B cells. when the cloning of the human gene for Oct-2 revealed it to be a member of a distinct family of homeobox genes, they sought to determine if the human Oct-2 cDNA could be used to identify homologous chicken homeobox genes. Using a human Oct-2 homeobox-specific DNA probe, they were able to identify 6-10 homeobox-containing genes in the chicken genome, demonstrating that the Oct-2-related subfamily of homeobox genes exists in avian species. DNA sequence analysis revealed it to be the chicken homologue of the human Oct-1 gene. Together, the data show that the POU-containing subfamily of homeobox genes have been highly conserved during vertebrate evolution, apparently as a result of selection for their DNA-binding and transcriptional regulatory properties.

  6. Biopsy proven pituitary sarcoidosis presenting as a possible adenoma.

    Science.gov (United States)

    Prayson, Richard A

    2016-12-01

    Sarcoidosis is a well-recognized systemic granulomatous process which involves the central nervous system in 5-15% of patients. One of the more frequent sites of central nervous system involvement is the pituitary and hypothalamic region. Involvement of the sellar region by sarcoidosis is overall an infrequent occurrence, comprising less than 1% of all intrasellar lesions. Patients typically present with an infiltrative lesion on imaging studies and clinically with symptoms related to diabetes insipidus or hyperprolactinemia. This report describes a 38-year-old woman who initially presented with a variety of symptoms including headaches, light sensitivity, nausea and vomiting, acute visual changes, cold intolerance, amenorrhea, decreased libido, fatigue and galactorrhea. She had an elevated serum prolactin level and evidence of oligoclonal bands in the cerebrospinal fluid. Imaging studies discovered a 1.8cm mass involving the pituitary gland and compressing the optic chiasm. The lesion was excised and microscopically was marked by a chronic inflammatory cell infiltrate and scattered nonnecrotizing granulomas. Stains and microbiologic cultures failed to demonstrate microorganisms. There was no evidence of other organ involvement on postoperative imaging. She was treated with prednisone with improvement of symptoms and subsequently required methotrexate to treat left eye pain and blurred vision, 29months after her surgery. Achieving treatment control in patients with pituitary and hypothalamic improvement in sarcoidosis still remains a challenge. Copyright © 2016 Elsevier Ltd. All rights reserved.

  7. Sarcoidosis in a dental surgeon: a case report

    Directory of Open Access Journals (Sweden)

    Mattia Daniele

    2010-08-01

    Full Text Available Abstract Introduction Although the causes of sarcoidosis are still unknown, past and current studies have provided evidence that this disease may be associated with occupational exposure to specific environmental agents. We describe a case of sarcoidosis in a dental surgeon with long exposure to inorganic dusts. To the best of our knowledge, this is the first report of this kind in the literature. Case presentation At the beginning of 2000, a 52-year-old Caucasian man, who worked as a dental surgeon, presented with shortness of breath during exercise, cough and retrosternal pain. After diagnosis of sarcoidosis, a scanning electronic microscopy with X-ray microanalysis of biopsy specimens was used in order to determine whether the disease could be traced to an occupational environmental agent. Results showed the presence of inorganic dust particles within sarcoidotic granulomas, and demonstrated that the material detected was identical to that found in a powder used by our patient for several years. Conclusions Although these results cannot be considered as definitive proof, they do however provide strong evidence that this disease may be associated with material used by dental surgeons.

  8. Sarcoidosis, cancer and molecular mimicry.

    Science.gov (United States)

    Tchernev, G; Wollina, U

    2013-01-01

    Molecular mimicry seems to be the most important factor for the heterogeneous clinical presentation and the immunopathogenesis of sarcoidosis. Molecular mimicry may occur as a result of altered activity of oncogenes. This can lead to crossed-type mediated body reactions targeting structurally similar sections or regions from the tissue homeostasis. Available data suggest that structural analogy between tissue and foreign or de novo-appearing peptides is not always reliable. Nevertheless, lack of amino acid identity between the tissue and the de novo-generated tumour antigens does not exclude the phenomenon of molecular mimicry as the major generator of sarcoidosis. There is growing evidence of the mimicry phenomena, caused not only by the similarity between the amino acids but also between the elements which connect segments in the immunological cascade and which may also be affected by external factors. Molecular mimicry may occur between two identified peptides having similar antigenic surfaces (transitory or not), in the absence of a primary homology in amino acid sequence. As far as tumour antigens are concerned, a structural analogy to the de novo-appearing tumour antigens is more likely than transitory imitation resulting from the additional interference of other physical forces. Further research should be performed to confirm, or reject, the transitory imitation thesis or hypothesis.

  9. [An unusual presentation of sarcoidosis].

    Science.gov (United States)

    Nakano, Y; Matsushita, H

    1997-02-01

    A 52-year-old man was admitted to our hospital with chief complaints of fever, general fatigue, and weight loss. A chest roentgenogram showed hyperinflation and many small nodular shadows in both lung fields. A chest CT scan showed centrilobular shadows distributed equally over all lung fields. Examination of a transbronchial lung biopsy specimen revealed a small non-caseating granuloma composed of a giant cell of Langhans type, epithelioid cells, and a few lymphocytes. There was a dark red eruption about 1 cm in diameter on the right forearm, which had been present for a few years, to the best of the patient's memory. Examination of a skin biopsy specimen revealed many non-caseating granulomas composed of epithelioid cells. Uveitis developed about 2 months after admission. Our diagnosis was sarcoidosis. Steroid therapy was started. The patient's symptoms rapidly disappeared and a chest roentgenogram showed remarkable improvement. We believe this was a very peculiar case of sarcoidosis: the patient had systemic symptoms only, and roentgenographic examinations showed hyperinflation and centrilobular shadows distributed over all lung fields.

  10. Local and Systemic CD4+ T Cell Exhaustion Reverses with Clinical Resolution of Pulmonary Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Charlene Hawkins

    2017-01-01

    Full Text Available Investigation of the Th1 immune response in sarcoidosis CD4+ T cells has revealed reduced proliferative capacity and cytokine expression upon TCR stimulation. In other disease models, such cellular dysfunction has been associated with a step-wise, progressive loss of T cell function that results from chronic antigenic stimulation. T cell exhaustion is defined by decreased cytokine production upon TCR activation, decreased proliferation, increased expression of inhibitory cell surface receptors, and increased susceptibility to apoptosis. We characterized sarcoidosis CD4+ T cell immune function in systemic and local environments among subjects undergoing disease progression compared to those experiencing disease resolution. Spontaneous and TCR-stimulated Th1 cytokine expression and proliferation assays were performed in 53 sarcoidosis subjects and 30 healthy controls. PD-1 expression and apoptosis were assessed by flow cytometry. Compared to healthy controls, sarcoidosis CD4+ T cells demonstrated reductions in Th1 cytokine expression, proliferative capacity (p<0.05, enhanced apoptosis (p<0.01, and increased PD-1 expression (p<0.001. BAL-derived CD4+ T cells also demonstrated multiple facets of T cell exhaustion (p<0.05. Reversal of CD4+ T cell exhaustion was observed in subjects undergoing spontaneous resolution (p<0.05. Sarcoidosis CD4+ T cells exhibit loss of cellular function during progressive disease that follows the archetype of T cell exhaustion.

  11. Who Is at Risk for Sarcoidosis?

    Science.gov (United States)

    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  12. Extra-pulmonary manifestations of sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Vardhanabhuti, V. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Venkatanarasimha, N. [St Michael' s Hospital, 30 Bond Street, Toronto, Ontario M5B 1W8 (Canada); Bhatnagar, G.; Maviki, M.; Iyengar, S.; Adams, W.M. [Radiology Department, Derriford Hospital, Plymouth (United Kingdom); Suresh, P., E-mail: sureshpriya2000@yahoo.com [Radiology Department, Derriford Hospital, Plymouth (United Kingdom)

    2012-03-15

    Although, the diagnosis and evaluation of sarcoidosis has traditionally remained confined to the chest, its multi-system nature has been widely recognized. Radiological features of pulmonary sarcoidosis are well known but extra-pulmonary manifestations can produce a plethora of non-specific imaging findings that can affect subcutaneous tissue, and the neurological, cardiac, gastrointestinal, urological, liver, spleen, and skeletal systems. In the literature, there are various case reports and specific system reviews but there are few reviews that encompass all the extra-pulmonary manifestations. In this paper, we comprehensively review the imaging features of extra-pulmonary sarcoidosis with characteristic features as well as atypical presentations. In addition, we discuss the emerging role of nuclear medicine in sarcoidosis.

  13. [Sarcoidosis in children with digestive manifestations].

    Science.gov (United States)

    Parlier, G; Josset, P; Charritat, J L; Tounian, P; Girardet, J P; Boccon-Gibod, L; Fontaine, J L

    1996-02-01

    Gastric involvement is the least rare among digestive localizations of sarcoidosis, as well in adults as in children. When it is to be seen at the beginning of the disease, it may cause difficulties in the diagnostic, especially with Crohn's disease. Gastric ulcers were detected in a 12 year-old girl, of African origin, who complained about epigastric pain. Eighteen months later, diarrhea, poor growing, uveitis and inflammatory biological signs led to a probable diagnostic of Crohn's disease. Endoscopy seemed to confirm this diagnostic with granulomatous lesions on gastric biopsies. The absence of radiological anomalies of the digestive tract and the poor efficiency of the medical treatment led to question this diagnosis and to assert that of sarcoidosis. This case allows to emphasize the rare involvement of the digestive tract in sarcoidosis and the aspects common both to Crohn's disease and sarcoidosis.

  14. Sarcoidosis in Melanoma Patients: Case Report and Literature Review

    Science.gov (United States)

    Beutler, Bryce D.; Cohen, Philip R.

    2015-01-01

    Sarcoidosis is a systemic inflammatory disease characterized by the development of noncaseating granulomas in multiple organ systems. Many hematologic malignancies and solid tumors, including melanoma, have been associated with sarcoidosis. We describe the clinical and pathologic findings of a 54-year-old man with melanoma-associated sarcoidosis. In addition, we not only review the literature describing characteristics of other melanoma patients with sarcoidosis, but also the features of melanoma patients with antineoplastic therapy-associated sarcoidosis. Sarcoidosis has been described in 80 melanoma patients; sufficient information for analysis was provided in 39 of these individuals. In 43.6% of individuals (17 out of 39), sarcoidosis was directly associated with melanoma; in 56.4% of oncologic patients (22 out of 39), sarcoidosis was induced by antineoplastic therapy that had been administered for the treatment of their metastatic melanoma. The discovery of melanoma preceded the development of sarcoidosis in 12 of the 17 (70.5%) individuals who did not receive systemic treatment. Pulmonary and/or cutaneous manifestations of sarcoidosis were common among both groups of patients. Most patients did not require treatment for sarcoidosis. Melanoma patients—either following antineoplastic therapy or without systemic treatment—may be at an increased risk to develop sarcoidosis. In antineoplastic therapy naive melanoma patients, a common etiologic factor—such as exposure to ultraviolet light—may play a role in their developing melanoma and sarcoidosis. PMID:26083934

  15. Sarcoidosis in Melanoma Patients: Case Report and Literature Review

    Energy Technology Data Exchange (ETDEWEB)

    Beutler, Bryce D., E-mail: brycebeutler@hotmail.com [School of Allied Health Sciences, University of Nevada, Las Vegas, 1060 Wiegand Road, Encinitas, CA 92024 (United States); Cohen, Philip R., E-mail: brycebeutler@hotmail.com [Department of Dermatology, University of California San Diego, 10991 Twinleaf Court, San Diego, CA 92131 (United States)

    2015-06-15

    Sarcoidosis is a systemic inflammatory disease characterized by the development of noncaseating granulomas in multiple organ systems. Many hematologic malignancies and solid tumors, including melanoma, have been associated with sarcoidosis. We describe the clinical and pathologic findings of a 54-year-old man with melanoma-associated sarcoidosis. In addition, we not only review the literature describing characteristics of other melanoma patients with sarcoidosis, but also the features of melanoma patients with antineoplastic therapy-associated sarcoidosis. Sarcoidosis has been described in 80 melanoma patients; sufficient information for analysis was provided in 39 of these individuals. In 43.6% of individuals (17 out of 39), sarcoidosis was directly associated with melanoma; in 56.4% of oncologic patients (22 out of 39), sarcoidosis was induced by antineoplastic therapy that had been administered for the treatment of their metastatic melanoma. The discovery of melanoma preceded the development of sarcoidosis in 12 of the 17 (70.5%) individuals who did not receive systemic treatment. Pulmonary and/or cutaneous manifestations of sarcoidosis were common among both groups of patients. Most patients did not require treatment for sarcoidosis. Melanoma patients—either following antineoplastic therapy or without systemic treatment—may be at an increased risk to develop sarcoidosis. In antineoplastic therapy naive melanoma patients, a common etiologic factor—such as exposure to ultraviolet light—may play a role in their developing melanoma and sarcoidosis.

  16. Comorbidity of Leishmania major with cutaneous sarcoidosis

    Directory of Open Access Journals (Sweden)

    Hamideh Moravvej

    2014-01-01

    Full Text Available Background: leishmaniasis infection might manifest as sarcoidosis; on the other hand, some evidences propose an association between sarcoidosis and leishmaniasis. Most of the times, it is impossible to discriminate idiopathic sarcoidosis from leishmaniasis by conventional histopathologic exam. Aim: We performed a cross-sectional study to examine the association of sarcoidosis with leishmaniasis in histopathologically diagnosed sarcoidal granuloma biopsy samples by polymerase chain reaction (PCR. Materials and Methods: We examined paraffin-embedded skin biopsy samples obtained from patients with clinical and histopathological diagnosis as naked sarcoidal granuloma, referred to Skin Research Center of Shaheed Beheshti Medical University from January 2001 to March 2010, in order to isolate Leishmania parasite. The samples were reassessed by an independent dermatopathologist. DNA extracted from all specimens was analyzed by the commercially available PCR kits (DNPTM Kit, CinnaGen, Tehran, Iran to detect endemic Leishmania species, namely leishmania major (L. major. Results: L. major was positive in PCR of Eight out of twenty-five examined samples. Conclusion: Cutaneous leishmaniasis may be misinterpreted as sarcoidosis; in endemic areas, when conventional methods fail to detect Leishmania parasite, PCR should be utilized in any granulomatous skin disease compatible with sarcoidosis, regardless of the clinical presentation or histopathological interpretation.

  17. Imaging aspects of the diagnosis of sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Spagnolo, Paolo [University of Modena and Reggio Emilia, Center for Rare Lung Diseases, Respiratory Disease Unit, Department of Oncology Haematology and Respiratory Diseases, Modena (Italy); Sverzellati, Nicola [University of Parma, Department of Surgical Sciences, section of Radiology, Parma (Italy); Wells, Athol U. [Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom); Hansell, David M. [Royal Brompton Hospital, Department of Radiology, London (United Kingdom)

    2014-04-15

    Sarcoidosis is a systemic granulomatous disorder of unknown aetiology with a wide spectrum of radiological appearances and almost invariably pulmonary involvement. Lung involvement accounts for most of the morbidity and much of the mortality associated with sarcoidosis. Imaging contributes significantly to the diagnosis and management of patients with sarcoidosis. In typical cases, chest radiography may be sufficient to establish the diagnosis with little margin of error and CT is not necessary. However, CT can play a critical role in several clinical settings: atypical clinical and/or radiographic findings; normal or near-normal chest radiograph but clinical suspicion of sarcoidosis; and detection of complications. Moreover, in many patients, CT findings are atypical and unfamiliar to most radiologists (e.g. sarcoidosis mimicking other lung diseases and vice versa), and in these cases histological confirmation of the diagnosis is recommended. CT is also useful in assessing disease extent and may help to discriminate between reversible and irreversible lung disease, thus providing critical prognostic information. This review concentrates on the more difficult imaging aspects of sarcoidosis, in particular differential diagnosis and disease complications. (orig.)

  18. Mechanism of granuloma formation in sarcoidosis.

    Science.gov (United States)

    Sakthivel, Priya; Bruder, Dunja

    2017-01-01

    The formation of noncaseating granuloma is a hallmark of pulmonary sarcoidosis. This review summarizes recent progress made to explain the cellular dynamics within the granuloma structure that may considerably differ between the two clinically distinct variants, that is, acute and chronic sarcoidosis. Compelling evidence exists that in acute but not chronic sarcoidosis CD4 T lymphocytes specifically recognizing the auto-antigen vimentin on human leukocyte antigen-DR3 molecules accumulate in sarcoid granuloma. These so-called TH17.1 cells produce high amounts of the TH17-related cytokines interleukin-17 (IL-17) and IL-22 in addition to interferon-γ. Moreover, regulatory T cells from patients with acute sarcoidosis are ICOS, providing a mechanistic link to the comparably high concentration of IL-10 exclusively found in the airways of these patients. Next to obvious differences in T effector cell and Treg subsets, alveolar macrophages harbor a functional mitochondrial system in acute sarcoidosis patients, while this system is impaired in patients with chronic disease. We provide a comprehensive update on the cellular components and their functional implications in sarcoid granuloma formation, with special emphasis on the specific characteristics of granuloma in acute versus chronic sarcoidosis. Moreover, the specific antigens thought to be involved in both forms of the disease are discussed.

  19. CT findings in severe thoracic sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Hennebicque, Anne-Sophie; Brillet, Pierre-Yves; Moulahi, Hassen; Brauner, Michel W. [UFR Bobigny, Department of Radiology, Federation MARTHA and EA 2363, Bobigny Cedex (France); Nunes, Hilario; Valeyre, Dominique [UFR Bobigny, Department of Pneumology, Federation MARTHA and EA 2363, Bobigny Cedex (France)

    2005-01-01

    Severe thoracic sarcoidosis includes manifestations with significant clinical and functional impairment and a risk of mortality. Severe thoracic sarcoidosis can take on various clinical presentations and is associated with increased morbidity. The purpose of this article was to describe the CT findings in severe thoracic sarcoidosis and to explain some of their mechanisms. Subacute respiratory insufficiency is a rare and early complication due to a high profusion of pulmonary lesions. Chronic respiratory insufficiency due to pulmonary fibrosis is a frequent and late complication. Three main CT patterns are identified: bronchial distortion, honeycombing and linear opacities. CT can be helpful in diagnosing some mechanisms of central airway obstruction such as bronchial distortion due to pulmonary fibrosis or an extrinsic bronchial compression by enlarged lymph nodes. An intrinsic narrowing of the bronchial wall by endobronchial granulomatous lesions may be suggested by CT when it shows evidence of bronchial mural thickening. Pulmonary hypertension usually occurs in patients with end-stage pulmonary disease and is related to fibrotic destruction of the distal capillary bed and to the resultant chronic hypoxemia. Several other mechanisms may contribute to the development of pulmonary hypertension including extrinsic compression of major pulmonary arteries by enlarged lymph nodes and secondary pulmonary veno-occlusive disease. Aspergilloma colonization of a cavity is the main cause of hemoptysis in sarcoidosis. Other rare causes are bronchiesctasis, necrotizing bronchial aspergillosis, semi-invasive pulmonary aspergillosis, erosion of a pulmonary artery due to a necrotic sarcoidosis lesion, necrosis of parenchymal sarcoidosis lesions and specific endobronchial macroscopic lesions. (orig.)

  20. Activated alveolar macrophage and lymphocyte alveolitis in extrathoracic sarcoidosis without radiological mediastinopulmonary involvement

    Energy Technology Data Exchange (ETDEWEB)

    Wallaert, B.; Ramon, P.; Fournier, E.C.; Prin, L.; Tonnel, A.B.; Voisin, C.

    1986-01-01

    Cellular characteristics of BAL were investigated in 18 patients with proved extrathoracic sarcoidosis (that is, sarcoidosis that affected the skin, eyes, parotid glands, stomach, nose, kidneys, or meninges) without clinical or radiological mediastinopulmonary involvement. Computed tomography of the thorax was performed on five patients: four patients were normal, and one had enlarged lymph nodes (these enlargements were not detectable on the patient's chest roentgenogram). The results of pulmonary function tests were normal in all patients. The total BAL cell count did not differ significantly between controls and patients. Abnormal percentages of alveolar lymphocytes (from 18 to 87%) were noted in 15 out of 18 patients. SACE levels were normal in 15 patients. No pulmonary gallium uptake was detected. The chemiluminescence of AM's, whether spontaneous or PMA induced, was increased in five out of seven patients. The percentages of T3+ lymphocytes in sarcoidosis patients did not significantly differ from those in controls. The T4+:T8+ ratio was normal in four patients and slightly increased in one. Follow-up of patients showed that alveolar lymphocytosis is as lasting as extrathoracic involvement. Our data demonstrate increased percentages of lymphocytes and activated AM's in the BAL of patients with extrathoracic sarcoidosis. This may be due to the initial involvement of the respiratory tract in extrathoracic sarcoidosis or to the diffusion of activated macrophages and lymphocytes from an extrathoracic site into the lung.

  1. Chest computed tomography-based scoring of thoracic sarcoidosis: Inter-rater reliability of CT abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Heuvel, D.A.V. den; Es, H.W. van; Heesewijk, J.P. van; Spee, M. [St. Antonius Hospital Nieuwegein, Department of Radiology, Nieuwegein (Netherlands); Jong, P.A. de [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Zanen, P.; Grutters, J.C. [University Medical Center Utrecht, Division Heart and Lungs, Utrecht (Netherlands); St. Antonius Hospital Nieuwegein, Center of Interstitial Lung Diseases, Department of Pulmonology, Nieuwegein (Netherlands)

    2015-09-15

    To determine inter-rater reliability of sarcoidosis-related computed tomography (CT) findings that can be used for scoring of thoracic sarcoidosis. CT images of 51 patients with sarcoidosis were scored by five chest radiologists for various abnormal CT findings (22 in total) encountered in thoracic sarcoidosis. Using intra-class correlation coefficient (ICC) analysis, inter-rater reliability was analysed and reported according to the Guidelines for Reporting Reliability and Agreement Studies (GRRAS) criteria. A pre-specified sub-analysis was performed to investigate the effect of training. Scoring was trained in a distinct set of 15 scans in which all abnormal CT findings were represented. Median age of the 51 patients (36 men, 70 %) was 43 years (range 26 - 64 years). All radiographic stages were present in this group. ICC ranged from 0.91 for honeycombing to 0.11 for nodular margin (sharp versus ill-defined). The ICC was above 0.60 in 13 of the 22 abnormal findings. Sub-analysis for the best-trained observers demonstrated an ICC improvement for all abnormal findings and values above 0.60 for 16 of the 22 abnormalities. In our cohort, reliability between raters was acceptable for 16 thoracic sarcoidosis-related abnormal CT findings. (orig.)

  2. Breast sarcoidosis appearing as a primary manifestation of sarcoidosis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hye Jeong; Kim, Eun Kyung; Kim, Min Jung; Oh, Ki Keun; Kim, Se Hoon [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2007-06-15

    Breast sarcoidosis is a rare disease entity and may have similar imaging findings as a breast malignancy. We report here a case of primary breast sarcoidosis that was diagnosed as nonspecific inflammation by sonography-guided 14-G automated needle biopsy. A directional vacuum assisted biopsy was helpful for a correct diagnosis.

  3. Common features of tuberculosis and sarcoidosis

    Directory of Open Access Journals (Sweden)

    Esmaeil Mortaz

    2016-01-01

    Full Text Available Tuberculosis (TB is a disease caused by Mycobacterium tuberculosis. Despite the availability of novel therapeutic approaches, TB is considered as one of the leading causes of death due to infectious diseases worldwide. Alveolar macrophages are the first line of defense against M. tuberculosis; they ingest and sequester the bacilli within granulomatous structures. Control and resolution of the infection requires activated T lymphocytes as well as Th1 cytokines. There are two forms of TB: active TB and latent TB. Latent TB is a state in which M. tuberculosis survives in the body without causing overt signs and symptoms. People with latent TB are noncontagious. However, M. tuberculosis can become active in the body, multiply, and cause overt TB. Sarcoidosis, on the other hand, is an autoimmune disease of unknown etiology which can affect multiple systems of the body. Nonspecific constitutional symptoms, such as fever, fatigue, malaise, and weight loss, are present in approximately one-third of patients. Chest X-ray usually shows hilar and mediastinal lymphadenopathy. Although the lungs are the most common sites of inflammation, sarcoidosis can also involve other organs, such as the eyes (intraocular and adnexal, skin, lymph nodes, salivary glands, heart, spleen, liver, and the nervous system. Recent investigations have provided further insights into the genetic basis of sarcoidosis and the way genotype determines the clinical presentation and phenotype of patients. Histopathologic features are usually insufficient for diagnosis of sarcoidosis. Diagnosis of sarcoidosis in endemic areas for TB can become a great challenge. Both TB and sarcoidosis are granulomatous diseases; TB is characterized by caseating granulomas, whereas sarcoidosis is characterized by noncaseating granulomas. New cases of sarcoidosis are increasingly being diagnosed in areas endemic for TB due to increased orientation of physicians and availability of diagnostic modalities

  4. Association of sarcoidosis and myasthenia gravis: Case Report ...

    African Journals Online (AJOL)

    Whereas the coexistence of different autoimmune or rheumatologic diseases with myasthenia gravis (MG) is well documented, its combination with sarcoidosis is extremely rare. Presented here is an interesting case with coexisting MG and sarcoidosis.

  5. Electrophysiological Features of Atrial Flutter in Cardiac Sarcoidosis: A Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Narayanan Namboodiri, MD

    2012-11-01

    Full Text Available We report two cases of systemic sarcoidosis with atrial flutter as the clinical manifestation. In one patient, who had symptoms of shorter duration, the arrhythmia was no longer inducible after a course of glucocorticoid therapy. Electroanatomical mapping in the other case revealed patchy fibrosis of the left atrial myocardium and multiple macro-reentrant circuits. Sinus rhythm could be restored with ablation of these reentrant circuits. To our knowledge, this is the first report on the demonstration of atrial scarring in a patient with sarcoidosis using 3-D electroanatomical mapping. These two cases illustrate that the inflammation of atrial myocardium is the primary mechanism of atrial arrhythmias in patients with cardiac sarcoidosis.

  6. Cardiac magnetic resonance imaging-indeterminate/negative cardiac sarcoidosis revealed by 18F-fluorodeoxyglucose-positron emission tomography: two case reports and a review of the literature

    Directory of Open Access Journals (Sweden)

    S. C. Sasson

    2017-10-01

    Full Text Available Abstract Background Sarcoidosis is an inflammatory disorder of immune dysregulation characterized by non-caseating granulomas that can affect any organ. Cardiac sarcoidosis is an under-recognized entity that has a heterogeneous presentation and may occur independently or with any severity of systemic disease. Diagnosing cardiac sarcoidosis remains problematic with endomyocardial biopsies associated with a high risk of complications. Several diagnostic algorithms are currently available that rely on histopathology or clinical and radiological measures. The dominant mode of diagnostic imaging to date for cardiac sarcoidosis has been cardiac magnetic resonance imaging with gadolinium enhancement. Case presentations We report the cases of two adult patients: case 1, a 50-year-old white man who presented with severe congestive cardiac failure; and case 2, a 37-year-old white woman who presented with complete heart block. Both patients had a background of untreated pulmonary sarcoidosis. Cardiac magnetic resonance imaging did not show evidence of sarcoidosis in either patient and both proceeded to 18F-fluorodeoxyglucose-positron emission tomography scans that were highly suggestive of cardiac sarcoidosis. Both patients were systemically immunosuppressed with orally administered prednisone and methotrexate and had subsequent improvement by clinical and nuclear medicine imaging measures. Conclusions Current consensus guidelines recommend all patients with sarcoidosis undergo screening for occult cardiac disease, with thorough history and examination, electrocardiogram, and transthoracic echocardiogram. If any abnormalities are detected, advanced cardiac imaging should follow. While cardiac magnetic resonance imaging identifies the majority of cardiac sarcoidosis, early disease may not be detected. These cases demonstrate 18F-fluorodeoxyglucose-positron emission tomography is warranted following an indeterminate or normal cardiac magnetic resonance

  7. ARA290 : a novel treatment for neuropathic pain in sarcoidosis

    NARCIS (Netherlands)

    Heij, L.

    2016-01-01

    The general aim of this thesis is to investigate small fiber neuropathy in sarcoidosis and to asses whether ARA290 is a possible new agent to treat the neuropathic complaints in sarcoidosis population. The results of the various ARA290 trials in painful sarcoidosis are discussed. Painful neuropathy

  8. Sarcoidosis in Lagos Revisited. A Retrospective Analysis of 15 ...

    African Journals Online (AJOL)

    Sarcoidosis amongst Nigerians remains a condition with limited information in literature despite the high prevalence of Sarcoidosis amongst Afro- Americans. This study was conceived to describe the presentation of the cases seen in an urban tertiary centre in Nigeria. A three year retrospective study of Sarcoidosis was ...

  9. Risk factors for uveitis in sarcoidosis | Kaye | South African Medical ...

    African Journals Online (AJOL)

    Uveitis is a potentially sight-threatening complication of sarcoidosis. The object of this study was to determine which patients with sarcoidosis are at greater risk of developing uveitis. We retrospectively assessed 136 patients with clinical, radiological and histological features of sarcoidosis. Of the 48 patients (35,3%) with ...

  10. The role of pattern recognition receptors in lung sarcoidosis

    NARCIS (Netherlands)

    Mortaz, Esmaeil|info:eu-repo/dai/nl/29141320X; Adcock, Ian M; Abedini, Atefhe; Kiani, Arda; Kazempour-Dizaji, Mehdi; Movassaghi, Masoud; Garssen, Johan|info:eu-repo/dai/nl/086369962

    2017-01-01

    Sarcoidosis is a granulomatous disorder of unknown etiology. Infection, genetic factors, autoimmunity and an aberrant innate immune system have been explored as potential causes of sarcoidosis. The etiology of sarcoidosis remains unknown, and it is thought that it might be caused by an infectious

  11. Cutaneous sarcoidosis: A retrospective case series and a hospital-based case-control study in Taiwan.

    Science.gov (United States)

    Liu, Kwei-Lan; Tsai, Wen-Chien; Lee, Chih-Hung

    2017-10-01

    Sarcoidosis is a systemic granulomatous disorder of unknown etiology often involving skin. Studies on cutaneous sarcoidosis and comorbidities are limited. This study is aimed to describe the clinical features of cutaneous sarcoidosis diagnosed in our hospital and to determine the relationships between cutaneous sarcoidosis and comorbidities.This retrospective study evaluates patients with cutaneous sarcoidosis in a tertiary center in Taiwan from 1996 to 2015. The records of 38 patients with cutaneous sarcoidosis were reviewed for clinical characteristics and evaluated by analysis of variance. A 1:4 case-control analysis was conducted with 152 age- and sex-matched controls who underwent biopsy for other benign skin tumors.The male to female ratio was 1:4.4. The average age at diagnosis was 51.7 years. Female patients were on average 13.9 years older than male patients. The correlation of age with gender was statistically significant (P = .037). The most common cutaneous lesions were plaques (47.4%) and confined to the face (71.1%). Of the 38 patients, 26.3% had diabetes mellitus. Age over 40 (P = .014) and female (P = .014) were associated with facial involvement. In the case-control study, a higher percentage of patients with cutaneous sarcoidosis than of control subjects had diabetes mellitus (P = .001), hearing loss (P = .031) and eye diseases (P = .047).The present study demonstrates a striking female predominance and high proportions of facial involvement. Diabetes mellitus, hearing loss, and eye diseases may be associated with Taiwanese patients with cutaneous sarcoidosis.

  12. Verrucous cutaneous sarcoidosis: case report and review of this unusual variant of cutaneous sarcoidosis.

    Science.gov (United States)

    Stockman, David L; Rosenberg, Jason; Bengana, Chafik; Suster, Saul; Plaza, Jose A

    2013-04-01

    Sarcoidosis is a multisystem disorder of unknown origin, characterized by the accumulation of lymphocytes and mononuclear histiocytes inducing the formation of noncaseating "naked" epithelioid granulomas. The lungs, lymphatic system, and skin are most often affected, but sarcoidosis may affect any organ. Cutaneous involvement of sarcoidosis is often the sentinel sign of the disease, with the skin sometimes being exclusively affected. We present a case of a 54-year-old African American woman with long-standing history of pulmonary sarcoidosis that presented with multiple verrucous cutaneous lesions on the upper and lower extremities mimicking carcinoma. The initial cutaneous biopsy was superficial in nature, and the pathologist raised the consideration of a possible keratoacanthoma. A deeper skin shave biopsy was performed, and the histopathology showed verrucous pseudoepitheliomatous epidermal hyperplasia with scattered noncaseating granulomas in the superficial dermis. Stains (acid-fast bacillus, Periodic acid-Schiff, and Gomori-Grocott methenamine silver stains) were negative for microorganisms. Given the clinical setting and histomorphology of the cutaneous lesions, the diagnosis of verrucous sarcoidosis was rendered. Verrucous sarcoidosis is a rare cutaneous manifestation of sarcoidosis that could be easily misdiagnosed if it is not appropriately biopsied. This hinders the precise evaluation of the histological specimen, overall clinical picture, and administration of appropriate therapy.

  13. [Advances in diagnosis of pulmonary sarcoidosis].

    Science.gov (United States)

    Ziora, Dariusz; Jastrzębski, Dariusz; Labus, Łukasz

    2012-01-01

    Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic systems of the body. The diagnosis of sarcoidosis is established on the basis of compatible clinical and radiologic findings, supported by histologic evidence in one or more organs of noncaseating epithelioid-cell granulomas. A diagnosis of sarcoidosis is reasonably certain without biopsy in patients who present with Löfgren's syndrome. In confirmation of sarcoidosis scale lymph node biopsy, endobronchial biopsy, mediastinoscopy, blind tranbronchial needle aspiration and transbronchial lung biopsy or broncho-alveolar lavage were used with diagnostic yields between 60-85%. At present in stage I and II of sarcoidosis the novel technics such as Endoscopic ultrasound-guided, fine-needle aspiration of intrathoracic lymph nodes (EBUS-FNA) and esophageal ultrasound-guided fine-needle aspiration (EUS-FNA) are performed. The combination of these two methods has been reported to provide a diagnostic yield of above 83-90% with about 100% specificity and may obviate the need for mediastinoscopy.

  14. EEG in Sarcoidosis Patients Without Neurological Findings.

    Science.gov (United States)

    Bilgin Topçuoğlu, Özgür; Kavas, Murat; Öztaş, Selahattin; Arınç, Sibel; Afşar, Gülgün; Saraç, Sema; Midi, İpek

    2017-01-01

    Sarcoidosis is a multisystem granulomatous disease affecting nervous system in 5% to 10% of patients. Magnetic resonance imaging (MRI) is accepted as the most sensitive method for detecting neurosarcoidosis. However, the most common findings in MRI are the nonspecific white matter lesions, which may be unrelated to sarcoidosis and can occur because of hypertension, diabetes mellitus, smoking, and other inflammatory or infectious disorders, as well. Autopsy studies report more frequent neurological involvement than the ante mortem studies. The aim of this study is to assess electroencephalography (EEG) in sarcoidosis patients without neurological findings in order to display asymptomatic neurological dysfunction. We performed EEG on 30 sarcoidosis patients without diagnosis of neurosarcoidosis or prior neurological comorbidities. Fourteen patients (46.7%) showed intermittant focal and/or generalized slowings while awake and not mentally activated. Seven (50%) of these 14 patients with EEG slowings had nonspecific white matter changes while the other half showed EEG slowings in the absence of MRI changes. We conclude that EEG slowings, when normal variants (psychomotor variant, temporal theta of elderly, frontal theta waves) are eliminated, may be an indicator of dysfunction in brain activity even in the absence of MRI findings. Hence, EEG may contribute toward detecting asymptomatic neurological dysfunction or probable future neurological involvement in sarcoidosis patients. © EEG and Clinical Neuroscience Society (ECNS) 2016.

  15. Pain and autonomic dysfunction in patients with sarcoidosis and small fibre neuropathy

    NARCIS (Netherlands)

    M. Bakkers (Mayienne); C.G. Faber (Carin); M. Drent (Marjolein); M.C.E. Hermans; S.I. van Nes (Sonja); G. Lauria (Giuseppe); M.H. de Baets (Marc); I.S.J. Merkies (Ingemar)

    2010-01-01

    textabstractSmall fibre neuropathy (SFN) has been demonstrated in sarcoidosis. However, a systematic analysis of neuropathic pain and autonomic symptoms, key features of SFN, has not been performed. Clinimetric evaluation of pain and autonomic symptoms using the neuropathic pain scale (NPS) and the

  16. Gingival enlargement unveiling sarcoidosis: Report of a rare case

    Directory of Open Access Journals (Sweden)

    Sabeeha Abbas Kadiwala

    2013-01-01

    Full Text Available Sarcoidosis is classified as an acquired systemic granulomatous disease. Because of the fact that sarcoidosis affects multiple tissues and organs, it is characterized by many potential signs and symptoms, as well as by the presence of non-caseating granulomas in the organs involved. Although oral sarcoidosis is relatively rare, it may however, present in the oral cavity. This report presents a rare case of sarcoidosis with the initial presenting symptom as severe generalized gingival enlargement. The gingival enlargement was treated by gingivectomy. After histopathological examination of gingival biopsy and certain special investigations, a diagnosis of sarcoidosis was made.

  17. Pleural effusion and sarcoidosis: an unusual combination.

    Science.gov (United States)

    Ferreiro, Lucía; San José, Esther; González-Barcala, Francisco Javier; Suárez-Antelo, Juan; Toubes, M Elena; Valdés, Luis

    2014-12-01

    Pleural involvement in sarcoidosis is uncommon and appears in several forms. To document the incidence and characteristics of pleural effusion in sarcoidosis patients, a review of the cases diagnosed in our centre between January 2001 and December 2012 was carried out. One hundred and ninety-five patients with sarcoidosis were identified; three (two men and one woman) presented with unilateral pleural effusion (1.5%): one in the right side and two in the left. Two were in stageii and one was in stageiv. The pleural fluid of the two patients who underwent thoracocentesis was predominantly lymphocytic. One of these patients presented chylothorax and the other had high CA-125levels. In general, these effusions are lymphocyte-rich, paucicellular, serous exudates (sometimes chylothorax) and contain proportionally higher levels of protein than LDH. Most cases are treated with corticosteroids, although it may resolve spontaneously. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

  18. Sonographic Appearance of Dermal and Subcutaneous Sarcoidosis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Jang, Ja Yoon; Bae, Young A; Hong, Hyeok Jin; Kwon, Kye Won [Dept. of Radiology, Bundang Jesaeng General Hospital, Seongnam (Korea, Republic of)

    2012-08-15

    Sarcoidosis is a systemic granulomatous disease of unknown origin that mainly involves lung and skin, but rarely involves subcutaneous tissue. While some studies have reported on CT or MR imaging findings of subcutaneous sarcoidosis, there is only one report on sonographic findings of subcutaneous sarcoidosis, recently published in the US. Familiarity with ultrasonographic findings of subcutaneous sarcoidosis might be helpful for the early diagnosis in patient with palpable nodules and image follow-up for subcutaneous sarcoidosis. Here we report on the sonographic appearance of subcutaneous sarcoidosis involving dermal and subcutaneous tissue over axilla and sole, a case diagnosed as sarcoidosis and improved by steroid treatment, along with a review of the relevant literature.

  19. [Sarcoidosis flare after autologous stem cell transplantation: An immune paradox?

    Science.gov (United States)

    Marchal, A; Charlotte, F; Maksud, P; Haroche, J; Lifferman, F; Miyara, M; Choquet, S; Amoura, Z; Cohen Aubart, F

    2017-09-01

    Sarcoidosis is a systemic granulomatous disorder of unknown cause. Apparition or flare of previously diagnosed sarcoidosis following hematopoietic stem cell transplantation (HSCT) has rarely been reported. We report a 62-year-old woman who presented a radiological flare of sarcoidosis post-autologous stem cell transplantation for a POEMS syndrome. Imaging findings and lymph node histology, which revealed non-caseating granuloma, were consistent with the sarcoidosis diagnosis. The patient was asymptomatic and was kept free of treatment. Sarcoidosis must be considered ahead of compatible clinicoradiological presentation occurring after HSCT. Sarcoidosis can mimic metastatic cancer or lymphatic relapse. Tissue biopsies and exclusion of differential diagnosis of granuloma diseases are warranted to confirm sarcoidosis diagnosis. Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  20. Cardiac Involvement in Sarcoidosis: Evolving Concepts in Diagnosis and Treatment

    Science.gov (United States)

    Lynch, Joseph P.; Hwang, Jennifer; Bradfield, Jason; Fishbein, Michael; Shivkumar, Kalyanam; Tung, Roderick

    2014-01-01

    Clinically evident sarcoidosis involving the heart has been noted in at least 2 to 7% of patients with sarcoidosis, but occult involvement is much higher (> 20%). Cardiac sarcoidosis is often not recognized antemortem, as sudden death may be the presenting feature. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Sarcoidosis can involve any part of the heart, with protean manifestations. Prognosis of cardiac sarcoidosis is related to extent and site(s) of involvement. Most deaths due to cardiac sarcoidosis are due to arrhythmias or conduction defects, but granulomatous infiltration of the myocardium may be lethal. The definitive diagnosis of isolated cardiac sarcoidosis is difficult. The yield of endomyocardial biopsies is low; treatment of cardiac sarcoidosis is often warranted even in the absence of histologic proof. Radionuclide scans are integral to the diagnosis. Currently, 18F-fluorodeoxyglucose positron emission tomography/computed tomography and gadolinium-enhanced magnetic resonance imaging scans are the key imaging modalities to diagnose cardiac sarcoidosis. The prognosis of cardiac sarcoidosis is variable, but mortality rates of untreated cardiac sarcoidosis are high. Although randomized therapeutic trials have not been done, corticosteroids (alone or combined with additional immunosuppressive medications) remain the mainstay of treatment. Because of the potential for sudden cardiac death, implantable cardioverter-defibrillators should be placed in any patient with cardiac sarcoidosis and serious ventricular arrhythmias or heart block, and should be considered for cardiomyopathy. Cardiac transplantation is a viable option for patients with end-stage cardiac sarcoidosis refractory to medical therapy. PMID:25007089

  1. Treatment of neuro-ophthalmic sarcoidosis.

    Science.gov (United States)

    Frohman, Larry P

    2015-03-01

    Because of the rarity of neuro-ophthalmic sarcoidosis, there are no therapeutic guidelines based on evidence-based medicine for this disorder. Review of literature combined with personal experience. Corticosteroids are the preferred initial therapy for neuro-ophthalmic sarcoidosis. If patients cannot tolerate the requisite dose of corticosteroid needed to control their disease, or if corticosteroids fail to adequately control the disease process, the choices of a second agent are based on the consideration of rapidity of clinical response and the safety profile. Although methotrexate and mycophenolate mofetil are the medications that are often selected after corticosteroid failure, more rapidly acting agents that have been used are infliximab and intravenous cyclophosphamide.

  2. Central skeletal sarcoidosis mimicking metastatic disease

    Energy Technology Data Exchange (ETDEWEB)

    Talmi, Danit; Smith, Stacy; Mulligan, Michael E. [University of Maryland School of Medicine, Department of Radiology, Baltimore, MD (United States)

    2008-08-15

    Sarcoidosis is a systemic disease that histologically typically shows non-caseating granulomas. The most common radiologic finding is hilar and mediastinal adenopathy. Patients with widely disseminated disease may show involvement of the peripheral appendicular skeleton in 1-13% of such cases. A primary skeletal presentation without other manifestations typical of the disease is rare. We present a case of sarcoidosis in a middle-aged Caucasian man in whom the disease presented with widespread lytic lesions in the axial skeleton and long bones, mimicking metastatic disease. There was no involvement of the peripheral skeleton, skin or lungs. (orig.)

  3. Coexistence of Sarcoidosis and Gouty Arthritis.

    Science.gov (United States)

    Semiz, Hüseyin; Kobak, Senol

    2017-08-21

    Sarcoidosis is an inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It may present with bilateral hilar lymphadenopathy, skin lesions, the involvement of eye and symptoms on the locomotor system. Gouty arthritis is an autoinflammatory disease characterized by hyperuricemia, recurrent arthritis attacks and the deposition of monosodium urate crystals in the joints and the surrounding tissues. We reported the coexistence of sarcoidosis and gouty arthritis in this paper. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  4. Gastric sarcoidosis in children: A case report and review of the ...

    African Journals Online (AJOL)

    Sarcoidosis is a systemic granulomatous disease of unknown etiology that is characterized by the formation of noncaseating granulomas. Gastrointestinal (GI) tract involvement in sarcoidosis is rare. Gastric sarcoidosis, particularly involving the antrum, affects approximately 10% of patients with systemic disease.

  5. Is sarcoidosis a rickettsiosis? An archival study

    DEFF Research Database (Denmark)

    Svendsen, Claus Bo; Milman, Nils; Andersen, Claus B

    2011-01-01

    Based on earlier research, Rickettsia helvetica could possibly be involved in the pathogenesis of sarcoidosis. Rickettsiae are transmitted to humans by a tick vector, Ixodes ricinus; this tick is highly prevalent in Northern Europe. We aimed to investigate the association between evidence...

  6. Sarcoidosis of the nose and paranasal sinuses.

    Science.gov (United States)

    McCaffrey, T V; McDonald, T J

    1983-10-01

    Sarcoidosis is a chronic systemic disease of unknown etiology characterized by non-caseating granulomatous inflammation of various organs. The records of 2319 patients with the diagnosis of sarcoidosis were reviewed to determine the incidence of nasal involvement. Seventeen patients or approximately 1% of the patients with sarcoidosis had histologically proven nasal mucosa involvement. These patients had symptoms of nasal crusting, congestion, epistaxis, pain, or anosmia. The clinical findings in these patients included friable nasal mucosa, nasal polyps, or a characteristic submucosal nodularity. Most patients also had abnormal sinus roentgenograms with either thickening of the sinus mucosa or opacification of the sinuses. Biopsy of the nasal mucosa shows typical non-caseating granulomas, but care must be exercised to exclude other causes of granulomatous inflammation of the nasal mucosa including tuberculosis, fungal infections, and other idiopathic granulomatous diseases such as Wegener's granulomatosis and Churg-Strauss syndrome. The treatment of nasal sarcoidosis has consisted of systemic steroids and in some cases topical beclomethasone dipropionate.

  7. Sarcoidosis of the vagina treated with methotrexate.

    Science.gov (United States)

    Şahin, N; Solak, A; Karaarslan, S; Doruk, S

    2016-06-01

    We describe the first case of mediolateral episiotomic sarcoidosis of the deep vaginal tissue, without involvement of the pulmonary parenchyma or pulmonary symptoms. A 68-year-old female was admitted with a vaginal mass that had developed about 1 month prior. On bimanual examination, we found a painful solid mass approximately 4 cm in diameter on the episiotomy line of the deep vaginal tissue. The patient underwent pelvic magnetic resonance imaging on suspicion of a malignancy, and a vaginal true-cut biopsy was performed. The biopsy specimen exhibited non-caseating, granulomatous inflammation and many multinucleated giant cells, strongly suggesting sarcoidosis. We had excluded other granulomatous diseases; a final diagnosis was made of stage-1 sarcoidosis in the deep vaginal mass. A 3-month course of methotrexate (2.5 mg/week) was commenced to treat a flare-up of rheumatoid arthritis. The vaginal mass resolved. To the best of our knowledge, this is the first case of sarcoidosis in a deep vaginal mass without pulmonary parenchymal or other solid-organ involvement.

  8. Management of extrapulmonary sarcoidosis: challenges and solutions

    Science.gov (United States)

    Al-Kofahi, Khalid; Korsten, Peter; Ascoli, Christian; Virupannavar, Shanti; Mirsaeidi, Mehdi; Chang, Ian; Qaqish, Naim; Saketkoo, Lesley A; Baughman, Robert P; Sweiss, Nadera J

    2016-01-01

    Background Sarcoidosis is a chronic multisystem disease of unknown etiology characterized by noncaseating granulomas that most often involves the lungs, but frequently has extrapulmonary manifestations, which might be difficult to treat in individual patients. Objective To review different disease manifestations, focusing on extrapulmonary organ systems, and to provide treatment options for refractory cases. Materials and methods We performed a literature search using Medline and Google Scholar for individual or combined keywords of “sarcoidosis, extrapulmonary, treatment, kidney, neurosarcoidosis, cardiovascular, gastrointestinal, transplantation, musculoskeletal, rheumatology, arthritis, and skin”. Peer-reviewed articles, including review articles, clinical trials, observational trials, and case reports that were published in English were included. References from retrieved articles were also manually searched for relevant articles. Results and conclusion Isolated involvement of a single organ or organ system is rare in sarcoidosis, and thus all patients must be thoroughly evaluated for additional disease manifestations. Cardiac sarcoidosis and neurosarcoidosis may be life-threatening. Clinicians need to assess patients comprehensively using clinical, laboratory, imaging, and histopathological data to recommend competently the best and least toxic treatment option for the individual patient. PMID:27853374

  9. Prevalence of asthma and atopy in sarcoidosis.

    Science.gov (United States)

    Wilsher, Margaret; Hopkins, Raewyn; Zeng, Irene; Cornere, Megan; Douglas, Richard

    2012-02-01

    We hypothesized that the prevalence of allergic disorders, characterized by the release of type 2 cytokines (IL-4, IL-5, IL-10), would be lower in sarcoidosis in which there is a dominant type 1 immune response (IL-2, interferon-gamma). The objective was to measure the prevalence of atopy and self-reported asthma in patients with sarcoidosis. Sarcoidosis patients (n = 136, 72 M, age range 22-75), recruited in the outpatient setting, completed a modified European Community Respiratory Health Survey. 123 of these patients provided blood for allergy testing. For the cohort as a whole the self-reported prevalence of asthma ever (21.5%) and asthma attack in the last 12 months (7.5%), was high as was wheezing (42.1%), breathlessness with wheeze (22.3%) and use of an asthma medication (13.1%). The prevalence of atopy was 34%. These data are not different from the previously reported prevalence of asthma and atopy in New Zealand. The same prevalence of asthma symptoms and atopy as in the normal population suggests that the immune system is not skewed away from mounting T helper type 2 immune responses in sarcoidosis. © 2011 The Authors. Respirology © 2011 Asian Pacific Society of Respirology.

  10. Medical image of the week: sarcoidosis

    Directory of Open Access Journals (Sweden)

    Hansra A

    2016-02-01

    Full Text Available No abstract available. Article truncated after 150 words. We present a 58-year-old African American man with a complicated medical history including long-standing sarcoidosis that has caused him chronic, unrelenting pain for two decades. He initially underwent placement of an intrathecal morphine pump, but recently began complaining of increasing pain. Consequently, he was seen at our hospital for interrogation of his pain pump by the interventional radiologist, and was incidentally noted to have bilateral calcified hilar lymphadenopathy on fluoroscopic imaging. A dedicated chest x-ray confirmed the abnormality, which was consistent with his known diagnosis of sarcoidosis. Sarcoidosis is a complex disease process characterized by noncaseous granulomas that can affect various organ systems, with pulmonary involvement in up to 90% of cases (1. Though sarcoidosis is a diagnosis of exclusion, clinicians should recognize that bilateral hilar lymphadenopathy is highly concerning for the underlying noncaseating granulomatous disease (2. The most common pattern of lymphadenopathy is well-defined, bilateral, symmetric hilar and right ...

  11. [Cardiac sarcoidosis: Diagnosis and therapeutic challenges].

    Science.gov (United States)

    Cohen Aubart, F; Nunes, H; Mathian, A; Haroche, J; Hié, M; Le-Thi Huong Boutin, D; Cluzel, P; Soussan, M; Waintraub, X; Fouret, P; Valeyre, D; Amoura, Z

    2017-01-01

    Sarcoidosis is a granulomatous disorder of unknown cause characterized by non-caseating granuloma in young adults. Cardiac involvement is rare and range from 2 to 75% depending on diagnostic criteria. Cardiac involvement in sarcoidosis may be asymptomatic or may manifest as rhythm/conduction troubles or congestive heart failure. The diagnosis and treatment of cardiac sarcoidosis may be challenging. However, advances have come in recent years from the use of cardiac MRI and 18FDG-TEP scanner, as well as from the stratification of the risk of ventricular tachycardia/fibrillation. Due to the rarity of the disease, there is no reliable prospective large study to guide therapeutic strategy for cardiac sarcoidosis. Corticosteroids are probably efficacious, in particular in case of atrio-ventricular block or moderate heart failure. Immunosuppressive drugs have not been largely studied but methotrexate could be helpful. In refractory forms, TNF-α antagonists have been used with success. Copyright © 2016 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  12. Risk factors for uveitis in sarcoidosis

    African Journals Online (AJOL)

    Sarcoidosis is a chronic granulomatous disease of unknown aetiology. The clinical marllfestations of the disorder depend on the organ system involved in the granulomatous process. The disease particularly. Departments of Ophthalmology and Medical Informatics,. Groote Schuur Hospital and University ofCape Town.

  13. Subcutaneous Sarcoidosis in a Nigerian female

    African Journals Online (AJOL)

    the body but intrathoracic involvement (pulmonary and hilar nodes) ... affecting both genders with slight female preponderance. (4). All age ... in a Nigerian female. Summary. Sarcoidosis is relatively uncommon in indigenous black Africans, especially along the West African coast. There has not been any report of isolated ...

  14. Acute Posterior Multifocal Placoid Pigment Epitheliopathy as the Initial Manifestation of Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Adil Darugar

    2011-01-01

    Full Text Available Purpose: To report an undiagnosed case of systemic sarcoidosis manifesting with bilateral acute posterior multifocal placoid pigment epitheliopathy (APMPPE. Case Report: A 26-year-old Caucasian man was referred for management of unilateral visual loss together with a paracentral scotoma developing 2 weeks after a flu-like syndrome. Clinical signs and ancillary diagnostic investigations suggested APMPPE. Laboratory tests demonstrated elevated serum angiotensin converting enzyme and lysozyme levels. Chest CT-scan disclosed moderate hilar lymph node calcifications but QuantiFERON-TB gold test was negative and bronchoalveolar lavage and biopsies were unremarkable. Accessory salivary gland biopsy disclosed epithelioid and gigantocellular granuloma formation without caseum, confirming a diagnosis of sarcoidosis. The fellow eye was involved a few days later and the patient complained of dyspnea. Echocardiography disclosed severe granulomatous myocardial infiltration and high dose corticosteroids and intravenous cyclophosphamide were initiated. Systemic treatment controlled both cardiac and ocular lesions, and was tapered accordingly. Conclusion: The constellation of "white dot syndromes" and systemic symptoms necessitates a general work-up to exclude granulomatous disorders such as sarcoidosis or tuberculosis. Delayed diagnosis of cardiac sarcoidosis may have life-threatening consequences and the ophthalmologist may be the first physician to diagnose the condition.

  15. Etiology of Sarcoidosis: Does Infection Play a Role?

    Science.gov (United States)

    Saidha, Shiv; Sotirchos, Elias S.; Eckstein, Christopher

    2012-01-01

    Sarcoidosis is a granulomatous inflammatory disorder of unclear etiology, which is known to affect multiple organ systems including the lungs, heart, skin, central nervous system, and eyes, among others. For this reason, sarcoidosis represents a systemic medical disorder that is clinically relevant to multiple medical sub-specialties. Despite extensive research, the etiology of sarcoidosis has yet to be elucidated, although most evidence supports that the pathogenetic mechanism of sarcoidosis is an aberrant immune response, driven by an unidentified antigen (or antigens) in genetically susceptible individuals. Multiple candidate etiologic agents, including microbial organisms and environmental agents, have been investigated, but study results are inconclusive. In this review, we describe the known histologic and immunologic features of sarcoidosis and discuss the evidence supporting a role for infectious processes in the pathogenesis of sarcoidosis. PMID:22461752

  16. Post herpes-zoster scar sarcoidosis with pulmonary involvement

    Directory of Open Access Journals (Sweden)

    Archana Singal

    2014-01-01

    Full Text Available Cutaneous sarcoidosis presents with a wide range of clinical presentations. An uncommon cutaneous manifestation is infiltration of old cutaneous scars with non-caseating granulomas known as scar sarcoidosis. Most of the patients with this clinical entity have other systemic manifestations, particularly pulmonary changes. We report a case of a 50 years old man, presenting with cutaneous sarcoidosis overlying scars of healed herpes zoster.

  17. Pulmonary sarcoidosis: the 'Great Pretender'

    Energy Technology Data Exchange (ETDEWEB)

    Hawtin, K.E., E-mail: katehawtin@doctors.org.u [Department of Radiology, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London (United Kingdom); Roddie, M.E. [Department of Radiology, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London (United Kingdom); Mauri, F.A. [Department of Histopathology, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London (United Kingdom); Copley, S.J. [Department of Radiology, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London (United Kingdom)

    2010-08-15

    Sarcoidosis has a wide spectrum of appearances within the thorax. This review will discuss and illustrate the range of pulmonary manifestations on high-resolution computed tomography and chest radiography, concentrating on atypical features and examples of sarcoidosis mimicking other lung diseases. All included cases have been histologically confirmed. Such variable imaging appearances should alert the radiologist to consider sarcoidosis as a differential diagnosis in the context of interstitial lung disease.

  18. Vertebral chondroblastoma

    Energy Technology Data Exchange (ETDEWEB)

    Ilaslan, Hakan; Sundaram, Murali [Department of Radiology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905 (United States); Unni, Krishnan K. [Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905 (United States)

    2003-02-01

    To determine the age distribution, gender, incidence, and imaging findings of vertebral chondroblastoma, and to compare our series with findings from case reports in the world literature.Design and patients Case records and imaging findings of nine histologically documented vertebral chondroblastomas were retrospectively reviewed for patient age, gender, vertebral column location and level, morphology, matrix, edema, soft tissue mass, spinal canal invasion, and metastases. Our findings were compared with a total of nine patients identified from previous publications in the world literature. The histologic findings in our cases was re-reviewed for diagnosis and specifically for features of calcification and secondary aneurysmal bone cyst (ABC). Clinical follow-up was requested from referring institutions. Nine of 856 chondroblastomas arose in vertebrae (incidence 1.4%; thoracic 5, lumbar 1, cervical 2, sacral 1). There were six males and three females ranging in age from 5 to 41 years (mean 28 years). Satisfactory imaging from seven patients revealed the tumor to arise from the posterior elements in four and the body in three. All tumors were expansive, six of seven were aggressive, and the spinal canal was significantly narrowed by bone or soft tissue mass in six. In one patient canal invasion was minimal. Calcification was pronounced in two and subtle in four. The sole nonaggressive-appearing tumor was heavily mineralized. Bony edema and secondary ABC were not seen on MR imaging. None of the cases had microscopic features of significant secondary ABC. Calcification, and specifically ''chicken wire'' calcification, was identified in two patients. Pulmonary metastases occurred in none. Vertebral chondroblastoma is a rare neoplasm that presents later in life than its appendicular counterpart. On imaging it is aggressive in appearance with bone destruction, soft tissue mass, and spinal canal invasion. The lesions contain variable amounts of mineral

  19. Current and emerging pharmacological treatments for sarcoidosis: a review

    Science.gov (United States)

    Beegle, Scott H; Barba, Kerry; Gobunsuy, Romel; Judson, Marc A

    2013-01-01

    The treatment of sarcoidosis is not standardized. Because sarcoidosis may never cause significant symptoms or organ dysfunction, treatment is not mandatory. When treatment is indicated, oral corticosteroids are usually recommended because they are highly likely to be effective in a relative short period of time. However, because sarcoidosis is often a chronic condition, long-term treatment with corticosteroids may cause significant toxicity. Therefore, corticosteroid sparing agents are often indicated in patients requiring chronic therapy. This review outlines the indications for treatment, corticosteroid treatment, and corticosteroid sparing treatments for sarcoidosis. PMID:23596348

  20. Sarcoidosis: Case Presentation and Literature Review

    Directory of Open Access Journals (Sweden)

    Dayana Alomá Fortún

    2016-09-01

    Full Text Available Sarcoidosis is a systemic disease of unknown etiology in which infectious agents have been implicated, inorganic powders or organic substances, characterized by the presence of necrotizing granulomatous inflammation with no accumulation of CD4 + lymphocytes and monocytes in the affected tissues. It is presented the case of a patient who went to the General University Hospital Dr. Gustavo Aldereguía Lima of Cienfuegos reporting dry cough, fever, chest tightness and slight weight loss three of three months evolution which did not improve despite receiving treatment in his health area. After several studies it was diagnosed a proliferative pulmonary sarcoidosis, systemic granulomatous disease whose etiology remains anonymous. Biopsy remains the basis for definitive diagnosis. As this is a difficult entity to explain and understand, besides being scarcely diagnosed in our area, it is decided to present that clinical case.

  1. Cardiac Sarcoidosis Culminating in Severe Biventricular Failure

    Directory of Open Access Journals (Sweden)

    Takefumi Ozaki

    2009-01-01

    Full Text Available A 59-year-old woman with a history of lung sarcoidosis developed general edema and exertional dyspnea. An electrocardiogram showed first-degree atrioventricular block with complete right bundle branch block. Chest X-ray showed cardiomegaly. Echocardiography showed diffuse and severe hypokinesis of the left ventricle (LV and biventricular enlargement with severe tricuspid regurgitation. Myocardial scintigraphy disclosed a perfusion defect at the ventricular septum and hypoperfusion at the posterior wall and the apex. On cardiac catheterization, pulmonary capillary wedge pressure, right ventricular, and right atrial pressures were elevated. Coronary angiograms were normal. Myocardial biopsy of the right ventricle histologically revealed epithelioid cell granuloma with infiltration of fibrous cells. The patient's symptom and LV function were improved with conventional medical therapy for heart failure. This is a rare case of cardiac sarcoidosis resulting in biventricular failure.

  2. Extrapulmonary sites of radiogallium accumulation in sarcoidosis

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    Sulavik, S.B.; Palestro, C.J.; Spencer, R.P.; Swyer, A.J.; Goldsmith, S.J.; Tierstein, A.S. (Univ. of Connecticut Health Center, Farmington (USA))

    1990-12-01

    In an effort to detect extrapulmonary sites of radiogallium accumulation in cases of sarcoidosis, 145 separate Ga-67 citrate studies of 114 patients with biopsy-proven sarcoidosis were examined. The most characteristic extrapulmonary radiogallium uptake pattern was the panda sign in 47 patients (41%). The most common site of prominent extrapulmonary radiogallium uptake was the lacrimal glands in 101 patients (88%). Second most common was activity in one or more superficial lymph node regions such as the cervical, axillary, femoral, or inguinal in 19 patients (17%). Other extrapulmonary sites included breast uptake in 6 out of 80 women (8%), prominent splenic and nasal uptake in 9 (8%) patients, periportal accumulation in 7 (6%), and cutaneous/subcutaneous activity in 4 (4%). Because many of these individuals were receiving corticosteroids, the natural (untreated) prevalence of extrapulmonary findings may be even higher. Although the sensitivity and specificity of extrapulmonary radiogallium accumulation has still to be determined, many of the sites may be accessible to biopsy both for diagnostic purposes and to follow the effects of medications. It is therefore suggested that whole-body imaging be performed when radiogallium is administered to patients with suspected or known sarcoidosis.

  3. [Pulmonary sarcoidosis developing during treatment with etanercept].

    Science.gov (United States)

    Kerjouan, M; Jouneau, S; Lena, H; Luraine, R; Desrues, B; Delaval, P

    2011-03-01

    TNF blockers are widely used to treat inflammatory rheumatic diseases and also in the treatment of extrapulmonary sarcoidosis. TNFα plays a major role in the development and persistence of sarcoid granulomata. However, recent studies have reported the involvement of anti-TNF therapies in the development of granulomatosis associated with the clinical and radiological features of sarcoidosis. A 54-years-old man with ankylosing spondylitis was treated with etanercept for two years. He was admitted with symptoms of bronchitis associated with radiological evidence of bilateral pulmonary nodules and a right upper lobe infiltrate. Anti-TNF therapy was stopped even though the patient had received 3 months of prophylactic treatment with rifampicin and isoniazid before starting etanercept. Bronchoalveolar lavage excluded infection, particularly tuberculosis. The chest CT-scan showed bilateral pulmonary nodules with peribronchovascular micronodules and enlarged mediastinal lymph nodes. Surgical lung biopsy was performed and revealed non-caseating granulomata. All the data were consistent with a diagnosis of pulmonary sarcoidosis. The patient remained symptomatic despite discontinuation of etanercept for ten months. Corticosteroids were then introduced, leading to a clinical, functional and radiological improvement. This case report underlines the importance of studying the pulmonary complications of TNF blockers. The first priority is to exclude tuberculosis but a diagnosis of sarcoid-like granulomatosis has to be considered. Twenty-three cases have been described in the literature to date. Copyright © 2011 SPLF. Published by Elsevier Masson SAS. All rights reserved.

  4. [Morpheiform sarcoidosis as atypical manifestation of sarcoidosis. Review of the literature and differential diagnosis].

    Science.gov (United States)

    Castellanos-González, María; Picazo Talavera, María Remedios

    2016-09-16

    Sarcoidosis is an idiopathic multisystem granulomatous disease that commonly involves the skin in 25% of affected patients. Because lesions assume a vast array of morphologies, a classification dividing them into specific (with presence of typical granulomas in the biopsy) or nonspecific (not containing granulomas) has been proposed. In the first group the variant morpheaform is considered exceptional. We review the cases reported in the literature and describe the possible differential diagnosis. We highlight the importance of recognizing the very atypical presentation of sarcoidosis and its ability to mimic morpheaform or sclerosis diseases in our patients. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  5. The prevalence of antinuclear antibodies in patients with sarcoidosis.

    Science.gov (United States)

    Kobak, Senol; Yilmaz, Hatice; Sever, Fidan; Duran, Arzu; Sen, Nazime; Karaarslan, Ahmet

    2014-01-01

    Introduction. Sarcoidosis, which is a chronic inflammatory granulomatous disease, can mimic different rheumatologic diseases including connective tissue diseases. Antinuclear antibodies are the markers used for connective tissue diseases. Aim. To determine antinuclear antibody frequency and any possible correlation with clinical and laboratory data in sarcoidosis patients. Material and Method. Forty-two sarcoidosis patients, 45 rheumatoid arthritis patients, and 45 healthy volunteers who were followed up in rheumatology outpatient clinic were included in this study. Demographic, clinical, serological, and radiological data of all patients were recorded. Antinuclear antibodies were determined with indirect immunofluorescent method and 1/100 titration was accepted as positive. The cases that were ANA positive were evaluated with immunoblot method. Results. Average age of the 42 patients (10 males) with sarcoidosis was 45.2 (20-70 years), and average disease duration was 3.5 years. ANA positivity was detected in 12 (28.5%) patients with sarcoidosis (1/100 in 10 patients, 1/320 in two patients), in 19 of RA patients (42.2%), and in two of healthy volunteers in low titer (P syndrome and scleroderma diagnosis, respectively. Discussion. The prevalence of ANA in patients with sarcoidosis was found to be significantly higher than healthy control group and lower than RA patients. This result shows that ANA may have an important role in the pathogenesis of sarcoidosis and also could be important in revealing the overlap syndromes of sarcoidosis-connective tissue diseases. Further studies with larger series are necessary in this subject.

  6. Pulmonary sarcoidosis shortly after spinal tuberculosis infection: a diagnostic challenge.

    Science.gov (United States)

    Luetkens, Julian A; Zoghi, Shahram; Rockstroh, Jürgen K; Naehle, Claas P

    2014-04-11

    Tuberculosis and sarcoidosis share similar histopathological findings. An aetiological connection between these diseases has been discussed. We report a case of pulmonary sarcoidosis, which occurred shortly after an isoniazid (INH)-resistant spinal tuberculosis was diagnosed which was suspected to be a miliary tuberculosis. This report illustrates the need to sensitise clinicians to two possible important causes of lung parenchyma alterations under tuberculostatic therapy.

  7. Cardiac sarcoidosis resembling panic disorder: a case report.

    Science.gov (United States)

    Tokumitsu, Keita; Demachi, Jun; Yamanoi, Yukichi; Oyama, Shigeto; Takeuchi, Junko; Yachimori, Koji; Yasui-Furukori, Norio

    2017-01-13

    Sarcoidosis is a systemic disease of unknown etiology, in which granulomas develop in various organs, including the skin, lungs, eyes, or heart. It has been reported that patients with sarcoidosis are more likely to develop panic disorder than members of the general population. However, there are many unknown factors concerning the causal relationship between these conditions. We present the case of a 57-year-old woman who appeared to have panic disorder, as she experienced repeated panic attacks induced by transient complete atrioventricular block, associated with cardiac sarcoidosis. Psychotherapy and pharmacotherapy were not effective in the treatment of her panic attacks. However, when we implanted a permanent pacemaker and initiated steroid treatment for cardiac sarcoidosis, panic attacks were ameliorated. Based on these findings, we diagnosed the patient's symptoms as an anxiety disorder associated with cardiac sarcoidosis, rather than panic disorder. This report highlights the importance of considering cardiac sarcoidosis in the differential diagnosis of panic disorder. This cardiac disease should be considered especially in patients have a history of cardiac disease (e.g., arrhythmia) and atypical presentations of panic symptoms. Panic disorder is a psychiatric condition that is typically diagnosed after other medical conditions have been excluded. Because the diagnosis of sarcoidosis is difficult in some patients, caution is required. The palpitations and symptoms of heart failure associated with cardiac sarcoidosis can be misdiagnosed as psychiatric symptoms of panic disorder. The condition described in the current case study appears to constitute a physical disease, the diagnosis of which requires significant consideration and caution.

  8. Sarcoidosis with heart involvement: a rare association of terrible ...

    African Journals Online (AJOL)

    Sarcoidosis is a multisystemic disorder of unknown etiology which is characterized by the formation of non-caseating granulomas in involved tissues. Cardiac involvement is one of the least common manifestations and it can occur at any point of time during the course of sarcoidosis. Here we present the case of 2 patients ...

  9. Health-related quality of life in sarcoidosis

    NARCIS (Netherlands)

    Korenromp, Ingrid H.E.; van de Laar, Mart A F J

    2014-01-01

    Purpose of review: The review presents an overview of the scientific publications in the field of health-related quality of life (HRQL) in sarcoidosis. Recent findings: Literature on HRQL in sarcoidosis is limited. HRQL was mainly used as a primary or secondary endpoint in intervention studies.

  10. The clinical and immunologic features of pulmonary fibrosis in sarcoidosis

    Science.gov (United States)

    PATTERSON, KAREN C.; HOGARTH, KYLE; HUSAIN, ALIYA N.; SPERLING, ANNE I.; NIEWOLD, TIMOTHY B.

    2014-01-01

    Sarcoidosis is a multisystem, granulomatous disease that most often affects the lungs. The clinical course is highly variable; many patients undergo spontaneous remission, but up to a third of patients progresses to a chronic disease course. The development of pulmonary fibrosis (PF) in a subset of patients with chronic disease has a negative impact on morbidity and mortality. While sarcoidosis-associated PF can be progressive, it is often referred to as “burnt out” disease, a designation reflecting inactive granulomatous inflammation. The immune mechanisms of sarcoidosis-associated PF are not well understood. It is not clear if fibrotic processes are active from the onset of sarcoidosis in predisposed individuals, or whether a profibrotic state develops as a response to ongoing inflammation. Transforming growth factor β (TGF-β) is an important profibrotic cytokine, and in sarcoidosis, distinct genotypes of TGF-β have been identified in those with PF. The overall cytokine profile in sarcoidosis-associated PF has not been well characterized, although a transition from a T helper 1 to a T helper 2 signature has been proposed. Macrophages have important regulatory interactions with fibroblasts, and the role of alveolar macrophages in sarcoidosis-associated PF is a compelling target for further study. Elucidating the natural history of sarcoidosis-associated PF will inform our understanding of the fundamental derangements, and will enhance prognostication and the development of therapeutic strategies. PMID:22683422

  11. Cardiac Sarcoidosis: Is it More Common in Men?

    Science.gov (United States)

    Martusewicz-Boros, Magdalena M; Boros, Piotr W; Wiatr, Elżbieta; Kempisty, Anna; Piotrowska-Kownacka, Dorota; Roszkowski-Śliż, Kazimierz

    2016-02-01

    Sarcoidosis is a systemic granulomatous disease which predominantly affects the lungs, although granulomas can also involve all other organs, including the heart. Cardiac sarcoidosis (CS) may occur at any stage of the disease and may be the cause of sudden cardiac death, even in a previously asymptomatic patient. The aim of this study was to evaluate the incidence of CS in a large group of patients diagnosed or followed up due to sarcoidosis. We performed a retrospective analysis of patients at our institution discharged with the final diagnosis "sarcoidosis" (ICD-10: D86) from January 2008 to October 2012. Only those with biopsy (from respiratory tract or lymph nodes) confirmed diagnosis of sarcoidosis were included. We then selected the subset of patients with cardiac involvement due to sarcoidosis confirmed by positive magnetic resonance imaging. The study covered 1375 consecutive sarcoidosis patients (51 % men), who were hospitalized during 5 years. Multiorgan disease was detected in 160 cases (11.7 %), and cardiac involvement was found in 64 patients (4.7 % of all), 70.3 % of whom were men. Twelve of those with CS were in stage I, 48 in stage II, and four in stage III. The odds ratio for having cardiac involvement in men compared to women was 2.3 (95 % CI 1.36-4.0, p = 0.002). Cardiac involvement in sarcoidosis was diagnosed in the similar percentage as in previously published data but was significantly more frequently in men.

  12. Decreased ADAM9 expression in patients with pulmonary sarcoidosis

    African Journals Online (AJOL)

    The pathogenesis of sarcoidosis is still poorly understood. Genome-wide gene expression profiling can provide novel genetic data involved in the pathogenesis of disease. In this study, using normal bronchoalveolar lavage (BAL) and sarcoidosis BAL as models for cDNA microarray analysis, we detected an elevation of ...

  13. Cardiac Sarcoidosis | Bajomo | Nigerian Journal of Clinical Practice

    African Journals Online (AJOL)

    Cardiac sarcoidosis is a rare complication which occurs in 1-5% of sarcoidosis. We report here a case seen in Olabisi Onnabanjo University Teaching Hospital, Sagamu, who presented with heart failure. Nigerian Journal of Clinical Practice Vol.6(2) 2003: 122-123 ...

  14. Systemic sarcoidosis complicated of acute renal failure: about 12 ...

    African Journals Online (AJOL)

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, ...

  15. Sensing of pathogens by Toll-like receptors in sarcoidosis

    NARCIS (Netherlands)

    Veltkamp, M.

    2011-01-01

    Sarcoidosis is a systemic disorder of unknown etiology characterized by the formation of non-caseating granulomas, most often affecting the lungs, lymph nodes and skin. Sarcoidosis is characterized by a strong Thelper-1 driven immune reaction, making viruses or intracellular pathogens, such as

  16. Sarcoidosis Presenting as Löfgren's Syndrome with Myopathy.

    Science.gov (United States)

    Kobak, Senol; Yalçin, Murat; Sever, Fidan; Oncel, Guray

    2013-01-01

    A 34-year-old female patient, who had proximal muscle weakness for 8 months, presented with erythema nodosum lesions on the pretibial region in addition to pain, swelling, and movement restriction in both ankles for the last one month. Thoracic CT demonstrated hilar and mediastinal lymphadenopathy. She underwent mediastinoscopic lymph node biopsy; biopsy result was consistent with noncaseating granuloma. Serum angiotensin converting enzyme level and muscle enzymes have been elevated. Muscular MRI and EMG findings were consistent with myositis. Muscle biopsy was done, and myopathy was found. The patient was diagnosed with sarcoidosis, Löfgren's syndrome, and sarcoid myopathy. The patient displayed remarkable clinical and radiological regression after 6-month corticosteroid and MTX therapy.

  17. Sarcoidosis Presenting as Löfgren’s Syndrome with Myopathy

    Directory of Open Access Journals (Sweden)

    Şenol Kobak

    2013-01-01

    Full Text Available A 34-year-old female patient, who had proximal muscle weakness for 8 months, presented with erythema nodosum lesions on the pretibial region in addition to pain, swelling, and movement restriction in both ankles for the last one month. Thoracic CT demonstrated hilar and mediastinal lymphadenopathy. She underwent mediastinoscopic lymph node biopsy; biopsy result was consistent with noncaseating granuloma. Serum angiotensin converting enzyme level and muscle enzymes have been elevated. Muscular MRI and EMG findings were consistent with myositis. Muscle biopsy was done, and myopathy was found. The patient was diagnosed with sarcoidosis, Löfgren's syndrome, and sarcoid myopathy. The patient displayed remarkable clinical and radiological regression after 6-month corticosteroid and MTX therapy.

  18. Myocardial imaging with {sup 18}F-fluoro-2-deoxyglucose positron emission tomography and magnetic resonance imaging in sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Ohira, Hiroshi; Tsujino, Ichizo; Ishimaru, Shinji; Sakaue, Shinji; Nishimura, Masaharu [Hokkaido University School of Medicine, First Department of Medicine, Sapporo, Hokkaido (Japan); Oyama, Noriko [Hokkaido University School of Medicine, Department of Radiology, Sapporo, Hokkaido (Japan); Takei, Toshiki; Tsukamoto, Eriko; Tamaki, Nagara [Hokkaido University School of Medicine, Department of Nuclear Medicine, Sapporo, Hokkaido (Japan); Miura, Masatake [Hokkaido University School of Medicine, Department of Cardiovascular Medicine, Sapporo, Hokkaido (Japan)

    2008-05-15

    Despite accumulating reports on the clinical value of {sup 18}F-fluoro-2-deoxyglucose positron emission tomography ({sup 18}F-FDG PET) and magnetic resonance imaging (MRI) in the assessment of cardiac sarcoidosis, no studies have systematically compared the images of these modalities. Twenty-one consecutive patients with suspected cardiac sarcoidosis underwent cardiac examinations that included {sup 18}F-FDG PET and MRI. The association of {sup 18}F-FDG PET and MRI findings with blood sampling data such as serum angiotensin converting enzyme levels was also evaluated. Eight of 21 patients were diagnosed as having cardiac sarcoidosis according to the Japanese Ministry of Health and Welfare Guidelines for Diagnosing Cardiac Sarcoidosis. Sensitivity and specificity for diagnosing cardiac sarcoidosis were 87.5 and 38.5%, respectively, for {sup 18}F-FDG PET, and 75 and 76.9%, respectively, for MRI. When the {sup 18}F-FDG PET and MRI images were compared, 16 of 21 patients showed positive findings in one (n = 8) or both (n = 8) of the two modalities. In eight patients with positive findings on both images, the distribution of the findings differed among all eight cases. The presence of positive findings on {sup 18}F-FDG PET was associated with elevated serum angiotensin-converting enzyme levels; this association was not demonstrated on MRI. Both {sup 18}F-FDG PET and MRI provided high sensitivity for diagnosing cardiac sarcoidosis in patients with suspected cardiac involvement, but the specificity of {sup 18}F-FDG PET was not as high as previously reported. The different distributions of the findings in the two modalities suggest the potential of {sup 18}F-FDG PET and MRI in detecting different pathological processes in the heart. (orig.)

  19. Sarcoidosis: radiographic manifestations in the nails and distal phalanges

    Energy Technology Data Exchange (ETDEWEB)

    Albers, Brittany K.; Garner, Hillary W. [Mayo Clinic, Department of Radiology, Jacksonville, FL (United States); Sluzevich, Jason C. [Mayo Clinic, Department of Dermatology, Jacksonville, FL (United States)

    2016-05-15

    Sarcoidosis is a granulomatous disease which can affect multiple organ systems. Clinical and radiologic manifestations depend on the organ system involved and the chronicity of disease. Nail involvement in sarcoidosis is rare, but is clinically relevant as it indicates chronic systemic disease. Nail abnormalities can be identified radiographically, and when seen in patients with known or suspected sarcoidosis, should prompt careful evaluation of the underlying bone for osseous involvement. We describe a case of sarcoidosis with radiographic findings in the nails and distal phalangeal tufts, which were indicative of nail and osseous sarcoid involvement and strongly supported the presence of chronic systemic disease. Although the nail findings resolved clinically and on radiographs after treatment, the osseous findings showed only minimal improvement. To our knowledge, the radiographic findings of nail sarcoidosis have not been previously addressed in the literature. (orig.)

  20. Rapidly Progressive Atrioventricular Block in a Patient with Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Nagham Saeed Jafar

    2014-01-01

    Full Text Available Cardiac sarcoidosis is a major cause of death in patients with systemic sarcoidosis. Cardiac manifestations are seen in 2.3% of the patients. Atrioventricular (AV block is one of the common manifestations of cardiac sarcoidosis. Other presentations of cardiac involvement include congestive heart failure, ventricular arrhythmias, and sudden cardiac death. The presence of AV block in young patients should raise the suspicion of sarcoidosis. AV block may be the only manifestation and patients may not have clinical evidence of pulmonary involvement. Here we describe a young male presented with exercise induced AV block rapidly progressing to complete heart block with recurrent syncope needing urgent pacemaker implantation. Factors that suggested an infiltrative process included his young age, rapidly progressive conduction abnormalities in the ECG in the absence of coronary disease, and previous history of cutaneous sarcoidosis.

  1. Recognition of distinctive patterns of gallium-67 distribution in sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Sulavik, S.B.; Spencer, R.P.; Weed, D.A.; Shapiro, H.R.; Shiue, S.T.; Castriotta, R.J. (Univ. of Connecticut School of Medicine, Farmington (USA))

    1990-12-01

    Assessment of gallium-67 ({sup 67}Ga) uptake in the salivary and lacrimal glands and intrathoracic lymph nodes was made in 605 consecutive patients including 65 with sarcoidosis. A distinctive intrathoracic lymph node {sup 67}Ga uptake pattern, resembling the Greek letter lambda, was observed only in sarcoidosis (72%). Symmetrical lacrimal gland and parotid gland {sup 67}Ga uptake (panda appearance) was noted in 79% of sarcoidosis patients. A simultaneous lambda and panda pattern (62%) or a panda appearance with radiographic bilateral, symmetrical, hilar lymphadenopathy (6%) was present only in sarcoidosis patients. The presence of either of these patterns was particularly prevalent in roentgen Stages I (80%) or II (74%). We conclude that simultaneous (a) lambda and panda images, or (b) a panda image with bilateral symmetrical hilar lymphadenopathy on chest X-ray represent distinctive patterns which are highly specific for sarcoidosis, and may obviate the need for invasive diagnostic procedures.

  2. Double pathology, sarcoidosis associated with multiple myeloma: A case report

    Directory of Open Access Journals (Sweden)

    Robert Dachs

    2014-05-01

    Full Text Available The association of sarcoidosis with multiple myeloma is not well known. Including this case report, 12 cases of patients with both sarcoidosis and multiple myeloma have been reported in the literature. The skeletal lesions of both conditions have many clinical and radiological similarities, and unless clinicians are aware of the association and the possibility of dual pathologies, the diagnosis of multiple myeloma in patients known with sarcoidosis may be missed. We present a case of a patient known with longstanding sarcoidosis who was found to have multiple lesions on magnetic resonance imaging (MRI involving the pelvis and both proximal femurs. Histological analysis revealed the presence of both non-necrotising granulomas consistent with sarcoidosis, and sheets of plasma cells consistent with a plasma cell neoplasm.

  3. Dual Analysis for Mycobacteria and Propionibacteria in Sarcoidosis BAL

    Science.gov (United States)

    Oswald-Richter, Kyra A.; Beachboard, Dia C.; Seeley, Erin H.; Abraham, Susamma; Shepherd, Bryan E.; Jenkins, Cathy A.; Culver, Daniel A.; Caprioli, Richard M.; Drake, Wonder P.

    2012-01-01

    Purpose Sarcoidosis is a non-caseating granulomatous disease for which a role for infectious antigens continues to strengthen. Recent studies have reported molecular evidence of mycobacteria or propionibacteria. We assessed for immune responses against mycobacterial and propionibacterial antigens in sarcoidosis bronchoalveolar lavage (BAL) using flow cytometry, and localized signals consistent with microbial antigens with sarcoidosis specimens, using matrix-assisted laser desorption ionization imaging mass spectrometry (MALDI-IMS). Methods BAL cells from 27 sarcoidosis, 14 PPD- controls, and 9 subjects with nontuberculosis mycobacterial (NTM) infections were analyzed for production of IFN-γ after stimulation with mycobacterial ESAT-6 and Propionibacterium acnes proteins. To complement the immunological data, MALDI-IMS was performed to localize ESAT-6 and Propionibacterium acnes signals within sarcoidosis and control specimens. Results CD4+ immunologic analysis for mycobacteria was positive in 17/27 sarcoidosis subjects, compared to 2/14 PPD-subjects, and 5/9 NTM subjects (p=00.008 and p=00.71 respectively, Fisher's exact test). There was no significant difference for recognition of P. acnes, which occurred only in sarcoidosis subjects that also recognized ESAT-6. Similar results were also observed for the CD8+ immunologic analysis. MALDI-IMS localized signals consistent with ESAT-6 only within sites of granulomatous inflammation, whereas P. acnes signals were distributed throughout the specimen. Conclusions MALDI-IMS localizes signals consistent with ESAT-6 to sarcoidosis granulomas, whereas no specific localization of P. acnes signals is detected. Immune responses against both mycobacterial and P. acnes are present within sarcoidosis BAL, but only mycobacterial signals are distinct from disease controls. These immunologic and molecular investigations support further investigation of the microbial community within sarcoidosis granulomas. PMID:22552860

  4. SNP Variants in Major Histocompatibility Complex Are Associated with Sarcoidosis Susceptibility—A Joint Analysis in Four European Populations

    Directory of Open Access Journals (Sweden)

    Elisa Laura Lahtela

    2017-04-01

    Full Text Available Sarcoidosis is a multiorgan inflammatory disorder with heritability estimates up to 66%. Previous studies have shown the major histocompatibility complex (MHC region to be associated with sarcoidosis, suggesting a functional role for antigen-presenting molecules and immune mediators in the disease pathogenesis. To detect variants predisposing to sarcoidosis and to identify genetic differences between patient subgroups, we studied four genes in the MHC Class III region (LTA, TNF, AGER, BTNL2 and HLA-DRA with tag-SNPs and their relation to HLA-DRB1 alleles. We present results from a joint analysis of four study populations (Finnish, Swedish, Dutch, and Czech. Patients with sarcoidosis (n = 805 were further subdivided based on the disease activity and the presence of Löfgren’s syndrome. In a joint analysis, seven SNPs were associated with non-Löfgren sarcoidosis (NL; the strongest association with rs3177928, P = 1.79E−07, OR = 1.9 and eight with Löfgren’s syndrome [Löfgren syndrome (LS; the strongest association with rs3129843, P = 3.44E−12, OR = 3.4] when compared with healthy controls (n = 870. Five SNPs were associated with sarcoidosis disease course (the strongest association with rs3177928, P = 0.003, OR = 1.9. The high linkage disequilibrium (LD between SNPs and an HLA-DRB1 challenged the result interpretation. When the SNPs and HLA-DRB1 alleles were analyzed together, independent association was observed for four SNPs in the HLA-DRA/BTNL2 region: rs3135365 (NL; P = 0.015, rs3177928 (NL; P < 0.001, rs6937545 (LS; P = 0.012, and rs5007259 (disease activity; P = 0.002. These SNPs act as expression quantitative trait loci (eQTL for HLA-DRB1 and/or HLA-DRB5. In conclusion, we found novel SNPs in BTNL2 and HLA-DRA regions associating with sarcoidosis. Our finding further establishes that polymorphisms in the HLA-DRA and BTNL2 have a role in sarcoidosis susceptibility. This multi

  5. Unmasking sarcoidosis following surgery for Cushing disease

    DEFF Research Database (Denmark)

    Diernæs, Jon Erik; Bygum, Anette; Poulsen, Per L

    2016-01-01

    We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman...... for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed...... successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease....

  6. The etiologic role of infectious antigens in sarcoidosis pathogenesis

    Science.gov (United States)

    Celada, Lindsay J.; Hawkins, Charlene; Drake, Wonder P.

    2015-01-01

    Sarcoidosis is a granulomatous disease of unknown etiology, characterized by a Th1 immunophenotype, most commonly involving the lung, skin, lymph node and eyes. Molecular and immunologic studies continue to strengthen the association of sarcoidosis with infectious antigens, particularly those derived from Propionibacterium and Mycobacterium species. Independent studies report the presence of microbial nucleic acids and proteins within sarcoidosis specimens. Complementary immunologic studies also support the role of infectious agents in sarcoidosis pathogenesis. Th-1 immune responses directed against mycobacterial virulence factors have been detected within sarcoidosis diagnostic bronchoalveolar lavage (BAL). Th1 and Th17 immune responses against propionibacteria have also been reported. More recently, case reports and clinical trials from Japanese, European and American investigators have emerged regarding the efficacy of antimicrobials against Propionibacterium and Mycobacterium species on pulmonary and cutaneous sarcoidosis. While these clinical investigations are not conclusive, they support increasing efforts to identify novel therapeutics, such as antimicrobials, that will impact the observed increase in sarcoidosis morbidity and mortality. PMID:26593133

  7. Sarcoidosis presenting as non-scarring non-scalp alopecia.

    Science.gov (United States)

    Dan, Luke; Relic, John

    2016-08-01

    In this article we describe a 39-year-old man who presented with non-scarring non-scalp alopecia of his limbs as the initial presentation of sarcoidosis. Alopecia is a rare cutaneous manifestation of sarcoidosis. A literature review has found only one other example of sarcoidosis presenting as non-scarring non-scalp alopecia in an area other than the scalp in a patient who was otherwise asymptomatic. Several reported cases have described scarring alopecia of the scalp, which is the area of skin most commonly affected by sarcoidosis. There has been one documented case of sarcoidosis manifesting as total body non-scarring alopecia in a patient who had systemic symptoms of sarcoidosis. Other cases have presented rare cutaneous manifestations of sarcoidosis but in all these cases several other organ systems have been involved, and the patient has had systemic symptoms on presentation or the cutaneous presentation did not include non-scalp non-scarring alopecia. © 2015 The Australasian College of Dermatologists.

  8. The association between osteopontin gene polymorphisms, osteopontin expression and sarcoidosis.

    Science.gov (United States)

    Lavi, Hadas; Assayag, Miri; Schwartz, Assaf; Arish, Nissim; Fridlender, Zvi G; Berkman, Neville

    2017-01-01

    Sarcoidosis is a systemic inflammatory disease of unknown etiology. Osteopontin (SPP1, OPN) is an extra cellular matrix glycoprotein and cytokine with a known role in granuloma formation and in autoimmune and inflammatory diseases. To determine whether plasma OPN levels are elevated in patients with sarcoidosis and compare the frequency of four single nucleotide polymorphism (SNPs) variants in the OPN gene in sarcoidosis patients compared to healthy controls. Demographic and clinical information, radiological studies and pulmonary function tests were evaluated in 113 patients with sarcoidosis and in 79 healthy controls. Blood samples were analyzed for SNPs of the OPN gene and for plasma OPN and CRP levels. Association between clinical features of disease and OPN levels as well as SNP frequencies was determined. Plasma OPN levels were higher in sarcoidosis patients than in healthy subjects, (median: 217 vs 122ng/ml, psarcoidosis patients and controls in the frequency of any of the SNPs evaluated. Presence of lung parenchymal involvement was associated with SNP distribution at rs1126772 (p = 0.02). We found no correlation between SNPs distribution and plasma OPN levels. Osteopontin protein levels are elevated in sarcoidosis. We found no evidence for an association between SNPs on the osteopontin gene and plasma OPN levels or the presence of sarcoidosis, however, an association between genotype and several phenotypic clinical parameters of disease was observed.

  9. A case of pulmonary sarcoidosis with endobronchial nodules.

    Science.gov (United States)

    Ji, Xiao-Qing; Wang, Li-Xia; Lu, De-Gan

    2016-01-01

    Sarcoidosis is a multisystemic disorder of unknown cause that is characterized pathologically by noncaseating granulomas and predominantly affects the lungs and the lymphatic system, especially intrathoracically. Diagnosis is based on the association of a compatible clinical and radiological presentation, the presence of characteristic histopathological lesions and the exclusion of other potential causes of granuloma. Although the lungs and mediastinal lymph nodes are almost involved, endobronchial nodular lesions of sarcoidosis with lung involvements are rare. We report a case of sarcoidosis with lung involvements and endobronchial nodules as confirmed by bronchial biopsy. © 2014 John Wiley & Sons Ltd.

  10. Brainstem Infarction and Panuveitis due to Sarcoidosis Successfully Treated with Steroid Pulse Therapy

    Directory of Open Access Journals (Sweden)

    Natsuyo Yoshida-Hata

    2012-01-01

    Full Text Available A 36-year-old man visited our hospital because of blurred vision and redness of the conjunctiva. Slit-lamp examination showed panuveitis. Two days later, he suddenly experienced dizziness, speech disturbance, paralysis of his right extremities, and gait disturbances. Neurological examinations suggested that his symptoms were caused by a left lateral medullary lesion. He also had erythema mainly on his trunk. Magnetic resonance imaging (MRI of his brain demonstrated a small infarct on the left side of the medulla oblongata. Clinical presentation and MRI findings were consistent with the diagnosis of a Wallenberg’s syndrome. He also had bilateral hilar lymphadenopathy. A skin biopsy showed granulomatous nodular dermatitis compatible with sarcoidosis. He was treated with steroid pulse therapy and his neurological and ocular symptoms immediately improved. Only seven similar cases of intracranical sarcoidosis have been reported, but none had been treated with steroid pulse therapy. We recommend that steroid pulse therapy be considered to treat patients with sarcoidosis with signs of lesions in the central nervous system.

  11. Dental extractions in patients with cardiac sarcoidosis.

    Science.gov (United States)

    Yoshimura, Yasuro; Nariai, Yoshiki; Yoshimura, Hitoshi

    2007-09-01

    To present data on hemodynamic changes during dental extractions in 5 patients with cardiac sarcoidosis, performed with electrocardiogram, heart rate, and blood pressure monitoring throughout the procedures, and to discuss the problems relating to the disease and dental extraction. The medical data for 5 patients, including medical records, physician correspondence, and laboratory data before the treatments, were assessed. Seven dental extractions were then performed while monitoring and recording the hemodynamic conditions. Heart rate, blood pressure, rate pressure products, and electrocardiographic findings were analyzed. Finally, posttreatment evaluations of the general and local conditions of the patients were conducted. All dental extractions were performed in nonactive stable periods, with no remarkable hemodynamic changes or complications, while maintaining a stable hemodynamic state throughout the extraction procedure. All patients received a pretreatment supplement of corticosteroid. Wound healing was similar to that in normal patients under antibiotic prophylaxis against infection originating from the dental extraction wound and the original dental lesion. Pretreatment general evaluation of patients with cardiac sarcoidosis should be performed through various examinations and physician consultation, and a stable hemodynamic change during the surgical procedure should be maintained under any hemodynamic monitors. Corticosteroid supplement and antibiotic coverage are also necessary for safe dental extraction and suitable healing.

  12. Unmasking sarcoidosis following surgery for Cushing disease.

    Science.gov (United States)

    Diernaes, Jon E F; Bygum, Anette; Poulsen, Per L

    2016-01-01

    We present a patient with Cushing disease apparently suppressing sarcoidosis, which was unmasked following surgical resection of a pituitary adrenocorticotropin (ACTH)-producing microadenoma. Case report and a short review of the literature published in this area. A 46-year-old Caucasian woman presented with symptoms of hypercortisolism such as progressive weight gain, Cushingoid appearance, proximal myopathy, easy bruising, and amenorrhea. Blood testing including inferior petrosal sinus sampling uncovered an ACTH-producing microadenoma in the right aspect of the anterior pituitary gland for which the patient underwent transphenoidal resection. Maintenance corticosteroid therapy was implemented, and the signs and symptoms of Cushing disease began to resolve. Three months after surgery, multiple erythematous painful nodules developed on the patient's arms. Erythema nodosum (EN) was diagnosed clinically and a suspicion of underlying sarcoidosis was substantiated by lung imaging and elevated plasma interleukin (IL)-2 receptor. One month later, the lesions spontaneously resolved without therapy other than maintenance glucocorticoid replacement. Physicians should be aware that patients undergoing successful treatment of Cushing syndrome may have a flare-up or emergence of a corticosteroid-responsive disease.

  13. Coexistence of systemic sclerosis and sarcoidosis.

    Science.gov (United States)

    Godziszewska, Sabina; Widuchowska, Małgorzata; Kopeć-Mędrek, Magdalena; Kucharz, Eugeniusz Józef

    Coexistence of systemic sclerosis (SSc) and sarcoidosis (SA) is rarely reported; 21 cases only were reported in the English medical literature before 2011. It is suggested that low incidence of overlap syndrome of SSc with SA is resulted from different immune mechanisms involved in pathogenesis of the diseases. In SSc patients, a role of Th2 lymphocytes is suggested while in patients with SA such role is attributed to Th1 lymphocytes. The paper presents a 47-year-old woman suffering from SSc for over 6 years. CT scan of the lungs revealed the nodulus of the right lung and enlarged mediastinal lymphatic nodes. Pathologic evaluation of the nodulus provided basis for diagnosis of sarcoidosis. Diagnosis of SSc was based on clinical and capilaroscopic evaluation as well as detection of anti-topoisomerase I antibodies. In the course of the disease, fibrosis of the lung, pulmonary hypertension and cardiac abnormalities with rhythm disturbances were developed. Treatment included cyclophosphamide, mycophenolate mofetil, sildenafil, losartan. Stabilization of the general state of the patient was achieved.

  14. Disfiguring Annular Sarcoidosis Improved by Adalimumab

    Directory of Open Access Journals (Sweden)

    C.A. Kaiser

    2011-05-01

    Full Text Available Depending on the location, dermatoses can produce blemishes that severely impair quality of life and require highly effective treatment that is otherwise used for extensive skin involvement. We report the case of a 39-year-old, otherwise healthy male disfigured by an 8 × 7-cm hypopigmented and centrally atrophic annular plaque with erythematous indurated borders in an area of scar tissue on his forehead. Skin biopsies revealed non-caseating granulomas, and hilar involvement was identified, leading to the diagnosis of systemic sarcoidosis stage II with cutaneous involvement. The lesions proved resistant to multiple therapies, but responded within 4 months to adalimumab with regression of the lesion and inflammatory infiltrate. The visual analogue scale of disease activity decreased from 7/10 to 3.5/10, and the Dermatology Life Quality Index from 16/30 to 3/30 points. In conclusion, TNF-α inhibition can control inflammation and disfigurement by cutaneous sarcoidosis and restore quality of life.

  15. Laryngeal Sarcoidosis in a Child: Case Report.

    Science.gov (United States)

    Vega-Cornejo, Gabriel; Ayala-Buenrostro, Priscila

    2017-11-01

    Sarcoidosis is a chronic, multisystemic, granulomatous disorder. Our patient was a 2-year-old girl with multiple airway conditions and a partial response to inhaled and systemic steroids. She was positive for acute phase reactants and negative for antibodies. Polymerase chain reaction revealed atypical Mycobacteria and she was negative for Mycobacterium tuberculosis. Laryngeal sarcoidosis was diagnosed by histopathology in a biopsy of larynx that revealed a chronic granulomatous inflammatory process with Langhans giant cells and acute and ulcerated areas with changes compatible with tuberculosis. Treatment consisted of monthly gammaglobulin for 6 months at doses of 2g/kg body weight, accompanied by Valmetrol™ and methotrexate. Immunomodulation with gammaglobulin was prescribed, with subsequent use of methotrexate-based immunosuppression. Currently, bronchoscopy shows no evidence of granulomas and she is negative for acute-phase reactants. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  16. Sarcoidosis with bilateral leg lymphedema as the initial presentation: a review of the literature

    Directory of Open Access Journals (Sweden)

    Hsin-Wei Huang

    2016-03-01

    Full Text Available Sarcoidosis is a granulomatous disease, characterized microscopically by noncaseating granulomas, which may involve multiple organs; however, the lung, skin, and lymph nodes are commonly affected. Sarcoidosis is a great imitator; in the skin, it presents with different cutaneous manifestations including lupus pernio, infiltrated plaques, maculopapular eruptions, infiltration of old scars, and subcutaneous sarcoidosis. Lymphedema as an initial presentation is extremely rare; cases are reported in African-American but not Asian patients. Lymphedema associated with sarcoidosis may result from lymphatic obstruction by infiltrating sarcoidosis. We present a case where the symptoms and signs of sarcoidosis were improved after treatment with systemic steroids.

  17. Sarcoidosis-Associated Aortoesophageal Fistula-Multistage Interdisciplinary Surgical Therapy for a Rare and Life-Threatening Condition.

    Science.gov (United States)

    Gombert, Alexander; Grommes, Jochen; Schick, Guido; Binnebösel, Marcel; Klink, Christian; Jacobs, Michael J; Kotelis, Drosos

    2017-02-01

    Aortoesophageal fistulas (AEFs) are rare and life-threatening conditions. Till date, an association between an AEF and sarcoidosis has not been reported yet. The aim of this report is to demonstrate a case of AEF secondary to sarcoidosis and its multistage interdisciplinary surgical therapy. A 66-year-old male was diagnosed with sarcoidosis in 2014. He has been treated with glucocorticoids since then and no severe health restrictions due to the disease have occurred. In December 2015, the patient presented with acute thoracic pain and hematemesis: an esophagogastroscopy revealed an AEF. First, stent-graft implantation in the thoracic aorta was urgently performed as a "bridging" procedure. Second, esophagectomy and local debridement were performed, followed by explantation of the stent graft and reconstruction by means of xenograft replacement of the stented aorta in a third operation. Finally, retrosternal gastric pull-up was performed in a fourth operative procedure. Sixteen days after the last operation the patient could be discharged to a rehabilitation clinic. Follow-up is uneventful so far; the antibiotic therapy was stopped at the time of hospital discharge. The pathogenesis of sarcoidosis, a rare autoimmunological disease, has not been completely clarified yet. The diagnosis relies on clinical symptoms and radiological as well as histopathological findings. Many cases of sarcoidosis show spontaneous regression, but severe complications may occur. While tracheoesophageal fistulas have been described in the literature, AEFs related to sarcoidosis have not been mentioned yet. Despite surgical and antibiotic treatment, the morbidity and mortality rates of AEF are high. Because the endovascular treatment has been established for emergency procedures of the aorta, it is considered as an appropriate first-line "bridging" treatment option. To achieve good long-term results, surgical treatment has to involve esophagectomy with secondary reconstruction of the upper

  18. What Are the Signs and Symptoms of Sarcoidosis?

    Science.gov (United States)

    ... related to the disease are more common in Japanese people. Lofgren's syndrome, a type of sarcoidosis, is ... and treating heart, lung, blood, and sleep disorders. Learn more about participating in a clinical trial . View ...

  19. Impaired survival of regulatory T cells in pulmonary sarcoidosis

    NARCIS (Netherlands)

    Broos, Caroline E.; van Nimwegen, Menno; Kleinjan, Alex; ten Berge, Bregje; Muskens, Femke; in 't Veen, Johannes C. C. M.; Annema, Jouke T.; Lambrecht, Bart N.; Hoogsteden, Henk C.; Hendriks, Rudi W.; Kool, Mirjam; van den Blink, Bernt

    2015-01-01

    Impaired regulatory T cell (Treg) function is thought to contribute to ongoing inflammatory responses in sarcoidosis, but underlying mechanisms remain unclear. Moreover, it is not known if increased apoptotic susceptibility of Tregs may contribute to an impaired immunosuppressive function in

  20. Gallium scanning in sarcoidosis. [/sup 67/Ga tracer technique

    Energy Technology Data Exchange (ETDEWEB)

    Israel, H.L.; Park, C.H.; Mansfield, C.M.

    1976-01-01

    The uptake of gallium-67 by pulmonary sarcoidosis was noted by Langhammer et al. in 1972 and by McKusick et al. in 1973. Heshiki et al. studied the application of this procedure in the diagnosis of sarcoidosis and concluded that although gallium-67 uptake in lungs and hilar regions correlated poorly with clinical activity, its measurement might prove useful in the diagnosis of sarcoidosis and in the measurement of response to therapy. We have undertaken to evaluate further the clinical usefulness of this technique. Thirty-six patients, 27 of whom had sarcoidosis, were given intravenous injections of 3 ml of commercially obtained (/sup 67/Ga) citrate. None were receiving corticosteroid therapy at the time of the initial study. Three days later simultaneous anterior and posterior scans of the thorax and the upper abdomen were performed in the supine position. 1 table.

  1. Sarcoidosis with heart involvement: a rare association of terrible ...

    African Journals Online (AJOL)

    Sarcoidosis with heart involvement: a rare association of terrible prognosis, a report of two cases. Asma Kefi, Nadia Ben Abdelhafidh, Sameh Sayhi, Rim Abid, Faida Ajili, Janet Laabidi, Salah Othmani ...

  2. Heredity In Sarcoidosis - A Registry-Based Twin Study

    DEFF Research Database (Denmark)

    Sverrild, Asger; Backer, Vibeke; Kyvik, Kirsten Ohm

    2008-01-01

    BACKGROUND: Sarcoidosis is a multiorgan, granulomatous, inflammatory disease with unknown aetiology. Familial clustering of cases and ethnic variation in the epidemiology suggests a genetic influence on the disease susceptibility. AIM: This paper reports twin concordance and heritability estimate...

  3. Impaired survival of regulatory T cells in pulmonary sarcoidosis

    NARCIS (Netherlands)

    C.E. Broos (Caroline); M. van Nimwegen (Menno); A. Kleinjan (Alex); B. ten Berge (Bregje); F. Muskens (Femke); J.C.C.M. in 't Veen (Johannes); J.T. Annema (Jouke); B.N.M. Lambrecht (Bart); H.C. Hoogsteden (Henk); R.W. Hendriks (Rudi); M. Kool (Mirjam); B. van den Blink (Bernt)

    2015-01-01

    textabstractBackground: Impaired regulatory T cell (Treg) function is thought to contribute to ongoing inflammatory responses in sarcoidosis, but underlying mechanisms remain unclear. Moreover, it is not known if increased apoptotic susceptibility of Tregs may contribute to an impaired

  4. Diffuse bronchiectasis as the primary manifestation of endobronchial sarcoidosis

    Directory of Open Access Journals (Sweden)

    Paul D. Hiles

    2017-01-01

    Full Text Available Sarcoidosis is an idiopathic disease that most commonly involves the lungs and is characterized by granulomatous inflammation. Bronchiectasis is one pulmonary manifestation of sarcoidosis, although it is almost always observed as traction bronchiectasis in the setting of fibrotic lung disease. A 50-year-old woman was evaluated for chronic cough and bronchiectasis with a small amount of peripheral upper lobe honeycombing and no significant pulmonary fibrosis or lymphadenopathy. After an extensive laboratory and imaging evaluation did not identify a cause of her bronchiectasis, bronchoscopy was performed to assess for primary ciliary dyskinesia and revealed a diffuse cobblestone appearance of the airway mucosa. Endobronchial biopsies and lymphocyte subset analysis of bronchoalveolar lavage fluid were consistent with a diagnosis of sarcoidosis. We believe endobronchial sarcoidosis should be included in the differential diagnosis of patients presenting with bronchiectasis.

  5. Endosonography vs conventional bronchoscopy for the diagnosis of sarcoidosis

    DEFF Research Database (Denmark)

    von Bartheld, Martin B; Dekkers, Olaf M; Szlubowski, Artur

    2013-01-01

    Tissue verification of noncaseating granulomas is recommended for the diagnosis of sarcoidosis. Bronchoscopy with transbronchial lung biopsies, the current diagnostic standard, has moderate sensitivity in assessing granulomas. Endosonography with intrathoracic nodal aspiration appears...

  6. CT findings in sarcoidosis with thoracic involvement; Hallazgos por TC en la sarcoidosis toracica

    Energy Technology Data Exchange (ETDEWEB)

    Diaz-Ruiz, M. J.; Tortajada, L.; Gallardo, X.; Castaner, E.; Carrasco, M.; Mata, J. M. [SDI-UDIAT Corporacion Sanitaria del Parc Tauli. Sabadell (Spain)

    2000-07-01

    Sarcoidosis is a systemic idiopathic disease characterized by the presence of noncaseating epithelioid granulomas in multiple organs. Thoracic involvement occurs in nearly all patients at some time during the course of the disease. We describe the findings in our patients with this disease using conventional computed tomography (CT) and high resolution CT (HRCT). We analyzed retrospectively the CT studies performed in our unit in 43 patients diagnosed as having sarcoidosis on the basis of biopsy and/or bronchoalveolar lavage. We classified the findings according to their intra parenchymal or extra parenchymal location, observing infrequent features such as pleural and pericardial thickening, lymphadenopathies presenting calcification or at uncommon sites, splenomegaly, laveolar pattern, trabecular pattern and air trapping. In patients with sarcoidosis, conventional CT and HRCT are more useful than plain radiology for the detection and study of the extension of intra parenchymal and extra parenchymal lesions and for the assessment of the disease course. It also serves to guide trans thoracic biopsy, should this procedure be necessary. (Author) 11 refs.

  7. The Prevalence of Antinuclear Antibodies in Patients with Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Senol Kobak

    2014-01-01

    Full Text Available Introduction. Sarcoidosis, which is a chronic inflammatory granulomatous disease, can mimic different rheumatologic diseases including connective tissue diseases. Antinuclear antibodies are the markers used for connective tissue diseases. Aim. To determine antinuclear antibody frequency and any possible correlation with clinical and laboratory data in sarcoidosis patients. Material and Method. Forty-two sarcoidosis patients, 45 rheumatoid arthritis patients, and 45 healthy volunteers who were followed up in rheumatology outpatient clinic were included in this study. Demographic, clinical, serological, and radiological data of all patients were recorded. Antinuclear antibodies were determined with indirect immunofluorescent method and 1/100 titration was accepted as positive. The cases that were ANA positive were evaluated with immunoblot method. Results. Average age of the 42 patients (10 males with sarcoidosis was 45.2 (20–70 years, and average disease duration was 3.5 years. ANA positivity was detected in 12 (28.5% patients with sarcoidosis (1/100 in 10 patients, 1/320 in two patients, in 19 of RA patients (42.2%, and in two of healthy volunteers in low titer (P<0.001. In the subgroup analysis made by immunblot test, one patient had anticentromere antibody, one had anti-Ro antibody, one had anti-Scl-70 antibody, one had anti-dsDNA antibody, and eight patients were negative. The two patients who had anticentromere and anti-Scl-70 antibodies had also Sjögren’s syndrome and scleroderma diagnosis, respectively. Discussion. The prevalence of ANA in patients with sarcoidosis was found to be significantly higher than healthy control group and lower than RA patients. This result shows that ANA may have an important role in the pathogenesis of sarcoidosis and also could be important in revealing the overlap syndromes of sarcoidosis-connective tissue diseases. Further studies with larger series are necessary in this subject.

  8. Cardiac Sarcoidosis: Sorting Fact from Fiction in This Rare Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Indranee Rajapreyar, MD

    2015-10-01

    Full Text Available Sarcoidosis is a rare condition of granulomatous infiltration of many tissues of the body, including the heart. Cardiac sarcoidosis has been challenging to study, as it is often asymptomatic, although the initial presentation can be sudden cardiac death. The incidence and prevalence rates have been difficult to establish and no expert agreed upon guidelines for diagnosis and management of cardiac sarcoidosis exist, and clinical manifestations are varied. The pathophysiology of granuloma formation in the myocardium as well as other tissues is governed by immune response to some environmental antigen. Genetics is also thought to play a role, although gene alterations have not been extensively studied, and no specific set of genetic mutations has been identified to aid in identification of individuals at risk of developing disease. Epigenetic factors likely play a significant role in modulation of gene expression with respect to immune response. There is no standardized screening tool for the identification of cardiac sarcoidosis. The presence of systemic sarcoidosis and new-onset third-degree heart block or ventricular arrhythmias warrants further investigation for cardiac sarcoidosis. MRI and PET are useful in helping to identify cardiac sarcoidosis but are not stand-alone tests. Endomyocardial biopsy is the gold standard but has a low yield owing to the patchy nature of granuloma formation in the myocardium. Therapy should be instituted early and involves immunosuppressive therapy with predominant use of corticosteroids. Arrhythmias, either ventricular or high-grade heart blocks, are managed with device therapy. Clinical presentation may warrant use of antiarrhythmic agents and/or catheter ablation. Survival and disease prognosis are dependent on early diagnosis and treatment. This review details the current understanding of cardiac sarcoidosis and highlights diagnostic strategies and treatment with the aim of guiding the clinician to early

  9. Racial Difference in Sarcoidosis Mortality in the United States

    Science.gov (United States)

    Machado, Roberto F.; Schraufnagel, Dean; Sweiss, Nadera J.; Baughman, Robert P.

    2015-01-01

    BACKGROUND: The clinical presentation and outcome of sarcoidosis varies by race. However, the race difference in mortality outcome remains largely unknown. METHODS: We studied mortality related to sarcoidosis from 1999 through 2010 by examining data on multiple causes of death from the National Center for Health Statistics. We compared the comorbid conditions between sarcoidosis-related deaths with deaths caused by car accidents (previously healthy control subjects) and rheumatoid arthritis (chronic disease control subjects) in both African Americans and Caucasians. RESULTS: From 1999 through 2010, sarcoidosis was reported as an immediate cause of death in 10,348 people in the United States with a combined overall mean age-adjusted mortality rate of 2.8 per 1 million person-years. Of these, 6,285 were African American and 3,984 Caucasian. The age-adjusted mortality rate for African Americans was 12 times higher than for Caucasians. African Americans died at an earlier age than Caucasians. African Americans living in the District of Columbia and North Carolina and Caucasians living in Vermont had higher mortality rates. Although the total sarcoidosis age-adjusted mortality rate had not changed over the 12 year period studied, this rate increased for Caucasians (R = 0.747, P = .005) but not for African Americans. Compared with the control groups, pulmonary hypertension was significantly more common in individuals with sarcoidosis. CONCLUSIONS: This nationwide population-based study exposes a significant difference in ethnicity and sex among people dying of sarcoidosis in the United States. Pulmonary hypertension investigation should be considered in all patients with sarcoidosis, especially African Americans. PMID:25188873

  10. Clinical Features of Pulmonary Sarcoidosis Complicated by Lung Cancer

    OpenAIRE

    Iijima, Yuki; Sugiyama, Yukihiko; Sawahata, Michiru; Nakayama, Masayuki; Bando, Masashi

    2017-01-01

    Objective For lung cancer complicated with sarcoidosis, there are no exact features that indicate whether lymphadenopathy is metastatic. This makes the validity of surgery uncertain for clinicians. The aim of this study was to clarify the clinical features of pulmonary sarcoidosis complicated by lung cancer, especially from the viewpoint of evaluating lymphadenopathy. Methods We retrospectively reviewed medical records from 2004 to 2013 at our institution, and 18 patients who were diagnosed w...

  11. Evidence for local dendritic cell activation in pulmonary sarcoidosis

    Directory of Open Access Journals (Sweden)

    Berge Bregje

    2012-04-01

    Full Text Available Abstract Background Sarcoidosis is a granulomatous disease characterized by a seemingly exaggerated immune response against a difficult to discern antigen. Dendritic cells (DCs are pivotal antigen presenting cells thought to play an important role in the pathogenesis. Paradoxically, decreased DC immune reactivity was reported in blood samples from pulmonary sarcoidosis patients. However, functional data on lung DCs in sarcoidosis are lacking. We hypothesized that at the site of disease DCs are mature, immunocompetent and involved in granuloma formation. Methods We analyzed myeloid DCs (mDCs and plasmacytoid DCs (pDCs in broncho-alveolar lavage (BAL and blood from newly diagnosed, untreated pulmonary sarcoidosis patients and healthy controls using 9-color flowcytometry. DCs, isolated from BAL using flowcytometric sorting (mDCs or cultured from monocytes (mo-DCs, were functionally assessed in a mixed leukocyte reaction with naïve allogeneic CD4+ T cells. Using Immunohistochemistry, location and activation status of CD11c+DCs was assessed in mucosal airway biopsies. Results mDCs in BAL, but not in blood, from sarcoidosis patients were increased in number when compared with mDCs from healthy controls. mDCs purified from BAL of sarcoidosis patients induced T cell proliferation and differentiation and did not show diminished immune reactivity. Mo-DCs from patients induced increased TNFα release in co-cultures with naïve allogeneic CD4+ T cells. Finally, immunohistochemical analyses revealed increased numbers of mature CD86+ DCs in granuloma-containing airway mucosal biopsies from sarcoidosis patients. Conclusion Taken together, these finding implicate increased local DC activation in granuloma formation or maintenance in pulmonary sarcoidosis.

  12. Hepatic sarcoidosis complicating treatment-naive viral hepatitis

    OpenAIRE

    Aravinthan, Aloysious; Gelson, William; Limbu, Anita; Brais, Rebecca; Richardson, Paul

    2012-01-01

    Hepatic sarcoidosis is usually asymptomatic but rarely leads to adverse liver-related outcome. Co-existence of viral hepatitis and hepatic sarcoidosis is a rare, but recognised phenomenon. Obtaining a balance between immune suppression and anti-viral therapy may be problematic. Immunosuppression in the presence of viral hepatitis can lead to rapid deterioration of liver disease. Similarly, anti-viral therapy may exacerbate granulomatous hepatitis. Here we present two cases of viral hepatitis ...

  13. Utility of angiotensin-converting enzyme activity in aqueous humor in the diagnosis of ocular sarcoidosis.

    Science.gov (United States)

    Mihailovic-Vucinic, Violeta; Popevic, Ljubica; Popevic, Spasoje; Stjepanovic, Mihailo; Aleksic, Andjelka; Stanojevic-Paovic, Anka

    2017-10-01

    Many studies include elevated activity of angiotensin-converting enzyme (ACE) in serum in sarcoidosis and in ocular sarcoidosis as well, but there are only a few analyzing ACE activities in aqueous humor. The aim of this study is to illuminate the diagnostic value of ACE in aqueous humor in patients with ocular sarcoidosis. We analyzed twenty patients with ocular sarcoidosis and 18 patients with nonocular involvement. All patients have biopsy-positive sarcoidosis of the lungs and/or mediastinal lymph nodes. Blood samples for ACE serum levels were obtained from all patients. Aqueous humor samples were taken by paracentesis with a 25-gauge needle in local anesthesia. With appropriate statistical tests, we compared ACE activity in serum and aqueous humor in patients with and without ocular sarcoidosis. The majority of our patients with ocular sarcoidosis were female (12/20), also in the group with systemic sarcoidosis and without ocular involvement (12/6). Mean age of the whole analyzed group of sarcoidosis patients was 45 ± 6 years. There is no statistically significant difference in ACE activity in serum between two groups of patients (with and without ocular sarcoidosis). There is statistically significant difference in ACE activity in aqueous humor among patients with ocular and nonocular sarcoidosis. ACE activity in aqueous humor is significantly higher in patients with ocular sarcoidosis. Increased ACE activity in aqueous humor can point to a diagnosis of ocular sarcoidosis, without the need for ocular biopsy.

  14. Cardiac Sarcoidosis Diagnosed by Incidental Lymph Node Biopsy.

    Science.gov (United States)

    Matsuda, Jun; Fujiu, Katsuhito; Roh, Solji; Tajima, Miyu; Maki, Hisataka; Kojima, Toshiya; Ushiku, Tetsuo; Nawata, Kan; Takeda, Norihiko; Watanabe, Masafumi; Akazawa, Hiroshi; Komuro, Issei

    2017-02-07

    Cardiac involvement in systemic sarcoidosis sometimes provokes life-threatening ventricular tachyarrhythmia. Steroid administration is one of the fundamental anti-arrhythmia therapies. For an indication of steroid therapy, a definitive diagnosis of sarcoidosis is required.1) However, cases that are clearly suspected of cardiac sarcoidosis based on their clinical courses sometimes do not meet the current diagnostic criteria and result in the loss of an appropriate opportunity to perform steroid therapy.Here we report a case that was diagnosed as sarcoidosis by incidental biopsy of an inguinal lymph node during cardiac resuscitation for cardiac tamponade.2) While the inguinal lymph node was not swollen on computed tomography, a specimen obtained from an incidental biopsy during the exposure of a femoral vessel for the establishment of extracorporeal cardio-pulmonary resuscitation showed a non-caseating granuloma.This findings suggest a non-swelling lymph node biopsy might be an alternative strategy for the diagnosis for sarcoidosis if other standard strategies do not result in a diagnosis of sarcoidosis.

  15. Ophthalmic and neuro-ophthalmic manifestations of sarcoidosis.

    Science.gov (United States)

    Kefella, Haben; Luther, Daniel; Hainline, Clotilde

    2017-11-01

    Sarcoidosis is a multisystemic inflammatory disease that commonly affects the eye and less often the neuro-ophthalmic pathways. The manifestations can be quite variable but can have characteristic signs and clinical features. This review provides a comprehensive overview of the various ocular and neuro-ophthalmic manifestations of sarcoidosis, emerging diagnostic measures and approach to treatment. Particular focus is given to recent advances in diagnostic approach and available treatment options. Laboratory investigations, chest and nuclear medicine imaging remain important techniques for helping to diagnose sarcoidosis. Recent evidence suggests a role for aqueous humor analysis in the diagnosis of ocular sarcoidosis. Characteristic neuroimaging may help differentiate neurosarcoidosis from other causes. The role of blind conjunctival biopsy for suspected neurosarcoidosis is discussed. The emerging role and use of biologics is delineated for the treatment of both ocular and neuro-ophthalmic sarcoidosis. Sarcoidosis can affect any part of the visual system: the most common ocular manifestation is uveitis and the most common neuro-ophthalmic manifestation is optic neuropathy. Although diagnosis remains challenging, recent advancements in diagnosis are promising. Emerging biologics with particular efficacy for ocular and neuro-ophthalmic disease provide expanding treatment options for sight-threatening disease.

  16. Usefulness of electron microscopy in the diagnosis of cardiac sarcoidosis.

    Science.gov (United States)

    Takemura, G; Takatsu, Y; Ono, K; Miyatake, T; Ono, M; Izumi, T; Fujiwara, H

    1995-01-01

    A 49-year-old man with cardiac sarcoidosis is presented. He suffered from congestive heart failure, and left ventricular asynergy and reduced function was evident by echocardiogram and left ventriculogram. A light microscopic examination of the endomyocardial biopsy revealed nonspecific myocarditis without giant cells or noncaseating granulomas. Under an electron microscope, however, several epithelioid cells were found in the specimen. The serum level of lysozyme was elevated. The patient had a past history of sarcoidosis of the eyes and lungs 22 years previously. Cardiac diseases presenting epithelioid cells other than sarcoidosis were clinically ruled out. Thus, the diagnosis of cardiac sarcoidosis was made based on both clinical and ultrastructural findings, and corticosteroid therapy was initiated. In the second biopsy, performed 4 months later, a noncaseating granuloma was found. Generally, the incidence of histological diagnosis of cardiac sarcoidosis by light microscopy is relatively low in endomyocardial biopsy specimens. The present case suggests that the addition of an ultrastructural examination may improve the diagnostic usefulness of the endomyocardial biopsy in cardiac sarcoidosis, since electron microscopy can clearly identify the presence of even one epithelioid cell.

  17. Long term follow-up of infliximab efficacy in pulmonary and extra-pulmonary sarcoidosis refractory to conventional therapy.

    Science.gov (United States)

    Russell, Eric; Luk, Francis; Manocha, Sonia; Ho, Tung; O'Connor, Carolyn; Hussain, Humaira

    2013-08-01

    Infliximab, a humanized, chimeric, monoclonal antibody against tumor necrosis alpha (TNF-α), has been shown to reduce the pulmonary and extra-pulmonary manifestations of sarcoidosis, however, there is little information regarding sustained efficacy with long-term use of infliximab. We retrospectively investigate whether a reduction in disease response is maintained, over a prolonged course of therapy (up to 85 months) with infliximab, and report on adverse events associated with its use. Subjects with multi-organ sarcoidosis were prescribed infliximab, between January 2000 to June 2010 due to failure of conventional therapy and were identified from the Drexel University College of Medicine sarcoidosis clinic. Retrospective patient reported symptom and objective clinical data analyses of pulmonary and extra-pulmonary findings were evaluated pre-infliximab and post or concurrent infliximab therapy. Any adverse events or reasons for discontinuation during infliximab therapy were reported. Twenty-six patients with biopsy proven sarcoidosis received anti-TNF therapy and met the criteria for study inclusion. Clinical evidence of sustained resolution or improvement was demonstrated in 58.5% of all organs assessed (p =<0.001). No clinical change in disease activity was seen in 35.8% of all organs evaluated. Despite infliximab treatment, 5.7% had progressive disease activity. Adverse events were seen in 57.7% of patients treated with infliximab over a 46.2 month average duration of therapy. Three (12%) patients had an adverse event that required permanent discontinuation. Infliximab is efficacious in the treatment of extra-pulmonary sarcoidosis and the efficacy is maintained with prolonged treatment. In patients with pulmonary sarcoid, sustained improvement in pulmonary imaging was seen after initiation of infliximab, however, post-treatment pulmonary function testing was not conclusive. Long-term infliximab therapy was well tolerated for our study group. Copyright © 2012

  18. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol

    Science.gov (United States)

    Koth, Laura L.; Maier, Lisa A.; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K.; Collman, Ronald G.; Hamzeh, Nabeel; Sweiss, Nadera J.; Zhang, Yingze; O’Neal, Scott; Senior, Robert M.; Becich, Michael; Hochheiser, Harry S.; Kaminski, Naftali; Wisniewski, Stephen R.; Gibson, Kevin F.

    2015-01-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant’s clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis. PMID:26193069

  19. Rationale and Design of the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) Study. Sarcoidosis Protocol.

    Science.gov (United States)

    Moller, David R; Koth, Laura L; Maier, Lisa A; Morris, Alison; Drake, Wonder; Rossman, Milton; Leader, Joseph K; Collman, Ronald G; Hamzeh, Nabeel; Sweiss, Nadera J; Zhang, Yingze; O'Neal, Scott; Senior, Robert M; Becich, Michael; Hochheiser, Harry S; Kaminski, Naftali; Wisniewski, Stephen R; Gibson, Kevin F

    2015-10-01

    Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation with tremendous clinical heterogeneity and uncertain pathobiology and lacking in clinically useful biomarkers. The Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis (GRADS) study is an observational cohort study designed to explore the role of the lung microbiome and genome in these two diseases. This article describes the design and rationale for the GRADS study sarcoidosis protocol. The study addresses the hypothesis that distinct patterns in the lung microbiome are characteristic of sarcoidosis phenotypes and are reflected in changes in systemic inflammatory responses as measured by peripheral blood changes in gene transcription. The goal is to enroll 400 participants, with a minimum of 35 in each of 9 clinical phenotype subgroups prioritized by their clinical relevance to understanding of the pathobiology and clinical heterogeneity of sarcoidosis. Participants with a confirmed diagnosis of sarcoidosis undergo a baseline visit with self-administered questionnaires, chest computed tomography, pulmonary function tests, and blood and urine testing. A research or clinical bronchoscopy with a research bronchoalveolar lavage will be performed to obtain samples for genomic and microbiome analyses. Comparisons will be made by blood genomic analysis and with clinical phenotypic variables. A 6-month follow-up visit is planned to assess each participant's clinical course. By the use of an integrative approach to the analysis of the microbiome and genome in selected clinical phenotypes, the GRADS study is powerfully positioned to inform and direct studies on the pathobiology of sarcoidosis, identify diagnostic or prognostic biomarkers, and provide novel molecular phenotypes that could lead to improved personalized approaches to therapy for sarcoidosis.

  20. Right ventricular cardiomyopathy meeting the arrhythmogenic right ventricular dysplasia revised criteria? Do not forget sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Vasaturo, Sabina; Ploeg, David E.; Zeppenfeld, Katja; Veselic-Charvat, Maud [Leiden University Medical Center, Leiden (Netherlands); Buitrago, Guadalupe [Dept. of Radiology, Hospital General Universitario Gregorio Maranon, Madrid (Spain)

    2015-06-15

    A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the {sup r}evised ARVD task force criteria{sup .} An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.

  1. The continuing search for Mycobacterium tuberculosis involvement in sarcoidosis: a study on archival biopsy specimens

    DEFF Research Database (Denmark)

    Svendsen, Claus Bo; Milman, Nils; Rasmussen, Erik Michael

    2011-01-01

    Increasing evidence indicates that mycobacteria may be involved in the aetiology and pathophysiology of sarcoidosis.......Increasing evidence indicates that mycobacteria may be involved in the aetiology and pathophysiology of sarcoidosis....

  2. Radiotherapy of vertebral hemangiomas

    Energy Technology Data Exchange (ETDEWEB)

    Sakata, Kohichi; Hareyama, Masato; Oouchi, Atushi; Sido, Mitsuo; Nagakura, Hisayasu; Tamakawa, Mituharu; Akiba, Hidenari; Morita, Kazuo [Dept. of Radiology, Sapporo Medical Univ., School of Medicine (Japan)

    1997-12-31

    Between 1975 and 1996, 14 patients (11 females, 3 males) with vertebral hemangioma received treatment with radiotherapy. Thirteen patients had a history of back pain or lumbago and 2 patients had neurological symptoms such as sensory impairment or paraplegia. The standard dose administered was 36 Gy in 18 fractions (five treatments per week). In the 13 patients with pain, this was completely or partially relieved. The condition of a man with hypesthesia of the legs deteriorated and a woman with paraplegia who was treated with decompressive laminectomy followed by radiotherapy recovered completely after irradiation. CT scan before irradiation showed thickened trabeculae as small punctate areas of sclerosis in all patients. At MR imaging before irradiation, T2-weighted MR images showed areas of high intensity in all patients and MR images demonstrated lesion enhancement. However, none of the patients who were treated successfully with radiation demonstrated any changes of the affected vertebra in the conventional radiographic films, CT scan or MR imaging, even 5 years after irradiation. Radiological imaging is indispensable for the diagnosis of vertebral hemangiomas but does not appear to be useful for evaluating the effects of radiotherapy. (orig.).

  3. Coeliac disease associated with sarcoidosis and antiphospholipid syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Melek Kechida

    2017-07-01

    Conclusion: Co-existence of sarcoidosis, CD and APS is extremely rare. APS should be recognized as an accompanying disorder of sarcoidosis and antiphospholipids measured especially when there is a history of thrombosis or miscarriages. CD should not be overlooked in association to sarcoidosis, given the shared immunological and genetic background, even in the absence of a typical presentation of the disease.

  4. Sarcoidosis with bilateral leg lymphedema as the initial presentation: a review of the literature

    OpenAIRE

    Huang, Hsin-Wei; Wong, Lai-San; Lee, Chih-Hung

    2016-01-01

    Sarcoidosis is a granulomatous disease, characterized microscopically by noncaseating granulomas, which may involve multiple organs; however, the lung, skin, and lymph nodes are commonly affected. Sarcoidosis is a great imitator; in the skin, it presents with different cutaneous manifestations including lupus pernio, infiltrated plaques, maculopapular eruptions, infiltration of old scars, and subcutaneous sarcoidosis. Lymphedema as an initial presentation is extremely rare; cases are reported...

  5. The role of pattern recognition receptors in lung sarcoidosis.

    Science.gov (United States)

    Mortaz, Esmaeil; Adcock, Ian M; Abedini, Atefhe; Kiani, Arda; Kazempour-Dizaji, Mehdi; Movassaghi, Masoud; Garssen, Johan

    2017-08-05

    Sarcoidosis is a granulomatous disorder of unknown etiology. Infection, genetic factors, autoimmunity and an aberrant innate immune system have been explored as potential causes of sarcoidosis. The etiology of sarcoidosis remains unknown, and it is thought that it might be caused by an infectious agent in a genetically predisposed, susceptible host. Inflammation results from recognition of evolutionarily conserved structures of pathogens (Pathogen-associated molecular patterns, PAMPs) and/or from reaction to tissue damage associated patterns (DAMPs) through recognition by a limited number of germ line-encoded pattern recognition receptors (PRRs). Due to the similar clinical and histopathological picture of sarcoidosis and tuberculosis, Mycobacterium tuberculosis antigens such early secreted antigen (ESAT-6), heat shock proteins (Mtb-HSP), catalase-peroxidase (katG) enzyme and superoxide dismutase A peptide (sodA) have been often considered as factors in the etiopathogenesis of sarcoidosis. Potential non-TB-associated PAMPs include lipopolysaccharide (LPS) from the outer membrane of Gram-negative bacteria, peptidoglycan, lipoteichoic acid, bacterial DNA, viral DNA/RNA, chitin, flagellin, leucine-rich repeats (LRR), mannans in the yeast cell wall, and microbial HSPs. Furthermore, exogenous non-organic antigens such as metals, silica, pigments with/without aluminum in tattoos, pesticides, and pollen have been evoked as potential causes of sarcoidosis. Exposure of the airways to diverse infectious and non-infectious agents may be important in the pathogenesis of sarcoidosis. The current review provides and update on the role of PPRs and DAMPs in the pathogenesis of sarcoidsis. Copyright © 2017 Elsevier B.V. All rights reserved.

  6. Sarcoidosis following HIV infection: evidence for CD4+ lymphocyte dependence.

    Science.gov (United States)

    Morris, David G; Jasmer, Robert M; Huang, Laurence; Gotway, Michael B; Nishimura, Stephen; King, Talmadge E

    2003-09-01

    The chronic granulomatous inflammation of sarcoidosis has been hypothesized to depend on the CD4+ T-helper lymphocyte. HIV infection, which depletes these cells, has been reported to attenuate the manifestations of sarcoidosis. We asked whether the development of symptomatic sarcoidosis in the context of preexisting HIV infection was dependent on the CD4+ lymphocyte count. We performed a retrospective standardized chart review of all patients who developed granulomatous inflammation following HIV infection at an urban academic referral center. We identified seven patients with sarcoidosis within this cohort and compared their CD4+ lymphocyte count to that in a cohort of 16 patients in whom similar granulomatous inflammation was found but who did not have sarcoidosis. We then compared our cases to all reported cases using a systematic literature review. The CD4+ lymphocyte count was > 200 cells/ microL in all of our patients with HIV infection when they developed subsequent sarcoidosis. In contrast, specific etiologies for granulomatous inflammation were found in all 10 HIV-infected patients who presented with granulomatous inflammation and a CD4+ lymphocyte count of 200 cells/ microL. We conclude that the development of the chronic granulomatous inflammation of sarcoidosis appears to depend on the preservation or restoration of the peripheral CD4+ lymphocyte count and that in most cases the CD4+ lymphocyte count exceeds 200 cells/ microL. Furthermore, alternative specific etiologies of granulomatous inflammation are generally identifiable in HIV-infected patients with peripheral CD4+ lymphocyte counts of < 200 cells/ microL.

  7. Mycobacterium tuberculosis Complex and Mycobacterial Heat Shock Proteins in Lymph Node Tissue from Patients with Pulmonary Sarcoidosis

    Science.gov (United States)

    Dubaniewicz, Anna; Dubaniewicz-Wybieralska, Mirosława; Sternau, Adam; Zwolska, Zofia; Iżycka-Świeszewska, Ewa; Augustynowicz-Kopeć, Ewa; Skokowski, Jarosław; Singh, Mahavir; Zimnoch, Lech

    2006-01-01

    We demonstrated that not whole Mycobacterium tuberculosis but its particular antigens, hsp70Mtb, hsp65Mtb, and hsp16Mtb, are present in lymph node tissues of patients with sarcoidosis (SA). hsp16Mtb occurs in the early stage of SA, whereas hsp70Mtb occurs in stage II of SA. hsp65Mtb is highly expressed in the capillary vessels in lymph node tissues in patients with SA. PMID:16954298

  8. Copy number variation of FCGR genes in etiopathogenesis of sarcoidosis.

    Directory of Open Access Journals (Sweden)

    Marlena Typiak

    Full Text Available We have previously revealed that, in contrast to polymorphism of FCGR2B and FCGR3B, polymorphism of FCGR2A, FCGR2C and FCGR3A genes, encoding receptors for Fc fragment of immunoglobulin G (Fcγ receptors, play a role in increased level of circulating immune complexes with occurrence of Mycobacterium tuberculosis heat shock proteins in patients with sarcoidosis. However, this immunocomplexemia might also be caused by decreased clearance by immune cells due to a changed copy number of FCGR genes. Thus, the next step of our study was to evaluate copy number variation of FCGR2A, FCGR2B, FCGR2C, FCGR3A and FCGR3B in this disease. The analysis was carried out by real-time quantitative PCR on 104 patients and 110 healthy volunteers. Despite previously detected variation in allele/genotype frequencies of FCGR in sarcoidosis and its particular stages, there was no copy number variation of the tested genes between sarcoidosis or its stages and healthy control, as well as between stages themselves. A relevant increase in copy number of FCGR2C and FCGR3B in Stage IV of sarcoidosis vs. other stages and controls was detected, but this observation was based on a limited number of Stage IV patients. Hence, polymorphism of FCGR genes seems to be more important than their copy number variation in etiopathogenesis of sarcoidosis in patients from the Polish population.

  9. Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis.

    Science.gov (United States)

    Mizuno, Kana; Nguyen, Chuyen Thi Hong; Ueda-Hayakawa, Ikuko; Okamoto, Hiroyuki

    2017-05-01

    Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62-year-old female was diagnosed with sarcoidosis by chest-abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non-caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  10. Successful treatment of recalcitrant cutaneous sarcoidosis with fumaric acid esters

    Directory of Open Access Journals (Sweden)

    Hanefeld Christoph

    2002-12-01

    Full Text Available Abstract Background Sarcoidosis is a multisystem disease of unknown origin characterized by the formation of noncaseating granulomas, in particular in the lungs, lymph nodes, eyes, and skin. Systemic treatment for cutaneous sarcoidosis can be used for large disfiguring lesions, generalized involvement, or recalcitrant lesions that did not respond to topical therapy. Case presentations We report three patients with recalcitrant cutaneous sarcoidosis who were treated with oral fumaric acid esters (FAE. Three female patients presented with cutaneous sarcoidosis that have proved to be refractory to various therapies, including corticosteroids and chloroquine. We treated the patients with FAE in tablet form using two formulations differing in strength (Fumaderm® initial, Fumaderm®. Dosage of FAE was performed according to the standard therapy regimen for psoriasis patients. After treatment with FAE (4–12 months, a complete clearance of skin lesions was achieved in the three patients. The side effects observed in this trial correspond to the well-known spectrum of adverse effects of FAE (flush, minor gastrointestinal complaints, lymphopenia. Conclusions On the basis of our findings FAE therapy seems to be a safe and effective regimen for patients with recalcitrant cutaneous sarcoidosis. Nevertheless further investigations are necessary to confirm our preliminary results.

  11. Successful treatment of recalcitrant cutaneous sarcoidosis with fumaric acid esters

    Science.gov (United States)

    Nowack, Ute; Gambichler, Thilo; Hanefeld, Christoph; Kastner, Ulrike; Altmeyer, Peter

    2002-01-01

    Background Sarcoidosis is a multisystem disease of unknown origin characterized by the formation of noncaseating granulomas, in particular in the lungs, lymph nodes, eyes, and skin. Systemic treatment for cutaneous sarcoidosis can be used for large disfiguring lesions, generalized involvement, or recalcitrant lesions that did not respond to topical therapy. Case presentations We report three patients with recalcitrant cutaneous sarcoidosis who were treated with oral fumaric acid esters (FAE). Three female patients presented with cutaneous sarcoidosis that have proved to be refractory to various therapies, including corticosteroids and chloroquine. We treated the patients with FAE in tablet form using two formulations differing in strength (Fumaderm® initial, Fumaderm®). Dosage of FAE was performed according to the standard therapy regimen for psoriasis patients. After treatment with FAE (4–12 months), a complete clearance of skin lesions was achieved in the three patients. The side effects observed in this trial correspond to the well-known spectrum of adverse effects of FAE (flush, minor gastrointestinal complaints, lymphopenia). Conclusions On the basis of our findings FAE therapy seems to be a safe and effective regimen for patients with recalcitrant cutaneous sarcoidosis. Nevertheless further investigations are necessary to confirm our preliminary results. PMID:12498617

  12. Fatigue in sarcoidosis and idiopathic pulmonary fibrosis: differences in character and severity between diseases.

    Science.gov (United States)

    Atkins, Christopher Peter; Gilbert, Daniel; Brockwell, Claire; Robinson, Sue; Wilson, Andrew Malcolm

    2016-08-01

    Sarcoidosis and idiopathic pulmonary fibrosis (IPF) are two common forms of interstitial lung disease. Fatigue is a recognised feature of sarcoidosis but an association between IPF and fatigue has not been investigated. To investigate the frequency and severity of fatigue in these groups, and variables affecting fatigue scores. A cross-sectional questionnaire study of patients with sarcoidosis and IPF followed-up at a single hospital was undertaken. Questionnaire data included validated measures of fatigue, anxiety, depression, sleepiness and dyspnoea, plus measures of disease severity including spirometry data. Questionnaires were administered to 232 patients (82 healthy volunteers, 73 sarcoidosis patients and 77 IPF patients). Sarcoidosis patients had statistically higher sleepiness scores but no significant difference was seen between overall measures of fatigue, anxiety or depression. Stratification by severity revealed a non-statistically significant tendency towards more severe fatigue scores in sarcoidosis. Regression analysis failed to identify any significant predictor variables measured in the sarcoidosis cohort, though in the IPF group both dyspnoea and sleepiness scores were significant predictors of fatigue (R2=0.74). Both sarcoidosis and IPF patients suffer with fatigue, although sarcoidosis patients tended towards reporting more severe fatigue scores, suggesting a subgroup with severe fatigue. The fatigue experienced by the two groups appears to be different; sarcoidosis patients report greater frequency of mental fatigue whereas IPF patients appear to suffer exhaustion, potentially related to dyspnoea. Dyspnoea and sleepiness scores modeled the majority of fatigue in the IPF group, whereas no single factor was able to predict fatigue in sarcoidosis.

  13. Up-to-date in pulmonary and extrapulmonary sarcoidosis.

    Science.gov (United States)

    Valeyre, D; Uzunhan, Y; Bouvry, D; Naccache, J-M; Nunes, H

    2008-01-01

    Sarcoidosis is a multisystemic immune disorder of unknown cause characterized by the formation of epithelioid granuloma in involved organs particularly the lung and the lymphatic system. Consistent recent advances have been made on the clinical, therapeutical and pathogenetic aspects. Various clinical phenotypes are better characterized and efforts to grade the severity could facilitate genetic studies and therapeutic trials. Pulmonary hypertension and some infrequent or atypical pulmonary manifestations as well as some rare extrapulmonary localizations have been thoroughly studied. Sarcoidosis-like granulomatosis due to identified causes constitute interesting models and have to be differentiated from sarcoidosis. THERAPEUTICAL ASPECTS: Anti-TNFalpha drugs have been investigated and infliximab has been proved efficient even though some limits to its efficiency have to be taken into account. PATHOGENETIC ASPECTS: Finally, advances concerning the pathogenesis, particularly genetic predisposing factors and phenotype/genotype correlations have been made.

  14. Sarcoidosis mimicking seborrheic dermatitis: another case of Sherlockian Dermatology

    Directory of Open Access Journals (Sweden)

    César Bimbi

    2014-10-01

    Full Text Available Even tiny despicable skin signs may eventually unveil systemic illnesses enabling a prompt start of medication and better prognosis. Here, we present a case of a 28-year-old female patient who came to the office complaining of a „minor irritation” on the left eyebrow that was a discrete and asymptomatic 5mm infiltrated apple jelly coloured papule that had started 6 months earlier. Biopsy of a second similar lesion discovered on left forearm showed granulomatous features of sarcoidosis. Under subsequent systemic investigation, the presence of sarcoidosis in other organs was found to be positive as stage II pulmonary sarcoidosis, and also with reticulo-endothelial involvement manifested by enlarged mediastinal lymph nodes. This case highlights the skin as a mirror to internal multisystemic disease and also the importance of investigating even small and discrete lesions with care and in depth.

  15. Sarcoidosis: a pattern of clinical and morphological presentation.

    Science.gov (United States)

    Valantine, H; McKenna, W J; Nihoyannopoulos, P; Mitchell, A; Foale, R A; Davies, M J; Oakley, C M

    1987-01-01

    The diagnosis of cardiac sarcoidosis, particularly when there is no overt systemic involvement, is frequently delayed because of its varied manifestations. Focal left ventricular wall motion abnormalities were recognised in five patients with sarcoidosis. Three patients showed abnormal regional wall motion in the basal portion of the ventricular septum and free wall with sparing of the apex. The angiographic appearances supported the echocardiographic findings which were atypical of ischaemic heart disease. The remaining two patients both had diffuse left ventricular hypokinesia, with a focal abnormality that was most pronounced in the anteroapical region; this pattern is often seen with coronary disease. The recognition by echocardiography or angiography of focal abnormalities of wall motion affecting the basal portion of the ventricular septum should suggest the possibility of myocardial sarcoidosis even in the absence of recognised systemic manifestations. Images Fig 1 Fig 2 Fig 3 Fig 4 Fig 5 Fig 6 Fig 7 PMID:3566984

  16. [Early-onset sarcoidosis/Blau syndrome].

    Science.gov (United States)

    Kambe, Naotomo; Satoh, Takashi; Nakano, Michiyo; Nakamura, Yuumi; Matsue, Hiroyuki

    2011-01-01

    Familial Blau syndrome and sporadic early-onset sarcoidosis (EOS) are both systemic granulomatous diseases evoked by the spontaneous activation of mutated NOD2. In Japan, the R334W amino acid substitution is more frequently identified, whereas the R334Q mutation is rare and, in contrast to western countries where disease causing mutations are typically hereditary, most Japanese cases derive from sporadic mutations. Recently, a case with a six-base deletion in the NOD2 gene was reported. This Blau syndrome/EOS patient presented with the unpainful soft swelling of the dorsal side of the wrist and ankles, as well as flexion contracture at the proximal interphalangeal joint that gradually appeared during their clinical course. These features are useful for the differential diagnosis of Blau syndrome/EOS from juvenile idiopathic arthritis. Owing to their characteristic clinical symptoms, Blau and EOS patients can be identified earlier if medical experts become more acquainted with these distinctions. Even though specific treatment based on pathophysiologic mechanism has not been explored yet, early diagnosis will prevent the progression to severe impairment, which can severely affect patients' lives.

  17. [Sarcoidosis : Organ involvement, diagnosis, current treatment].

    Science.gov (United States)

    Weidenthaler-Barth, B; Steinbrink, K; Kümmel, A; von Stebut, E

    2015-07-01

    Sarcoidosis is characterized by the appearance of noncausating, epitheloid cell granulomas, primarily in skin and lung. Hereditary disposition is well known; additional infection-associated triggers play a role for the development of inflammation mediated by T helper (Th)1 cells. Clinically, various disease courses can be observed that are characterized by the formation of skin papules at typical sites of the body which differ in their tendency to be associated with systemic organ involvement. Systemic disease without skin affections is also possible. The diagnosis is based on the typical clinical appearance, biopsy of affected tissue (e.g. skin, lung) and laboratory investigations. Additional systemic involvement needs to be excluded. In most cases, the disease is self-limited, but can also be life threatening due to organ fibrosis. The degree of (extra-)cutaneous involvement and level of discomfort are used to select the type of treatment, which ranges from topical immune suppressive agents to systemic therapy with corticosteroids. In nonresponders, additional modern immunosuppressive/immunomodulating therapeutic options are available.

  18. Graves’ Disease and Papillary Thyroid Carcinoma in a Patient with Active Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Şefika Burçak Polat

    2012-12-01

    Full Text Available Sarcoidosis is a systemic granulomatous disease of unknown etiology. In most cases, mediastinal lymph nodes and lung parenchyma are involved. In addition, the eyes, skin, abdominal organs, central nervous system or the joints might be involved during the course of the disease. Sarcoidosis has been found to be related with other autoimmune diseases such as thyroiditis. In this report, we present the case of a patient in whom hyperthroidism was found and Graves’ disease and papillary thyroid carcinoma were diagnosed while being investigated with a preliminary diagnosis of sarcoidosis. A 50-year-old male patient was admitted to our chest diseases clinic with the complaints of cough with colorless sputum, joint pain, weakness, weight loss (36 kgs, palpitations, tremor of the hands and pain in the right eye. Mediastinal lympadenopathy was detected on CT scan. Laboratory tests demonstrated hypercalcemia and low levels of parathyroid hormone (PTH. Eye examination revealed uveitis. Bronchoscopy was planned to confirm the diagnosis of sarcoidosis. Thyroid function test was performed and the patient turned out to have hyperthyroidism with high free T4 and low TSH levels. TSH receptor antibody titer was 71 U/L (0- 14 U/L. Ultrasonographic examination demonstrated enlarged thyroid gland and heterogeneous parenchyma with increased blood flow pattern. 4- and 24-hour radioiodine uptakes were 30% and 60%, respectively and scintigraphy findings were compatible with diffuse toxic goiter. On Hertel exophthalmometer, the measurements of the right and left eye were 23 and 24 mm, respectively. The diagnosis of Graves’ disease was made and thionamide therapy was started. Since active ophthalmopathy was present and the patient did not want to receive medical therapy for a long time, surgery was the best option for definitive therapy. The patient was prepared for the surgery with lugol solution (3x10 drops daily and after eutyroidism was achieved, bilateral total

  19. Hybrid Magnetic Resonance Imaging and Positron Emission Tomography With Fluorodeoxyglucose to Diagnose Active Cardiac Sarcoidosis.

    Science.gov (United States)

    Dweck, Marc R; Abgral, Ronan; Trivieri, Maria Giovanna; Robson, Philip M; Karakatsanis, Nicolas; Mani, Venkatesh; Palmisano, Anna; Miller, Marc A; Lala, Anuradha; Chang, Helena L; Sanz, Javier; Contreras, Johanna; Narula, Jagat; Fuster, Valentin; Padilla, Maria; Fayad, Zahi A; Kovacic, Jason C

    2017-06-09

    The purpose of this study was to explore the diagnostic usefulness of hybrid cardiac magnetic resonance (CMR) and positron emission tomography (PET) using 18F-fluorodeoxyglucose (FDG) for active cardiac sarcoidosis. Active cardiac sarcoidosis (aCS) is underdiagnosed and has a high mortality. Patients with clinical suspicion of aCS underwent hybrid CMR/PET with late gadolinium enhancement (LGE) and FDG to assess the pattern of injury and disease activity, respectively. Patients were categorized visually as magnetic resonance (MR)+PET+ (characteristic LGE aligning exactly with increased FDG uptake), MR+PET- (characteristic LGE but no increased FDG), MR-PET- (neither characteristic LGE nor increased FDG), and MR-PET+ (increased FDG uptake in absence of characteristic LGE) and further characterized as aCS+ (MR+PET+) or aCS- (MR+PET-, MR-PET-, MR-PET+). FDG uptake was quantified using maximum target-to-normal-myocardium ratio and the net uptake rate (Ki) from dynamic Patlak analysis. Receiver operating characteristic methods were used to identify imaging biomarkers for aCS. FDG PET was assessed using computed tomography/PET in 19 control subjects with healthy myocardium. A total of 25 patients (12 males; 54.9 ± 9.8 years of age) were recruited prospectively; 8 were MR+PET+, suggestive of aCS; 1 was MR+PET-, consistent with inactive cardiac sarcoidosis; and 8 were MR-PET-, with no imaging evidence of cardiac sarcoidosis. Eight patients were MR-PET+ (6 with global myocardial FDG uptake, 2 with focal-on-diffuse uptake); they demonstrated distinct Ki values and hyperintense maximum standardized uptake value compared with MR+PET+ patients. Similar hyperintense patterns of global (n = 9) and focal-on-diffuse (n = 2) FDG uptake were also observed in control patients, suggesting physiological myocardial uptake. Maximum target-to-normal-myocardium ratio values were higher in the aCS+ group (p < 0.001), demonstrating an area under the curve of 0.98 on receiver operating

  20. A Nodular Type of Subcutaneous Sarcoidosis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Kyu Ho; Choi, Yun Sun; Kim, Byoung Suck; Joo, Jong Eun; Jung, Yoon Young; Cho, Young Kwon; An, Jin Kyung; Kim, Hyun Sook; Woo, Jung Joo [Eulji University Hospital, Daejeon (Korea, Republic of)

    2009-01-15

    Sarcoidosis is a granulomatous multisystemic disorder that rarely involves subcutaneous tissue. We describe the MR imaging findings of a subcutaneous sarcoidosis in a patient that presented with a nontender, palpable soft tissue mass on the left buttock, which was confirmed after surgical excision. The MR images showed the presence of a subcutaneous mass that breached the adjacent fascia with an irregular outline and homogeneous, slightly higher signal intensity than the surrounding muscle as seen on a T2-weighted image and with homogeneous enhancement after contrast injection. The lesion could not be differentiated from a sarcoma or a malignancy.

  1. ACE Inhibitor in the treatment of cutaneous and lymphatic sarcoidosis.

    Science.gov (United States)

    Kaura, Vinod; Kaura, Samantha H; Kaura, Claire S

    2007-01-01

    Angiotensin-converting enzyme is used as a marker for sarcoid activity. We describe a case of remission of cutaneous and lymphatic sarcoidosis in a patient treated with an ACE inhibitor for congestive heart failure and hypertension; the remission has continued over 4 years of follow-up. Because this is a report of only one case, there is a possibility of sampling error. Whether the patient's remission in this case was a serendipitous spontaneous remission that happened to occur during ACE inhibitor therapy or whether ACE inhibitor therapy can play a role in the treatment of sarcoidosis needs to be determined in a large clinical trial.

  2. Proton MRI in the evaluation of pulmonary sarcoidosis: Comparison to chest CT

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Jonathan H., E-mail: chungj@njhealth.org [Radiology, National Jewish Health, Denver, CO (United States); Little, Brent P., E-mail: bplittle2@hotmail.com [Radiology, Emory University School of Medicine, Atlanta, GA (United States); Forssen, Anna V., E-mail: ForssenA@NJHealth.org [Division of Biostatistics and Bioinformatics, National Jewish Health, Denver, CO (United States); Yong, Jin, E-mail: JinG@NJHealth.org [Radiology, National Jewish Health, Denver, CO (United States); Diagnostic Radiology, Chonbuk National University Medical School, Jeonbuk (Korea, Republic of); Nambu, Atsushi, E-mail: nambu-a@gray.plala.or.jp [Radiology, National Jewish Health, Denver, CO (United States); Radiology, University of Yamanashi, Chuo City (Japan); Kazlouski, Demitry, E-mail: demitry.kazlouski@gmail.com [Radiology, National Jewish Health, Denver, CO (United States); Puderbach, Michael, E-mail: m.puderbach@dkfz-heidelberg.de [Diagnostic and Interventional Radiology, Chest Clinics at University Hospital, Heidelberg (Germany); Biederer, Juergen, E-mail: juergen.biederer@uni-heidelberg.de [Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg (Germany); Lynch, David A., E-mail: LynchD@NJHealth.org [Radiology, National Jewish Health, Denver, CO (United States)

    2013-12-01

    Purpose: The purpose of this study was to determine the feasibility of proton MRI of the lung in sarcoidosis patients and the agreement between the imaging appearance of pulmonary sarcoidosis on MRI and CT. Materials and methods: Chest CT scans and dedicated pulmonary MRI scans (including HASTE, VIBE, and TrueFISP sequences) were performed within 90 days of each other in 29 patients. The scans were scored for gross parenchymal opacification, reticulation, nodules, and masses using a 3-point lobar scale. Total and subset scores for corresponding MRI and CT scans were compared using the Spearman correlation test, Bland–Altman plots, and Cohen's quadratic-weighted kappa analysis. MRI scores were compared to CT by lobe and disease category, using percentage agreement, Spearman rank correlation, and Cohen's quadratic-weighted kappa. Results: The mean (±s.d.) time between MRI and CT scans was 33 ± 32 days. There was substantial correlation and agreement between total disease scoring on MRI and CT with a Spearman correlation coefficient of 0.774 (p < 0.0001) and a Cohen's weighted kappa score of 0.646. Correlation and agreement were highest for gross parenchymal opacification (0.695, 0.528) and reticulation (0.609, 0.445), and lowest in the setting of nodules (0.501, 0.305). Agreement testing was not performed for mass scores due to low prevalence. Upper lobe scoring on MRI and CT demonstrated greater agreement compared to the lower lobes (average difference in Cohen's weighted kappa score of 0.112). Conclusion: There is substantial correlation and agreement between MRI and CT in the scoring of pulmonary sarcoidosis, though MRI evaluation in the upper lobes may be more accurate than in the lower lobes.

  3. Ichthyosiform sarcoidosis: report of three cases from Japan and literature review.

    Science.gov (United States)

    Miura, Takako; Kato, Yasunobu; Yamamoto, Toshiyuki

    2016-12-23

    Cutaneous lesions of sarcoidosis present with various manifestations including specific and non-specific cutaneous lesions. Ichthyosiform sarcoidosis is a rare form of cutaneous sarcoidosis, presenting with asymptomatic, adherent, polygonal scales, mainly appearing on the lower limbs.  Ichthyosiform sarcoidosis has a predilection for dark-skinned races, and cases affecting Japanese patients have rarely been reported in English literature.  We herein describe three Japanese cases of ichthyosiform sarcoidosis on the lower limbs. All of the patients were female, with an age range of 57-69 years old.  Histologically, sarcoidal granulomas were located in the mid- to lower dermis.  All cases had scar sarcoidosis on the knees.  Furthermore, Case 1 presented with papular sarcoidosis on the back, and Case 3 presented with subcutaneous nodules on the buttock as well as erythema nodosum-like lesions on the lower legs.  All patients had lung sarcoidosis, but ocular sarcoidosis was seen in only Case 2. Case 3 showed Heerfordt syndrome with facial nerve paralysis. Histological features showed that the granular layers were scarcely detected in the overlying epidermis; however, filaggrin expression was not decreased.  Sarcoidal granulomas accumulated around the sweat glands in one case, whereas those features were not detected in the other two cases. In conclusion, ichthyosiform cutaneous sarcoidosis may be overlooked or misdiagnosed as xerotic dry skin which is frequently found in elderly people, and ichthyosiform cutaneous lesions may be more prevalent than previously estimated.

  4. ACCENTUATION OF PERSONALITY TRAITS IN THE PATIENTS WITH GRANULOMATOUS LESIONS OF RESPIRATORY ORGANS IN CASE OF SARCOIDOSIS AND TUBERCULOSIS

    Directory of Open Access Journals (Sweden)

    A. Yu. Chernikov

    2017-01-01

    Full Text Available 404 sarcoidosis and 404 tuberculosis patients were examined in order to detect correlations between clinical manifestations, psychological adaptation and accentuation of personality traits and granulomatous lesions of respiratory organs in case of sarcoidosis and tuberculosis. All patients had subjective and objective examinations and answered the following questionnaires: Schmieschek questionnaire to identify accentuation of personality traits, clinical questionnaire to detect and evaluate neurotic disorders, Taylor Manifest Anxiety Scale to detect the level of anxiety. It has been found out that sarcoidosis patients are characterized both by asymptomatic course of the disease as well as diverse clinical manifestations: pain syndrome, nodal fever, intoxication with expressed general fatigue, respiratory insufficiency. It is combined with stuck, pedant, cycloid, exalted, emotive accentuations of personality traits, with psychological maladaptation as per the scores of autonomic imbalance, neurotic depression, asthenia, with average high level of anxiety. The following is typical of tuberculosis patients: syndrome of bronchial tree lesions and respiratory insufficiency; distymny, demonstrative, excitable, exalted, anxiety-hypochondriac accentuations of personality traits, with psychological maladaptation as per the scores of autonomic imbalance, obsessive-phobic disorders and hysteria; average high level of anxiety. The strong correlation has been found between the degree of symptoms expression, level of anxiety and psychological maladaptation and the type of patient's accentuation of personality traits.

  5. Systemic sarcoidosis complicated of acute renal failure: about 12 cases

    Science.gov (United States)

    Mahfoudhi, Madiha; Mamlouk, Habiba; Turki, Sami; Kheder, Adel

    2015-01-01

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, biological and histological profile in these cases and then to indicate the interest to consider the diagnosis of sarcoidosis in cases of unexplained renal failure. Extra-renal complications, therapeutic modalities and the outcome were determined in all patients. Our series involved 12 women with an average age of 40 years. Biological investigations showed an abnormal normocalcemia in 7 cases, a hypercalcemia in 5 cases, a hypercalciuria in 10 cases and polyclonal hypergammaglobulinemia in 7 cases. An acute renal failure was found in all patients with a median creatinin of 520 umol/L. For all patients, the renal echography was normaln however, the kidney biopsy showed tubulo-interstitial nephritis. The extra-renal signs highlighting pulmonary interstitial syndrome in 5 cases, a sicca syndrome in 4 cases, mediastinal lymph nodes in 2 cases, a lymphocytic alveolitis in 3 cases, an anterior granulomatous uveitis in 2 cases and a polyarthritis in 5 cases. Five patients benefited of hemodialysis. The treatment consisted of corticosteroid in all cases. The follow up was marked by complete resolution of clinical and biological signs. The diagnosis of renal sarcoidosis must be done quickly to prevent renal failure. PMID:26834928

  6. Association between physical functions and quality of life in sarcoidosis

    NARCIS (Netherlands)

    Drent, M.; Marcellis, R. G. J.; Lenssen, A. F.; de Vries, J.

    2014-01-01

    BACKGROUND: Quality of life (QOL) is often reduced in patients with sarcoidosis. Studies of the associations between physical functions and QOL are lacking. OBJECTIVES: So the aims of this study were (i) to evaluate the associations between QOL and physical functions, including muscle strength and

  7. Hepatic sarcoidosis: a case series | Ennaifer | Pan African Medical ...

    African Journals Online (AJOL)

    Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary ...

  8. Sarcoidosis, Celiac Disease and Deep Venous Thrombosis: a Rare Association

    Directory of Open Access Journals (Sweden)

    Gökhan Çelik

    2011-11-01

    Full Text Available Sarcoidosis is a multisystem granulomatous disorder of unknown etiology and it may rarely be associated with a second disorder. Celiac disease is an immune-mediated enteropathy characterized with malabsorption caused by gluten intolerance, and several reports indicate an association between celiac disease and sarcoidosis. In addition, although celiac disease is associated with several extraintestinal pathologies, venous thrombosis has been rarely reported. Herein we present a rare case report of a patient with a diagnosis of sarcoidosis, celiac disease and deep venous thrombosis because of the rare association of these disorders. The patient was admitted with abdominal pain, weight loss, chronic diarrhea and a 5-day history of swelling in her right leg. A diagnosis of deep venous thrombosis was achieved by doppler ultrasonographic examination. The diagnosis of celiac disease was made by biopsy of duodenal mucosa and supported with elevated serum level of anti-gliadin IgA and IgG, and a diagnosis of sarcoidosis was achieved by transbronchial needle aspiration from the subcarinal lymph node during flexible bronchoscopy.

  9. Nature of fatigue moderates depressive symptoms and anxiety in sarcoidosis

    NARCIS (Netherlands)

    de Kleijn, W.P.E.; Drent, M.; de Vries, J.

    2013-01-01

    Objectives Fatigue, depressive symptoms, and anxiety are frequently reported in sarcoidosis. However, the relationship between these debilitating symptoms is unclear. Therefore, the purpose of this prospective follow-up study was to identify the prevalence of depressive symptoms and anxiety in

  10. Case study: sarcoidosis | Woolley | South Sudan Medical Journal

    African Journals Online (AJOL)

    South Sudan Medical Journal. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 7, No 3 (2014) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. Case study: sarcoidosis. Andrew Woolley, Michaela Lynn, ...

  11. Characteristics of sarcoidosis in Maori and Pacific Islanders.

    Science.gov (United States)

    Wilsher, Margaret L; Young, Lisa M; Hopkins, Raewyn; Cornere, Megan

    2017-02-01

    Ethnicity is strongly associated with variable clinical presentation in sarcoidosis but the association between ethnicity and clinical characteristics has not previously been described in patients of Polynesian ancestry, Maori and Pacific Islander (PI). The objective of this study was to describe the clinical characteristics of sarcoidosis in Maori and PI patients and determine if those were different to European patients. A retrospective review of the medical records of 406 patients (69 Maori/PI) attending a specialist interstitial lung disease (ILD) clinic. The population (207 females, mean age at presentation: 36) reflected the current New Zealand census data (2013) with only people of Indian ethnicity over-represented. Parenchymal lung involvement was uncommon in Maori and PI patients (21% Scadding stage 2, 2% stage 3), and no patient had extensive pulmonary fibrosis (stage 4). Computed tomography (CT) patterns of sarcoid parenchymal lung involvement were less commonly reported for Maori/PI. There were no differences in respect of baseline lung function or requirement for treatment. Ocular and skin involvement occurred more frequently in Maori and PI (P = 0.0045, P = 0.03), and erythema nodosum was more common in Caucasians (P = 0.0008). People of Polynesian ancestry appear to have less pulmonary and more extra-pulmonary manifestations of sarcoidosis. This adds to our knowledge that sarcoidosis heterogeneity is influenced by ethnicity. © 2016 Asian Pacific Society of Respirology.

  12. An epidemiological perspective of the pathology and etiology of sarcoidosis.

    Science.gov (United States)

    Sawahata, Michiru; Sugiyama, Yukihiko

    2016-08-01

    To update current knowledge on the pathology and etiology of sarcoidosis, here we review previous epidemiological research and discuss age-related differences and historical changes in the clinical characteristics of sarcoidosis we identified over the last four decades in Japan. Extrathoracic lymph node involvement was more common in young patients, while extrathoracic involvement of non-lymphatic organs and hypercalcemia were more common in older patients. Most patients in their 20s presented with bilateral hilar lymphadenopathy, but this was consistently less common among older patients. Over time, the distribution of age at diagnosis has shifted toward the older age group in the United States, Denmark, and Japan. In Japan, the incidence rate has been decreasing among young people, but there has consistently been a second peak among postmenopausal women. Age-related differences in the clinical presentation of sarcoidosis may reflect the pathways of causative antigens and the strengthening of immunoregulatory mechanisms with age. Internal and external environmental factors, such as exposure to diverse microorganisms, ovarian insufficiency, and active vitamin D deficiency, that may contribute to the onset of sarcoidosis must be identified in order to develop strategies for prevention and treatment.

  13. Clinical Features of Pulmonary Sarcoidosis Complicated by Lung Cancer.

    Science.gov (United States)

    Iijima, Yuki; Sugiyama, Yukihiko; Sawahata, Michiru; Nakayama, Masayuki; Bando, Masashi

    2017-01-01

    Objective For lung cancer complicated with sarcoidosis, there are no exact features that indicate whether lymphadenopathy is metastatic. This makes the validity of surgery uncertain for clinicians. The aim of this study was to clarify the clinical features of pulmonary sarcoidosis complicated by lung cancer, especially from the viewpoint of evaluating lymphadenopathy. Methods We retrospectively reviewed medical records from 2004 to 2013 at our institution, and 18 patients who were diagnosed with sarcoidosis and lung cancer were thus found to be eligible. We investigated the relationship between the clinical and pathological findings of their swollen lymph nodes. Results Of 18 patients, 11 conducted surgery, and the postoperative pathological evaluation of swollen lymph nodes was done in 8 of the patients. Postoperative N factor in all these patients was 0, even though lymphatic metastasis had been suspected preoperatively because of the unbalanced distribution of lymphadenopathy or the accumulation of fluorodeoxyglucose. Conclusion In patients with lung cancer complicated by sarcoidosis, the clinical assessment of the state of lymphadenopathy is difficult to make. However, as many of them tend to be benign, we suggest that surgical resection should be considered for a complete cure in the absence of any remote metastasis.

  14. Systemic sarcoidosis complicated of acute renal failure: about 12 cases.

    Science.gov (United States)

    Mahfoudhi, Madiha; Mamlouk, Habiba; Turki, Sami; Kheder, Adel

    2015-01-01

    The sarcoidosis is a systemic granulomatosis affecting most frequently the lungs and the mediastinum. An acute renal failure reveals exceptionally this disease. It's a retrospective study implicating 12 cases of sarcoidosis complicated of acute renal failure. The aim of this study is to determine epidemiological, clinical, biological and histological profile in these cases and then to indicate the interest to consider the diagnosis of sarcoidosis in cases of unexplained renal failure. Extra-renal complications, therapeutic modalities and the outcome were determined in all patients. Our series involved 12 women with an average age of 40 years. Biological investigations showed an abnormal normocalcemia in 7 cases, a hypercalcemia in 5 cases, a hypercalciuria in 10 cases and polyclonal hypergammaglobulinemia in 7 cases. An acute renal failure was found in all patients with a median creatinin of 520 umol/L. For all patients, the renal echography was normal however, the kidney biopsy showed tubulo-interstitial nephritis. The extra-renal signs highlighting pulmonary interstitial syndrome in 5 cases, a sicca syndrome in 4 cases, mediastinal lymph nodes in 2 cases, a lymphocytic alveolitis in 3 cases, an anterior granulomatous uveitis in 2 cases and a polyarthritis in 5 cases. Five patients benefited of hemodialysis. The treatment consisted of corticosteroid in all cases. The follow up was marked by complete resolution of clinical and biological signs. The diagnosis of renal sarcoidosis must be done quickly to prevent renal failure.

  15. Multifactorial Etiology Pulmonary Hypertension in a Patient with Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Barreto Ana Terra Fonseca

    2016-01-01

    Full Text Available Differential diagnosis between pre- and postcapillary pulmonary hypertension (PH in patients with diastolic heart failure (DHF is a challenge in clinical practice. The presence of PH is implicated in worse prognosis in patients with this disease. This case report approaches the process of investigation of pulmonary hypertension in adult patient with DHF, double mitral lesion, and sarcoidosis with poor clinical outcome.

  16. Quantiferon test for tuberculosis screening in sarcoidosis patients

    DEFF Research Database (Denmark)

    Milman, Nils; Søborg, Bolette; Svendsen, Claus Bo

    2011-01-01

    Tumour necrosis factor-alpha (TNF-α) inhibitors have been introduced in the treatment of refractory sarcoidosis. These biologics may reactivate latent tuberculosis infection (LTBI). Despite its known limitations, the tuberculin skin test (TST) is currently used for the diagnosis of LTBI in Danish...

  17. Systemic sarcoidosis with necrobiosis lipoidica-like scalp lesions

    DEFF Research Database (Denmark)

    Andersen, Klaus Ejner

    1977-01-01

    A 78-year-old woman is described, having systemic sarcoidosis for 15 years, involving the lungs, lymph nodes and the skin. Over the last 6 years she developed a progressive cicatricial alopecia with histologic changes of the granulomatous type of necrobiosis lipoidica surrounded by sarcoid...

  18. Sarcoidosis and multiple myeloma: Concurrent presentation of an unusual association

    Directory of Open Access Journals (Sweden)

    Vidya Nair

    2016-01-01

    Full Text Available Literature on concurrent association of sarcoidosis with lymphoproliferative malignancies other than lymphoma e.g. multiple myeloma is meager. The rarity of the situation prompted us to report this patient who was a 51-year-old woman with a 2-years history of breathlessness, cough with expectoration, chest pain and backache. Initial evaluation revealed mild anemia, increased alkaline phosphatase with chest skiagram showing both lower zone non homogenous opacities with calcified hilar lymph nodes. CECT chest showed mediastinal with bilateral hilar lymphadenopathy, parenchymal fibrosis, traction bronchiectasis, ground glass opacities, septal and peribronchovascular thickening affecting mid and lower lung zones bilaterally. MRI Dorsolumbar spine was suggestive of marrow infiltrative disorder. EBUS FNA of intrathoracic nodes, EBB and TBLB confirmed sarcoidosis. PET CT revealed hyper metabolic activity in lung, multiple lymph nodes and lytic bone lesions. Serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin IgG kappa type. Bone marrow biopsy revealed an increase in plasma cells (15%, but no granulomas. Diagnosis of Indolent or multiple myeloma with sarcoidosis was established. 12 cases of sarcoidosis and multiple myeloma have been reported in literature, and mostly preceding the onset of multiple myeloma by many years, in our case both were diagnosed concurrently.

  19. Effectiveness of infliximab in refractory FDG PET-positive sarcoidosis

    NARCIS (Netherlands)

    Vorselaars, Adriane D M; Crommelin, Heleen A; Deneer, Vera H M; Meek, Bob; Claessen, Anke M E; Keijsers, Ruth G M; van Moorsel, Coline H M; Grutters, JC

    Inconclusive evidence for the efficacy of infliximab in sarcoidosis hinders the global use of this potentially beneficial drug. To study infliximab efficacy in a clinical setting, we performed a prospective open-label trial in patients refractory to conventional treatment. Patients (n=56) received

  20. Resistant thyrotoxicosis: A case of sarcoidosis of thyroid

    Directory of Open Access Journals (Sweden)

    Uday Yanamandra

    2013-01-01

    Full Text Available Autoimmune endocrinopathies and, less commonly, thyroid autoimmune disease have been reported in patients with sarcoidosis. Similarities exist in the pathogenesis of these two conditions. Concomitant sarcoidosis in the thyroid gland in patients with Graves′ disease may contribute to the resistance to antithyroid drugs and radioiodine therapy. We present the clinical, laboratory, imaging, and pathologic findings of a patient with Graves′ disease who was unresponsive to medical management. This 37-year-old man presented with thyrotoxicosis. Thyroid hormone assays and 99m Technitium findings were consistent with Graves′ disease. He was also found to have hilar lymphadenopathy. Patient failed to achieve remission with high doses of antithyroid drugs and 2 sessions of radioiodine ablative therapy.Histopathology of lymph nodesdisclosed noncaseating granulomas, consistent with sarcoidosis. Patient′s thyrotoxicosis subsided only following steroid administration. The histopathology of the thyroid gland on aspiration and the subsidence of symptoms with steroids reiterate the possibility of thyroid sarcoidosis. This diagnosis needs biopsy for confirmation, which our patient didn′t consent for.

  1. Subcutaneous Sarcoidosis in a Nigerian female | Yakubu | Annals of ...

    African Journals Online (AJOL)

    There has not been any report of isolated cutaneous sarcoidosis from our center. We present a patient who was reviewed with an isolated ganglion-like subcutaneous swelling on the ankle region. Excision biopsy revealed a non caseating granulomatous lesion. Systemic evaluation was not remarkable and the lesion was ...

  2. Elevated Aminotransaminases As the First Manifestation of Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Georges Nawfal

    2009-01-01

    Full Text Available Sarcoidose is a rare disease in children. The aminotransaminase level is often normal to moderately elevated (2 to 3 folds of the normal level. We report the case of a child who presented an aminotransaminase level that was 10 times the normal level, as the first manifestation of sarcoidosis.

  3. Identification of Immune-Relevant Factors Conferring Sarcoidosis Genetic Risk

    Science.gov (United States)

    Fischer, Annegret; Ellinghaus, David; Nutsua, Marcel; Hofmann, Sylvia; Montgomery, Courtney G.; Iannuzzi, Michael C.; Rybicki, Benjamin A.; Petrek, Martin; Mrazek, Frantisek; Pabst, Stefan; Grohé, Christian; Grunewald, Johan; Ronninger, Marcus; Eklund, Anders; Padyukov, Leonid; Mihailovic-Vucinic, Violeta; Jovanovic, Dragana; Sterclova, Martina; Homolka, Jiri; Nöthen, Markus M.; Herms, Stefan; Gieger, Christian; Strauch, Konstantin; Winkelmann, Juliane; Boehm, Bernhard O.; Brand, Stephan; Büning, Carsten; Schürmann, Manfred; Ellinghaus, Eva; Baurecht, Hansjörg; Lieb, Wolfgang; Nebel, Almut; Müller-Quernheim, Joachim; Franke, Andre

    2015-01-01

    Rationale: Genetic variation plays a significant role in the etiology of sarcoidosis. However, only a small fraction of its heritability has been explained so far. Objectives: To define further genetic risk loci for sarcoidosis, we used the Immunochip for a candidate gene association study of immune-associated loci. Methods: Altogether the study population comprised over 19,000 individuals. In a two-stage design, 1,726 German sarcoidosis cases and 5,482 control subjects were genotyped for 128,705 single-nucleotide polymorphisms using the Illumina Immunochip for the screening step. The remaining 3,955 cases, 7,514 control subjects, and 684 parents of affected offspring were used for validation and replication of 44 candidate and two established risk single-nucleotide polymorphisms. Measurements and Main Results: Four novel susceptibility loci were identified with genome-wide significance in the European case-control populations, located on chromosomes 12q24.12 (rs653178; ATXN2/SH2B3), 5q33.3 (rs4921492; IL12B), 4q24 (rs223498; MANBA/NFKB1), and 2q33.2 (rs6748088; FAM117B). We further defined three independent association signals in the HLA region with genome-wide significance, peaking in the BTNL2 promoter region (rs5007259), at HLA-B (rs4143332/HLA-B*0801) and at HLA-DPB1 (rs9277542), and found another novel independent signal near IL23R (rs12069782) on chromosome 1p31.3. Conclusions: Functional predictions and protein network analyses suggest a prominent role of the drug-targetable IL23/Th17 signaling pathway in the genetic etiology of sarcoidosis. Our findings reveal a substantial genetic overlap of sarcoidosis with diverse immune-mediated inflammatory disorders, which could be of relevance for the clinical application of modern therapeutics PMID:26051272

  4. Sarcoidosis in the eastern region of Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Al-Khouzaie Thamer

    2011-01-01

    Full Text Available Aim: To review a general hospital′s experience with sarcoidosis and the clinical pattern of the disease among Saudis. Methods: A retrospective file review was carried out on all patients with a proven diagnosis of sarcoidosis in a general hospital in Eastern Saudi Arabia over a period of 11 years (1998-2008. Results: Sixty-nine patients, of whom 33 cases were included in the analyses, were diagnosed to have sarcoidosis during the study period. There were 18 females and 15 males. The mean age was 44.5 years (SD 17. The most common presentations were cough (48%, dyspnea (21%, joint pain (18%, splenomegaly (12%, hepatomegaly (9%, and lymphadenopathy (5%. The biochemical analysis showed elevated calcium levels in 6% and elevated angiotensin converting enzyme (ACE in 14 (46.7%. The tuberculin skin test was negative in all tested patients (n = 29 except one patient. The patients were classified using the modified Scadding classification system. None of the patients was in stage 0, 39.4% were in stage 1, 45% were in stage 2 and 15% were in stage 3.. The diagnosis in all patients was proven histologically. The outcome was favorable in most patients (85%, and in 6% of the patients, the course was chronic and progressive, although 66% received active treatment. Conclusion: Sarcoidosis does occur in native Saudis. The clinical presentation of these patients was similar to the western pattern of disease with some differences such as relative lack of cardiac, eye, parotid, and central nervous system involvement. The rarity of cardiac and central nervous system involvement was comparable with other Middle Eastern studies. Sarcoidosis, though rare in our community, should still be considered in the differential diagnosis of patients with the typical presentation after excluding tuberculosis.

  5. GSTT1 and GSTM1 gene polymorphisims in sarcoidosis.

    Science.gov (United States)

    Coskun, Funda; Karkucak, Mutlu; Yilmaz, Dilber; Yakut, Tahsin; Uzaslan, Esra

    2016-10-07

    Sarcoidosis is a granulomatous disease of unknown cause, which affects all systems, especially the lungs and the lymphatic system. Genetic and environmental factors are held accountable for the etiology. Based on the general opinion, sarcoidosis develops after exposure to a specific environmental agent by genetically susceptible individuals.  The present study aimed to evaluate the disease susceptibility of the GSTT1 and GSTM1 gene polymorphisms in the patients with sarcoidosis. The present study included 78 patients; 38 patients with histopathologically verified sarcoidosis and 40 control subjects. Multiplex PCR method was used to determine the GSTT1 and GSTM1 gene polymorphisms. The genotype was determined based on the bands formed in the agarose gel electrophoresis. The statistical analysis was done using the chi-square test. The positive/negative genotype rates were 79%/21% and 53%/47%, respectively in the case group for the GSTT1 and GSTM1 gene polymorphisms, whereas the positive/negative genotype rates were 77%/23% and 55%/45% in the control group. There was no statistically significant difference in the positive and negative genotypes compared with the case group and the control group for the GSTT1 and GSTM1 gene polymorphisms (p > 0.05). The results from the present study suggest that there is not any association with the control group for the disease susceptibility of the GSTT1 and GSTM1 gene polymorphisms in patients with sarcoidosis, and this result should be supported by large-scale studies because of the limited number of cases in the present study.

  6. Clinical presentation of sarcoidosis and diagnostic work-up.

    Science.gov (United States)

    Valeyre, Dominique; Bernaudin, Jean-François; Uzunhan, Yurdagul; Kambouchner, Marianne; Brillet, Pierre-Yves; Soussan, Michael; Nunes, Hilario

    2014-06-01

    Sarcoidosis is a systemic disease of unknown cause characterized by the formation of immune granulomas which most often involve the lung and the lymphatic system. Sarcoidosis may encompass numerous different clinical presentations. Typical presentations often prompt a rapid diagnosis while in 25 to 50% of cases, diverse and less typical presentations may lead to delayed diagnosis. The mediastinopulmonary sphere is involved in 85 to 95% of cases, associated with extrapulmonary localizations in half of cases while extrapulmonary localizations without lung involvement may be seen in 5 to 15% of cases. Bilateral hilar lymphadenopathy is the most typical sign at chest radiography. Computed tomography (CT) is essential face for atypical manifestations of the disease to avoid confusion with differential diagnoses and, sometimes, comorbidities. CT typically evidences diffuse pulmonary perilymphatic micronodules, with a perilobular and fissural distribution and upper and posterior predominance, even when an atypical CT pattern is predominant. CT allows deciphering pulmonary lesions in cases of pulmonary fibrosis, pulmonary hypertension, and airflow limitation. Pulmonary function tests generally correlate with the overall disease process. Forced vital capacity is the simplest and most accurate parameter to reflect the impact of pulmonary sarcoidosis. Cardiopulmonary exercise testing helps in understanding the mechanism behind dyspnea of uncertain origin. Endoscopic transbronchial needle aspiration is an extra tool to support diagnosis in addition to more classical biopsy means. Bronchoalveolar lavage (BAL) may be used for individual patients while it is not really decisive for the diagnosis of sarcoidosis for most patients. Diagnosis relies on compatible clinical and radiological presentation, evidence of noncaseating granulomas and exclusion of other diseases with a similar presentation or histology. The probability of diagnosis at presentation is variable from case to case

  7. Tuberculosis lymphadenitis in a well managed case of sarcoidosis.

    Science.gov (United States)

    Mohapatra, P R; Garg, Kranti; Singhal, Niti; Aggarwal, D; Gupta, R; Khurana, A; Janmeja, A K

    2013-01-01

    Differentiation between tuberculosis (TB) and sarcoidoisis is sometimes extremely difficult. Sequential occurrence of sarcoidosis and TB in the same patient is uncommon. We present the case of a young man, with a proven diagnosis of sarcoidosis who later developed TB after completion of treatment for sarcoidosis. A 32-year-old male patient presented with low-grade fever since two months. Physical examination revealed cervical lymphadenopathy. Initial fine needle aspiration cytology (FNAC) of the cervical lymph node was suggestive of granulomatous inflammation; the chest radiograph was normal. Repeat FNAC from the same lymph node was suggestive of reactive lymphoid hyperplasia. The patient was treated with antibiotics and followed-up. He again presented with persistence of fever and lymphadenopathy and blurring of vision. Ophthalmological examination revealed uveitis, possibly due to a granulomatous cause. His repeat Mantoux test again was non-reactive; serum angiotensin converting enzyme (ACE) levels were raised. This time an excision biopsy of the lymph node was done which revealed discrete, non-caseating, reticulin rich granulomatous inflammation suggestive of sarcoidosis. The patient was treated with oral prednisolone and imporved symptomatically. Subsequently, nearly nine months after completion of corticosteroid treatment, he presented with low-grade, intermittent fever and a lymph node enlargement in the right parotid region. FNAC from this lymph node showed caseating granulomatous inflammation and the stain for acid-fast bacilli was positive. He was treated with Category I DOTS under the Revised National Tuberculosis Control Programme and improved significantly. The present case highlights the need for further research into the aetiology of TB and sarcoidosis.

  8. Symptomatic vertebral hemangiomas during pregnancy.

    Science.gov (United States)

    Moles, Alexis; Hamel, Olivier; Perret, Christophe; Bord, Eric; Robert, Roger; Buffenoir, Kevin

    2014-05-01

    Symptomatic vertebral hemangiomas during pregnancy are rare, as only 27 cases have been reported in the literature since 1948. However, symptomatic vertebral hemangiomas can be responsible for spinal cord compression, in which case they constitute a medical emergency, which raises management difficulties in the context of pregnancy. Pregnancy is a known factor responsible for deterioration of these vascular tumors. In this paper, the authors report 2 clinical cases of symptomatic vertebral hemangiomas during pregnancy, including 1 case of spontaneous fracture that has never been previously reported in the literature. The authors then present a brief review of the literature to discuss emergency management of this condition. The first case was a 28-year-old woman at 35 weeks of gestation, who presented with paraparesis. Spinal cord MRI demonstrated a vertebral hemangioma invading the body and posterior arch of T-3 with posterior epidural extension. Laminectomy and vertebroplasty were performed after cesarean section, allowing neurological recovery. The second case involved a 35-year-old woman who presented with spontaneous fracture of T-7 at 36 weeks of gestation, revealing a vertebral hemangioma with no neurological deficit, but it was responsible for pain and local instability. Treatment consisted of postpartum posterior interbody fusion. With a clinical and radiological follow-up of 2 years, no complications and no modification of the hemangiomas were observed. A review of the literature reveals discordant management of these rare cases, which is why the treatment course must be decided by a multidisciplinary team as a function of fetal gestational age and maternal neurological features.

  9. A prospective study evaluating the presence of Rickettsia in Danish patients with sarcoidosis

    DEFF Research Database (Denmark)

    Svendsen, Claus Bo; Milman, Nils; Nielsen, Henrik Winther

    2009-01-01

    Rickettsia helvetica has previously been proposed as an aetiological agent in sarcoidosis. The purpose of the present study was to detect possible signs of Rickettsia infection in a Danish population of patients with sarcoidosis. Twenty-six patients with newly diagnosed sarcoidosis were prospecti......Rickettsia helvetica has previously been proposed as an aetiological agent in sarcoidosis. The purpose of the present study was to detect possible signs of Rickettsia infection in a Danish population of patients with sarcoidosis. Twenty-six patients with newly diagnosed sarcoidosis were...... prospectively enrolled in the study. The diagnosis was confirmed by biopsy in 18 and by clinical characteristics in 8 patients; 11 patients with different non-sarcoid lung diseases were recruited as controls. We obtained information regarding tick exposure and sarcoid disease manifestations by a structured...

  10. Peripheral blood gene expression as a novel genomic biomarker in complicated sarcoidosis.

    Directory of Open Access Journals (Sweden)

    Tong Zhou

    Full Text Available Sarcoidosis, a systemic granulomatous syndrome invariably affecting the lung, typically spontaneously remits but in ~20% of cases progresses with severe lung dysfunction or cardiac and neurologic involvement (complicated sarcoidosis. Unfortunately, current biomarkers fail to distinguish patients with remitting (uncomplicated sarcoidosis from other fibrotic lung disorders, and fail to identify individuals at risk for complicated sarcoidosis. We utilized genome-wide peripheral blood gene expression analysis to identify a 20-gene sarcoidosis biomarker signature distinguishing sarcoidosis (n = 39 from healthy controls (n = 35, 86% classification accuracy and which served as a molecular signature for complicated sarcoidosis (n = 17. As aberrancies in T cell receptor (TCR signaling, JAK-STAT (JS signaling, and cytokine-cytokine receptor (CCR signaling are implicated in sarcoidosis pathogenesis, a 31-gene signature comprised of T cell signaling pathway genes associated with sarcoidosis (TCR/JS/CCR was compared to the unbiased 20-gene biomarker signature but proved inferior in prediction accuracy in distinguishing complicated from uncomplicated sarcoidosis. Additional validation strategies included significant association of single nucleotide polymorphisms (SNPs in signature genes with sarcoidosis susceptibility and severity (unbiased signature genes - CX3CR1, FKBP1A, NOG, RBM12B, SENS3, TSHZ2; T cell/JAK-STAT pathway genes such as AKT3, CBLB, DLG1, IFNG, IL2RA, IL7R, ITK, JUN, MALT1, NFATC2, PLCG1, SPRED1. In summary, this validated peripheral blood molecular gene signature appears to be a valuable biomarker in identifying cases with sarcoidoisis and predicting risk for complicated sarcoidosis.

  11. Peripheral Blood Gene Expression as a Novel Genomic Biomarker in Complicated Sarcoidosis

    Science.gov (United States)

    Sweiss, Nadera J.; Chen, Edward S.; Moller, David R.; Knox, Kenneth S.; Ma, Shwu-Fan; Wade, Michael S.; Noth, Imre; Machado, Roberto F.; Garcia, Joe G. N.

    2012-01-01

    Sarcoidosis, a systemic granulomatous syndrome invariably affecting the lung, typically spontaneously remits but in ∼20% of cases progresses with severe lung dysfunction or cardiac and neurologic involvement (complicated sarcoidosis). Unfortunately, current biomarkers fail to distinguish patients with remitting (uncomplicated) sarcoidosis from other fibrotic lung disorders, and fail to identify individuals at risk for complicated sarcoidosis. We utilized genome-wide peripheral blood gene expression analysis to identify a 20-gene sarcoidosis biomarker signature distinguishing sarcoidosis (n = 39) from healthy controls (n = 35, 86% classification accuracy) and which served as a molecular signature for complicated sarcoidosis (n = 17). As aberrancies in T cell receptor (TCR) signaling, JAK-STAT (JS) signaling, and cytokine-cytokine receptor (CCR) signaling are implicated in sarcoidosis pathogenesis, a 31-gene signature comprised of T cell signaling pathway genes associated with sarcoidosis (TCR/JS/CCR) was compared to the unbiased 20-gene biomarker signature but proved inferior in prediction accuracy in distinguishing complicated from uncomplicated sarcoidosis. Additional validation strategies included significant association of single nucleotide polymorphisms (SNPs) in signature genes with sarcoidosis susceptibility and severity (unbiased signature genes - CX3CR1, FKBP1A, NOG, RBM12B, SENS3, TSHZ2; T cell/JAK-STAT pathway genes such as AKT3, CBLB, DLG1, IFNG, IL2RA, IL7R, ITK, JUN, MALT1, NFATC2, PLCG1, SPRED1). In summary, this validated peripheral blood molecular gene signature appears to be a valuable biomarker in identifying cases with sarcoidoisis and predicting risk for complicated sarcoidosis. PMID:22984568

  12. Sarcoidosis in gastric cancer at the time of diagnosis: A case report

    OpenAIRE

    Jiao, Yang; NING, JIE; ZHAO, WEN-DI; Li, Yan-Li; WU, HONG-YANG; GU, KANG-SHENG

    2015-01-01

    Sarcoidosis is a multisystemic inflammatory disease that commonly affects the lungs and lymphatic system and is characterized by the formation of non-caseating granulomas. Although the association between sarcoidosis and malignant diseases has been well described, it remains controversial whether this association is merely a coincidence or the consequence of a common pathophysiological mechanism. The present study reports a rare case of sarcoidosis that was present in a patient with gastric c...

  13. Sarcoidosis with involvement of the paranasal sinuses - a retrospective analysis of 12 biopsy-proven cases

    Science.gov (United States)

    2013-01-01

    Background Extrapulmonary involvement by sarcoidosis is observed in about 30–40% of patients with sarcoidosis. Little is known about the frequency and clinical characteristics of sinonasal sarcoidosis. Methods We retrospectively analyzed 12 cases of biopsy-proven sinonasal sarcoidosis. Patients were identified from a patient population of 1360 patients with sarcoidosis at the Outpatient Clinic for Sarcoidosis and Rare Lung Diseases at LungClinic Grosshansdorf, a tertiary care hospital for respiratory medicine. Results The most frequent signs and symptoms were nasal polyps (4 cases), epistaxis (3 cases), nasal crusts (8 cases) and anosmia (5 cases). Pulmonary sarcoidosis of the patients was staged as stage I (n = 1) and stage II (n = 11) on chest radiographs. Spirometry was normal in 11 patients. 7 patients had a diffusion capacity of the lung for carbon monoxide of less than 90% of predicted. Other organs were affected in 8 patients. All patients received systemic corticosteroid treatment and most patients received topical steroids. 5 patients received steroid sparing agents. Repeated sinus surgery had to be performed in 4 patients. Conclusions Sinonasal involvement is a rare disease manifestation of sarcoidosis with a frequency slightly lower than 1% in our patient population. The clinical course of sinonasal sarcoidosis can be complicated by relapse despite systemic immunosuppressive treatment and repeated sinus surgery. PMID:24070015

  14. Pulmonology meets rheumatology in sarcoidosis: a review on the therapeutic approach.

    Science.gov (United States)

    Drent, Marjolein; Cremers, Johanna P; Jansen, Tim L

    2014-05-01

    Sarcoidosis is a systemic disease characterized by the formation of granulomas in various organs, mainly lungs and lymphatic system. Joint, muscle, and bone involvement is also rather common. Recent studies on its pathogenesis and therapeutic management are reviewed here. The pathogenesis of sarcoidosis is not fully elucidated. An exaggerated granulomatous reaction after exposure to unidentified antigens in genetically susceptible individuals evokes a clinical situation which we call sarcoidosis. No firm guidelines exist on whether, when, and how treatment should be started. Treatment is dependent on the presentation and the distribution, extensiveness and severity of sarcoidosis. Treatment of Löfgren's triad-related symptoms starts with NSAIDs; in other more extensive manifestations of sarcoidosis, the initiating dosage of glucocorticosteroids is approximately 20 mg daily. In terms of evidence-based treatment for sarcoidosis, only a few randomized controlled trials have been done. There is no cure for chronic sarcoidosis, and treatment only changes the granulomatous process and its clinical consequences. Identified associations of certain polymorphisms with severity of the disease and treatment response suggest future research questions as well as finding the cause(s) of sarcoidosis, and the elucidation of relevant biomarkers and new efficient treatments. Between 20 and 70% of patients need systemic therapy. The increased awareness of long-term side-effects of glucocorticosteroids and the emergence of new drugs have changed the treatment of sarcoidosis. Alternative or additional options to corticosteroids should be assessed.

  15. Endometrial Cancer with Sarcoidosis in Regional Lymph Nodes: A Case Report

    Directory of Open Access Journals (Sweden)

    Satoshi Tamauchi

    2015-10-01

    Full Text Available Sarcoidosis is a chronic, multisystemic disease commonly affecting the lungs and lymphatic system and is characterized by the formation of noncaseating granulomas. Although several reports are available on cases developing both sarcoidosis and cancer metachronously, cases of simultaneous diagnosis of these diseases have rarely been reported. A 67-year-old woman diagnosed with endometrial cancer had developed systemic lymph node swelling, including bilateral hilar, paraaortic, and a few pelvic lymph nodes, as observed on preoperative imaging. During surgery, frozen sections of a paraaortic lymph node were examined, revealing noncaseating granulomas compatible with sarcoidosis. Next, modified radical hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy were performed. Postoperative pathological analysis revealed endometrioid adenocarcinoma of the uterus, and no metastasis but noncaseating granulomas were detected in the resected lymph nodes. Postoperatively, we identified cutaneous sarcoidosis and uveitis in the presence of a tuberculin-negative test. On the basis of these findings, we diagnosed the patients with endometrial cancer complicated by sarcoidosis. She underwent adjuvant chemotherapy, and at the 1-year follow-up, the lymph node swelling due to sarcoidosis was stable, and no recurrence of the cancer was observed. This turned out to be a case of early endometrial cancer mimicking advanced cancer by sarcoidosis. Histological confirmation and additional examination for sarcoidosis are necessary in cancer patients suspected of sarcoidosis.

  16. Endometrial Cancer with Sarcoidosis in Regional Lymph Nodes: A Case Report.

    Science.gov (United States)

    Tamauchi, Satoshi; Shimomura, Yuji; Hayakawa, Hiromi

    2015-01-01

    Sarcoidosis is a chronic, multisystemic disease commonly affecting the lungs and lymphatic system and is characterized by the formation of noncaseating granulomas. Although several reports are available on cases developing both sarcoidosis and cancer metachronously, cases of simultaneous diagnosis of these diseases have rarely been reported. A 67-year-old woman diagnosed with endometrial cancer had developed systemic lymph node swelling, including bilateral hilar, paraaortic, and a few pelvic lymph nodes, as observed on preoperative imaging. During surgery, frozen sections of a paraaortic lymph node were examined, revealing noncaseating granulomas compatible with sarcoidosis. Next, modified radical hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy were performed. Postoperative pathological analysis revealed endometrioid adenocarcinoma of the uterus, and no metastasis but noncaseating granulomas were detected in the resected lymph nodes. Postoperatively, we identified cutaneous sarcoidosis and uveitis in the presence of a tuberculin-negative test. On the basis of these findings, we diagnosed the patients with endometrial cancer complicated by sarcoidosis. She underwent adjuvant chemotherapy, and at the 1-year follow-up, the lymph node swelling due to sarcoidosis was stable, and no recurrence of the cancer was observed. This turned out to be a case of early endometrial cancer mimicking advanced cancer by sarcoidosis. Histological confirmation and additional examination for sarcoidosis are necessary in cancer patients suspected of sarcoidosis.

  17. Fulminant musculoskeletal and neurologic sarcoidosis: case report and literature update

    Energy Technology Data Exchange (ETDEWEB)

    Sweeney, Ashley; Hammer, Richard; Evenski, Andrea; Crim, Julia [University of Missouri at Columbia, Columbia, MO (United States)

    2016-11-15

    We report a case of fulminant sarcoidosis in a 28-year-old man presenting with skin nodules, multifocal small and large joint arthralgias, and blurred vision. Characteristic bone, soft tissue, articular, and CNS findings were evident on multimodality imaging. Bony abnormalities included near-complete destruction of a distal phalanx, ''lace-like'' lucent lesions, erosive arthritis, lytic lesions with and without sclerotic margins, and bone marrow replacement visible only on MRI. The extent of bony disease at time of presentation was unusual. We review the widely varying reported prevalence of imaging findings of bony sarcoidosis in the literature, and discuss reasons for this variability. We found that musculoskeletal findings at US and MRI were less specific than radiographic and CT findings, but were useful in quantifying extent of disease. (orig.)

  18. [Sudden cardiac death due to sarcoidosis. Case report].

    Science.gov (United States)

    Sejben, István; Som, Zoltán; Cserni, Gábor

    2017-07-01

    Sarcoidosis is a systemic granulomatous disease of unknown aetiology, which is characterized by bilateral hilar lymphadenopathy and pulmonary disease. Clinically detected cardiac involvement occurs in 5% of sarcoid patients, although cardiac manifestations are discovered in 25% of the cases at autopsy. Sarcoid heart disease frequently causes atrioventricular block. The authors present the case of a 44-year-old man with bradycardia. On admission, second degree Mobitz II, then third degree atrioventricular block was diagnosed. Coronarography showed normal coronary arteries. 2.5 years following artificial Biotronik Entovis DR type pacemaker implantation, sudden cardiac death occurred. Autopsy revealed sarcoidosis with cardiac, pulmonary, splenic, renal and lymph node involvement. In case of young or middle-aged patients with atrioventricular block, it is best to search for other causes if the most common coronary origin can be excluded. Orv Hetil. 2017; 158(27): 1067-1070.

  19. Magnetic resonance imaging of lacrimal gland in sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Matsunaga, Nobuhiko; Mizoguchi, Takanori; Tashiro, Junko; Amemiya, Tsugio; Takeda, Hiroyuki (Nagasaki Univ. (Japan). School of Medicine)

    1991-04-01

    We evaluated the magnetic resonance imaging (MRI) of the lacrimal gland in 18 patients with sarcoidosis. All the cases were imaged by conventional spin-echo technique using a 1.5 T super-conductive MR system by General Electric. We identified enlargement of the lacrimal gland in 7 patients. The signal intensity of lacrimal glands in these cases were characterized by isointensity on T1-weighted and proton density image and by high intensity on T2-weighted images. Hilar lymphadenopathy was present in 5 of them. Other ocular disorders related to sarcoidosis were present in 15 patients. Enlargement of the lacrimal gland was correlated with retinal vasculitis and not with hilar lymphadenopathy. (author).

  20. Sarcoidosis of the lacrimal gland as a first manifestation.

    Science.gov (United States)

    Peralta-Gómez, M Y; Ávila-Ocampo, K A; Huerta-Velázquez, S; Rivera-Salgado, M I

    2017-06-11

    The first manifestation of sarcoidosis is usually at the pulmonary level. The case is described of a 40-year-old female patient, who presented with an increased volume of the lacrimal gland and mechanical ptosis of upper left eyelid as the first expression of this disease. The diagnosis of systemic sarcoidosis with primary presentation of the lacrimal gland was made after performing several immunological studies with negative results, imaging studies, and taking of glandular and lymph node biopsies. A favourable response was achieved with oral methotrexate treatment. Copyright © 2017 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  1. Cranial Nerve Palsies: Sarcoidosis to Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Fawad Aslam

    2013-01-01

    Full Text Available Cranial palsies are a very rare feature of SLE. Similarly, peripheral sensory-motor axonal neuropathy is very uncommon in SLE. The combination of the two as the presenting symptoms of SLE is a diagnostic challenge particularly in an elderly male patient with a known diagnosis of sarcoidosis. This case serves to highlight the diagnostic considerations in such a patient. The lack of response to standard therapy and the presence of subtle clues like anemia, proteinuria, and mild serositis should prompt the physician to look for alternate diagnoses. The potential association of SLE and sarcoidosis is also discussed. SLE can be present in elderly male patients with cranial and peripheral neuropathy.

  2. Sarcoidosis with Major Airway, Vascular and Nerve Compromise

    Directory of Open Access Journals (Sweden)

    Hiroshi Sekiguchi

    2013-01-01

    Full Text Available The present report describes a 60-year-old Caucasian woman who presented with progressive dyspnea, cough and wheeze. A computed tomography scan of the chest showed innumerable bilateral inflammatory pulmonary nodules with bronchovascular distribution and a mediastinal and hilar infiltrative process with calcified lymphadenopathy leading to narrowing of lobar bronchi and pulmonary arteries. An echocardiogram revealed pulmonary hypertension. Bronchoscopy showed left vocal cord paralysis and significant narrowing of the bilateral bronchi with mucosal thickening and multiple nodules. Transbronchial biopsy was compatible with sarcoidosis. Despite balloon angioplasty of the left lower lobe and pulmonary artery, and medical therapy with oral corticosteroids, her symptoms did not significantly improve. To the authors’ knowledge, the present report describes the first case of pulmonary sarcoidosis resulting in major airway, vascular and nerve compromise due to compressive lymphadenopathy and suspected concurrent granulomatous infiltration. Its presentation mimicked idiopathic mediastinal fibrosis.

  3. Economic burden of sarcoidosis in a commercially-insured population in the United States.

    Science.gov (United States)

    Rice, J Bradford; White, Alan; Lopez, Andrea; Conway, Alexandra; Wagh, Aneesha; Nelson, Winnie W; Philbin, Michael; Wan, George J

    2017-10-01

    Sarcoidosis is a multi-system inflammatory disorder characterized by the presence of non-caseating granulomas in involved organs. Patients with sarcoidosis have a reduced quality-of-life and are at an increased risk for several comorbidities. Little is known about the direct and indirect cost of sarcoidosis following the initial diagnosis. To provide an estimate of the healthcare resource utilization (HCRU) and costs borne by commercial payers for sarcoidosis patients in the US. Patients with a first diagnosis of sarcoidosis between January 1, 1998 and March 31, 2015 ("index date") were selected from a de-identified privately-insured administrative claims database. Sarcoidosis patients were required to have continuous health plan enrollment 12 months prior to and following their index dates. Propensity-score (1:1) matching of sarcoidosis patients with non-sarcoidosis controls was carried out based on a logistic regression of baseline characteristics. Burden of HCRU and work loss (disability days and medically-related absenteeism) were compared between the matched groups over the 12-month period following the index date ("outcome period"). A total of 7,119 sarcoidosis patients who met the selection criteria were matched with a control. Overall, commercial payers incurred $19,714 in mean total annual healthcare costs per sarcoidosis patient. The principle cost drivers were outpatient visits ($9,050 2015 USD, 46%) and inpatient admissions ($6,398, 32%). Relative to controls, sarcoidosis patients had $5,190 (36%) higher total healthcare costs ($19,714 vs $14,524; p economic burden to payers in the first year following diagnosis.

  4. Sarcoidosis in an Italian province. Prevalence and environmental risk factors

    Science.gov (United States)

    Beghè, Deborah; Dall’Asta, Luca; Garavelli, Claudia; Pastorelli, Augusto Alberto; Muscarella, Marilena; Saccani, Gloria; Aiello, Marina; Corradi, Massimo; Stacchini, Paolo; Chetta, Alfredo; Bertorelli, Giuseppina

    2017-01-01

    Background and aim Sarcoidosis is a systemic granulomatous inflammatory disease whose causes are still unknown and for which epidemiological data are often discordant. The aim of our study is to investigate prevalence and spatial distribution of cases, and identify environmental exposures associated with sarcoidosis in an Italian province. Methods After georeferentiation of cases, the area under study was subdivided with respect to Municipality and Health Districts and to the altitude in order to identify zonal differences in prevalence. The bioaccumulation levels of 12 metals in lichen tissues were analyzed, in order to determine sources of air pollution. Finally, the analysis of the correlation between metals and between pickup stations was performed. Results 223 patients were identified (58.3% female and 41.7% male of total) and the mean age was 50.6±15.4 years (53.5±15.5 years for the females and 46.5±14.4 for the males). The mean prevalence was 49 per 100.000 individuals. However, we observed very heterogeneous prevalence in the area under study. The correlations among metals revealed different deposition patterns in lowland area respect to hilly and mountain areas. Conclusions The study highlights a high prevalence of sarcoidosis cases, characterized by a very inhomogeneous and patchy distribution with phenomena of local aggregation. Moreover, the bioaccumulation analysis was an effective method to identify the mineral particles that mostly contribute to air pollution in the different areas, but it was not sufficient to establish a clear correlation between the onset of sarcoidosis and environmental risk factors. PMID:28475583

  5. Development of a T7 Phage Display Library to Detect Sarcoidosis and Tuberculosis by a Panel of Novel Antigens

    Directory of Open Access Journals (Sweden)

    Harvinder Talwar

    2015-04-01

    Full Text Available Sarcoidosis is a granulomatous inflammatory disease, diagnosed through tissue biopsy of involved organs in the absence of other causes such as tuberculosis (TB. No specific serologic test is available to diagnose and differentiate sarcoidosis from TB. Using a high throughput method, we developed a T7 phage display cDNA library derived from mRNA isolated from bronchoalveolar lavage (BAL cells and leukocytes of sarcoidosis patients. This complex cDNA library was biopanned to obtain 1152 potential sarcoidosis antigens and a microarray was constructed to immunoscreen two different sets of sera from healthy controls and sarcoidosis. Meta-analysis identified 259 discriminating sarcoidosis antigens, and multivariate analysis identified 32 antigens with a sensitivity of 89% and a specificity of 83% to classify sarcoidosis from healthy controls. Additionally, interrogating the same microarray platform with sera from subjects with TB, we identified 50 clones that distinguish between TB, sarcoidosis and healthy controls. The top 10 sarcoidosis and TB specific clones were sequenced and homologies were searched in the public database revealing unique epitopes and mimotopes in each group. Here, we show for the first time that immunoscreenings of a library derived from sarcoidosis tissue differentiates between sarcoidosis and tuberculosis antigens. These novel biomarkers can improve diagnosis of sarcoidosis and TB, and may aid to develop or evaluate a TB vaccine.

  6. Anomalies in the dominant sarcoidosis paradigm justify its displacement.

    Science.gov (United States)

    Reich, Jerome M

    2017-04-01

    The prevailing paradigm defines sarcoidosis as a disease of unknown etiology characterized by a systemic noncaseating epithelioid granulomatous response (SGR). This formulation fails to account for the elusiveness of the etiological agent, the nature of the Kveim response, the paradox of cutaneous delayed type hypersensitivity anergy in a setting of intense immune response and the appearance of SGR, indistinguishable from sarcoidosis, in persons with lymphohematogenous and solid neoplasms and a variety of cellular immune deficiencies. Displacing this formulation with an evidence-based alternative in which the SGR is viewed as an etiologically diverse, primitive immunological fallback due to inefficient cellular immune processing eliminates these shortcomings, providing a unifying accounting for the puzzles and violations of expectations associated with the prevailing paradigm. Its clinical import resides in the reported annual three-percent increase in sarcoidosis mortality in the past two decades (Swigris et al., 2011), which may be attributable, in part, to unneeded suppressive treatment. Copyright © 2017 Elsevier GmbH. All rights reserved.

  7. Where does the antigen of cutaneous sarcoidosis come from?

    Science.gov (United States)

    Kurata, Atsushi; Terado, Yuichi; Izumi, Miki; Fujioka, Yasunori; Franke, Folker Ernst

    2010-02-01

    The antigen pathway of cutaneous sarcoidosis remains obscure. We have investigated topographic involvement of inflammatory cells and lymphatic vessels. Eleven cutaneous biopsies from eight patients were studied, along with controls from other granulomatous disorders and various skin lesions. Markers for lymphocytes, dendritic cells (DCs), and lymphatic vessel endothelial cells were detected using immunohistochemistry. S100(+) and CD1a(+) immature DCs (Langerhans cells) occurred more frequently within the epidermis, whereas S100(+), fascin(+), or CD83(+) maturing DCs occurred more frequently beneath the epithelium in cutaneous sarcoidosis cases than in controls (e.g. CD83, cutaneous sarcoidosis vs. other granulomatous disorders: r = 0.557, p = 0.011). Fascin(+) and CD83(+) mature DCs were often closely attached to CD3(+) T-lymphocytes around dermal granulomas. D2-40(+) lymphatic vessels were often found surrounding dermal granulomas, especially those located in the deeper dermis, in contrast to fascin(+) blood vessels. Antigen-capturing by immature DCs seems to take place initially in the epidermis, followed by maturation of DCs. These mature DCs may present the processed antigen to T-lymphocytes that cause dermal granulomas either in the interstitium of the upper dermis, or in or around lymphatic vessels of the lower dermis. Environmental antigen could be verified by skin test.

  8. Acute vision loss: a fuzzy presentation of sarcoidosis.

    Science.gov (United States)

    Austin, Andrea L; Day, Luke T; Bishop, Frank M

    2013-04-01

    Acute vision loss is a devastating problem for patients and a challenging diagnostic dilemma for Emergency Physicians. This chief complaint is one in which we must be adept at quickly evaluating and initiating either care or referral. This case reviews the approach to acute vision loss and shows the importance of expanding the differential in atypical and complex presentations. A 31-year-old, previously healthy, white woman presented to the Emergency Department (ED) with 1 day of painless right eye vision loss. Ocular ultrasound and slit-lamp examination were unremarkable. Fundoscopic examination revealed retinal hemorrhages and papillitis. Her chest X-ray study was significant for bilateral hilar adenopathy, and subsequent lymph node biopsy confirmed the diagnosis of sarcoidosis. Although sarcoidosis is more common in African Americans, it must be considered in all patients in the appropriate clinical context. Sarcoidosis is an important diagnosis to include on the differential of many chief complaints that present to the ED, including acute vision loss and dyspnea. Published by Elsevier Inc.

  9. Vertebral osteomyelitis without disc involvement

    Energy Technology Data Exchange (ETDEWEB)

    Kamani, I.; Syed, I.; Saifuddin, A. E-mail: asaifuddin@aol.com; Green, R.; MacSweeney, F

    2004-10-01

    Vertebral osteomyelitis is most commonly due to pyogenic or granulomatous infection and typically results in the combined involvement of the intervertebral disc and adjacent vertebral bodies. Non-infective causes include the related conditions of chronic recurrent multifocal osteomyelitis (CRMO) and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome. Occasionally, these conditions may present purely within the vertebral body, resulting in various combinations of vertebral marrow oedema and sclerosis, destructive lesions of the vertebral body and pathological vertebral collapse, thus mimicking neoplastic disease. This review illustrates the imaging features of vertebral osteomyelitis without disc involvement, with emphasis on magnetic resonance imaging (MRI) findings.

  10. Health-related quality of life in adult survivors of childhood sarcoidosis

    DEFF Research Database (Denmark)

    Milman, Nils; Svendsen, Claus Bo; Hoffmann, Anne Lisbeth

    2009-01-01

    AIM: To describe health-related quality of life (hrQOL) in adult subjects who had sarcoidosis in childhood. METHODS: Forty-six children (24 boys), all ethnic Danes......AIM: To describe health-related quality of life (hrQOL) in adult subjects who had sarcoidosis in childhood. METHODS: Forty-six children (24 boys), all ethnic Danes...

  11. New treatment strategies for pulmonary sarcoidosis : antimetabolites, biological drugs, and other treatment approaches

    NARCIS (Netherlands)

    Baughman, Robert P; Grutters, JC|info:eu-repo/dai/nl/258116129

    2015-01-01

    About half of patients with sarcoidosis will need systemic therapy for their disease. Oral glucocorticoids are the standard first-line treatment for sarcoidosis. With time, patients might develop substantial morbidity from long-term use of high doses of these drugs. We propose a step-wise approach

  12. Transcriptional blood signatures distinguish pulmonary tuberculosis, pulmonary sarcoidosis, pneumonias and lung cancers.

    Science.gov (United States)

    Bloom, Chloe I; Graham, Christine M; Berry, Matthew P R; Rozakeas, Fotini; Redford, Paul S; Wang, Yuanyuan; Xu, Zhaohui; Wilkinson, Katalin A; Wilkinson, Robert J; Kendrick, Yvonne; Devouassoux, Gilles; Ferry, Tristan; Miyara, Makoto; Bouvry, Diane; Valeyre, Dominique; Dominique, Valeyre; Gorochov, Guy; Blankenship, Derek; Saadatian, Mitra; Vanhems, Phillip; Beynon, Huw; Vancheeswaran, Rama; Wickremasinghe, Melissa; Chaussabel, Damien; Banchereau, Jacques; Pascual, Virginia; Ho, Ling-Pei; Lipman, Marc; O'Garra, Anne

    2013-01-01

    New approaches to define factors underlying the immunopathogenesis of pulmonary diseases including sarcoidosis and tuberculosis are needed to develop new treatments and biomarkers. Comparing the blood transcriptional response of tuberculosis to other similar pulmonary diseases will advance knowledge of disease pathways and help distinguish diseases with similar clinical presentations. To determine the factors underlying the immunopathogenesis of the granulomatous diseases, sarcoidosis and tuberculosis, by comparing the blood transcriptional responses in these and other pulmonary diseases. We compared whole blood genome-wide transcriptional profiles in pulmonary sarcoidosis, pulmonary tuberculosis, to community acquired pneumonia and primary lung cancer and healthy controls, before and after treatment, and in purified leucocyte populations. An Interferon-inducible neutrophil-driven blood transcriptional signature was present in both sarcoidosis and tuberculosis, with a higher abundance and expression in tuberculosis. Heterogeneity of the sarcoidosis signature correlated significantly with disease activity. Transcriptional profiles in pneumonia and lung cancer revealed an over-abundance of inflammatory transcripts. After successful treatment the transcriptional activity in tuberculosis and pneumonia patients was significantly reduced. However the glucocorticoid-responsive sarcoidosis patients showed a significant increase in transcriptional activity. 144-blood transcripts were able to distinguish tuberculosis from other lung diseases and controls. Tuberculosis and sarcoidosis revealed similar blood transcriptional profiles, dominated by interferon-inducible transcripts, while pneumonia and lung cancer showed distinct signatures, dominated by inflammatory genes. There were also significant differences between tuberculosis and sarcoidosis in the degree of their transcriptional activity, the heterogeneity of their profiles and their transcriptional response to treatment.

  13. Nodular hepatic and splenic sarcoidosis in a patient with a normal ...

    African Journals Online (AJOL)

    Most patients with sarcoidosis usually have an abnormal chest radiograph, while nodular lesions of both the liver and the spleen is an unusual manifestation of sarcoidosis.We report a patient with multiple nodular hepatic and splenic hypodense lesions with an unremarkable chest x-ray, and biopsy of the liver revealed ...

  14. T helper 17 cells and Regulatory T cells in Pulmonary Sarcoidosis : It takes two to tangle

    NARCIS (Netherlands)

    C.E. Broos (Caroline)

    2017-01-01

    markdownabstractSarcoidosis is an intriguingly complex immunological disorder. It is characterized by the formation of non-necrotizing granulomas that are most commonly found in the mediastinal lymph nodes and lungs of patients. Gaps in knowledge on sarcoidosis disease etiology and determinants of

  15. [Dust particles mainly containing silicon found in the cells of granulomas of sarcoidosis].

    Science.gov (United States)

    Liu, Hong-gang

    2006-06-01

    To investigate if there are dust particles in the cells of granulomas of sarcoidosis and analyze its composition and the possible correlation between the sarcoidosis and the composition of dust particles. The samples of 50 patients with sarcoidosis and 6 patients with silicosis as control were collected by biopsy and/or autopsy sampling. The pathological varieties of the granulomas and the situation of the dust particles within the cells of granulomas were observed using hematoxylin and eosin staining, Warthin-Starry silver staining, immunohistochemistry staining as well as the transmission electron microscope, and the X-Ray spectrum chemical element analysis in the samples from these two kinds of diseases. The dust particles containing silicon as the main chemical element were present in epithelioid cells and multinucleate giant cells in all the samples. The quantity of the particles deposited in sarcoidosis was less than that in the silicosis. Moreover, the dust cells were found in the granulomas and the visceral pleural lymphatic vessels in the pleura and the hilar pulmonary lymph nodes in sarcoidosis and silicosis. The pathological varieties of the granulomas in sarcoidosis and silicosis were similar. There are less silicon dust particles in the cells of granulomas of sarcoidosis than in those of the silicotic nodules, which indicates that the silicon dust particles may be related to the sarcoidosis.

  16. Massive splenomegaly and pancytopenia reuealing sarcoidosis in a child.

    Science.gov (United States)

    Viprey, M; Donadieu, J; Epaud, R; Coulomb, A; Le Pointe, H Ducou; Clement, A; Fauroux, B; Corvol, H

    2013-08-01

    Clinical presentation of sarcoidosis in children is very variable and dependant upon age. Herein, we report the first association of massive splenomegaly and pancytopenia as the revealing mode of sarcoidosis in an 8-year-old girl who, despite bone marrow involvement, had a remarkable good outcome following steroid therapy.

  17. Dendritic Cells and T Lymphocytes in the Pathogenesis of Pulmonary Sarcoidosis

    NARCIS (Netherlands)

    B. ten Berge (Bregje)

    2011-01-01

    textabstractThe history of sarcoidosis begins in 1899 when the Norwegian dermatologist Ceasar Boeck described nodular skin lesions characterized by epitheloid cells and a few giant cells as multiple benign sarcoid of the skin. Now, many years later, a lot more is known about sarcoidosis. The

  18. Sarcoidosis in Ireland: regional differences in prevalence and mortality from 1996-2005.

    LENUS (Irish Health Repository)

    Nicholson, T T

    2010-07-01

    Sarcoidosis is a common multisystem disease of unknown cause and Ireland is among the countries with the highest reported prevalence of disease worldwide. Despite this, reports on the geographical distribution of disease and differences in mortality due to sarcoidosis within Northern Ireland (NI) and the Republic of Ireland (ROI) are currently lacking.

  19. Cardiac sarcoidosis and heart transplantation: a report of four consecutive patients

    DEFF Research Database (Denmark)

    Milman, N.; Andersen, Claus Bøgelund; Mortensen, Sven Aage

    2008-01-01

    to HTx. In three patients, all with dilated cardiomyopathy due to myocardial sarcoidosis, the final diagnosis was obtained by examination of the explanted heart. Arrythmias (supraventricular and ventricular), heart block, mitral valve insufficiency and dilated cardiomyopathy were prominent clinical......Heart transplantation (HTx) is a well-established treatment for severe cardiac failure. However, HTx for cardiac sarcoidosis is rare; less than 80 patients have been reported worldwide. In many patients, the diagnosis was not made prior to HTx. The aim of this study was to describe the use of HTx...... in the treatment of severe cardiac sarcoidosis. The series was comprised of four Caucasian patients (1 male, 3 females), aged 25-57 years. HTx were performed in the period 1990-2006. None of the patients had clinically overt extra-cardiac sarcoidosis. In one patient the diagnosis of sarcoidosis was proven prior...

  20. Sudden cardiac death and sarcoidosis of the heart in a young patient.

    Science.gov (United States)

    Jotterand, Morgane; Grabherr, Silke; Lobrinus, Johannes Alexandre; Michaud, Katarzyna

    Sarcoidosis is a granulomatous disease of unknown etiology affecting any organ, microscopically characterized by noncaseating granulomata. Cardiac involvement in sarcoidosis has been reported. It might be symptomatic or not and even revealed by sudden death. Heart conduction system is rarely investigated at autopsy, even in cases of sudden cardiac death. We present a case of a 32-year-old woman who died suddenly. The examination of the heart conduction system revealed a cardiac sarcoidosis that could explain the sudden death. The review of clinical data of the patient revealed some symptoms consistent/in agreement with this hypothesis. Cardiac sarcoidosis remains a diagnostic challenge and can be easily missed, clinically and pathologically. The retrospective analysis of clinical data and autopsy results of fatal and unusual cases might help to better understand sarcoidosis and its clinical presentations. Examination of the cardiac conduction system is crucial in selected cases of sudden cardiac death. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. HLA-G polymorphisms and HLA-G expression in sarcoidosis

    DEFF Research Database (Denmark)

    Hviid, Thomas Vauvert F; Milman, Nils; Hylenius, Sine

    2006-01-01

    was investigated in granulomas from sarcoidosis patients with the use of immunohistochemistry. RESULTS: The HLA-G*010102/-G*0106 alleles were observed more often in sarcoidosis patients (39.4%) than in controls (26.4%), p = 0.025 (Fisher's exact test); however, not significant after correction (p(c) = 0.15). When...... HLA-G expression was investigated by immunohistochemistry in granulomas from sarcoidosis patients, weak HLA-G expression was observed in only one patient. CONCLUSIONS: HLA-G alleles that include a 14-bp sequence polymorphism in exon 8 of the HLA-G gene are observed more often in sarcoidosis patients...... than in controls. The sequence variation may influence HLA-G mRNA stability and influence the expression of soluble isoforms of HLA-G. Only rare and weak expression of HLA-G was observed in granulomas from sarcoidosis patients. More studies are needed to further elucidate the possible role for HLA...

  2. Papulo-Nodular Reactions in Black Tattoos as Markers of Sarcoidosis

    DEFF Research Database (Denmark)

    Sepehri, Mitra; Hutton Carlsen, Katrina; Serup, Jørgen

    2016-01-01

    of black pigment forming foreign bodies may in the predisposed individual trigger widespread reaction in the skin and internal organs. CONCLUSION: Black tattoos with papulo-nodular reactions should be seen as markers of sarcoidosis. Papulo-nodular reactions may, as triggers, induce widespread reactions...... phenomenon" with concomitant reaction in many other black tattoos, triggered by a recent tattoo with a papulo-nodular reaction, was observed in 70% in the sarcoidosis group and 28% in the non-sarcoidosis group, indicating a predisposing factor which may be autoimmune and linked with sarcoidosis. Agglomerates...... in other black tattoos - a "rush phenomenon" - depending on individual predisposition. Sarcoidosis is estimated to be 500-fold increased in papulo-nodular reactions compared to the prevalence in the general population, and the association with black tattoos is strong....

  3. The spectrum of pulmonary sarcoidosis: Variations of high-resolution CT findings and clues for specific diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Nishino, Mizuki; Lee, Karen S. [Department of Radiology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215 (United States); Itoh, Harumi [Department of Radiology, University of Fukui, Faculty of Medical Sciences, Matsuoka-cho, Yoshida-gun, Fukui (Japan); Hatabu, Hiroto [Department of Radiology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215 (United States)], E-mail: hhatabu@bidmc.harvard.edu

    2010-01-15

    Sarcoidosis is a systemic disease of unknown cause, characterized by widespread non-caseating granulomas. There is a wide spectrum of radiologic manifestations in pulmonary sarcoidosis, providing challenges to radiologists. However, recognition of the key features of sarcoidosis with knowledge of its pathologic background can often allow for specific diagnosis. In this review, we describe the variety of high-resolution CT findings in pulmonary sarcoidosis along with its pathologic features as the basis for radiographic manifestations, and discuss the key features on high-resolution CT for the specific diagnosis of pulmonary sarcoidosis.

  4. Prevalence of Vertebral Fractures in Children with Suspected Osteoporosis.

    Science.gov (United States)

    Kyriakou, Andreas; Shepherd, Sheila; Mason, Avril; Ahmed, S Faisal

    2016-12-01

    To explore the prevalence and anatomic distribution of vertebral fractures in disease groups investigated for primary and secondary osteoporosis, using vertebral fracture assessment (VFA). VFA was performed independently by 2 nonradiologists, in 165 children (77 males, 88 females) as part of their investigation for osteoporosis. Vertebral bodies from T6 to L4 were assessed for vertebral fractures using the Genant scoring system. The common readings for the presence of vertebral fractures were used for evaluating the prevalence and anatomic distribution of vertebral fractures. The median age of the subjects was 13.4 years (range, 3.6, 18). Of the 165 children, 24 (15%) were being investigated for primary bone disease, and the remainder had a range of chronic diseases known to affect bone health. Vertebral fractures were identified in 38 (23%) children. The distribution of the vertebral fractures was bimodal, with vertebral fractures peaks centered at T9 and L4. Conditions associated with increased odds for vertebral fractures were inflammatory bowel disease (OR, 3.3; 95% CI, 1.4, 8.0; P = .018) and osteogenesis imperfecta (OR, 2.3; 95% CI, 1.04, 5.8; P = .022). Among children with vertebral fractures, those with Duchenne muscular dystrophy (P = .015) and osteogenesis imperfecta (P = .023) demonstrated higher number of vertebral fractures than the other disease groups. VFA identified the presence of vertebral fractures, in a bimodal distribution, in both primary bone disease and chronic disease groups. VFA is a practical screening tool for identification of vertebral fractures in children and adolescents at risk of fragility fractures. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Vertebral Fracture Prediction

    DEFF Research Database (Denmark)

    2008-01-01

    Vertebral Fracture Prediction A method of processing data derived from an image of at least part of a spine is provided for estimating the risk of a future fracture in vertebraeof the spine. Position data relating to at least four neighbouring vertebrae of the spine is processed. The curvature...

  6. Endplates Changes Related to Age and Vertebral Segment

    Directory of Open Access Journals (Sweden)

    Carlos Fernando P. S. Herrero

    2014-01-01

    Full Text Available Endplate separations are defined as the presence of a space between the hyaline cartilage and the cortical bone of the adjacent vertebral body. This study evaluates endplate separations from the vertebral body and intervertebral discs and verifies if endplate separation is related to age and the spinal level. Groups were formed based on age (20–40 and 41–85 years old and the vertebral segment (T7-T8 and L4-L5 segments. Histological analysis included assessment of the length of the vertebral endplates, the number and dimensions of the separations, and orientation of the collagen fibers, in the mid-sagittal slice. Two indexes were created: the separation index (number of separations/vertebral length and separation extension index (sum of all separations/vertebral length. The results of the study demonstrated a direct relationship between the density of separations in the endplate and two variables: age and spinal level.

  7. Influence of physical activity on vertebral strength during late adolescence.

    Science.gov (United States)

    Junno, Juho-Antti; Paananen, Markus; Karppinen, Jaro; Tammelin, Tuija; Niinimäki, Jaakko; Lammentausta, Eveliina; Niskanen, Markku; Nieminen, Miika T; Järvelin, Marjo-Riitta; Takatalo, Jani; Tervonen, Osmo; Tuukkanen, Juha

    2013-02-01

    Reduced vertebral strength is a clear risk factor for vertebral fractures. Men and women with vertebral fractures often have reduced vertebral size and bone mineral density (BMD). Vertebral strength is controlled by both genetic and developmental factors. Malnutrition and low levels of physical activity are commonly considered to result in reduced bone size during growth. Several studies have also demonstrated the general relationship between BMD and physical activity in the appendicular skeleton. In this study, we wanted to clarify the role of physical activity on vertebral bodies. Vertebral dimensions appear to generally be less pliant than long bones when lifetime changes occur. We wanted to explore the association between physical activity during late adolescence and vertebral strength parameters such as cross-sectional size and BMD. The association between physical activity and vertebral strength was explored by measuring vertebral strength parameters and defining the level of physical activity during adolescence. The study population consisted of 6,928 males and females who, at 15 to 16 and 19 years of age, responded to a mailed questionnaire inquiring about their physical activity. A total of 558 individuals at the mean age of 21 years underwent magnetic resonance imaging (MRI) scans. We measured the dimensions of the fourth lumbar vertebra from the MRI scans of the Northern Finland Birth Cohort 1986 and performed T2* relaxation time mapping, reflective of BMD. Vertebral strength was based on these two parameters. We analyzed the association of physical activity on vertebral strength using the analysis of variance. We observed no association between the level of physical activity during late adolescence and vertebral strength at 21 years. Copyright © 2013 Elsevier Inc. All rights reserved.

  8. [Manifestations of sarcoidosis in the upper respiratory tract].

    Science.gov (United States)

    Aguilar-García, J; de la Torre-Lima, J; Prada-Pardal, J L

    2006-02-01

    Sarcoidosis is a multisystemic disease having unknown cause, characterized by non-caseating granulomatous inflammation of the organs involved. It predominantly affects the respiratory tract, with preference for the lower tract and less frequently affects the upper respiratory tract (nose, paranasal sinuses and larynx). It manifests non-specifically, with symptoms secondary to the obstruction of the airway. It can be confused with other more common disorders in our setting, such as tuberculosis. We conduct a review, fundamentally focusing on the diagnosis and treatment due to their difficulty.

  9. Sarcoidosis HLA class II genotyping distinguishes differences of clinical phenotype across ethnic groups

    Science.gov (United States)

    Sato, Hiroe; Woodhead, Felix A.; Ahmad, Tariq; Grutters, Jan C.; Spagnolo, Paolo; van den Bosch, Jules M.M.; Maier, Lisa A.; Newman, Lee S.; Nagai, Sonoko; Izumi, Takateru; Wells, Athol U.; du Bois, Roland M.; Welsh, Kenneth I.

    2010-01-01

    The HLA class II (DRB1 and DQB1) associations with sarcoidosis have been studied by several groups but often without consistent results. In this paper, we consider the hypothesis that observed inconsistencies relate to distinct, genetically encoded disease phenotypes which differ in prevalence between centres. We therefore typed HLA-DRB1 and DQB1 in 340 UK, 139 Dutch and 163 Japanese sarcoidosis patients and, respectively, 354, 218 and 168 healthy controls from these populations. We applied consistent phenotyping and genotyping and investigated associations between HLA class II alleles and distinct disease phenotypes within and between ethnic groups. DRB1*01 and DQB1*0501 are protective against all manifestations of sarcoidosis. Lung-predominant sarcoidosis is associated with DRB1*12 and *14. Löfgren's syndrome is a common sarcoidosis phenotype in the Dutch and is strongly associated with the DRB1*0301 allele. This phenotype is not seen among the Japanese in whom DRB1*0301 is absent. The same allele is protective for UK uveitis. Sarcoid uveitis is common in Japan. The DRB1*04–DQB1*0301 haplotype is a risk factor for this disease manifestation in Japanese and UK subjects but protective for sarcoidosis overall. We show that distinct sarcoidosis phenotypes have similar genetic associations across ethnic groups. The disease case mix differs between centres and may be explained by different ethnic allelic frequencies. PMID:20685690

  10. Sarcoidosis-Like Lesions: Another Paradoxical Reaction to Anti-TNF Therapy?

    Science.gov (United States)

    Decock, Amelie; Van Assche, Gert; Vermeire, Séverine; Wuyts, Wim; Ferrante, Marc

    2017-03-01

    Since the introduction of anti-tumour necrosis factor [TNF] therapy in inflammatory diseases, paradoxical reactions are increasingly being reported. One of these paradoxical reactions is the development of sarcoidosis-like lesions. This presentation is paradoxical since anti-TNF therapy can also be therapeutic in refractory cases of sarcoidosis. We report two cases of sarcoidosis-like lesions under anti-TNF therapy. Both were patients with inflammatory bowel disease [IBD], treated successfully with adalimumab. Next, we reviewed the literature for similar cases. Medical subject heading terms 'adalimumab', 'infliximab', 'etanercept', 'golimumab' or 'certolizumab', and 'sarcoidosis' were used to perform key word searches of the PubMed database. We identified 90 reported cases of sarcoidosis-like lesions, which developed during anti-TNF therapy. In most cases, the anti-TNF drug involved was etanercept. The median age was 43 years and there was a predominance of female patients. The underlying disease was rheumatoid arthritis in most cases, followed by ankylosing spondylitis and psoriasiform arthritis. In six cases, the underlying disease was IBD. In 71 cases there was at least a partial resolution by discontinuation of the anti-TNF treatment, initiation of steroids or both. Re-initiation of anti-TNF therapy gave relapse in seven out of 20 cases. Sarcoidosis-like lesions are increasingly reported during anti-TNF treatment. Vigilance is appropriate when patients present with symptoms compatible with sarcoidosis.

  11. A Case of Sarcoidosis Associated With Anti–Tumor Necrosis Factor Treatment

    Directory of Open Access Journals (Sweden)

    Ayse Baha MD

    2015-02-01

    Full Text Available Sarcoidosis is a systemic chronic granulomatous disease of unknown etiology. It predominantly involves the lungs but can affect many organs or tissues in the body, such as the lymphatic system, skin, eyes, and liver. Typical histopathological lesions are noncaseating granulomas in the affected organ or tissue. Indications, type of treatment, and duration of sarcoidosis treatment is currently debated. Despite studies showing that anti–tumor necrosis factor-α (TNF-α treatment can successfully be used in refractory sarcoidosis, there are some case reports regarding the development of sarcoidosis with these agents. There have been reports of 47 anti-TNF-associated cases of sarcoidosis until 2012. The patient is a 54-year-old Caucasian male. During routine examinations of the patient who had been followed for psoriasis vulgaris for 20 years and who had been on several anti-TNF regimens thereafter, new pulmonary pathologies due to sarcoidosis were detected. We present here a case of sarcoidosis that developed after infliximab treatment and showed obvious radiologic regression with discontinuation of treatment. During anti-TNF treatment, it should be kept in mind that autoimmune and granulomatous diseases may develop and particular care should be given to patient follow-ups.

  12. A Case of Sarcoidosis Associated With Anti-Tumor Necrosis Factor Treatment.

    Science.gov (United States)

    Baha, Ayse; Hanazay, Cigdem; Kokturk, Nurdan; Turktas, Haluk

    2015-01-01

    Sarcoidosis is a systemic chronic granulomatous disease of unknown etiology. It predominantly involves the lungs but can affect many organs or tissues in the body, such as the lymphatic system, skin, eyes, and liver. Typical histopathological lesions are noncaseating granulomas in the affected organ or tissue. Indications, type of treatment, and duration of sarcoidosis treatment is currently debated. Despite studies showing that anti-tumor necrosis factor-α (TNF-α) treatment can successfully be used in refractory sarcoidosis, there are some case reports regarding the development of sarcoidosis with these agents. There have been reports of 47 anti-TNF-associated cases of sarcoidosis until 2012. The patient is a 54-year-old Caucasian male. During routine examinations of the patient who had been followed for psoriasis vulgaris for 20 years and who had been on several anti-TNF regimens thereafter, new pulmonary pathologies due to sarcoidosis were detected. We present here a case of sarcoidosis that developed after infliximab treatment and showed obvious radiologic regression with discontinuation of treatment. During anti-TNF treatment, it should be kept in mind that autoimmune and granulomatous diseases may develop and particular care should be given to patient follow-ups.

  13. Splenomegaly in sarcoidosis: Frequency, treatment, prognosis and long-term follow-up

    Directory of Open Access Journals (Sweden)

    Pavlović-Popović Zora

    2015-01-01

    Full Text Available Introduction. The splenic involvement is common in sarcoidosis, but its real frequency is still obscure, depending doubtless on the method of splenomegaly detection. Splenomegaly may be accompanied with pain or anemia, leucopenia and thrombocytopenia. Objective. The aim of this study was to investigate the frequency of splenomegaly related to clinical characteristics of sarcoidosis and to solve the dilemma - whether to introduce medicaments, and when to perform splenectomy. Methods. The method of the study is a retrospective and prospective analysis of the patients’ material. Results. The study included 540 patients with sarcoidosis in a 20-year period. Of them, 26% had splenomegaly detected by computerized tomography screening. Splenomegaly was more frequently registered in the patients with a longer history of sarcoidosis (38%, as compared to those with a shorter history of the disease (23% (p<0.05. Splenomegaly was more frequently registered in the patients with other extrapulmonary lesions detected (33% than in those who had no extrapulmonary manifestations of sarcoidosis (17% (p<0.01. Indications, possible benefits and complications of splenectomy were analysed in 11 sarcoidosis patients undergoing this intervention for various reasons, of which the follow-up period ranged from one to 20 years. Conclusion. Splenomegaly was more frequent in chronic cases or in the patients with established sarcoid lesions of other extrapulmonary organs. The primary treatment of uncomplicated symptomatic splenic sarcoidosis includes medicamentous therapy. Occasionally, splenectomy is required. Prognostically, splenomegaly indicates an unfavorable course of the disease.

  14. Evolution of the vertebrate claudin gene family: insights from a basal vertebrate, the sea lamprey.

    Science.gov (United States)

    Mukendi, Christian; Dean, Nicholas; Lala, Rushil; Smith, Jeramiah; Bronner, Marianne E; Nikitina, Natalya V

    2016-01-01

    Claudins are major constituents of tight junctions, contributing both to their intercellular sealing and selective permeability properties. While claudins and claudin-like molecules are present in some invertebrates, the association of claudins with tight junctions has been conclusively documented only in vertebrates. Here we report the sequencing, phylogenetic analysis and comprehensive spatiotemporal expression analysis of the entire claudin gene family in the basal extant vertebrate, the sea lamprey. Our results demonstrate that clear orthologues to about half of all mammalian claudins are present in the lamprey, suggesting that at least one round of whole genome duplication contributed to the diversification of this gene family. Expression analysis revealed that claudins are expressed in discrete and specific domains, many of which represent vertebrate-specific innovations, such as in cranial ectodermal placodes and the neural crest; whereas others represent structures characteristic of chordates, e.g. pronephros, notochord, somites, endostyle and pharyngeal arches. By comparing the embryonic expression of claudins in the lamprey to that of other vertebrates, we found that ancestral expression patterns were often preserved in higher vertebrates. Morpholino mediated loss of Cldn3b demonstrated a functional role for this protein in placode and pharyngeal arch morphogenesis. Taken together, our data provide novel insights into the origins and evolution of the claudin gene family and the significance of claudin proteins in the evolution of vertebrates.

  15. Clinicopathological study of glomerular diseases associated with sarcoidosis: a multicenter study

    Science.gov (United States)

    2013-01-01

    Background The association between sarcoidosis and glomerular diseases has not been extensively investigated in a large series and the potential features of this uncommon association remain to be determined. Methods We retrospectively identified 26 patients with biopsy-proven glomerular lesions that occurred in a sarcoidosis context. Potential remission of glomerular disease and sarcoidosis under specific treatment (steroid and/or immunosuppressive agents) was recorded for all patients. Demographic, clinical and biological characteristics were assessed at the time of kidney biopsy for each patient. Therapeutic data were analyzed for all patients. Results Glomerular disease occurred after the diagnosis of sarcoidosis in 11 of 26 cases (42%) (mean delay of 9.7 years). In six patients (23%), the glomerulopathy preceded the sarcoidosis diagnosis (mean delay 8 years). In the last nine patients (35%), both conditions occurred simultaneously. The most frequent glomerular disease occurring in sarcoidosis patients was membranous nephropathy in eleven cases. Other glomerular lesions included IgA nephropathy in six cases, focal segmental glomerulosclerosis in four patients, minimal change nephrotic syndrome for three patients and proliferative lupus nephritis in two patients. Granulomatous interstitial nephritis was associated with glomerular disease in six patients and was exclusively found in patients in whom the both disease occurred simultaneously. In nine patients with simultaneous glomerular and sarcoidosis diseases, we observed a strong dissociation between glomerular disease and sarcoidosis in terms of steroid responsiveness. At the end of the follow-up (mean of 8.4 years), six patients had reached end-stage renal disease and three patients had died. Conclusions A wide spectrum of glomerular lesions is associated with sarcoidosis. The close temporal relationship observed in some patients suggests common causative molecular mechanisms of glomerular injury but

  16. Upper Airway Obstruction Requiring Emergent Tracheostomy Secondary to Laryngeal Sarcoidosis: A Case Report.

    Science.gov (United States)

    Ryu, Changwan; Herzog, Erica L; Pan, Hongyi; Homer, Robert; Gulati, Mridu

    2017-02-13

    BACKGROUND Laryngeal sarcoidosis is a rare extrapulmonary manifestation of sarcoidosis, accounting for 0.33-2.1% of cases. A life-threatening complication of laryngeal sarcoidosis is upper airway obstruction. In this report we describe our experience in the acute and chronic care of a patient who required an emergent tracheostomy, with the aim to provide further insight into this difficult to manage disease. CASE REPORT A 37-year-old African American female with a 10-year history of stage 1 sarcoidosis presented with severe dyspnea. Laryngeal sarcoidosis was diagnosed three years previously, and she remained stable on low-dose prednisone until six months prior to admission, at which time she self-discontinued her prednisone for the homeopathic treatment Nopalea cactus juice. Her physical examination was concerning for impending respiratory failure as she presented with inspiratory stridor and hoarseness. Laryngoscopy showed a retroflexed epiglottis obstructing the glottis with edematous arytenoids and aryepiglottic folds. Otolaryngology performed an emergent tracheostomy to secure her airway and obtained epiglottic biopsies, which were consistent with sarcoidosis. She was eventually discharged home on prednisone 60 mg daily. Following months of corticosteroids, a laryngoscopy showed the epiglottis continuing to obstruct the glottis. The addition of methotrexate to a tapered dosage of prednisone 10 mg daily was unsuccessful, and she remains on prednisone 20 mg daily for disease control. CONCLUSIONS Laryngeal sarcoidosis, a rare extrapulmonary manifestation of sarcoidosis, uncommonly presents as the life-threatening complication of complete upper airway obstruction. As such, laryngeal sarcoidosis is associated with significant morbidity and mortality, requiring a high index of suspicion for timely diagnosis and treatment.

  17. Appendectomy, tonsillectomy, and risk for sarcoidosis - A hospital-based case-control study in Japan.

    Science.gov (United States)

    Sawahata, Michiru; Nakamura, Yosikazu; Sugiyama, Yukihiko

    2017-05-01

    The role of surgery in the onset of sarcoidosis is unclear. We investigated whether surgery is an internal environmental factor for sarcoidosis onset within the Japanese population. We enrolled 222 patients diagnosed with sarcoidosis (78 men, 144 women) who were admitted to our department between 1984 and 2012. We also enrolled 529 control subjects (251 men, 278 women), who were matched for sex, age at admission, and year of admission. Surgical history, family history, and smoking status were evaluated. Multivariate analysis correlated history of appendectomy (OR, 1.55; 95% CI, 1.05-2.29) and tonsillectomy (OR, 2.79; 95% CI, 0.91-8.56) with the occurrence of sarcoidosis; other surgical procedures had no correlation. In women, appendectomy had a stronger association with sarcoidosis (OR, 1.69; 95% CI, 1.05-2.73), as opposed to that in men (OR, 1.39; 95% CI, 0.68-2.85). This association was greater in women aged ≥45 years than in those aged sarcoidosis in women (OR, 3.30; 95% CI, 0.88-12.39), than in men (OR, 1.26; 95% CI, 0.10-16.52). ORs for sarcoidosis were 5.55 (95% CI, 2.02-15.27) and 0.97 (95% CI, 0.52-1.84) in women aged ≥45 years with a history of appendectomy at sarcoidosis onset, suggesting their potential protective role against sarcoidosis development. Further studies are needed to minimize possible confounding factors. Copyright © 2017 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

  18. Value of neutrophil/lymphocyte ratio in the differential diagnosis of sarcoidosis and tuberculosis

    Directory of Open Access Journals (Sweden)

    Sinem Iliaz

    2014-01-01

    Full Text Available Introduction: The differential diagnosis of sarcoidosis creates a challange due to tuberculosis also having lung and lymph node involvement. Because both diseases show granulomatous inflammation, it may not be possible to distinguish tuberculosis and sarcoidosis in pathological specimens. As a result of the complexity in the differential diagnosis of sarcoidosis and tuberculosis, new markers for differentiation are being investigated. Objective: The aim of our study is to investigate the value of neutrophil/lymphocyte ratio (NLR as a possible marker in differentiating sarcoidosis and tuberculosis. Materials and Methods: In our study, 51 acid-fast bacilli (AFB positive and/or culture-positive patients with pulmonary tuberculosis, ​​40 patients with biopsy-proven sarcoidosis and a control group consisting of 43 patients were included. In our study, information was collected retrospectively based on hospital records. Results: Leukocyte and neutrophil counts, NLR, erythrocyte sedimentation rate (ESR, C-reactive protein (CRP were significantly higher, and albumin was significantly lower in the tuberculosis group compared with sarcoidosis (for all parameters P < 0.001. The most appropriate cut-off value of NLR to distinguish tuberculosis from sarcoidosis was determined as 2.55. For this cut-off value of NLR there was 79% sensitivity, 69% specificity, 73% positive predictive value (PPV, 75% negative predictive value (NPV, and area under the curve (AUC was 0.788. For differentiation of sarcoidosis from tuberculosis, accuracy of the NLR test according to this cut-off value was found as 76%. Conclusion: NLR as a little known marker in respiratory medicine was found to be supportive in differentiation of tuberculosis and sarcoidosis. More studies on this issue is needed.

  19. Multiple flow rates measurement of exhaled nitric oxide in patients with sarcoidosis: a pilot feasibility study.

    Science.gov (United States)

    Choi, J; Hoffman, L A; Sethi, J M; Zullo, T G; Gibson, K F

    2009-07-01

    Fraction of end tidal exhaled nitric oxide (FeNO) has been introduced as a non-invasive marker of airway inflammation in patients with asthma and may have value in monitoring disease activity in patients with sarcoidosis. This pilot study explored: 1) feasibility of the multiple flow rates maneuver to estimate alveolar (C(AlV)NO) and airway wall (J(AW)NO) NO in patients with sarcoidosis; and 2) utility of exhaled NO (FeNO, C(Alv)NO and J(AW)NO) measurements to detect and monitor treatment response in patients with active pulmonary sarcoidosis. Patients with sarcoidosis (n = 42) and healthy non-smokers (n = 20) underwent FeNO measurement at 7 flow-rates (50 to 400 ml/s). Using the Tsoukias and George (1998) model, C(Alv)NO and J(AW)NO were estimated. Both patients and healthy non-smokers were able to perform the multiple flow rates maneuver without discomfort, with first measurement success rate of 57% and 65%, respectively. No significant difference was found between patients with sarcoidosis and healthy non-smokers in exhaled NO. None were correlated with pulmonary function tests, except a significant negative correlation between C(Alv)NO and FVC% (p = 0.001) and DLCO% (p = 0.012). In 8 patients with active sarcoidosis, FeNO, C(Alv)NO or J(AW)NO were not different from those of patients with inactive sarcoidosis. Treatment of active sarcoidosis using oral prednisone and methotrexate did not show any consistent pattern of changes in C(Alv)NO or J(AW)NO. Due to a large inter-subject variability and difficulty controlling use of the inhaled corticosteroids, exhaled NO measurement did not appear to be a clinically useful method of monitoring disease progression in sarcoidosis.

  20. Risk of Malignancy Among Patients With Sarcoidosis: A Population-Based Cohort Study.

    Science.gov (United States)

    Ungprasert, Patompong; Crowson, Cynthia S; Matteson, Eric L

    2017-01-01

    To investigate the risk of malignancy in patients with sarcoidosis in a population-based cohort. A cohort of Olmsted County, Minnesota, residents diagnosed with sarcoidosis between January 1, 1976 and December 31, 2013 was identified based on individual medical record review. For each sarcoidosis subject, 2 sex- and age-matched comparator subjects without sarcoidosis were randomly selected. Cases and comparators were then cross-indexed with the Mayo Clinic Cancer Registry, which collected data on every type of malignancy except for nonmelanoma skin cancer, for malignancy ascertainment. A total of 345 incident cases of sarcoidosis and 690 comparators were identified. There was no difference in the prevalence of malignancy at the index date between the 2 groups (4.3% among cases and 4.3% among comparators; P = 1.0). During followup, 36 patients with sarcoidosis and 91 subjects without sarcoidosis developed malignancy, with a cumulative incidence at 10 years of 3.8% and 7.1%, respectively. The difference corresponded to a hazard ratio (HR) of 0.72 (95% confidence interval [95% CI] 0.49-1.06). The cumulative incidences at 10 years for individual types of malignancy were also similar between the 2 groups, with nonsignificant HRs. However, subgroup analysis found that cases with extrathoracic involvement were at higher risk of incident hematologic malignancy compared with cases without extrathoracic involvement (HR 1.87 [95% CI 1.09-3.22]). Risk of malignancy was similar among patients with sarcoidosis compared to nonsarcoidosis subjects. However, the risk of incident hematologic malignancy was significantly higher among patients with sarcoidosis with extrathoracic involvement compared to patients without extrathoracic disease. © 2016, American College of Rheumatology.

  1. Predicting vertebral bone strength by vertebral static histomorphometry

    DEFF Research Database (Denmark)

    Thomsen, Jesper Skovhus; Ebbesen, Ebbe Nils; Mosekilde, Lis

    2002-01-01

    The study investigates the relationship between static histomorphometry and bone strength of human lumbar vertebral bone. The ability of vertebral histomorphometry to predict vertebral bone strength was compared with that of vertebral densitometry, and also with histomorphometry and bone strength...... of the entire vertebral bodies (L-2) were used for histomorphometry. The other iliac crest biopsies and the L-3 were destructively tested by compression. High correlation was found between BV/TV or Tb.Sp and vertebral bone strength (absolute value of r = 0.86 in both cases). Addition of Tb.Th significantly...... of improving the prediction of bone strength of the vertebral body. The correlations between BV/TV of L-2 and bone strength of L-3 were comparable with the correlation obtained by quantitative computed tomography (QCT), peripheral QCT (pQCT), and dual-energy X-ray absorptrometry (DEXA) of L-3 and bone strength...

  2. Pulmonary arterial hypertension in a patient with stage II sarcoidosis and Hashitoxicosis

    Directory of Open Access Journals (Sweden)

    S. Ocak

    2009-06-01

    Full Text Available Although pulmonary arterial hypertension is usually associated with advanced stages of sarcoidosis, its occurrence in early stage disease is rare. Herein, a case of associated pulmonary arterial hypertension in the setting of Hashitoxicosis and stage II pulmonary sarcoidosis is reported. The case of associated pulmonary arterial hypertension occurred in a young female without clinically significant medical history and who completely recovered after receiving oral corticotherapy only. Furthermore, this case report suggests the presence of an interaction between pulmonary arterial hypertension, sarcoidosis and Hashitoxicosis.

  3. Dilemma of diagnosing thoracic sarcoidosis in tuberculosis endemic regions: An imaging-based approach. Part 1

    Directory of Open Access Journals (Sweden)

    Ashu S Bhalla

    2017-01-01

    Full Text Available Sarcoidosis is a multi-systemic disorder of unknown etiology, although commonly believed to be immune-mediated. Histologically, it is characterized by noncaseating granuloma which contrasts against the caseating granuloma seen in tuberculosis (TB, an infectious disease that closely mimics sarcoidosis, both clinically as well as radiologically. In TB-endemic regions, the overlapping clinico-radiological manifestations create significant diagnostic dilemma, especially since the management options are markedly different in the two entities. Part 1 of this review aims to summarize the clinical, laboratory, and imaging features of sarcoidosis, encompassing both typical and atypical manifestations, in an attempt to distinguish between the two disease entities.

  4. Sarcoidosis on tattoos: a review of the literature from 1939 to 2011.

    Science.gov (United States)

    Kluger, N

    2013-08-01

    Sarcoidosis is an autoimmune disease of unknown etiology characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs. Cutaneous sarcoidosis occurs in approximately 25% of the cases. Sarcoid reactions on old scars, traumatized skin sites and around embedded foreign material have long been observed. For the past 70 years, sarcoidal granulomas on tattoos and permanent make-up have also been documented. Granulomatous and sarcoidal tattoo reactions may be the first and sometimes only cutaneous manifestation of systemic sarcoidosis. This review summarizes the currently available data on this topic and discusses the issues related to the diagnosis, management and physiopathogeny of sarcoidal reactions on tattoos.

  5. Cigarette smoking and male sex are independent and age concomitant risk factors for the development of ocular sarcoidosis in a new orleans sarcoidosis population

    Science.gov (United States)

    Janot, Adam C.; Huscher, Dörte; Walker, McCall; Grewal, Harmanjot K.; Yu, Mary; Lammi, Matthew R.; Saketkoo, Lesley Ann

    2016-01-01

    Introduction Sarcoidosis is a multi-organ system granulomatous disease of unknown origin with an incidence of 1–40/100,000. Though pulmonary manifestations are predominant, ocular sarcoidosis (OS) affects 25–50% of patients with sarcoidosis and can lead to blindness. Methods A retrospective, single-center chart review of sarcoidosis cases investigated variables associated with the development of OS. Inclusion criteria were biopsy-proven sarcoidosis, disease duration greater than 1 year, documented smoking status on chart review and documentation of sarcoid-related eye disease. Multivariate analysis identified independent risk factors for OS. Results Of 269 charts reviewed, 109 patients met inclusion criteria. The OS group had a significantly higher proportion of smokers (71.4%) than without OS (42.0%, p=0.027) with no difference (p=0.61) in median number of pack years. Male sex was significantly higher in the OS group (57.1% versus 26.1%, p=0.009). Median duration of sarcoidosis was higher in the OS group (10 versus 4 years, p=0.031). Multivariate regression identified tobacco exposure (OR=5.25, p=0.007, 95% CI 1.58–17.41), male sex (OR=7.48, p=0.002, 95% CI 2.15–26.01), and age (OR=1.114, p=0.002, 95% CI 1.04–1.19) as concomitant risk factors for the development of OS. Conclusion To date, there are few dedicated investigations of risk factors for OS, especially smoking. This investigation identified male sex, age, and tobacco exposure as independent risk factors for OS. Though disease duration did not withstand regression analysis in this moderately sized group, age at chart review suggests screening for OS should not remit but rather intensify in aging patients with sarcoidosis. PMID:26278693

  6. Sex reversal in vertebrates

    OpenAIRE

    2016-01-01

    This special topic issue of Sexual Development gives an overview of sex reversal in vertebrates, from fishes naturally changing their sex, to rodents escaping the mammalian SRY-determining system. It offers eight up-to-date reviews on specific subjects in sex reversal, considering fishes, amphibians, reptiles, birds, marsupials, and placental mammals, including humans. The broad scope of represented animals makes this ideal for students and researchers, especially those interested in the...

  7. Esclerose sistêmica e sarcoidose Scleroderma and sarcoidosis

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    Fernanda Guidolin

    2005-10-01

    Full Text Available Os autores descrevem o caso de uma paciente com esclerose sistêmica (ES - forma limitada - com comprometimento pulmonar tipo fibrose intersticial. Após sete anos sem acompanhamento, foram identificados gânglios mediastinais e esplenomegalia. A biópsia de linfonodos mostrou granuloma não caseoso sugestivo de sarcoidose. Estamos mostrando, neste caso, a associação de ES e sarcoidose, para chamar a atenção para esse fato e enfatizar que a sarcoidose deve ser lembrada no diagnóstico diferencial das complicações pulmonares da esclerodermia.The authors describe the case of a patient with limited scleroderma and interstitial lung disease. Follow-up was lost for seven years, when patient returned presenting nodular mediastinal enlargement and splenomegaly. Lymph node biopsy showed granulomatous lesions without caseum suggestive of sarcoidosis. This case is being presented to remind the association of scleroderma and sarcoidosis as a possible differential diagnosis of scleroderma pulmonary complications.

  8. Building the Vertebrate Spine

    Science.gov (United States)

    Pourquié, Olivier

    2008-03-01

    The vertebrate body can be subdivided along the antero-posterior (AP) axis into repeated structures called segments. This periodic pattern is established during embryogenesis by the somitogenesis process. Somites are generated in a rhythmic fashion from the paraxial mesoderm and subsequently differentiate to give rise to the vertebrae and skeletal muscles of the body. Somite formation involves an oscillator-the segmentation clock-whose periodic signal is converted into the periodic array of somite boundaries. This clock drives the dynamic expression of cyclic genes in the presomitic mesoderm and requires Notch and Wnt signaling. Microarray studies of the mouse presomitic mesoderm transcriptome reveal that the segmentation clock drives the periodic expression of a large network of cyclic genes involved in cell signaling. Mutually exclusive activation of the Notch/FGF and Wnt pathways during each cycle suggests that coordinated regulation of these three pathways underlies the clock oscillator. In humans, mutations in the genes associated to the function of this oscillator such as Dll3 or Lunatic Fringe result in abnormal segmentation of the vertebral column such as those seen in congenital scoliosis. Whereas the segmentation clock is thought to set the pace of vertebrate segmentation, the translation of this pulsation into the reiterated arrangement of segment boundaries along the AP axis involves dynamic gradients of FGF and Wnt signaling. The FGF signaling gradient is established based on an unusual mechanism involving mRNA decay which provides an efficient means to couple the spatio-temporal activation of segmentation to the posterior elongation of the embryo. Another striking aspect of somite production is the strict bilateral symmetry of the process. Retinoic acid was shown to control aspects of this coordination by buffering destabilizing effects from the embryonic left-right machinery. Defects in this embryonic program controlling vertebral symmetry might lead

  9. A rare case of eyelid sarcoidosis presenting as an orbital mass

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    Balan Louis Gaspar

    2016-01-01

    Full Text Available Intraorbital sarcoidosis presenting externally as a solitary eyelid mass has been described in the literature as isolated case reports. We describe a rare case of asymptomatic sarcoidosis with orbital mass as the presenting feature in a young woman. The lesion was excised with the clinical possibility of a thrombosed varix. On histology, the lesion was characterized by numerous nonnecrotizing epithelioid cell granulomas with several multinucleated giant cells containing abundant asteroid bodies and oxalate crystals. No tubercular bacilli were detected. A diagnosis of sarcoidosis was rendered and on further clinical work-up, she was detected to have hilar lymphadenopathy. Sarcoidosis should be considered in the differential diagnosis of orbital mass as it could be the initial manifestation of the disease process.

  10. Co-existing sarcoidosis and Takayasu arteritis: report of a case

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    Hamzaoui Amira

    2011-02-01

    Full Text Available Abstract Introduction Takayasu arteritis (TA is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. It has been described in association with various auto-immune disorders (mainly inflammatory digestive tract diseases. However, only few cases of TA associated with sarcoidosis have been reported, raising the question of an association by chance. Case report We report a case of a 34 year-old woman, with one year history of sarcoidosis, who presented with asymmetric high hypertension revealing inflammatory humeral, axillary and subclavian arteritis related to TA, successfully treated by steroid and immunosuppressive therapy(MethotrexateR. Conclusion TA and sarcoidosis may be related, rising the hypothesis that TA or Takayasu arteritis-like granulomatous vasculitis may be, in fact, a complication of sarcoidosis.

  11. A rare case of sarcoidosis involving the middle turbinates: an incidental diagnosis

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    Tosun Emine

    2006-11-01

    Full Text Available Abstract Background Sarcoidosis is a chronic, systemic granulomatous disease of unknown etiology that features noncaseating granulomas in many body regions. Sinonasal involvement is rare but is also suspected to be underreported. Case presentation We present the case of a 39-year-old woman who was incidentally diagnosed with isolated sarcoidosis involving the middle turbinates. Histopathologic examination of resected concha bullosa material and an extensive panel of diagnostic tests revealed a diagnosis of isolated sarcoidosis. Since no systemic manifestations were detected, topical corticosteroid (nasal spray was administered in the postoperative period. Throughout the 12 months after surgery, the patient remained free of symptoms and all nasal endoscopy examinations were normal. Conclusion Although isolated nasal involvement of sarcoidosis is rare, otorhinolaryngologists should consider this condition in a differential diagnosis for sinonasal complaints.

  12. HLA-G polymorphisms and HLA-G expression in sarcoidosis

    DEFF Research Database (Denmark)

    Hviid, TVF; Milman, N; Hylenius, S

    2006-01-01

    HLA-G expression was investigated by immunohistochemistry in granulomas from sarcoidosis patients, weak HLA-G expression was observed in only one patient. CONCLUSIONS: HLA-G alleles that include a 14-bp sequence polymorphism in exon 8 of the HLA-G gene are observed more often in sarcoidosis patients......BACKGROUND: The MHC class Ib molecule Human Leukocyte Antigen (HLA)-G may be important in induction and maintenance of immunological tolerance, and HLA-G expression may have a role in different cancers, in certain diseases with associations to HLA, and in organ transplantation. Sarcoidosis...... is a systemic granulomatous disease with unknown etiology but at the molecular level several studies have shown HLA associations. METHODS: In the present study, HLA-G alleles/polymorphisms were studied in sarcoidosis patients (n = 47) and controls (n = 129) by PCR techniques and HLA-G protein expression...

  13. Pulmonary hypertension in end-stage pulmonary sarcoidosis: therapeutic effect of sildenafil?

    DEFF Research Database (Denmark)

    Milman, N.; Burton, C.M.; Iversen, M.

    2008-01-01

    BACKGROUND: The objectives of this study were to assess the frequency and severity of pulmonary hypertension (PH) and the effect of sildenafil treatment in patients with recalcitrant pulmonary sarcoidosis. METHODS: This investigation was a single-center, retrospective study of all patients (n = 25......) with end-stage pulmonary sarcoidosis referred for lung transplantation. Hemodynamic measurements were evaluated by right-side cardiac catheterization in 24 of 25 patients. Sildenafil treatment for patients with sarcoidosis-associated PH was introduced in April 2004. RESULTS: The study group of 24 patients...... (16 men, 8 women) had a median age of 45 (range 35 to 58) years, and duration of sarcoidosis of 11 (range 2 to 38) years. Mean pulmonary arterial pressure (MPAP) was median 36 (range 18 to 73) mm Hg. PH (MPAP >25 mm Hg) was present in 19 of 24 patients (79%). Sildenafil was administered to 12 of 13...

  14. Oral manifestation of sarcoidosis: A case report and review of the literature

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    Vladimíra Radochova

    2016-01-01

    Full Text Available Sarcoidosis is a common multisystemic granulomatous disease characterized by the formation of nonnecrotizing epithelioid granulomas in various body organs. The most commonly affected organs in the head-and-neck region include salivary glands and cervical lymph nodes. Involvement of the oral mucosa is very rare. The purpose of this report is to present an atypical oral manifestation of sarcoidosis with ulcerations of the buccal mucosa in a 59-year-old female.

  15. Interleukin-2 Receptor and Angiotensin-Converting Enzyme as Markers for Ocular Sarcoidosis

    Science.gov (United States)

    Gundlach, Enken; Hoffmann, Michael Marcus; Prasse, Antje; Heinzelmann, Sonja; Ness, Thomas

    2016-01-01

    Purpose To study the impact of soluble IL2 receptor (sIL2R), chest x-ray (CxR), and angiotensin-converting enzyme (ACE) as markers for sarcoidosis in uveitis patients. Design Retrospective study. Methods Serum concentrations of sIL2R and ACE were measured in patients with active uveitis. Those with elevated sIL2R and /or ACE values were examined for suspected systemic sarcoidosis. Main Outcome Measure Our main outcome parameters were the specificity and sensitivity of sIL2R, CxR and ACE in screening for ocular sarcoidosis. Results We measured 261 patients with uveitis for sarcoidosis using sIL2R and ACE between January 2008 and November 2011; sarcoidosis was been diagnosed using other tests (e.g. computer tomography, brochoalveolar lavage, biopsy) in 41 of 53 patients with elevated sIL2R values (>639 U/ml) and in one patient with normal sIL2R (582 U/ml). Their mean sIL2R value was 1310 U/ml, extending from 582 to 8659 U/ml. Only 9 patients, however, presented elevated ACE (>82 U/l). Their mean ACE value was 116.4 U/l, ranging from 84.1 to 175.5 U/l. IL2R specificity was 94% with 98% sensitivity. In contrast, ACE had a specificity of 99.5%, but a sensitivity of only 22%; the chest x-ray had a specificity of 100% with 50% sensitivity in detecting sarcoidosis. We observed the entire spectrum of uveitis: sixteen patients suffered from anterior, 8 from intermediate, 16 from posterior, and 2 from panuveitis. Conclusions An elevated level of soluble IL2R suggests sarcoidosis with uveitis more convincingly than ACE, making sIL2R a more effective marker parameter for sarcoidosis than ACE or chest x-ray in uveitis patients. PMID:26799486

  16. Clinical Characteristics and Outcome of Hepatic Sarcoidosis: A Population-Based Study 1976-2013.

    Science.gov (United States)

    Ungprasert, Patompong; Crowson, Cynthia S; Simonetto, Douglas A; Matteson, Eric L

    2017-10-01

    Data on clinical manifestations and outcome of hepatic sarcoidosis are scarce. This study aimed to use a population-based cohort of patients with incident sarcoidosis to better describe the characteristics of hepatic sarcoidosis. A cohort of incident cases of sarcoidosis in Olmsted County, MN, USA, from 1976 to 2013 was identified from the database. Diagnosis was verified by individual medical record review. Confirmed cases of sarcoidosis were then reviewed for liver involvement. Data on clinical manifestations, imaging study, liver biochemical tests, treatment, and outcome were collected. Cumulative incidence of cirrhosis adjusted for the competing risk of death was estimated. A total of 345 cases of incident sarcoidosis were identified. Of these, 19 cases (6%) had liver involvement (mean age 46.1 years, 53% female and 79% Caucasian). Most patients had asymptomatic liver disease and were discovered in pursuit of abnormal biochemical tests and imaging studies. Alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) were elevated in the majority of patients (88 and 90%, respectively). Elevated transaminases were less common and less severe. About half of patients had abnormal imaging study with hypodense nodular lesions being the most common abnormality (six patients) followed by hepatomegaly (three patients). Liver biopsy revealed non-caseating granuloma in 88% (14 of 16 patients). A total of four patients developed cirrhosis. Involvement of the liver by sarcoidosis was seen in 6% of patients with sarcoidosis. The majority of patients were asymptomatic. Elevated ALP and GGT were the most common abnormal biochemical tests. Liver biopsy revealed non-caseating granuloma in almost all cases. Cirrhosis was seen in a significant number of patients. Generalizability of the observations to other populations may be limited, as the studied population was predominantly Caucasian. The prevalence of liver disease may be higher in more diverse populations.

  17. Endometrial Cancer with Sarcoidosis in Regional Lymph Nodes: A Case Report

    OpenAIRE

    Satoshi Tamauchi; Yuji Shimomura; Hiromi Hayakawa

    2015-01-01

    Sarcoidosis is a chronic, multisystemic disease commonly affecting the lungs and lymphatic system and is characterized by the formation of noncaseating granulomas. Although several reports are available on cases developing both sarcoidosis and cancer metachronously, cases of simultaneous diagnosis of these diseases have rarely been reported. A 67-year-old woman diagnosed with endometrial cancer had developed systemic lymph node swelling, including bilateral hilar, paraaortic, and a few pelvic...

  18. A Case of Sarcoidosis Associated With Anti–Tumor Necrosis Factor Treatment

    OpenAIRE

    Ayse Baha MD; Cigdem Hanazay MD; Nurdan Kokturk MD; Haluk Turktas MD

    2015-01-01

    Sarcoidosis is a systemic chronic granulomatous disease of unknown etiology. It predominantly involves the lungs but can affect many organs or tissues in the body, such as the lymphatic system, skin, eyes, and liver. Typical histopathological lesions are noncaseating granulomas in the affected organ or tissue. Indications, type of treatment, and duration of sarcoidosis treatment is currently debated. Despite studies showing that anti–tumor necrosis factor-α (TNF-α) treatment can successfully ...

  19. Transcriptional blood signatures distinguish pulmonary tuberculosis, pulmonary sarcoidosis, pneumonias and lung cancers.

    Directory of Open Access Journals (Sweden)

    Chloe I Bloom

    Full Text Available RATIONALE: New approaches to define factors underlying the immunopathogenesis of pulmonary diseases including sarcoidosis and tuberculosis are needed to develop new treatments and biomarkers. Comparing the blood transcriptional response of tuberculosis to other similar pulmonary diseases will advance knowledge of disease pathways and help distinguish diseases with similar clinical presentations. OBJECTIVES: To determine the factors underlying the immunopathogenesis of the granulomatous diseases, sarcoidosis and tuberculosis, by comparing the blood transcriptional responses in these and other pulmonary diseases. METHODS: We compared whole blood genome-wide transcriptional profiles in pulmonary sarcoidosis, pulmonary tuberculosis, to community acquired pneumonia and primary lung cancer and healthy controls, before and after treatment, and in purified leucocyte populations. MEASUREMENTS AND MAIN RESULTS: An Interferon-inducible neutrophil-driven blood transcriptional signature was present in both sarcoidosis and tuberculosis, with a higher abundance and expression in tuberculosis. Heterogeneity of the sarcoidosis signature correlated significantly with disease activity. Transcriptional profiles in pneumonia and lung cancer revealed an over-abundance of inflammatory transcripts. After successful treatment the transcriptional activity in tuberculosis and pneumonia patients was significantly reduced. However the glucocorticoid-responsive sarcoidosis patients showed a significant increase in transcriptional activity. 144-blood transcripts were able to distinguish tuberculosis from other lung diseases and controls. CONCLUSIONS: Tuberculosis and sarcoidosis revealed similar blood transcriptional profiles, dominated by interferon-inducible transcripts, while pneumonia and lung cancer showed distinct signatures, dominated by inflammatory genes. There were also significant differences between tuberculosis and sarcoidosis in the degree of their

  20. Increased risk of ventricular tachycardia in patients with sarcoidosis during the very long term follow-up.

    Science.gov (United States)

    Te, Abigail Louise D; Lin, Yenn-Jiang; Chen, Yun-Yu; Chung, Fa-Po; Chang, Shih-Lin; Lo, Li-Wei; Hu, Yu-Feng; Tuan, Ta-Chuan; Chao, Tze-Fan; Liao, Jo-Nan; Lin, Chin-Yu; Chang, Yao-Ting; Chien, Kuo-Liong; Chen, Shih-Ann

    2017-02-01

    Sarcoidosis is an important diagnostic consideration in patients with ventricular tachycardia (VT) of unknown origin. The clinical course of VT as the primary presentation in patients with sarcoidosis is mostly unknown. This study aimed to investigate the incidence of life-threatening VT and mortality during long term follow-up in patients with sarcoidosis. We analyzed the epidemiological features of sarcoidosis in Taiwan using the National Health Insurance Research Database from 2000 to 2004. Patients with sarcoidosis were identified, and healthy controls without prior histories of structural heart disease were matched with a 1:1 propensity-score to the sarcoidosis group. The risk of life-threatening VT and mortality with sarcoidosis was analyzed. A total of 2237 sarcoidosis cases were enrolled with a matching number of healthy controls, and the baseline characteristics between the two groups were similar. After a mean follow-up of 11.4±2.15years (IQR: 12, 11.3-12), the VT incidence in the sarcoidosis group was higher than in healthy controls (0.94% [85 per 100,000 person-year] in the sarcoidosis group, and 0.09% [8 per 100,000 person-year] in healthy controls). After a multivariate adjustment including the sex, age, and other comorbidities, the VT risk was still higher in the sarcoidosis group (hazard ratio: 12.7, 95% confidence interval: 2.82-56.9; PSarcoidosis may increase the predisposition to ventricular arrhythmias with a cumulative incidence of 0.94% during a very long term follow-up of nearly 10years from initially diagnosing sarcoidosis. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  1. [Sarcoidosis after adalimumab treatment in inflammatory rheumatic diseases: a report of two cases and literature review].

    Science.gov (United States)

    Scailteux, Lucie-Marie; Guedes, Claudie; Polard, Elisabeth; Perdriger, Aleth

    2015-01-01

    TNF α antagonists (anti-TNF α) are widely used in inflammatory rheumatic diseases: rheumatoid arthritis (RA) and spondylarthropathy (SpA). The efficacy of the anti-TNF α monoclonal antibodies was also observed in unresponsive sarcoidosis to conventional therapy. In contrast, sarcoidosis in patients with inflammatory rheumatic disease treated with anti-TNF α keep on growing, with a suspected role of anti-TNF α in this pathological process. We presented here two cases of sarcoidosis developing while the patient was on adalimumab (ADA) therapy for inflammatory rheumatic disease. In one case, the reintroduction of ADA led to increase in symptomatology. We also analyzed the 16 other cases of sarcoidosis developing under ADA treatment published in literature, mostly in RA patients. These cases show a possible paradoxical effect of ADA in sarcoidosis development in patients treated with anti-TNFα monoclonal antibodies. The iatrogenic mechanism remains unclear. These cases underline the importance of a drug-induced etiology survey facing any symptomatology suggesting the development of sarcoidosis in patients treated with anti-TNF α for an inflammatory rheumatic disease. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  2. Scar sarcoidosis on a finger mimicking a rapidly growing soft tissue tumour: a case report

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    Henrichs Marcel-Philipp

    2012-10-01

    Full Text Available Abstract Background Scar sarcoidosis is a rare and uncommon but specific cutaneous manifestation of sarcoidosis. In general it arises in pre-existing scars deriving from mechanical traumas. As most surgeons dealing with scars might not be aware of cutaneous sarcoidosis and its different types of appearance the appropriate staging and treatment might be missed or at least delayed. To our knowledge this is the first case in literature of scar sarcoidosis on a finger. Case presentation We present a case of a 33-year-old carpenter who developed scar sarcoidosis on his right index finger 4 years after the tendon of the long digital flexor got accidentally cut by an angle grinder. He was referred due to a swelling of the finger suspected to be a malignant soft tissue tumour. The circumference of the affected finger had almost doubled, adding up to 94 mm. Incision biopsy revealed typical noncaseating granulomas. Further investigation showed a systemic extent of the disease with involvement of the lung. A systemic treatment with oral steroids led to an almost full regression of the swelling with restoration of function and resolution of lung infiltrates. Conclusion In case of a suspicious and/or progressive swelling a definite diagnosis should be achieved by biopsy within a short time to enable a proper treatment. If scar sarcoidosis is proven further investigation is necessary to exclude a systemical involvement. A surgical treatment of the swelling is not indicated.

  3. Scar sarcoidosis on a finger mimicking a rapidly growing soft tissue tumour: a case report.

    Science.gov (United States)

    Henrichs, Marcel-Philipp; Streitbürger, Arne; Gosheger, Georg; Surke, Carsten; Dierkes, Christian; Hardes, Jendrik

    2012-10-02

    Scar sarcoidosis is a rare and uncommon but specific cutaneous manifestation of sarcoidosis. In general it arises in pre-existing scars deriving from mechanical traumas. As most surgeons dealing with scars might not be aware of cutaneous sarcoidosis and its different types of appearance the appropriate staging and treatment might be missed or at least delayed. To our knowledge this is the first case in literature of scar sarcoidosis on a finger. We present a case of a 33-year-old carpenter who developed scar sarcoidosis on his right index finger 4 years after the tendon of the long digital flexor got accidentally cut by an angle grinder. He was referred due to a swelling of the finger suspected to be a malignant soft tissue tumour. The circumference of the affected finger had almost doubled, adding up to 94 mm. Incision biopsy revealed typical noncaseating granulomas. Further investigation showed a systemic extent of the disease with involvement of the lung. A systemic treatment with oral steroids led to an almost full regression of the swelling with restoration of function and resolution of lung infiltrates. In case of a suspicious and/or progressive swelling a definite diagnosis should be achieved by biopsy within a short time to enable a proper treatment. If scar sarcoidosis is proven further investigation is necessary to exclude a systemical involvement. A surgical treatment of the swelling is not indicated.

  4. Differential Expression of MicroRNA and Predicted Targets in Pulmonary Sarcoidosis

    Science.gov (United States)

    Crouser, Elliott D.; Julian, Mark W.; Crawford, Melissa; Shao, Guohong; Yu, Lianbo; Planck, Stephen R.; Rosenbaum, James T.; Nana-Sinkam, S. Patrick

    2014-01-01

    Background Recent studies show that various inflammatory diseases are regulated at the level of RNA translation by small non-coding RNAs, termed microRNAs (miRNAs). We sought to determine whether sarcoidosis tissues harbor a distinct pattern of miRNA expression and then considered their potential molecular targets. Methods and Results Genome-wide microarray analysis of miRNA expression in lung tissue and peripheral blood mononuclear cells (PBMCs) was performed and differentially expressed (DE)-miRNAs were then validated by real-time PCR. A distinct pattern of DE-miRNA expression was identified in both lung tissue and PBMCs of sarcoidosis patients. A subgroup of DE-miRNAs common to lung and lymph node tissues were predicted to target transforming growth factor (TGFβ)-regulated pathways. Likewise, the DE-miRNAs identified in PBMCs of sarcoidosis patients were predicted to target the TGFβ-regulated “wingless and integrase-1” (WNT) pathway. Conclusions This study is the first to profile miRNAs in sarcoidosis tissues and to consider their possible roles in disease pathogenesis. Our results suggest that miRNA regulate TGFβ and related WNT pathways in sarcoidosis tissues, pathways previously incriminated in the pathogenesis of sarcoidosis. PMID:22209793

  5. Upregulation of neurokinin-1 receptor expression in the lungs of patients with sarcoidosis.

    LENUS (Irish Health Repository)

    O'Connor, Terence M

    2012-02-03

    Substance P (SP) is a proinflammatory neuropeptide that is secreted by sensory nerves and inflammatory cells. Increased levels of SP are found in sarcoid bronchoalveolar lavage fluid. SP acts by binding to the neurokinin-1 receptor and increases secretion of tumor necrosis factor-alpha in many cell types. We sought to determine neurokinin-1 receptor expression in patients with sarcoidosis compared with normal controls. Neurokinin-1 receptor messenger RNA and protein expression were below the limits of detection by reverse transcriptase-polymerase chain reaction and immunohistochemistry in peripheral blood mononuclear cells of healthy volunteers (n = 9) or patients with stage 1 or 2 pulmonary sarcoidosis (n = 10), but were detected in 1\\/9 bronchoalveolar lavage cells of controls compared with 8\\/10 patients with sarcoidosis (p = 0.012) and 2\\/9 biopsies of controls compared with 9\\/10 patients with sarcoidosis (p = 0.013). Immunohistochemistry localized upregulated neurokinin-1 receptor expression to bronchial and alveolar epithelial cells, macrophages, lymphocytes, and sarcoid granulomas. The patient in whom neurokinin-1 receptor was not detected was taking corticosteroids. Incubation of the type II alveolar and bronchial epithelial cell lines A549 and SK-LU 1 with dexamethasone downregulated neurokinin-1 receptor expression. Upregulated neurokinin-1 receptor expression in patients with sarcoidosis may potentiate substance P-induced proinflammatory cytokine production in patients with sarcoidosis.

  6. Imbalance between Th17 and Regulatory T-Cells in Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Hui Huang

    2013-10-01

    Full Text Available Sarcoidosis is a systemic granulomatous disease, which is thought to result from an aberrant immune response. CD4+ T lymphocytes play an important role in the development of granulomas. Previously, the immunopathogenesis of sarcoidosis was focused on Th1/Th2 disturbances. The aim of this study was to evaluate the balance between newer CD4+ T lymphocytes, i.e., Treg and Th17 cells. In our studies, a decrease in Treg cells and an increase in Th17 cells were observed in the peripheral blood and BALF of sarcoidosis patients. A significant increase in the Th17/Treg cell ratio was observed in sarcoidosis patients. After treatment with prednisone, the expression of Foxp3 mRNA was elevated in the peripheral blood, and expression of (RORγt mRNA showed a downward trend. These findings suggest that sarcoidosis is associated with an imbalance between Th17 and Treg cells in peripheral blood and BALF. Therefore, targeting the cytokines that affect the Th17/Treg ratio could provide a new promising therapy for pulmonary sarcoidosis.

  7. Sublethal consequences of urban life for wild vertebrates

    National Research Council Canada - National Science Library

    Gallagher, Austin J; Peiman, Kathryn S; de Bruijn, Robert; Cooke, Steven J; Birnie-Gauvin, Kim

    2016-01-01

    ... — while others have not. Here we present a review of the sublethal consequences of life in the city for wild vertebrates, and demonstrate that urban animals face an almost completely different set of physiological...

  8. Sarcoidosis and Histoplasmosis: Is One a Consequence of the Other? A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anupam Bansal

    2015-01-01

    Full Text Available Sarcoidosis involves abnormal collections of inflammatory cells (granulomas which may form as nodules in multiple organs. 90% of affected patients have respiratory tract abnormalities. We present a 61-year-old male with sarcoidosis who was admitted for respiratory distress. Fibrosing mediastinitis was seen in the chest computograph. Management was conservative and included steroids, antibiotics, and oxygen therapy. Sarcoidosis and fibrosing mediastinitis are rare. Fibrosing mediastinitis is more commonly seen with histoplasmosis. We explore the clinical similarities between histoplasmosis and sarcoidosis. We also explore the potential cause and effect relationship and workup for each disease entity.

  9. Routine needle biopsy during vertebral augmentation procedures. Is it necessary?

    Science.gov (United States)

    Pneumaticos, Spiros G; Chatziioannou, Sofia N; Savvidou, Christiana; Pilichou, Anastasia; Rontogianni, Dimitra; Korres, Dimitrios S

    2010-11-01

    Vertebral augmentation procedures are currently widely performed to treat vertebral compression fractures. The purpose of this study was to determine the frequency of underlying previously unrecognized etiology in a consecutive series of patients undergoing kyphoplasty to treat vertebral compression fractures. A prospective histological evaluation of vertebral body biopsy specimens from presumed osteoporotic vertebral compression fractures were performed in order to identify aforementioned causes. Over a 2-year period, vertebral body biopsies from 154 vertebral levels were performed in 75 patients undergoing kyphoplasty for vertebral compression fractures. All patients received a preoperative workup that included plain radiographs, MRI, whole body bone scan, and laboratory examinations. Bone specimens were obtained from affected vertebral bodies and submitted for histologic evaluation to identify the prevalence of an underlying cause. All specimens demonstrated fragmented bone with variable amounts of unmineralised bone, signs of bone-remodeling and/or fracture-healing. In 11 patients underlying pathology other than osteoporosis was identified (prostate cancer, 1; pancreatic cancer, 1; colon cancer, 1; breast cancer, 2; multiple myeloma, 3; leukemia, 1; and lung cancer, 2). In all but one patient the results of the biopsy confirmed the diagnosis suspected from the preoperative workup. For the last patient, namely the one with pancreatic cancer, the workup did not identify the origin of the primary tumor, although the patient was considered to have a compression fracture secondary to metastatic disease of unknown origin, the vertebral biopsy suggested the presence of adenocarcinoma which eventually was proven to be pancreatic cancer. In augmentation procedures for vertebral compression fractures, bone biopsy should be reserved for the patients where the preoperative evaluation raises the suspicion of a non-osteoporotic etiology.

  10. Humoral immune profiling of mycobacterial antigen recognition in sarcoidosis and Löfgren’s syndrome using high-content peptide microarrays

    Directory of Open Access Journals (Sweden)

    Giovanni Ferrara

    2017-03-01

    Conclusions: Specific IgG recognition patterns for M. tuberculosis antigens in sarcoidosis patients re-affirm mycobacterial involvement in sarcoidosis, providing biologically relevant targets for future studies pertaining to diagnostics and immunotherapy.

  11. Isolated dysphagia unmasking bulbar neurosarcoidosis and pulmonary sarcoidosis.

    Science.gov (United States)

    Abdallah, Tarek; Abdallah, Mokhtar; Elsayegh, Dany; Chalhoub, Michel; Khoueiry, Georges; Glatman, Alex; Maniatis, Theodore

    2014-06-01

    Dysphagia is a rare manifestation of sarcoidosis. It is more commonly the result of esophageal compression by enlarged mediastinal lymph nodes rather than direct esophageal involvement and rarely secondary to neurosarcoidosis and oropharyngeal dysphagia. We report a 54 year old female presenting with a six month history of worsening dysphagia. She denied respiratory symptoms. Physical exam was normal. ESR was 61 mm/hr. Serum ACE level was 65 mcg/L. Chest X-ray was normal. Esophagram revealed a large amount of contrast pooling in pharyngeal recesses with intermittent laryngeal aspiration. Swallow videofluorography showed a decreased retraction of the base of the tongue, limited laryngeal elevation, and a large amount of contrast pooling in pharyngeal recesses with intermittent laryngeal aspiration. EGD showed a normal opening of the upper esophageal sphincter and the cricopharyngeus appeared normal. Proximal esophageal biopsies were normal. Brain MRI with gadolinium was normal. Lumbar puncture was performed. CSF showed a moderate pleocytosis, a WBC count of 19 with 97% lymphocytes, an elevated total protein level of 85 mg/dl (15-60). Neck CT scan showed no oropharyngeal tissue thickening or infiltration, no masses or enlarged lymph nodes. Chest CT scan showed enlarged intrathoracic lymph nodes and no esophageal compression. Bronchoscopy showed the vocal cords to be intact, and the CD4/CD8 ratio in BAL was 5.3. Subcarinal lymph node EBUS biopsy revealed non caseating granulomas. The patient was started on IV methylprednisolone. Three days later, the swallow videofluorography showed a near complete response to steroids. The patient tolerated regular consistency diet with thin liquids, and she was discharged on a slow taper of prednisone over a period of three months. A unique case of isolated dysphagia unmasking bulbar neurosarcoidosis and pulmonary sarcoidosis is herein reported. Copyright © 2014 Arab Journal of Gastroenterology. Published by Elsevier Ltd. All

  12. Unsuspected exposure to beryllium: potential implications for sarcoidosis diagnoses.

    Science.gov (United States)

    Laczniak, Andrew N; Gross, Nathan A; Fuortes, Laurence J; Field, R William

    2014-07-21

    Exposure to Beryllium (Be) can cause sensitization (BeS) and chronic beryllium disease (CBD) in some individuals.  Even relatively low exposures may be sufficient to generate an asymptomatic, or in some cases a symptomatic, immune response. Since the clinical presentation of CBD is similar to that of sarcoidosis, it is helpful to have information on exposure to beryllium in order to reduce misdiagnosis. The purpose of this pilot study is to explore the occurrence of Be surface deposits at worksites with little or no previous reported use of commercially available Be products.  The workplaces chosen for this study represent a convenience sample of businesses in eastern Iowa. One hundred thirty-six surface dust samples were collected from 27 businesses for analysis of Be. The results were then divided into categories by the amount of detected Be according to U.S. Department of Energy guidelines as described in 10 CFR 850.30 and 10 CFR 850.31. Overall, at least one of the samples at 78% of the work sites tested contained deposited Be above the analytical limit of quantitation (0.035 µg beryllium per sample).  Beryllium was detected in 46% of the samples collected. Twelve percent of the samples exceeded 0.2 µg/100 cm² and 4% of the samples exceeded a Be concentration of 3 µg/100 cm². The findings from this study suggest that there may be a wider range and greater number of work environments that have the potential for Be exposure than has been documented previously.  These findings could have implications for the accurate diagnosis of sarcoidosis.

  13. Proteomic Analysis of Kveim Reagent Identifies Targets of Cellular Immunity in Sarcoidosis.

    Science.gov (United States)

    Eberhardt, Christian; Thillai, Muhunthan; Parker, Robert; Siddiqui, Nazneen; Potiphar, Lee; Goldin, Rob; Timms, John F; Wells, Athol U; Kon, Onn M; Wickremasinghe, Melissa; Mitchell, Donald; Weeks, Mark E; Lalvani, Ajit

    2017-01-01

    Kveim-reagent (Kv) skin testing was a historical method of diagnosing sarcoidosis. Intradermal injection of treated sarcoidosis spleen tissue resulted in a granuloma response at injection site by 4-6 weeks. Previous work indicates proteins as the possible trigger of this reaction. We aimed to identify Kv-specific proteins and characterise the ex vivo response of Peripheral Blood Mononuclear Cells (PBMCs) from sarcoidosis, tuberculosis and healthy control patients when stimulated with both Kv and selected Kv-specific proteins. Kv extracts were separated by 1D-SDS-PAGE and 2D-DIGE and then underwent mass spectrometric analysis for protein identification. Sarcoidosis and control PBMCs were first stimulated with Kv and then with three selected recombinant protein candidates which were identified from the proteomic analysis. PBMC secreted cytokines were subsequently measured by Multiplex Cytokine Assay. We observed significantly increased IFN-γ and TNF-α secretion from Kv-stimulated PBMCs of sarcoidosis patients vs. PBMCs from healthy volunteers (IFN-γ: 207.2 pg/mL vs. 3.86 pg/mL, p = 0.0018; TNF-α: 2375 pg/mL vs. 42.82 pg/mL, p = 0.0003). Through proteomic approaches we then identified 74 sarcoidosis tissue-specific proteins. Of these, 3 proteins (vimentin, tubulin and alpha-actinin-4) were identified using both 1D-SDS-PAGE and 2D-DIGE. Data are available via ProteomeXchange with identifier PXD005150. Increased cytokine secretion was subsequently observed with vimentin stimulation of sarcoidosis PBMCs vs. tuberculosis PBMCs (IFN-γ: 396.6 pg/mL vs 0.1 pg/mL, p = 0.0009; TNF-α: 1139 pg/mL vs 0.1 pg/mL, psarcoidosis PBMCs compared to PBMCs from healthy controls (IFN-γ: 396.6 pg/mL vs. 0.1 pg/mL, p = 0.014; TNF-α: 1139 pg/mL vs 42.29 pg/mL, p = 0.027). No difference was found in cytokine secretion between sarcoidosis and control PBMCs when stimulated with either tubulin or alpha-actinin-4. Stimulation with both Kveim reagent and vimentin induces a specific pro

  14. Diagnosis and Management of Vertebral Compression Fractures.

    Science.gov (United States)

    McCarthy, Jason; Davis, Amy

    2016-07-01

    Vertebral compression fractures (VCFs) are the most common complication of osteoporosis, affecting more than 700,000 Americans annually. Fracture risk increases with age, with four in 10 white women older than 50 years experiencing a hip, spine, or vertebral fracture in their lifetime. VCFs can lead to chronic pain, disfigurement, height loss, impaired activities of daily living, increased risk of pressure sores, pneumonia, and psychological distress. Patients with an acute VCF may report abrupt onset of back pain with position changes, coughing, sneezing, or lifting. Physical examination findings are often normal, but can demonstrate kyphosis and midline spine tenderness. More than two-thirds of patients are asymptomatic and diagnosed incidentally on plain radiography. Acute VCFs may be treated with analgesics such as acetaminophen, nonsteroidal anti-inflammatory drugs, narcotics, and calcitonin. Physicians must be mindful of medication adverse effects in older patients. Other conservative therapeutic options include limited bed rest, bracing, physical therapy, nerve root blocks, and epidural injections. Percutaneous vertebral augmentation, including vertebroplasty and kyphoplasty, is controversial, but can be considered in patients with inadequate pain relief with nonsurgical care or when persistent pain substantially affects quality of life. Family physicians can help prevent vertebral fractures through management of risk factors and the treatment of osteoporosis.

  15. Vertebral contour in spondylolisthesis

    Energy Technology Data Exchange (ETDEWEB)

    Lee, J. S.; Rho, J. C.; Park, J. H.; Choi, H. Y.; Kim, B. K. [Wallace memorial Baptist Hospital, Busan (Korea, Republic of)

    1981-09-15

    The defect in the pars interarticularis of spondylolisthesis may be dependent on contributing factors related to trauma and stress to which the neural arch is subjected, superimposed on a hereditary diasthesis. Posterior wedging of 5th lumber vertebral body in lumbosacral spondylolisthesis together with the degree of slip have been measured. The average wedging in spondylolisthesis is significantly greater than patient without this condition, and forms a characteristic radiological sign. The degree of wedging and slip show a statistically valid correlation. The diagnosis of spondylolisthesis is becoming more prevalent as the complexity of our society result in the increasing use of roentgenography of the lumbar spine. Isolated lateral deviation and rotation of spinous process seen in anteroposterior radiographs of the lumbar spine seems to be associated with pathology in the pars interarticularis.

  16. In-Hospital Disease Burden of Sarcoidosis in Switzerland from 2002 to 2012

    Science.gov (United States)

    Pohle, Susanne; Baty, Florent; Brutsche, Martin

    2016-01-01

    Sarcoidosis is a multisystem disease with an unpredictable and sometimes fatal course while the underlying pathomechanism is still unclear. Reasons of the increasing hospitalization rate and mortality in the United States remain in dispute but incriminated are a number of distinct comorbidities and risk factors as well as the application of more aggressive therapeutic agents. Studies reflecting the recent development in central Europe are lacking. Our aim was to investigate the recent mortality and hospitalization rates as well as the underlying comorbidities of hospitalized sarcoidosis patients in Switzerland. In this longitudinal, nested case-control study, a nation-wide database provided by the Swiss Federal Office for Statistics enclosing every hospital entry covering the years 2002–2012 (n = 15,627,573) was analyzed. There were 8,385 cases with a diagnosis of sarcoidosis representing 0.054% (8,385 / 15,627,573) of all hospitalizations in Switzerland. These cases were compared with age- and sex-matched controls without the diagnosis of sarcoidosis. Hospitalization and mortality rates in Switzerland remained stable over the observed time period. Comorbidity analysis revealed that sarcoidosis patients had significantly higher medication-related comorbidities compared to matched controls, probably due to systemic corticosteroids and immunosuppressive therapy. Sarcoidosis patients were also more frequently re-hospitalized (median annual hospitalization rate 0.28 [IQR 0.15-0.65] vs. 0.19 [IQR 0.13-0.36] per year; p sarcoidosis patients having co-occurrence of associated respiratory diseases. Moreover, age was an important risk factor for re-hospitalization. PMID:26987059

  17. T wave amplitude in lead aVR as a novel diagnostic marker for cardiac sarcoidosis.

    Science.gov (United States)

    Tanaka, Yoshihiro; Konno, Tetsuo; Yoshida, Shohei; Tsuda, Toyonobu; Sakata, Kenji; Furusho, Hiroshi; Takamura, Masayuki; Yoshimura, Kenichi; Yamagishi, Masakazu; Hayashi, Kenshi

    2017-03-01

    It is vital to identify cardiac involvement (CI) in patients with sarcoidosis as the condition could initially lead to sudden cardiac death. Although the T wave amplitude in lead aVR (TWAaVR) is reportedly associated with adverse cardiac events in various cardiovascular diseases, only scarce data are available concerning the utility of lead aVR in identifying CI in patients with sarcoidosis. We retrospectively investigated the diagnostic values of TWAaVR in patients with sarcoidosis in comparison with conventional electrocardiography parameters such as bundle branch block (BBB). From January 2006 to December 2014, 93 consecutive patients with sarcoidosis were enrolled (mean age, 55.7 ± 15.7 years; male, 31 %; cardiac involvement, n = 26). TWAaVR showed the greatest sensitivity (39 %) and specificity (92 %) in distinguishing between sarcoidosis patients with and without CI, at a cutoff value of -0.08 mV. The diagnostic value of BBB for cardiac involvement was significantly improved when combined with TWAaVR (sensitivity: 61-94 %, specificity: 97-89 %, area under the curve: 0.79-0.92, p = 0.018). Multivariate logistic regression analysis indicated that TWAaVR and BBB were independent electrocardiography parameters associated with CI. In summary, we observed that sarcoidosis patients exhibiting a high TWAaVR were likely to have CI. Thus, the application of a combination of BBB with TWAaVR may be useful when screening for CI in sarcoidosis patients.

  18. Screening for Differentially Expressed Proteins Relevant to the Differential Diagnosis of Sarcoidosis and Tuberculosis.

    Directory of Open Access Journals (Sweden)

    Shan-Shan Du

    Full Text Available In this study, we sought to identify differentially expressed proteins in the serum of patients with sarcoidosis or tuberculosis and to evaluate these proteins as markers for the differential diagnosis of sarcoidosis and sputum-negative tuberculosis.Using protein microarrays, we identified 3 proteins exhibiting differential expression between patients with sarcoidosis and tuberculosis. Elevated expression of these proteins was verified using the enzyme-linked immunosorbent assay (ELISA and was further confirmed by immunohistochemistry. Receiver operating characteristic (ROC curve, logistic regression analysis, parallel, and serial tests were used to evaluate the diagnostic efficacy of the proteins.Intercellular Adhesion Molecule 1(ICAM-1 and leptin were screened for differentially expressed proteins relevant to sarcoidosis and tuberculosis. Using ROC curves, we found that ICAM-1 (cutoff value: 57740 pg/mL had an area under the curve (AUC, sensitivity, and specificity of 0.718, 62.3%, and 79.5% respectively, while leptin (cutoff value: 1193.186 pg/mL had an AUC, sensitivity, and specificity of 0.763, 88.3%, and 65.8%, respectively. Logistic regression analysis revealed that the AUC, sensitivity, and specificity of combined leptin and ICAM-1 were 0.787, 89.6%, and 65.8%, respectively, while those of combined leptin, ICAM-1, and body mass index (BMI were 0.837, 90.9%, and 64.4%, respectively, which had the greatest diagnostic value. Parallel and serial tests indicated that the BMI-leptin parallel with the ICAM-1 serial was the best diagnostic method, achieving a sensitivity and specificity of 86.5% and 73.1%, respectively. Thus, our results identified elevated expression of ICAM-1 and leptin in serum and granulomas of sarcoidosis patients.ICAM-1 and leptin were found to be potential markers for the diagnosis of sarcoidosis and differential diagnosis of sarcoidosis and sputum-negative tuberculosis.

  19. Functional imaging in extrapulmonary sarcoidosis: FDG-PET/CT and MR features.

    Science.gov (United States)

    Soussan, Michael; Augier, Alexandre; Brillet, Pierre-Yves; Weinmann, Pierre; Valeyre, Dominique

    2014-02-01

    Sarcoidosis is a multiorgan granulomatous disease of unknown etiology that primarily involves the lungs and the lymphatic system. Extrapulmonary sarcoidosis is common, occurring in 30 to 50% of patients. In this review, we describe and illustrate the role of F-FDG PET/CT and MR imaging in patients with extrapulmonary sarcoidosis. FDG-PET/CT and MR can improve the accuracy of the diagnosis of extrapulmonary involvement, specify the respective contributions of active and fibrotic components of lesions, guide the selection of the biopsy site, provide prognostic information, and guide therapeutic management. We focus on suggestive patterns that help to improve lesion characterization, especially when these lesions are clinically occult. In cardiac sarcoidosis, the combined use of FDG-PET/CT and cardiac MR may provide optimal detection of the disease by enabling the differentiation between patients with active granulomatous inflammation and those with fibrous lesions. In cases with central nervous system involvement, the T2 hypointensity of the dural and parenchymal lesions is helpful for identifying sarcoidosis. Granulomatous bone marrow infiltration in the axial skeleton can be sensitively detected by both FDG-PET/CT and MR. Muscular sarcoidosis can have a characteristic appearance with the "dark star" sign on MR and a thick linear FDG uptake that predominantly involves the lower legs, designated as the "tiger man" sign. Extrathoracic lymphadenopathy is commonly observed on FDG-PET/CT imaging; however, its features are not specific, and the differentiation of extrathoracic lymphadenopathy from metastatic disease, tuberculosis, or lymphoma may be difficult. Familiarity with the functional imaging features in extrapulmonary sarcoidosis in various anatomical locations plays a crucial role in the diagnosis and management of patients.

  20. A conceptual model of health-related quality of life in sarcoidosis.

    Science.gov (United States)

    Victorson, David E; Cella, David; Grund, Heidi; Judson, Marc A

    2014-02-01

    Sarcoidosis is a multisystem disease that commonly affects the lung, eye, skin, and lymphatic systems. Organ function has been a major focus of treatment outcome with less attention given to more subjective impacts, such as health-related quality of life (HRQL). The purpose of this paper is to present a conceptual model of HRQL in sarcoidosis, which was developed through patient and clinician input. We surveyed sarcoidosis clinical experts (n = 5) regarding disease-specific symptoms and their impact on their patient's lives. We also conducted three sarcoidosis patient focus groups (n = 22) that reflected major sarcoidosis typologies (lung, skin, and eye). Data were coded and summarized using qualitative methodologies. Clinicians highlighted the following domains as being important (relative frequencies for comments are in parentheses): emotional distress (17%), lung problems (14%), pain (14%), physical limitations (14%), fatigue (10%), social limitations (10%), eye problems (7%), skin problems (7%), sleep disturbance (3%), and constitutional symptoms (3%). Similarly, patients highlighted the following domains: social limitations (14%), skin problems (12%), pain (10%), coping (10%), emotional distress (9%), lung problems (8%), eye problems (7%), negative impact of corticosteroids (7%), physical limitations (6%), fatigue (6%), sleep disturbance (3%), constitutional symptoms (2%), comorbidities (2%), other systems affected (2%), environmental factors (1%), and positive impact of corticosteroids (1%). Clinician and patient responses overlapped in several domains, including emotional distress, physical and social limitations, and sarcoidosis-specific impacts, such as eye, skin, and lung problems. These findings support the HRQL impact of sarcoidosis and provide the basis for a conceptual model which has the potential to inform new patient-reported outcomes measures for this population.

  1. Case of sarcoidosis in childhood. With special reference to serum ACE, serum lysozyme and WXGa scan as an index of disease activity in sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Suzuki, Goro

    1984-12-01

    The patient was an 8-years-old girl with bilateral hilar lymphadenopathy (BHL) on chest roentogenogram and negativity of tuberculin reaction, but without superficial lymphadenopathy. Serum angiotensin-converting enzyme (ACE) and serum lysozyme were elevated. So sarcoidosis was suspected and WXGa scan, liver biopsy and ophthalmologic examination were performed. WXGa scan disclosed the abnormal accumulation to BHL, but liver biopsy and ophthalmologic examination were negative. Taking into account absence of superficial lymphadenopaty and the patient's age, lymphnode biopsy and another invasive examinations were not performed. None of the examinations suggested malignant disease. Though histological diagnosis was failed, the results of noninvasive examinations as WXGa scan, serum ACE and serum lysozyme intensively supported the diagnosis of sarcoidosis. And after a while, with improvement of BHL and tuberculin reaction, WXGa scan, serum ACE and serum lysozyme were normalized and the inactive change of disease was considered. These noninvasive examinations would be repeatable and more sensitive. So the combination of these noninvasive examinations as WXGa scan, serum ACE and serum lysozyme was considered to be available to diagnosis of sarcoidosis and as an index of disease activity in sarcoidosis.

  2. Contribution the scintigraphy with gallium 67 in the sarcoidosis; Apport de la scintigraphie au gallium 67 dans la sarcoidose

    Energy Technology Data Exchange (ETDEWEB)

    Elbez, I.; Sellem, A.; Rejeb, O.; Elkadri, N.; Hammami, H. [Service de medecine nucleaire, hopital militaire, Tunis, (Tunisia)

    2009-05-15

    The objective is to show the interest of the scintigraphy with citrates of gallium 67 in the diagnosis and follow up of sarcoidosis injuries. The conclusions are that the scintigraphy with gallium 67 constitutes an imaging technique of good performance, non invasive in the diagnosis, le injuries evaluation and the sarcoidosis follow-up. (N.C.)

  3. Humoral immune profiling of mycobacterial antigen recognition in sarcoidosis and Löfgren's syndrome using high-content peptide microarrays.

    Science.gov (United States)

    Ferrara, Giovanni; Valentini, Davide; Rao, Martin; Wahlström, Jan; Grunewald, Johan; Larsson, Lars-Olof; Brighenti, Susanna; Dodoo, Ernest; Zumla, Alimuddin; Maeurer, Markus

    2017-03-01

    Sarcoidosis is considered an idiopathic granulomatous disease, although similar immunological and clinical features with tuberculosis (TB) suggest mycobacterial involvement in its pathogenesis. High-content peptide microarrays (HCPM) may help to decipher mycobacteria-specific antibody reactivity in sarcoidosis. Serum samples from patients with sarcoidosis, Löfgren's syndrome, and TB, as well as from healthy individuals (12/group), were tested on HCPM containing 5964 individual peptides spanning 154 Mycobacterium tuberculosis proteins displayed as 15-amino acid stretches. Inclusion/exclusion and significance analyses were performed according to published methods. Each study group recognized 68-78% M. tuberculosis peptides at least once. M. tuberculosis epitope recognition by sarcoidosis patient sera was 42.7%, and by TB patient sera was 39.1%. Seven and 16 peptides were recognized in 9/12 (75%) and 8/12 (67%) sarcoidosis patient sera but not in TB patient sera, respectively. Nine (75%) and eight (67%) out of twelve TB patient sera, respectively recognized M. tuberculosis peptides that were not recognized in sarcoidosis patient sera. Specific IgG recognition patterns for M. tuberculosis antigens in sarcoidosis patients re-affirm mycobacterial involvement in sarcoidosis, providing biologically relevant targets for future studies pertaining to diagnostics and immunotherapy. Copyright © 2017. Published by Elsevier Ltd.

  4. 18F-FDG PET, genotype-corrected ACE and sIL-2R in newly diagnosed sarcoidosis.

    NARCIS (Netherlands)

    Keijsers, R.G.; Verzijlbergen, F.J.; Oyen, W.J.G.; Bosch, J.M. van den; Ruven, H.J.; Velzen-Blad, H. van; Grutters, J.C.

    2009-01-01

    PURPOSE: Angiotensin-converting enzyme (ACE) and soluble interleukin-2 receptor (sIL-2R) are serological markers, widely used for determining sarcoidosis activity. (18)F-FDG PET has proven to be a sensitive technique in the imaging of sarcoidosis. The aim of this study was to determine sensitivity

  5. Tartrate-resistant acid phosphatase 5a in sarcoidosis: Further evidence for a novel macrophage biomarker in chronic inflammation

    Directory of Open Access Journals (Sweden)

    Yi-Ying Wu

    2014-06-01

    Conclusion: TRACP5a protein is expressed abundantly in the granulomatous tissues and may be elevated in a significant proportion of sarcoidosis patients. These findings further support our hypothesis that serum TRACP5a is derived from systemic inflammatory MΦ and thereby may be a biomarker of inflammation for sarcoidosis and also reflect its disease activity.

  6. PROGNOSTIC ALGORITHM FOR DISEASE FLOW IN PULMONARY AND THORACIC LYMPH NODES SARCOIDOSIS

    Directory of Open Access Journals (Sweden)

    S. A. Terpigorev

    2014-01-01

    Full Text Available Background: Sarcoidosis is a systemic granulomatosis commonly affecting respiratory system. Variable and often unpredictable flow of the disease provides rationale for the development of prognostic algorithm. Aim: To detect predictive parameters in pulmonary and thoracic lymph nodes sarcoidosis; to develop prognostic algorithm. Materials and methods: The results of examination of 113 patients (85 women and 28 men, 19–77 years old with morphologically verified sarcoidosis has been assessed. Clinical manifestations, functional, radiographic (including CT numerical scores and morphological features of the disease were analyzed against 3-year outcomes in prednisolon/hydroxychloroquine-treated or treatment-naive patients. Results: Radiographic stage, CT-pattern scores, several parameters of pulmonary function tests (DLCO, RV, FEV1, FVC and dyspnoe had the greatest prognostic significance for disease flow. Prognostic accuracy was 87.8% and increased to 94.5% after one-year dynamics of symptoms was taken into account. Therapy with systemic glucocorticosteroids did not influence outcomes in sarcoidosis with asymptomatic enlargement of thoracic lymph nodes. Conclusion: We have developed an algorithm for prognosis assessment in pulmonary sarcoidosis. Taking into account the results of patients follow-up significantly improves the accuracy of the prognosis.

  7. Early detection of cardiac involvement in sarcoidosis with 2-dimensional speckle-tracking echocardiography.

    Science.gov (United States)

    Schouver, Elie-Dan; Moceri, Pamela; Doyen, Denis; Tieulie, Nathalie; Queyrel, Viviane; Baudouy, Delphine; Cerboni, Pierre; Gibelin, Pierre; Leroy, Sylvie; Fuzibet, Jean-Gabriel; Ferrari, Emile

    2017-01-15

    Cardiac sarcoidosis (CS) is associated with high morbidity and sudden death. The absence of specific symptoms and lack of diagnostic gold standard technique is challenging. New imaging methods could improve the diagnosis of CS. The aim of our study was to assess the role of left ventricular (LV) longitudinal and circumferential strain as estimated by 2D speckle-tracking imaging in patients with diagnosed sarcoidosis without cardiac involvement according to the current guidelines. We investigated the prevalence of LV strain impairment in this population and assessed its relationship with clinical outcomes, composite of mortality, heart failure, arrhythmia and/or secondarily development of CS and cardiac device implantation. We performed a prospective case-control longitudinal study including 35 patients with diagnosed sarcoidosis and normal cardiac function as assessed by standard transthoracic echocardiography and 35 healthy age- and gender-matched controls. All patients underwent a comprehensive echocardiographic study. Mean age of patients was 47.9±14.8years old (22 women). Compared with controls, global LV longitudinal strain (LV GLS) was reduced in sarcoidosis patients: (-17.2±3.1 vs -21.3±1.5%, psarcoidosis patients that was associated with outcomes. LV GLS may represent an early marker of myocardial involvement in sarcoidosis patients that needs to be studied further. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  8. Prognostic Value of Myocardial Scarring on CMR in Patients With Cardiac Sarcoidosis.

    Science.gov (United States)

    Coleman, G Cameron; Shaw, Peter W; Balfour, Pelbreton C; Gonzalez, Jorge A; Kramer, Christopher M; Patel, Amit R; Salerno, Michael

    2017-04-01

    This study sought to perform a systematic review and meta-analysis to understand the prognostic value of myocardial scarring as evidenced by late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging in patients with known or suspected cardiac sarcoidosis. Although CMR is increasingly used for the diagnosis of cardiac sarcoidosis, the prognostic value of CMR has been less well described in this population. PubMed, Cochrane CENTRAL, and metaRegister of Controlled Trials were searched for CMR studies with ≥1 year of prognostic data. Primary endpoints were all-cause mortality and a composite outcome of arrhythmogenic events (ventricular arrhythmia, implantable cardioverter-defibrillator shock, sudden cardiac death) plus all-cause mortality during follow-up. Summary effect estimates were generated with random-effects modeling. Ten studies were included, involving a total of 760 patients with a mean follow-up of 3.0 ± 1.1 years. Patients had a mean age of 53 years, 41% were male, 95.3% had known extracardiac sarcoidosis, and 21.6% had known cardiac sarcoidosis. The average ejection fraction was 57.8 ± 9.1%. Patients with LGE had higher odds for all-cause mortality (odds ratio [OR]: 3.06; p sarcoidosis, the presence of LGE on CMR imaging is associated with increased odds of both all-cause mortality and arrhythmogenic events. Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  9. Diagnostic efficacy of ultrasound-guided core-needle biopsy of peripheral lymph nodes in sarcoidosis.

    Science.gov (United States)

    Boussouar, S; Medjhoul, A; Bernaudin, J F; Tayebjee, O; Soussan, M; Uzunhan, Y; Nunes, H; Kambouchner, M; Martin, A; Valeyre, D; Brillet, P Y

    2015-09-14

    Core-needle biopsy guided by ultrasound can be performed for investigating peripheral lymph node (PLN). The aim of this study was to determine the efficacy of this technique in sarcoidosis. Retrospective review of files of all patients in the database of the radiology department of Avicenne university hospital who underwent PLN biopsies guided by ultrasound from January 2008 to June 2011 (n=292). Cases with either granulomas at histology with the procedure or with a final diagnosis of sarcoidosis were included in the study. The histological specimens were adequate in 282 out of 292 cases (96%) showing non-caseating granulomas in 22 cases (n=20 patients with a final diagnosis of sarcoidosis and n=2 patients with tuberculosis). After reviewing clinical files of the 282 patient, 22 were confirmed to have sarcoidosis, at initial presentation (n=19) or later during flare-up or relapse (n=3) with only 2 patients having no granuloma on PLN biopsy. PLN were palpable in 18 cases and only detected by (18F)FDG-PET/CT showing increased PLN uptake in 4 cases. The sensitivity and specificity of adequate biopsy were 91 and 99% and the positive and negative predictive values were 91 and 99%, respectively. Core-needle biopsy guided by ultrasound has a high efficacy for evidencing granulomas in sarcoidosis patients with PLN involvement either clinically palpable or in the presence of (18F)FDG-PET/CT uptake.

  10. Sarcoidosis with renal damage: report of one case and literature review

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    Yan HUANG

    2013-09-01

    Full Text Available Objective To improve the ability of clinicians in the recognition, diagnosis and treatment of sarcoidosis with renal damage through analyzing the clinical data of one case of sarcoidosis with renal damage and literature review. Methods One case of sarcoidosis with renal damage was reported. The case history, clinical manifestations, pathological features, diagnosis and treatment of the patient were analyzed, and the related literature published domestically and abroad was reviewed to summarize the experiences in diagnosis and treatment of the disease. Results The patient was a 39 years old woman. The primary main clinical manifestations were cough and fever. Renal damage manifesting proteinuria and microscopic hematuria appeared one month later, and chest CT showed mediastinum and bilateral hilar lymphadenectasis. Renal and tracheal mucosa biopsy revealed noncaseating necrotized granuloma, so the diagnosis of sarcoidosis with renal damage was confirmed. Administration of glucocorticoid combined with cyclophosphamide achieved good therapeutic effect: hypercalciuria and proteinuria that lymph nodes in were abated with urinary calcium and protein returned to normal level, and microscopic hematuria disappeared. Chest CT showed mediastinum and hilum on both sides reduced significantly in size compared with those before the treatment. Conclusion  Sarcoidosis with renal damage is a rare disease with atypical clinical manifestations. Early diagnosis and timely treatment may achieve good effects.

  11. The emerging role of whole-body 18F-fluorodeoxyglucose positron emission tomography in patients with sarcoidosis

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    Silvia Taralli

    2012-01-01

    Full Text Available IntroductionThe purpose of this article is to examine the emerging role of whole-body positron emission tomography (PET with 18F-fluorodeoxyglucose (FDG in patients with sarcoidosis.Materials and methodsWe reviewed the literature on the use of FDG-PET in patients with sarcoidosis to identify how this technique is being applied in clinical practice.Results and discussionOur review shows that: 1 sarcoidosis is commonly associated with increased FDG uptake. Therefore, positive findings should be interpreted with caution when FDG-PET is being used to distinguish benign from malignant abnormalities; 2 FDG-PET seems to be a very useful molecular imaging method for staging sarcoidosis, identification of occult sites of involvement, guiding biopsy procedures, and monitoring patients’ responses to treatment; and 3 in patients with sarcoidosis, the diagnostic accuracy of FDG-PET is superior to that of 67Ga scintigraphy.

  12. A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease

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    Shinji Sato

    2014-01-01

    Full Text Available Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM with rapidly progressive interstitial lung disease (RP-ILD was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis.

  13. A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease

    Science.gov (United States)

    Nogi, Shinichi; Sasaki, Noriko; Chinen, Naofumi; Honda, Kiri; Saito, Eiko; Wakabayashi, Takayuki; Yamada, Chiho; Suzuki, Yasuo

    2014-01-01

    Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM) from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis. PMID:25431723

  14. Papulo-Nodular Reactions in Black Tattoos as Markers of Sarcoidosis: Study of 92 Tattoo Reactions from a Hospital Material.

    Science.gov (United States)

    Sepehri, Mitra; Hutton Carlsen, Katrina; Serup, Jørgen

    2016-01-01

    Sarcoidosis is, from historical data, suggested to be more prevalent among patients with tattoo reactions. We aimed to evaluate this association in a systematic study. This is a consecutive study of patients with tattoo complications, diagnosed in the "Tattoo Clinic" at Bispebjerg University Hospital in Copenhagen, Denmark, from 2008 to 2015, based on clinical assessment and histology. From the overall group of 494 tattoo complications in 406 patients, 92 reactions in 72 patients showed a papulo-nodular pattern studied for local and systemic sarcoidosis, since sarcoidosis is expected to be nodular. Of the 92 reactions with a papulo-nodular pattern, 27 (29%) reactions in 19 patients were diagnosed as cutaneous or systemic sarcoidosis, supported by histology; 65 (71%) were diagnosed as non-sarcoidosis due to histology and no clinical sarcoid manifestations. "Rush phenomenon" with concomitant reaction in many other black tattoos, triggered by a recent tattoo with a papulo-nodular reaction, was observed in 70% in the sarcoidosis group and 28% in the non-sarcoidosis group, indicating a predisposing factor which may be autoimmune and linked with sarcoidosis. Agglomerates of black pigment forming foreign bodies may in the predisposed individual trigger widespread reaction in the skin and internal organs. Black tattoos with papulo-nodular reactions should be seen as markers of sarcoidosis. Papulo-nodular reactions may, as triggers, induce widespread reactions in other black tattoos - a "rush phenomenon" - depending on individual predisposition. Sarcoidosis is estimated to be 500-fold increased in papulo-nodular reactions compared to the prevalence in the general population, and the association with black tattoos is strong. © 2017 S. Karger AG, Basel.

  15. Title: Toll-like receptor 3 L412F polymorphism promotes a persistent clinical phenotype in pulmonary sarcoidosis.

    Science.gov (United States)

    Cooke, Gordon; Kamal, Ijaz; Strengert, Monika; Hams, Emily; Mawhinney, Leona; Tynan, Aisling; O' Reilly, Ciaran; O' Dwyer, David N; Kunkel, Steven L; Knaus, Ulla G; Shields, Denis C; Moller, David R; Bowie, Andrew G; Fallon, Padraic G; Hogaboam, Cory M; Armstrong, Michelle E; Donnelly, Seamas C

    2017-12-11

    Sarcoidosis is a multisystemic disorder of unknown etiology, characterised by the presence of non-caseating granulomas in target organs. In ninety percent of cases, there is thoracic involvement. Fifty to seventy percent of pulmonary sarcoidosis patients will experience acute, self-limiting disease. For the subgroup of patients who develop persistent disease, no targeted therapy is currently available. To investigate the potential of the single nucleotide polymorphism (SNP), Toll-like receptor 3 Leu412Phe (TLR3 L412F; rs3775291), as a causative factor in the development of, and in disease persistence in pulmonary sarcoidosis. To investigate the functionality of TLR3 L412F in vitro in primary human lung fibroblasts from pulmonary sarcoidosis patients. Cohorts of Irish sarcoidosis patients (n = 228), healthy Irish controls (n = 263) and a secondary cohort of American sarcoidosis patients (n = 123) were genotyped for TLR3 L412F. Additionally, the effect of TLR3 L412F in primary lung fibroblasts from pulmonary sarcoidosis patients was quantitated following TLR3 activation in the context of cytokine and type I interferon production, TLR3 expression, and apoptotic- and fibroproliferative-responses. We report a significant association between TLR3 L412F and persistent clinical disease in two cohorts of Irish and American Caucasians with pulmonary sarcoidosis. Furthermore, activation of TLR3 in primary lung fibroblasts from 412F-homozygous pulmonary sarcoidosis patients resulted in reduced IFN-β and TLR3 expression, reduced apoptosis- and dysregulated fibroproliferative-responses compared with TLR3 wild-type patients. This study identifies defective TLR3 function as a previously unidentified factor in persistent clinical disease in pulmonary sarcoidosis and reveals TLR3 L412F as a candidate biomarker.

  16. Diminished activity of tartrate resistant acid phosphatase in alveolar macrophages from patients with active sarcoidosis.

    Science.gov (United States)

    Barth, J; Kreipe, H; Kiemle-Kallee, J; Radzun, H J; Parwaresch, M R; Petermann, W

    1988-11-01

    Alveolar macrophages differ from their percursors in blood, monocytes, by expressing strong activity of the tartrate resistant variant of acid phosphatase (TAcP). A study was carried out to analyse the expression of this enzyme cytochemical marker by alveolar macrophages from bronchoalveolar lavage cells from 34 patients with sarcoidosis and 12 control subjects. Alveolar macrophages from control subjects displayed a strong and homogeneous staining pattern and only 0.1% of cells were negative after staining. Macrophages from patients with sarcoidosis showed reduced TAcP activity and up to 7% of the cells were negative. The percentage of TAcP negative macrophages was correlated with the percentage of lymphocytes and with the ratio of CD4 to CD8 lymphocytes among cells recovered by bronchoalveolar lavage. The reduced TAcP activity in alveolar macrophages from patients with sarcoidosis may be due to an increased recruitment of immature precursors from blood.

  17. Sarcoidosis and sarcoid-like reaction following Hodgkin's disease. Report of two cases.

    Science.gov (United States)

    de Hemricourt, Erard; De Boeck, Koenraad; Hilte, Frank; Abib, Abdel; Kockx, Mark; Vandevivere, Johan; De Bock, Robrecht

    2003-01-01

    The occurrence of a sarcoidosis associated with Hodgkin's disease is an infrequent but well-described event. Moreover it has been described for many decades that some malignancies may present with simultaneous granulomatous manifestations named sarcoid-like reactions. To differentiate sarcoidosis from the sarcoid-like reactions is not an easy step in the final diagnosis. No author in the past has included the use of 2-deoxy-2[18F]fluoro-D-glucose positron emission tomography (FDG-PET) in order to help in this differentiation. We report two patients with Hodgkin's disease complicated with a typical form of sarcoidosis and with diffuse sarcoid-like reactions. We discuss the use of FDG-PET as a tool in the making of the final diagnosis and the difficulties associated with this technique in the interpretation of different hypermetabolic tissues.

  18. Genotyping in the MHC locus: potential for defining predictive markers in sarcoidosis

    Directory of Open Access Journals (Sweden)

    Seitzer Ulrike

    2001-10-01

    Full Text Available Abstract In sarcoidosis, host genetic factors are discussed as contributing to disease susceptibility and course. Since tumor necrosis factor (TNF-α is a central mediator of granuloma formation and since elevated TNF-α levels are found during active phases of sarcoidosis, genetic polymorphisms correlating with influences on TNF-α levels are of special interest. The complete sequencing of the MHC region and the increase in the number of identified gene polymorphisms in this locus associated with TNF-α production offer the opportunity of detecting new genes associated with sarcoidosis and perhaps of defining disease-associated haplotypes that bear the potential of serving as predictive markers for this disease.

  19. Multisystem sarcoidosis in a patient on interferon-α therapy for chronic hepatitis C

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    Bouchra Oudghiri

    2012-01-01

    Full Text Available Sarcoidosis is a chronic multisystemic granulomatous disease that is triggered by an autoimmune process. Nowadays, this pathology represents a well-recognized but uncommon complication for antiviral treatment in hepatitis C virus (HCV infection. Herein, we report a remarkable case of 47-year-old woman treated for chronic HCV infection; the patient has developed interferon alfa-induced sarcoidosis involving the central nervous system. The evolution was fatal despite disrupting the antiviral therapy and initiating a high-dose corticotherapy. This complication of interferon alfa treatment was reported in the literature in only one case. Through this case and a review of the literature, we aim to underline the importance of screening for sarcoidosis before and during the follow-up of HCV patients undergoing antiviral therapy.

  20. Acute kidney injury with granulomatous interstitial nephritis and vasculitis revealing sarcoidosis

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    Amel Harzallah

    2017-01-01

    Full Text Available Sarcoidosis is an inflammatory disease that affects mostly the lungs and lymph glands. Renal involvement is rare and especially vasculitis. We report a case who presented an acute kidney failure and had sarcoidosis with vasculitis and nodular splenic involvement. A 35-year-old woman presenting a Lofgren syndrome was hospitalized for acute renal failure with cervical lymphadenopathy without other clinical findings. Laboratory data disclosed elevated angiotensin converting enzyme serum level. Abdominal ultrasound showed a multinodular spleen. Renal histology revealed granulomatous interstitial nephritis with necrotizing vasculitis. Outcome was favorable after the institution of high dose corticosteroids along with cyclophosphamide. Renal involvement is rare in sarcoidosis. However, the diagnostic delay should be avoided to improve the outcome.

  1. Atypical radiological manifestations of thoracic sarcoidosis: A review and pictorial essay

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    Hamdan Al-Jahdali

    2013-01-01

    Full Text Available Thoracic sarcoidosis is a common disease, with well-described and recognizable radiographic features. Nevertheless, most physicians are not familiar with the rare atypical often-confusing manifestations of thoracic sarcoid. Although these findings have been previously reviewed, but more recent advances in imaging and laboratory science, need to be incorporated. We present a review of literature and illustrate the review with unpublished data, intended to provide a more recent single comprehensive reference to assist with the diagnosis when atypical radiographic findings of thoracic sarcoidosis are encountered. Thoracic involvement accounts for most of morbidity and mortality associated with sarcoidosis. An accurate timely identification is required to minimize morbidity and mortality. It is essential to recognize atypical imaging findings and relate these to clinical manifestations and histology.

  2. Vertebral Malformations in French Bulldogs

    OpenAIRE

    KURICOVÁ, Mária; LEDECKÝ, Valent; KVETKOVÁ, Jaroslava; LIPTÁK, Tomáš

    2017-01-01

    The aim of this study was to detect vertebral malformations among French Bulldogs admitted between the years 2011 – 2016 due to the high popularity of the breed and the intentions to increase the breed welfare by reducing the occurrence of congenital anomalies. Besides, we aimed to look for gender predisposition, possible vertebral predisposition, occurrence of clinical symptoms and radiographic findings. A total of 73 French Bulldogs met the inclusion criteria (radiographs of the whole spine...

  3. Genetic Variation in Osteopontin Gene Is Associated with Susceptibility to Sarcoidosis in Slovenian Population

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    A. Maver

    2009-01-01

    Full Text Available Sarcoidosis is a systemic inflammatory disease characterised by appearance of granulomas. Precise etiology has not been elucidated. Osteopontin (Opn is a Th1 cytokine whose levels have been found increased in granulomas and serum samples from patients with sarcoidosis. We investigated whether genetic variation in Osteopontin gene (OPN gene contributes to susceptibility to sarcoidosis. Haplotype-block structure in the OPN gene region was investigated using data from HapMap project. Three representative SNPs have been selected from each block of SNPs in linkage disequilibrium (rs11730582-C/T, rs11728697-C/T and rs4754-C/T. Genotyping was performed using TaqMan SNP Genotyping Assays on a sample of 165 patients and 284 controls. Statistical analyses of association were performed using Chi-Square test and algorithms implemented in the haplo.stats and PHASE packages. Genotyping analysis revealed a significant difference in genotype frequencies at rs4754 polymorphism in groups of patients and controls under recessive genetic model (p=0.036, OR=1.99, 95%CI=1.04-3.82, CC homozygotes being significantly over-represented in the patients group. However these results failed to reach significance after correction for multiple testing (p=0.25. The frequencies of predicted haplotypes differed between patient and control groups, frequency of TTT haplotype was found to be significantly decreased in the group of patients with sarcoidosis (p=0.014, OR=0.40, 95%CI=0.20-0.79. Our results suggest that variation in the OPN gene might be significantly associated with sarcoidosis and that the TTT haplotype in OPN may act as a protective factor in sarcoidosis.

  4. Enhanced proliferation and decreased apoptosis in lung lavage cells of sarcoidosis patients.

    Science.gov (United States)

    Petzmann, Susanna; Maercker, Christian; Markert, Eva; Kern, Izidor; Osolnik, Katharina; Pohl, Wolfgang; Popper, Helmut H

    2006-10-01

    Sarcoidosis is a systemic autoimmune disease where an inflammatory reaction involving alveolar macrophages, T-helper lymphocytes, and epitheloid cells is mounted against unknown antigens. A genetic predisposition for sarcoidosis is supposed by studies in twins, by geographical and racial distribution. In the current investigation we compared the expression patterns between slow onset and acute sarcodosis using a whole-genome cDNA array. Bronchoalveolar lavage was performed in six patients with slow onset sarcoidosis and four patients with acute sarcoidosis (Löfgren's disease) and obtained cells were used for gene expression profiling. The results were confirmed by RT- and Taqman-PCR. In addition, protein expression was examined on paraffin sections of sarcoid granulomas by immunohistochemistry. In T-helper lymphocytes and alveolar macrophages we found an upregulation of genes belonging to the phosphoinositol-3-kinase/v-akt murine thymoma viral oncogene homolog/signal transducer and activator of transcription 3 pathway, as well as a downregulation of genes of the extrinsic and intrinsic apoptotic signaling cascades. In addition an upregulation of the genes encoding fatty acid binding protein 4 and 5, as well as peroxisome proliferative activated receptor delta in Löfgren's disease was detected. Differences in gene expression between slow onset sarcoidosis and Löfgren's syndrome were found mainly within genes of the major histocompatibility complex. In sarcoidosis enhanced cell proliferation and decreased apoptosis result in accumulation and prolonged survival of antigen-primed T-helper lymphocytes and activated macrophages. This is enhanced in Löfgren's disease, probably by hyper-stimulation via the peroxisome proliferation signaling, providing a larger pool of antigen-primed immune cells.

  5. Triggering receptor expressed on myeloid cells-1 and 2 in bronchoalveolar lavage fluid in pulmonary sarcoidosis.

    Science.gov (United States)

    Suchankova, Magda; Bucova, Maria; Tibenska, Elena; Tedlova, Eva; Demian, Juraj; Majer, Ivan; Novosadova, Helena; Tedla, Miroslav; Paulovicova, Ema; Kantarova, Daniela

    2013-04-01

    Pulmonary sarcoidosis (PS) is characterized by the formation of granulomas in the lungs and has been associated with infection by microorganisms. Triggering receptor expressed on the surface of myeloid cells (TREM)-1 is overexpressed in response to infection while TREM-2 is involved in granuloma formation. We hypothesized that these receptors are overexpressed in PS and might be useful for diagnostic testing. Cell surface TREM-1 and TREM-2 expression in cells obtained at bronchoalveolar lavage (BAL) was measured in individuals with sarcoidosis (n = 26) and compared with that seen in individuals with other interstitial lung diseases (ILD) (n = 27). TREM-1 and TREM-2 expression was significantly increased in sarcoidosis compared with other ILD: total number of TREM-1, P = 0.0039 (23.81 vs 13.50 cells/μl), TREM-2, P < 0.0001 (32.81 vs 7.76 cells/μl); percentage of TREM-1: P = 0.0002 (41.30% vs 15.70%), TREM-2: P < 0.0001 (34% vs 9.60%); and mean fluorescence of TREM-1: P = 0.0005 (5.43 vs 1.96), TREM-2: P = 0.0011 (6.85 vs 2.77). Increase in both of these receptors seems to be typical for PS. In discriminating sarcoidosis from other ILD, the specificity (96%) and sensitivity (72%) of the combination of TREM-1 and TREM-2 was high. Increased TREM-1 and TREM-2 cell surface expression is observed in sarcoidosis. Evaluation of BAL cell expression of both of these receptors may serve as a diagnostic marker for sarcoidosis. © 2013 The Authors. Respirology © 2013 Asian Pacific Society of Respirology.

  6. Fungal exposure in homes of patients with sarcoidosis - an environmental exposure study

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    Harlander Matevz

    2011-01-01

    Full Text Available Abstract Background There is increasing evidence that exposure to moulds (fungi may influence the development of sarcoidosis. To assess the influence of the environmental exposure, a study was undertaken to determine the exposure to fungi in homes of subjects with sarcoidosis. Methods Subjects were patients with clinically established sarcoidosis recruited during the period September 2007 till June 2010. Of these 55 were newly diagnosed and currently under treatment for less than one year, 25 had been treated and had no recurrence and 27 had been treated but had recurrence of the disease. Controls were healthy subjects without any respiratory symptoms (n = 30. Samples of air (about 2.5 m3 were taken in the bedroom of the subjects using a portable pump and cellulose ester filters. The filters were analysed for the content of the enzyme N-acetylhexosaminidase (NAHA as a marker of fungal cell biomass, using a specific substrate and a fluorescent technique and expressed as NAHA units (U/m3. Results Compared to controls, subjects undergoing treatment of the disease (newly diagnosed or with recurrence had significantly higher activities of NAHA in their homes than controls (33.6 and 39.9 vs 10.0 U/m3, p 3. In homes of subjects with newly diagnosed disease with treatment less than one year, values above 14 NAHA U/m3 were found among 35 out of 55 and among those with recurrent disease among 18 out of 27. Conclusions The higher activities of NAHA enzyme found in homes of subjects with active and recurrent sarcoidosis suggest that exposure to fungi is related to the risk of sarcoidosis. Further environmental studies to assess the importance of this exposure for subjects with sarcoidosis are warranted. The results suggest that remedial actions in homes with high levels of fungi may be justified.

  7. An in silico modeling approach to understanding the dynamics of sarcoidosis.

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    Baltazar D Aguda

    Full Text Available BACKGROUND: Sarcoidosis is a polygenic disease with diverse phenotypic presentations characterized by an abnormal antigen-mediated Th1 type immune response. At present, progress towards understanding sarcoidosis disease mechanisms and the development of novel treatments is limited by constraints attendant to conducting human research in a rare disease in the absence of relevant animal models. We sought to develop a computational model to enhance our understanding of the pathological mechanisms of and predict potential treatments of sarcoidosis. METHODOLOGY/RESULTS: Based upon the literature, we developed a computational model of known interactions between essential immune cells (antigen-presenting macrophages, effector and regulatory T cells and cytokine mediators (IL-2, TNFα, IFNγ of granulomatous inflammation during sarcoidosis. The dynamics of these interactions are described by a set of ordinary differential equations. The model predicts bistable switching behavior which is consistent with normal (self-limited and "sarcoidosis-like" (sustained activation of the inflammatory components of the system following a single antigen challenge. By perturbing the influence of model components using inhibitors of the cytokine mediators, distinct clinically relevant disease phenotypes were represented. Finally, the model was shown to be useful for pre-clinical testing of therapies based upon molecular targets and dose-effect relationships. CONCLUSIONS/SIGNIFICANCE: Our work illustrates a dynamic computer simulation of granulomatous inflammation scenarios that is useful for the investigation of disease mechanisms and for pre-clinical therapeutic testing. In lieu of relevant in vitro or animal surrogates, our model may provide for the screening of potential therapies for specific sarcoidosis disease phenotypes in advance of expensive clinical trials.

  8. DMRT genes in vertebrate gametogenesis.

    Science.gov (United States)

    Zarkower, David

    2013-01-01

    Genes containing the DM domain DNA-binding motif regulate sex determination and sexual differentiation in a broad variety of metazoans, including nematodes, insects, and vertebrates. They can function in primary sex determination or downstream in sexual differentiation, and they can act either throughout the body or in highly restricted cell types. In vertebrates, several DM domain genes--DMRT genes--play critical roles in gonadal differentiation or gametogenesis. DMRT1 has the most prominent role and likely regulates testicular differentiation in all vertebrates. In the mammalian gonad, DMRT1 exerts both intrinsic and extrinsic control of gametogenesis; it is required for germ cell differentiation in males and regulates meiosis in both sexes, and it is required in supporting cells for the establishment and maintenance of male fate in the testis. These varied functions of DMRT1 serve to coordinate gonadal development and function. In other vertebrates, DMRT1 regulates gonadal differentiation, and it also appears to have played a central role in the evolution of new sex-determining mechanisms in at least three vertebrate clades. This chapter focuses on the regulation of vertebrate gametogenesis by DMRT1. Copyright © 2013 Elsevier Inc. All rights reserved.

  9. MR manifestations of vertebral artery injuries in cervical spine trauma

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    Yu, Jeong Sik; Chung, Tae Sub; Kim, Young Soo; Cho, Yong Eun; Kang, Byung Chul; Kim, Dong Ik [Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-11-01

    To assess the diagnostic efficacy of magnetic resonance (MR) imaging in the detection of a vertebral artery injury occurring from major cervical spine trauma. Conventional MR findings of 63 patients and 63 control subjects were compared to detect a possible change in the vertebral arteries resulted from trauma. Plain films, CT and clinical records were also reviewed to correlate the degree of cervical spine injury with vascular change. Nine cases of absent flow signals in vessel lumen were observed in eight patients and one was observed in the control group. Patients more frequently demonstrated other abnormalities such as intraluminal linear signals (n=3) or focal luminal narrowing (n=9) but there was no statistical significance. There was a close relationship between degree of cord damage and occlusion of the vertebral artery. Conventional MR imaging is useful in the detection of vertebral artery occlusion resulting from cervical spine trauma.

  10. Sarcoidosis en la infancia. Una rara enfermedad sistémica

    OpenAIRE

    Antonio Zamora-Chávez; Stanislaw Sadowinski-Pine; Carlos Serrano-Bello; Luis Velázquez-Jones; Omar Josué Saucedo-Ramírez; Jonathan Palafox-Flores; Erandi Josefina Mata-Vázquez

    2016-01-01

    Introducción: La sarcoidosis es una enfermedad sistémica de etiología desconocida que raramente se presenta en la infancia. Generalmente afecta los pulmones; sin embargo, puede involucrar diversos órganos. Ocasionalmente afecta el estado general, y origina fiebre, hepatomegalia y esplenomegalia. Caso clínico: Se presenta el caso de un adolescente de doce años de edad con sarcoidosis infantil de inicio tardío, cuyo diagnóstico fue confirmado con un estudio histopatológico de ganglio linfáti...

  11. The BTNL2 A allele variant is frequent in Danish patients with sarcoidosis

    DEFF Research Database (Denmark)

    Milman, Nils; Svendsen, Claus Bo; Nielsen, Finn Cilius

    2011-01-01

    The butyrophilin-like 2 (BTNL2) gene is located on chromosome 6p21.3 close to the HLA-class II genes. An association has been reported between sarcoidosis and a single nucleotide polymorphism in BTNL2, rs2076530, also termed the A allele.......The butyrophilin-like 2 (BTNL2) gene is located on chromosome 6p21.3 close to the HLA-class II genes. An association has been reported between sarcoidosis and a single nucleotide polymorphism in BTNL2, rs2076530, also termed the A allele....

  12. Dual AV Nodal Nonreentrant Tachycardia Resulting in Inappropriate ICD Therapy in a Patient with Cardiac Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Ankur A. Karnik, MD

    2014-01-01

    Full Text Available Dual atrioventricular nodal nonreentrant tachycardia (DAVNNT occurs due to concurrent antegrade conduction over fast and slow atrioventricular nodal pathways and is treated by slow pathway modification. We describe a unique case of a patient with cardiac sarcoidosis who received inappropriate ICD shocks for DAVNNT. Atrial and ventricular device electrograms satisfied both rate and V>A criteria for ventricular tachycardia. We postulate that alterations in refractoriness and conduction as is seen in cardiac sarcoidosis (CS may have contributed to occurrence of DAVNNT.

  13. Recombination drives vertebrate genome contraction.

    Science.gov (United States)

    Nam, Kiwoong; Ellegren, Hans

    2012-01-01

    Selective and/or neutral processes may govern variation in DNA content and, ultimately, genome size. The observation in several organisms of a negative correlation between recombination rate and intron size could be compatible with a neutral model in which recombination is mutagenic for length changes. We used whole-genome data on small insertions and deletions within transposable elements from chicken and zebra finch to demonstrate clear links between recombination rate and a number of attributes of reduced DNA content. Recombination rate was negatively correlated with the length of introns, transposable elements, and intergenic spacer and with the rate of short insertions. Importantly, it was positively correlated with gene density, the rate of short deletions, the deletion bias, and the net change in sequence length. All these observations point at a pattern of more condensed genome structure in regions of high recombination. Based on the observed rates of small insertions and deletions and assuming that these rates are representative for the whole genome, we estimate that the genome of the most recent common ancestor of birds and lizards has lost nearly 20% of its DNA content up until the present. Expansion of transposable elements can counteract the effect of deletions in an equilibrium mutation model; however, since the activity of transposable elements has been low in the avian lineage, the deletion bias is likely to have had a significant effect on genome size evolution in dinosaurs and birds, contributing to the maintenance of a small genome. We also demonstrate that most of the observed correlations between recombination rate and genome contraction parameters are seen in the human genome, including for segregating indel polymorphisms. Our data are compatible with a neutral model in which recombination drives vertebrate genome size evolution and gives no direct support for a role of natural selection in this process.

  14. Diagnostic and therapeutic evaluation of multiple choroidal granulomas in a patient with confirmed sarcoidosis using enhanced depth imaging optical coherence tomography.

    Science.gov (United States)

    Ishihara, Mami; Shibuya, Etsuko; Tanaka, Shin; Mizuki, Nobuhisa

    2017-10-05

    Sarcoidosis is a major cause of granulomatous uveitis but rarely manifests as multiple choroidal granulomas. This report describes the use of enhanced depth imaging optical coherence tomography (EDI-OCT) to visualize changes occurring in multiple choroidal granulomas during treatment. The patient was a 36-year-old Japanese man with histopathologically confirmed sarcoidosis, who was examined using EDI-OCT and showed multiple yellowish-white subretinal lesions in the peripapillary region and the arcade of the right eye. EDI-OCT revealed homogeneous hyporeflective choroidal lesions with choriocapillaris thinning, consistent with a diagnosis of choroidal granulomas. Subretinal fluid adjacent to one of the peripapillary choroidal lesions was also apparent. EDI-OCT during oral prednisolone administration revealed a decrease in lesion size at as early as 3 weeks and complete resolution of the lesions after 6 months of treatment. However, 2 months after prednisolone discontinuation, EDI-OCT revealed recurrence of choroidal granulomas in the peripapillary region and the arcade of the right eye. After injection of triamcinolone acetonide into the posterior sub-Tenon's capsule (sub-Tenon's injection), EDI-OCT demonstrated a reduction in granuloma lesion size within 3 months of the injection. EDI-OCT allowed detailed morphologic visualization of the choroidal granulomas caused by sarcoidosis. This imaging technique was useful for monitoring changes in granuloma size in response to steroid administration and for early detection of recurrence. Injection of triamcinolone acetonide into the posterior sub-Tenon's capsule was as effective as oral prednisolone for the treatment of choroidal granulomas.

  15. Transmission of ranavirus between ectothermic vertebrate hosts.

    Science.gov (United States)

    Brenes, Roberto; Gray, Matthew J; Waltzek, Thomas B; Wilkes, Rebecca P; Miller, Debra L

    2014-01-01

    Transmission is an essential process that contributes to the survival of pathogens. Ranaviruses are known to infect different classes of lower vertebrates including amphibians, fishes and reptiles. Differences in the likelihood of infection among ectothermic vertebrate hosts could explain the successful yearlong persistence of ranaviruses in aquatic environments. The goal of this study was to determine if transmission of a Frog Virus 3 (FV3)-like ranavirus was possible among three species from different ectothermic vertebrate classes: Cope's gray treefrog (Hyla chrysoscelis) larvae, mosquito fish (Gambusia affinis), and red-eared slider (Trachemys scripta elegans). We housed individuals previously exposed to the FV3-like ranavirus with naïve (unexposed) individuals in containers divided by plastic mesh screen to permit water flow between subjects. Our results showed that infected gray treefrog larvae were capable of transmitting ranavirus to naïve larval conspecifics and turtles (60% and 30% infection, respectively), but not to fish. Also, infected turtles and fish transmitted ranavirus to 50% and 10% of the naïve gray treefrog larvae, respectively. Nearly all infected amphibians experienced mortality, whereas infected turtles and fish did not die. Our results demonstrate that ranavirus can be transmitted through water among ectothermic vertebrate classes, which has not been reported previously. Moreover, fish and reptiles might serve as reservoirs for ranavirus given their ability to live with subclinical infections. Subclinical infections of ranavirus in fish and aquatic turtles could contribute to the pathogen's persistence, especially when highly susceptible hosts like amphibians are absent as a result of seasonal fluctuations in relative abundance.

  16. The timing of Timezyme diversification in vertebrates.

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    Damien Cazaméa-Catalan

    Full Text Available All biological functions in vertebrates are synchronized with daily and seasonal changes in the environment by the time keeping hormone melatonin. Its nocturnal surge is primarily due to the rhythmic activity of the arylalkylamine N-acetyl transferase AANAT, which thus became the focus of many investigations regarding its evolution and function. Various vertebrate isoforms have been reported from cartilaginous fish to mammals but their origin has not been clearly established. Using phylogeny and synteny, we took advantage of the increasing number of available genomes in order to test whether the various rounds of vertebrate whole genome duplications were responsible for the diversification of AANAT. We highlight a gene secondary loss of the AANAT2 in the Sarcopterygii, revealing for the first time that the AAANAT1/2 duplication occurred before the divergence between Actinopterygii (bony fish and Sarcopterygii (tetrapods, lobe-finned fish, and lungfish. We hypothesize the teleost-specific whole genome duplication (WDG generated the appearance of the AANAT1a/1b and the AANAT2/2'paralogs, the 2' isoform being rapidly lost in the teleost common ancestor (ray-finned fish. We also demonstrate the secondary loss of the AANAT1a in a Paracantopterygii (Atlantic cod and of the 1b in some Ostariophysi (zebrafish and cave fish. Salmonids present an even more diverse set of AANATs that may be due to their specific WGD followed by secondary losses. We propose that vertebrate AANAT diversity resulted from 3 rounds of WGD followed by previously uncharacterized secondary losses. Extant isoforms show subfunctionalized localizations, enzyme activities and affinities that have increased with time since their emergence.

  17. The appearance of 'Lambda' and 'Panda' sign on Ga-67 scintigraphy in sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Yoshimizu, Tazuko; Suga, Kazuyoshi; Orihashi, Norihiro; Soejima, Kyoko; Kaneko, Takafumi; Kawamura, Mitsutoshi; Nakanishi, Takashi; Utsumi, Hiromoto; Yamada, Norimasa (Yamaguchi Univ., Ube (Japan). School of Medicine)

    1991-10-01

    The lesions of sarcoidosis generally show relatively high Ga-67 uptake and the usefulness of scintigraphy using this agent in the evaluation of lesion activity is well known. In this report, we assessed characteristic uptake of Ga-67 in intrathoracic lymph nodes, the shape of which resembled the Greek letter Lambda (Lambda sign) and a symmetrical accumulation in bilateral lacrimal and salivary glands which resembled a Panda face (Panda sign) in patients with sarcoidosis. Our review of Ga-67 scans obtained from 15 patients with sarcoidosis and 1,779 patients with other disorders during the past 3 years revealed that the simultaneous presence of both Lambda and Panda signs was specific to patients with sarcoidosis and was not found in patients with various other disorders, although one of these signs might be observed in patients without sarcoidosis. When Lambda-like sign was observed, chest radiogram or CT was necessary for the differential diagnosis of sarcoidosis. Panda sign was observed frequently among patients who had previously received irradiation of the neck. Our results confirmed that sarcoidosis must be suspected when both Lambda and Panda signs were observed. (author).

  18. Reconstructing the ancestral vertebrate brain.

    Science.gov (United States)

    Sugahara, Fumiaki; Murakami, Yasunori; Pascual-Anaya, Juan; Kuratani, Shigeru

    2017-05-01

    Highly complicated morphologies and sophisticated functions of vertebrate brains have been established through evolution. However, the origin and early evolutionary history of the brain remain elusive, owing to lack of information regarding the brain architecture of extant and fossil species of jawless vertebrates (agnathans). Comparative analyses of the brain of less studied cyclostomes (only extant agnathan group, consisting of lampreys and hagfish) with the well-known sister group of jawed vertebrates (gnathostomes) are the only tools we have available to illustrate the ancestral architecture of the vertebrate brain. Previous developmental studies had shown that the lamprey lacked well-established brain compartments that are present in gnathostomes, such as the medial ganglionic eminence and the rhombic lip. The most accepted scenario suggested that cyclostomes had fewer compartments than that of the gnathostome brain and that gnathostomes thus evolved by a stepwise addition of innovations on its developmental sequence. However, recent studies have revealed that these compartments are present in hagfish embryos, indicating that these brain regions have been acquired before the split of cyclostomes and gnathostomes. By comparing two cyclostome lineages and gnathostomes, it has become possible to speculate about a more complex ancestral state of the brain, excluding derived traits in either of the lineages. In this review, we summarize recent studies on the brain development of the lamprey and hagfish. Then, we attempt to reconstruct the possible brain architecture of the last common ancestor of vertebrates. Finally, we discuss how the developmental plan of the vertebrate brain has been modified independently in different vertebrate lineages. © 2017 Japanese Society of Developmental Biologists.

  19. Third-generation percutaneous vertebral augmentation systems

    OpenAIRE

    Vanni, Daniele; Galzio, Renato; Kazakova, Anna; Pantalone, Andrea; Grillea, Giovanni; Bartolo, Marcello; Salini, Vincenzo; Magliani, Vincenzo

    2016-01-01

    Currently, there is no general consensus about the management of osteoporotic vertebral fractures (OVF). In the past, conservative treatment for at least one month was deemed appropriate for the majority of vertebral fractures. When pain persisted after conservative treatment, it was necessary to consider surgical interventions including: vertebroplasty for vertebral fractures with less than 30% loss of height of the affected vertebral body and kyphoplasty for vertebral fractures with greater...

  20. Pregnancy related symptomatic vertebral hemangioma

    Directory of Open Access Journals (Sweden)

    Meena Gupta

    2014-01-01

    Full Text Available Vertebral hemangiomas are benign vascular tumors of the spine that remain asymptomatic in most cases and incidentally encountered on imaging. Rarely, altered hemodynamic and hormonal changes during pregnancy may expand these benign lesions resulting in severe cord compression. The management of symptomatic vertebral hemangioma during pregnancy is controversial as modalities like radiotherapy and embolization are not suitable and surgery during pregnancy has a risk of preterm labor. Few cases of pregnancy related symptomatic vertebral hemangioma with marked epidural component have been reported in the literature. We report a case of 23-year-old primigravida who developed rapidly progressive paraparesis at 28 weeks of gestation and spine magnetic resonance imaging (MRI revealed upper thoracic vertebral hemangioma with extensive extra-osseous extension and spinal cord compression. Laminectomy and surgical decompression of the cord was performed at 32 weeks of the pregnancy. There was significant improvement in muscle power after a week of surgery. Six weeks postoperatively she delivered a full term normal baby with subsequent improvement of neurologic deficit. Repeat MRI of dorsal spine performed at 3 months postoperatively showed reduced posterior and anterior epidural components of vertebral hemangioma.

  1. Childhood sarcoidosis in Denmark 1979-1994: incidence, clinical features and laboratory results at presentation in 48 children

    DEFF Research Database (Denmark)

    Hoffmann, A L; Milman, N; Byg, K E

    2004-01-01

    AIM: To describe the incidence, clinical presentation and paraclinical findings in childhood sarcoidosis in Denmark, 1979-1994. METHODS: Patients (n = 5536) with a diagnosis of sarcoidosis were drawn from the nationwide Patient Registry; 81 patients were diagnosis...... of sarcoidosis was reconfirmed in 48/81 (59%) patients. In 35/48 (73%) patients, diagnosis was verified by histology, and in 13 it was substantiated by paraclinical/clinical findings. RESULTS: The series comprised 26 boys and 22 girls (male/female ratio 1.18). Median age at diagnosis was 13 y (range 0...

  2. Independent regulation of vertebral number and vertebral identity by microRNA-196 paralogs.

    Science.gov (United States)

    Wong, Siew Fen Lisa; Agarwal, Vikram; Mansfield, Jennifer H; Denans, Nicolas; Schwartz, Matthew G; Prosser, Haydn M; Pourquié, Olivier; Bartel, David P; Tabin, Clifford J; McGlinn, Edwina

    2015-09-01

    The Hox genes play a central role in patterning the embryonic anterior-to-posterior axis. An important function of Hox activity in vertebrates is the specification of different vertebral morphologies, with an additional role in axis elongation emerging. The miR-196 family of microRNAs (miRNAs) are predicted to extensively target Hox 3' UTRs, although the full extent to which miR-196 regulates Hox expression dynamics and influences mammalian development remains to be elucidated. Here we used an extensive allelic series of mouse knockouts to show that the miR-196 family of miRNAs is essential both for properly patterning vertebral identity at different axial levels and for modulating the total number of vertebrae. All three miR-196 paralogs, 196a1, 196a2, and 196b, act redundantly to pattern the midthoracic region, whereas 196a2 and 196b have an additive role in controlling the number of rib-bearing vertebra and positioning of the sacrum. Independent of this, 196a1, 196a2, and 196b act redundantly to constrain total vertebral number. Loss of miR-196 leads to a collective up-regulation of numerous trunk Hox target genes with a concomitant delay in activation of caudal Hox genes, which are proposed to signal the end of axis extension. Additionally, we identified altered molecular signatures associated with the Wnt, Fgf, and Notch/segmentation pathways and demonstrate that miR-196 has the potential to regulate Wnt activity by multiple mechanisms. By feeding into, and thereby integrating, multiple genetic networks controlling vertebral number and identity, miR-196 is a critical player defining axial formulae.

  3. The origin of conodonts and of vertebrate mineralized skeletons

    Science.gov (United States)

    Murdock, Duncan J.E.; Dong, Xi-Ping; Repetski, John E.; Marone, Federica; Stampanoni, Marco; Donoghue, Philip C.J.

    2013-01-01

    Conodonts are an extinct group of jawless vertebrates whose tooth-like elements are the earliest instance of a mineralized skeleton in the vertebrate lineage, inspiring the ‘inside-out’ hypothesis that teeth evolved independently of the vertebrate dermal skeleton and before the origin of jaws. However, these propositions have been based on evidence from derived euconodonts. Here we test hypotheses of a paraconodont ancestry of euconodonts using synchrotron radiation X-ray tomographic microscopy to characterize and compare the microstructure of morphologically similar euconodont and paraconodont elements. Paraconodonts exhibit a range of grades of structural differentiation, including tissues and a pattern of growth common to euconodont basal bodies. The different grades of structural differentiation exhibited by paraconodonts demonstrate the stepwise acquisition of euconodont characters, resolving debate over the relationship between these two groups. By implication, the putative homology of euconodont crown tissue and vertebrate enamel must be rejected as these tissues have evolved independently and convergently. Thus, the precise ontogenetic, structural and topological similarities between conodont elements and vertebrate odontodes appear to be a remarkable instance of convergence. The last common ancestor of conodonts and jawed vertebrates probably lacked mineralized skeletal tissues. The hypothesis that teeth evolved before jaws and the inside-out hypothesis of dental evolution must be rejected; teeth seem to have evolved through the extension of odontogenic competence from the external dermis to internal epithelium soon after the origin of jaws.

  4. The origin of conodonts and of vertebrate mineralized skeletons.

    Science.gov (United States)

    Murdock, Duncan J E; Dong, Xi-Ping; Repetski, John E; Marone, Federica; Stampanoni, Marco; Donoghue, Philip C J

    2013-10-24

    Conodonts are an extinct group of jawless vertebrates whose tooth-like elements are the earliest instance of a mineralized skeleton in the vertebrate lineage, inspiring the 'inside-out' hypothesis that teeth evolved independently of the vertebrate dermal skeleton and before the origin of jaws. However, these propositions have been based on evidence from derived euconodonts. Here we test hypotheses of a paraconodont ancestry of euconodonts using synchrotron radiation X-ray tomographic microscopy to characterize and compare the microstructure of morphologically similar euconodont and paraconodont elements. Paraconodonts exhibit a range of grades of structural differentiation, including tissues and a pattern of growth common to euconodont basal bodies. The different grades of structural differentiation exhibited by paraconodonts demonstrate the stepwise acquisition of euconodont characters, resolving debate over the relationship between these two groups. By implication, the putative homology of euconodont crown tissue and vertebrate enamel must be rejected as these tissues have evolved independently and convergently. Thus, the precise ontogenetic, structural and topological similarities between conodont elements and vertebrate odontodes appear to be a remarkable instance of convergence. The last common ancestor of conodonts and jawed vertebrates probably lacked mineralized skeletal tissues. The hypothesis that teeth evolved before jaws and the inside-out hypothesis of dental evolution must be rejected; teeth seem to have evolved through the extension of odontogenic competence from the external dermis to internal epithelium soon after the origin of jaws.

  5. Exercise capacity, muscle strength, and fatigue in sarcoidosis : A follow-up study

    NARCIS (Netherlands)

    Marcellis, R.G.; Lenssen, A.F.; Kleynen, S.; de Vries, J.; Drent, M.

    2013-01-01

    Purpose The purpose of this study was to examine changes in the prevalence of exercise intolerance, reduced muscle strength, and fatigue and the changes in these parameters in individual patients during a 2-year follow-up study. Methods Ninety sarcoidosis patients (62 males and 28 females; mean age:

  6. Sarcoidosis cardíaca: trasplante cardíaco y recidiva de la enfermedad

    Directory of Open Access Journals (Sweden)

    César A. Belziti

    2010-01-01

    Full Text Available RESUMENLa sarcoidosis cardíaca es una enfermedad granulomatosa multisistémica de causa desconocida.El compromiso aislado del corazón es poco frecuente y el pronóstico se correlacionacon el grado de afectación de este órgano. El tratamiento inmunosupresor y el trasplantecardíaco han demostrado que prolongan la sobrevida; no obstante, existen comunicacionesde recidiva de la enfermedad en el órgano implantado. En esta presentación se describe uncaso de recidiva de sarcoidosis cardíaca en un paciente con miocardiopatía dilatada coninsuficiencia cardíaca avanzada, sometido a un trasplante cardíaco y en quien el diagnósticode sarcoidosis cardíaca se estableció por el estudio anatomopatológico del corazón explantado.La evolución clínica fue favorable a pesar de la recidiva de la sarcoidosis en el órgano implantado.REV ARGENT CARDIOL 2010;78:358-360.

  7. In Vivo Evaluation of TNF-Alpha in the Lungs of Patients Affected by Sarcoidosis

    Science.gov (United States)

    Pietrangeli, Vittorio; Ricci, Alberto; Bruno, Pierdonato; Ragni, Paola; Scopinaro, Francesco; Mariotta, Salvatore; Signore, Alberto

    2015-01-01

    Introduction. Sarcoidosis is a multisystemic granulomatous disorder characterized by multiple noncaseating granulomas involving intrathoracic lymph nodes and lung parenchyma. Recently, the use of anti-tumor necrosis factor alpha (anti-TNFα) agents has been introduced for therapy of chronic and refractory sarcoidosis with controversial results. Infliximab (Remicade) is a chimeric monoclonal antibody (mAb) that recognizes and binds TNFα, neutralizing its biological effects. In the present study,   99mTc labelled infliximab was used to study the expression of TNFα in sarcoid lesions and to evaluate its role as a predictive marker in response to therapy with Remicade. Material and Methods. A total of 10 patients with newly diagnosed sarcoidosis were enrolled together with 10 control patients affected by rheumatoid arthritis. All patients were studied by planar imaging of the chest with   99mTc-infliximab at 6 h and 24 h and total body [18F]-FDG PET/CT. Regions of interest were drawn over the lungs and the right arm and target-to-background ratios were analysed for   99mTc-infliximab. SUVmean and SUVmax were calculated over lungs for FDG. Results and Discussion. Image analysis showed low correlation between T/B ratios and BAL results in patients despite positivity at [18F]-FDG PET. Conclusion. In conclusion, patients with newly diagnosed pulmonary sarcoidosis, with FDG-PET and BAL positivity, showed a negative   99mTc-infliximab scintigraphy. PMID:25866780

  8. In Vivo Evaluation of TNF-Alpha in the Lungs of Patients Affected by Sarcoidosis

    Directory of Open Access Journals (Sweden)

    Filippo Galli

    2015-01-01

    Full Text Available Introduction. Sarcoidosis is a multisystemic granulomatous disorder characterized by multiple noncaseating granulomas involving intrathoracic lymph nodes and lung parenchyma. Recently, the use of anti-tumor necrosis factor alpha (anti-TNFα agents has been introduced for therapy of chronic and refractory sarcoidosis with controversial results. Infliximab (Remicade is a chimeric monoclonal antibody (mAb that recognizes and binds TNFα, neutralizing its biological effects. In the present study, Tc 99m labelled infliximab was used to study the expression of TNFα in sarcoid lesions and to evaluate its role as a predictive marker in response to therapy with Remicade. Material and Methods. A total of 10 patients with newly diagnosed sarcoidosis were enrolled together with 10 control patients affected by rheumatoid arthritis. All patients were studied by planar imaging of the chest with Tc 99m-infliximab at 6 h and 24 h and total body [18F]-FDG PET/CT. Regions of interest were drawn over the lungs and the right arm and target-to-background ratios were analysed for Tc 99m-infliximab. SUVmean and SUVmax were calculated over lungs for FDG. Results and Discussion. Image analysis showed low correlation between T/B ratios and BAL results in patients despite positivity at [18F]-FDG PET. Conclusion. In conclusion, patients with newly diagnosed pulmonary sarcoidosis, with FDG-PET and BAL positivity, showed a negative Tc 99m-infliximab scintigraphy.

  9. Multifocal choroiditis as the first sign of systemic sarcoidosis associated with pembrolizumab

    Directory of Open Access Journals (Sweden)

    Qu-Knafo Lise

    2017-04-01

    Conclusions and importance: Pembrolizumab is an immune checkpoint inhibitor therapy used in the treatment of metastatic melanoma. We report a pembrolizumab-associated sarcoidosis revealed by a panuveitis with multifocal choroiditis. Physicians should be aware of the potential inflammatory and autoimmune disease that may be induced by immunomodulatory therapies.

  10. An electronic nose discriminates exhaled breath of patients with untreated pulmonary sarcoidosis from controls

    NARCIS (Netherlands)

    Dragonieri, Silvano; Brinkman, Paul; Mouw, Evert; Zwinderman, Aeilko H.; Carratú, Pierluigi; Resta, Onofrio; Sterk, Peter J.; Jonkers, Rene E.

    2013-01-01

    Sarcoidosis is a systemic granulomatous disease of unknown cause that affects the lungs in over 90% of cases. Breath analysis by electronic nose technology provides exhaled molecular profiles that have potential in the diagnosis of several respiratory diseases. We hypothesized that exhaled molecular

  11. Age-related and historical changes in the clinical characteristics of sarcoidosis in Japan.

    Science.gov (United States)

    Sawahata, Michiru; Sugiyama, Yukihiko; Nakamura, Yosikazu; Nakayama, Masayuki; Mato, Naoko; Yamasawa, Hideaki; Bando, Masashi

    2015-02-01

    National surveys conducted in Japan between 1960 and 2004 suggest a gradually increasing incidence of sarcoidosis in women >50 years old with increased involvement of the eye, skin, and heart. However, whether this involvement is due to the increased age at diagnosis is still unclear. We aimed here to identify the age-related differences in organ involvement in sarcoidosis in Japan, as well as the historical changes in clinical characteristics and the age-specific distribution of cases at diagnosis. We reviewed 588 consecutive Japanese patients newly diagnosed with sarcoidosis between 1974 and 2012 at Jichi Medical University Hospital. We compared organ involvement between subgroups differentiated by sex and age (lymphatic extrathoracic organs such as the eye, heart, muscle, and kidney. The age at diagnosis has consistently increased over the past four decades. The monophasic distribution in men has tended to become biphasic, and the biphasic distribution in women monophasic. Increasing trends were apparent for hypercalcemia and involvement of the gastrointestinal tract, skin, nervous system, muscle, and kidney. Elderly patients at diagnosis had various extrathoracic involvement including eye, skin, and cardiac lesions. Moreover, the age at diagnosis of sarcoidosis has continued to increase in both sexes, influencing the recent trends in clinical characteristics. Copyright © 2015 Elsevier Ltd. All rights reserved.

  12. A case of chylothorax in a patient with sarcoidosis: a rare and potentially fatal complication

    Directory of Open Access Journals (Sweden)

    Bikash Bhattarai

    2015-09-01

    Full Text Available Obstruction of the thoracic duct may lead to accumulation of a lymphatic fluid rich in triglycerides named chyle. When chyle accumulates in the pleural cavity, it becomes a chylothorax. Malignancy, particularly lymphoma, is the most common cause of chylothorax; however, any pathology leading to obstruction or destruction of the thoracic duct can lead to a chylothorax. This particular case investigates an incidence of chylothorax in sarcoidosis. A 54-year-old African American woman with a medical history of sarcoidosis, congestive heart failure, and smoking presented to the emergency department with complaints of bilateral foot swelling and exertional shortness of breath 3 days in duration. Physical examination was positive for bilateral crepitations with decreased air entry, abdominal ascites, and bilateral 2+ pitting edema. Both chest X-ray and chest CT were positive for stable bilateral pleural effusions (when compared to imaging done 3 years previously, and thoracocentesis and paracentesis were positive for chylous fluid accumulation. Chylothorax was diagnosed, and based on the previous medical history, the lymphadenopathy of sarcoidosis was determined to cause the occlusion of the thoracic duct. Lymphoscintigraphy and surgical intervention were advised; however, the family decided on conservative management and the patient expired intubated in the ICU. Chylothorax is a rare manifestation of sarcoidosis and high index of suspicion should be there to diagnose this, as there is high morbidity and mortality associated with it.

  13. A case of chylothorax in a patient with sarcoidosis: a rare and potentially fatal complication

    Science.gov (United States)

    Bhattarai, Bikash; Schmidt, Frances; Devkota, Ashok; Policard, Geraldine; Manhas, Saveena; Oke, Vikram; Agu, Chidozie Charles; Basunia, Md Rawshan; Enriquez, Danilo; Quist, Joseph; Kharel, Prakash

    2015-01-01

    Obstruction of the thoracic duct may lead to accumulation of a lymphatic fluid rich in triglycerides named chyle. When chyle accumulates in the pleural cavity, it becomes a chylothorax. Malignancy, particularly lymphoma, is the most common cause of chylothorax; however, any pathology leading to obstruction or destruction of the thoracic duct can lead to a chylothorax. This particular case investigates an incidence of chylothorax in sarcoidosis. A 54-year-old African American woman with a medical history of sarcoidosis, congestive heart failure, and smoking presented to the emergency department with complaints of bilateral foot swelling and exertional shortness of breath 3 days in duration. Physical examination was positive for bilateral crepitations with decreased air entry, abdominal ascites, and bilateral 2+ pitting edema. Both chest X-ray and chest CT were positive for stable bilateral pleural effusions (when compared to imaging done 3 years previously), and thoracocentesis and paracentesis were positive for chylous fluid accumulation. Chylothorax was diagnosed, and based on the previous medical history, the lymphadenopathy of sarcoidosis was determined to cause the occlusion of the thoracic duct. Lymphoscintigraphy and surgical intervention were advised; however, the family decided on conservative management and the patient expired intubated in the ICU. Chylothorax is a rare manifestation of sarcoidosis and high index of suspicion should be there to diagnose this, as there is high morbidity and mortality associated with it. PMID:26333861

  14. Pulmonology meets rheumatology in sarcoidosis: a review on the therapeutic approach

    NARCIS (Netherlands)

    Drent, M.; Cremers, J.P.; Jansen, T.L.Th.A.

    2014-01-01

    PURPOSE OF REVIEW: Sarcoidosis is a systemic disease characterized by the formation of granulomas in various organs, mainly lungs and lymphatic system. Joint, muscle, and bone involvement is also rather common. Recent studies on its pathogenesis and therapeutic management are reviewed here. RECENT

  15. Incidence of respiratory tract sarcoidosis in southern and northern regions of Ukraine

    Directory of Open Access Journals (Sweden)

    Gumenyuk G.L.

    2014-06-01

    Full Text Available We conducted a comparative survey of incidence and prevalence of pulmonary sarcoidosis in Southern (Crimean AR and Northern (Zhytomyr region regions of Ukraine by means of analysis of self-referred cases and cases, revealed at prophylactic radiological examination in 2011. The incidence of sarcoidosis in Crimea was 1.08 per 100 000 adult inhabitants; the prevalence was 4,59 per 100 000. In Zhytomyr region the incidence value (2,62 per 100 000 exceeded an equivalent value in Crimea by 2,4 times. The prevalence (7,86 per 100 000 was also higher than in Crimea by 1,7 times. Taking into account a significant impact of climate factor on sarcoidosis epidemiology, the incidence and prevalence of disease in Northern and Southern regions should be considered polar as for the regions of Ukraine. This makes it possoble to conclude that mean incidence of sarcoidosis in Ukraine ranges from 1,1 to 2,6 per 100 000 adult population, whereas the prevalence – from 4,6 to 7,9 per 100 000, which is equivalent to the level of Southern European countries.

  16. Efficacy of additional treatment with azathioprine in a patient with prednisolone-dependent gastric sarcoidosis.

    Science.gov (United States)

    Murata, Masaki; Sugimoto, Mitsushige; Yokota, Yoshihiro; Ban, Hiromitsu; Inatomi, Osamu; Bamba, Shigeki; Kushima, Ryoji; Andoh, Akira

    2016-12-21

    Gastric sarcoidosis with noncaseating granuloma is rare. Although corticosteroid produces a dramatic clinical response, it is unknown whether azathioprine show efficacy in prednisolone-dependent cases. Here, we report a case of gastric sarcoidosis in a 25-year-old man with severe epigastlargia. Gastroendoscopy revealed multiple map-like ulcerations. Histological examination showed multiple noncaseating granulomatous lesions in gastric mucosa, which were incompatible with diagnoses of Crohn's disease or tuberculosis. He was started on prednisolone at 30 mg/d, and his symptoms improved within 7-d. The prednisolone was gradually tapered by 5 mg every 2-wk, but oral azathioprine at 50 mg was added after symptoms recurred at tapered dose of 10 mg. Endoscopy 4-wk later showed healing ulcers, and, lymphocytic infiltration was absent. The efficacy of additional azathioprine in gastric sarcoidosis is not well defined. Here, we report a case of prednisolone-dependent gastric sarcoidosis that improved after additional azathioprine, and also review the literature concerning the treatment, especially for prednisolone-dependent cases.

  17. Vertebrate pressure-gradient receivers

    DEFF Research Database (Denmark)

    Christensen-Dalsgaard, Jakob

    2011-01-01

    The eardrums of all terrestrial vertebrates (tetrapods) are connected through Eustachian tubes or interaural canals. In some of the animals, these connections create pressure-gradient directionality, an enhanced directionality by interaction of sound arriving at both sides of the eardrum and stro......The eardrums of all terrestrial vertebrates (tetrapods) are connected through Eustachian tubes or interaural canals. In some of the animals, these connections create pressure-gradient directionality, an enhanced directionality by interaction of sound arriving at both sides of the eardrum....... Recent vertebrates form a continuum from perfect interaural transmission (0 dB in a certain frequency band) and pronounced eardrum directionality (30-40 dB) in the lizards, over somewhat attenuated transmission and limited directionality in birds and frogs, to the strongly attenuated interaural...

  18. In-Hospital Disease Burden of Sarcoidosis in Switzerland from 2002 to 2012.

    Science.gov (United States)

    Pohle, Susanne; Baty, Florent; Brutsche, Martin

    2016-01-01

    Sarcoidosis is a multisystem disease with an unpredictable and sometimes fatal course while the underlying pathomechanism is still unclear. Reasons of the increasing hospitalization rate and mortality in the United States remain in dispute but incriminated are a number of distinct comorbidities and risk factors as well as the application of more aggressive therapeutic agents. Studies reflecting the recent development in central Europe are lacking. Our aim was to investigate the recent mortality and hospitalization rates as well as the underlying comorbidities of hospitalized sarcoidosis patients in Switzerland. In this longitudinal, nested case-control study, a nation-wide database provided by the Swiss Federal Office for Statistics enclosing every hospital entry covering the years 2002-2012 (n = 15,627,573) was analyzed. There were 8,385 cases with a diagnosis of sarcoidosis representing 0.054% (8,385 / 15,627,573) of all hospitalizations in Switzerland. These cases were compared with age- and sex-matched controls without the diagnosis of sarcoidosis. Hospitalization and mortality rates in Switzerland remained stable over the observed time period. Comorbidity analysis revealed that sarcoidosis patients had significantly higher medication-related comorbidities compared to matched controls, probably due to systemic corticosteroids and immunosuppressive therapy. Sarcoidosis patients were also more frequently re-hospitalized (median annual hospitalization rate 0.28 [IQR 0.15-0.65] vs. 0.19 [IQR 0.13-0.36] per year; p < 0.001), had a longer hospital stay (6 [IQR 2-13] vs. 4 [IQR 1-8] days; p < 0.001), had more comorbidities (4 [IQR 2-7] vs. 2 [IQR 1-5]; p < 0.001), and had a significantly higher in-hospital mortality (2.6% [95% CI 2.3%-2.9%] vs. 1.8% [95% CI 1.5%-2.1%] (p < 0.001). A worse outcome was observed among sarcoidosis patients having co-occurrence of associated respiratory diseases. Moreover, age was an important risk factor for re-hospitalization.

  19. Comparison of {sup 18}F-FDG and {sup 67}Ga-citrate in sarcoidosis imaging

    Energy Technology Data Exchange (ETDEWEB)

    Prager, E.; Bisail, B.; Schwarz, T.; Lanz, H.; Aigner, R.M. [Div. of Nuclear Medicine, Medical Univ. of Graz (Austria); Wehrschuetz, M.; Sorantin, E. [Dept. of General and Pediatric Radiology, Medical Univ. of Graz (Austria); Woltsche, M. [Div. of Pulmonology, LKH Hoergas/Enzenbach (Austria)

    2008-07-01

    Aim: {sup 67}Ga citrate has been used long and successfully to diagnose and stage sarcoidosis. {sup 18}F-fluorodeoxyglucose ({sup 18}F-FDG) has been suggested as a positron emission tomography (PET) tracer for sarcoidosis imaging. This study aimed to analyze possible advantages of {sup 18}F-FDG-PET over {sup 67}Ga citrate scintigraphy during the primary assessment of patients with sarcoidosis. Patients and methods: twenty-four patients (11 men, 13 women, aged 52 years {+-} 12.4) with histologically proven sarcoidosis were investigated with {sup 18}F-FDG and {sup 67}Ga citrate. Equipment included a full-ring PET scanner (ECAT EXACT HR+, Siemens/CTI, Knoxville TN, USA) and a double-headed gamma camera (ECAM, Siemens, Illinois, USA) for scintigraphy. The mean time difference between the two studies was 6.5 days (range: 5-8 days). Results: there was a significant difference in the detection of pulmonary and nonpulmonary sarcoidosis lesions between planar {sup 67}Ga citrate scans and {sup 18}F-FDG-PET images (< 0.0021). A total of 64 lesions were detected with {sup 67}Ga citrate scans in the thorax and elsewhere with a mean of 2.6 lesions (4%) per patient, while 85 lesions were found with {sup 18}F-FDG-PET, with a mean of 3.5 lesions (4.1%) per patient. There was complete agreement between {sup 18}F-FDG and {sup 67}Ga citrate in thoracic manifestations in four (16.6%) patients, and in non-thoracic manifestations in five (20.8%) patients. The interobserver variability showed a kappa value of 0.79. Conclusion: {sup 67}Ga citrate and {sup 18}F-FDG are useful tracers for diagnostic evaluation of thoracic sarcoidosis. {sup 18}F-FDG seems to be more suitable for imaging the mediastinum, the bi-hilar lymph nodes, the posterior regions of the lungs and non-thoracic lesions. Further prospective studies are needed to clarify the role of both tracers in early diagnosis and staging of sarcoidosis, and to resolve questions concerning medical treatment and follow-up. (orig.)

  20. Outcome of Patients With Cardiac Sarcoidosis Who Received Cardiac Resynchronization Therapy: Comparison With Dilated Cardiomyopathy Patients.

    Science.gov (United States)

    Yufu, Kunio; Kondo, Hidekazu; Shinohara, Tetsuji; Kawano, Kyoko; Ishii, Yumi; Miyoshi, Miho; Imamura, Takaaki; Saito, Shotaro; Okada, Norihiro; Akioka, Hidefumi; Teshima, Yasushi; Nakagawa, Mikiko; Takahashi, Naohiko

    2017-02-01

    Cardiac resynchronization therapy (CRT) has been shown to be effective for patients with chronic heart failure; however, the efficacy of CRT in patients with cardiac sarcoidosis (CS) has not been established. We compared the outcomes of patients with CS who received CRT to patients with dilated cardiomyopathy (DCM). The incidence of major adverse cerebral and cardiovascular events (MACCE) in 11 consecutive CS patients (8 females; mean age, 66 ± 8.0 years) who received CRT were compared with 29 DCM patients (9 females; mean age, 70 ± 8.9 years). Females and patients with previous right ventricular pacing were largely included in the comparison of CS and DCM patients (P < 0.05 and P < 0.0001, respectively). During the mean follow-up period (465 ± 383 days for CS and 729 ± 393 days for DCM), MACCE were evident in 9 patients (23%); specifically, 5 CS and 4 DCM patients developed MACCE (45% vs. 14%, P < 0.05), respectively. Kaplan-Meier survival analysis demonstrated that CS patients had a higher prevalence of MACCE than DCM patients (log rank = 6.306, P = 0.0120; and Wilcoxon = 7.1333, P = 0.0076). Based on univariate analysis, the etiology of CS was associated with MACCE. Our results suggest that the long-term outcome of CRT in patients with CS was very poor compared with DCM patients. Thus, caution should be exercised regarding the indication of CRT in patients with CS. © 2016 Wiley Periodicals, Inc.

  1. MRI in cardiac sarcoidosis and amyloidosis; MRT bei kardialer Sarkoidose und Amyloidose

    Energy Technology Data Exchange (ETDEWEB)

    Bauner, K.U. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); Wintersperger, B. [Klinikum der Ludwig-Maximilians-Universitaet Muenchen, Campus Grosshadern, Institut fuer Klinische Radiologie, Muenchen (Germany); University of Toronto, Department of Medical Imaging, Toronto General Hospital, Toronto, ON (Canada)

    2013-01-15

    Sarcoidosis and amyloidosis are both multisystem disorders, which may involve the heart; however, isolated cardiac disease is rare. Diagnosis of cardiac sarcoidosis and amyloidosis is crucial because the patient prognosis is dependent on cardiac involvement and early treatment. Echocardiography is the first line imaging modality in the diagnostic work-up of both diseases, possibly giving hints towards the correct diagnosis. Besides myocardial biopsy and radionuclide studies cardiac magnetic resonance imaging (MRI) is routinely performed in patients suspect of having infiltrative cardiomyopathy. The T1 mapping procedure is currently being evaluated as a new technique for detection and quantification of global myocardial enhancement, as seen in cardiac amyloidosis. Sensitivities and specificities for detection of cardiac sarcoidosis and amyloidosis can be significantly improved by MRI, especially with late gadolinium enhancement (LGE) imaging. In cardiac sarcoidosis the use of LGE is outcome-related while in amyloidosis analysis of T1-mapping may be of prognostic value. If cardiac involvement in sarcoidosis or amyloidosis is suspected cardiac MRI including LGE should be performed for establishing the diagnosis. (orig.) [German] Die Sarkoidose und Amyloidose sind Multisystemerkrankungen, in deren Verlauf es zu einer kardialen Beteiligung kommen kann. Bildgebend wird als primaeres Verfahren die Echokardiographie eingesetzt. Zur weiteren Diagnostik wird neben der Biopsie und nuklearmedizinischen Verfahren v. a. die MRT herangezogen. Als neuere Technik zur Darstellung globaler diffuser Kontrastmittelanreicherungen, wie sie im Rahmen der Amyloidose vorkommen, wird z. Z. das T1-Mapping evaluiert. Durch den Einsatz der MRT, insbesondere des Late-Gadolinium-Enhancements (LGE), koennen die Sensitivitaet und Spezifitaet in der Diagnostik der kardialen Sarkoidose und Amyloidose entscheidend verbessert werden. Bei der Sarkoidose stellt das Vorhandensein eines LGE einen

  2. [Nine cases of pulmonary sarcoidosis predominantly affecting the lower lung fields].

    Science.gov (United States)

    Matsui, Yoshinori; Akagawa, Shinobu; Masuda, Kimihiko; Yamato, Azusa; Ohshima, Nobuharu; Matsui, Hirotoshi; Teramoto, Shinji; Tamura, Atsuhisa; Nagai, Hideaki; Hebisawa, Akira

    2010-12-01

    Pulmonary sarcoidosis which predominantly affects the lower lung fields is relatively rare. We performed this study to clarify the clinical manifestations of this type of sarcoidosis. Over a period of 13 years, we diagnosed pulmonary sarcoidosis in 119 patients. Among these, we reviewed the clinical characteristics of 9 patients (3 men, 6 women, mean age 62 years) with pulmonary lesions predominantly affecting the lower lung fields. Four patients had a history of dust inhalation and 6 had symptoms of dyspnea. All patients had ocular lesions and 5 had cutaneous lesions. Serum KL-6 levels were elevated in all patients, whereas angiotensin-converting-enzyme (ACE) levels were elevated in 3. Pulmonary function tests revealed stenosis in 4 patients, and decreased diffusion capacity in 7. Chest CT findings in the lower lung fields revealed bronchovascular thickening, micronodular opacities in the vessels and chest wall, and interlobular septal thickening in 8 patients; ground-glass opacities in 5; curvilinear shadows in 4; and patchy shadows, traction bronchiectasis, and pleural effusion in 3. Histopathologic findings of lung biopsy specimens featured granulomas in all patients, and pulmonary interstitium fibrosis and small round-cell infiltration in the alveoli of most patients. Patients with sarcoidosis affecting the lower lung fields often had symptoms of dyspnea, extrapulmonary lesions in the eye and/or on the skin, and elevated serum KL-6 levels but not ACE. Chest CT showed findings typical of sarcoidosis, such as lymphatic distribution, but also showed unusual findings such as ground-glass opacities, curvilinear shadows, patchy shadows, traction bronchiectasis and pleural effusion. We speculated that 1 patient with ground-glass opacities and traction bronchiectasis without lymphatic distribution on CT, and fibroblastic foci with active alveolitis histopathologically, had complications of a different type of interstitial pneumonia.

  3. A Diagnostic Dilemma: Metastatic Testicular Cancer and Systemic Sarcoidosis – A Review of the Literature

    Directory of Open Access Journals (Sweden)

    R. Gupta

    2011-03-01

    Full Text Available Sarcoidosis is a multisystem disease that most commonly involves the lungs and the lymph nodes, but with genitourinary tract involvement, can easily mimic testicular cancer with metastasis to the lungs. We describe the case of a 30-year-old African-American male who presented with complaints of a headache, skin lesions, and a scrotal mass. A computed tomography scan of the head showed lesions in the frontotemporal and pons region, causing obstructive hydrocephalus. An ultrasound of the scrotum showed an enlarged epididymis bilaterally as well as a solid hypoechoic ill-defined mass on the right side, separate from the intact testis. Given the high suspicion for testicular malignancy with brain metastasis, a right orchiectomy was completed. The pathology revealed non-caseating necrotizing granulomas that stained negative for tubercular and fungal organisms, which was consistent with sarcoidosis. Additionally, the patient’s skin and central nervous system (CNS lesions improved on steroids that had been started for cerebral edema. Given the predilection of testicular cancer for CNS metastasis, neurosarcoidosis can also be mistaken for testicular cancer metastasis to the CNS, as seen in our case. Differentiating testicular cancer from genitourinary sarcoidosis is difficult but can be clarified using a combination of clinical presentation, epidemiology, serum markers (ACE, AFP, B-HCG, biopsies from skin/lymph nodes, and sometimes imaging. It is critical to differentiate genitourinary sarcoidosis from malignancy, as a misdiagnosis can lead to unnecessary surgical interventions, which have important implications for future fertility. There can also be a coexistence of as well as an association between testicular cancer and sarcoidosis, which should be recognized by health care providers. Both authors contributed equally to the manuscript.

  4. Efficacy of adalimumab in sarcoidosis patients who developed intolerance to infliximab.

    Science.gov (United States)

    Crommelin, Heleen A; van der Burg, Leone M; Vorselaars, Adriane D M; Drent, Marjolein; van Moorsel, Coline H M; Rijkers, Ger T; Deneer, Vera H M; Grutters, Jan C

    2016-06-01

    Tumor necrosis factor-alpha (TNF-α) inhibitors are regarded as the third-line therapy in sarcoidosis, the first choice generally being infliximab. To date, data regarding response to adalimumab in sarcoidosis patients intolerant to infliximab are lacking. The objective of this retrospective observational study was to establish if adalimumab could achieve stabilization or improvement of the disease in refractory sarcoidosis patients who developed intolerance to infliximab. Sarcoidosis patients referred to St Antonius Interstitial Lung Diseases Center of Excellence, Nieuwegein, The Netherlands, between January 2008 and April 2015 who switched from infliximab to adalimumab were included. Changes in organ function, inflammatory biomarker levels, and adverse events were retrieved from medical records. Out of 142 infliximab treated patients, 18 (13%) had to discontinue treatment due to antibody formation or severe adverse events and switched to adalimumab therapy. Organ function improved in 7 patients (39%), was stable in 6 patients (33%), and worsened in 5 patients (28%) after 12 months of treatment or after 6 months if evaluation after 12 months was not available (n = 4). In none of the patients biomarker levels of soluble interleukin-2 receptor (sIL-2R) deteriorated. Median decrease in sIL-2R was 3614 pg/mL. Most reported adverse event was infection (n = 10). Adalimumab is an effective alternative for patients intolerant to infliximab. The switch to adalimumab achieved clinical improvement in 39% and stabilization in 33% of patients intolerant to infliximab. Further research is needed to develop guidelines on how to use adalimumab for sarcoidosis in terms of dosing regimen. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Tested Demonstrations.

    Science.gov (United States)

    Gilbert, George L., Ed.

    1978-01-01

    Presents the following chemistry lecture demonstrations and experiments: (1) a versatile kinetic demonstration; (2) the Bakelite Demonstration; (3) applying Beer's law; and (4) entropy calculations. (HM)

  6. Endobronchial Ultrasound-guided Transbronchial Needle Aspiration versus Standard Bronchoscopic Modalities for Diagnosis of Sarcoidosis: A Meta-analysis

    Directory of Open Access Journals (Sweden)

    Li-Xing Hu

    2016-01-01

    Conclusions: The results of this meta-analysis suggest that EBUS-TBNA + TBLB + EBB could be used for the diagnosis of sarcoidosis, if available. At medical centers without EBUS-TBNA, TBNA + TBLB + EBB could be used instead.

  7. Dupla meningocele na coluna vertebral

    Directory of Open Access Journals (Sweden)

    Ehrenfried O. Wittig

    1968-03-01

    Full Text Available É relatado caso de dupla meningocele de coluna vertebral, respectivamente nas regiões cervico-torácica e tóraco-lombar, no qual ocorreu hidrocéfalo após pneumoventriculografia. Para a compensação do hidrocéfalo foi feita drenagem ventrículo-peritonial.

  8. Sarcoidosis-associated pulmonary hypertension: acute vasoresponsiveness to inhaled nitric oxide and the relation to long-term effect of sildenafil

    DEFF Research Database (Denmark)

    Milman, Nils; Svendsen, Claus Bo; Iversen, Martin

    2009-01-01

    Abstract Background: Severe pulmonary sarcoidosis is often complicated by pulmonary hypertension (PH) caused by different pathophysiological mechanisms. Objectives: To assess the acute vasoresponsiveness in patients with sarcoidosis and PH and the relation to the therapeutic effect of sildenafil....... Methods: A retrospective chart review of 25 patients with recalcitrant pulmonary sarcoidosis being evaluated for lung transplantation at our centre. Haemodynamics were evaluated by right heart catheterisation in 24 patients of whom 19 had PH. Eight of the 19 patients received vasodilator challenge...

  9. Rapid response of tattoo-associated cutaneous sarcoidosis to minocycline: case report and review of the literature.

    Science.gov (United States)

    Sheu, Johanna; Saavedra, Arturo P; Mostaghimi, Arash

    2014-08-17

    Cutaneous sarcoidosis can present in pre-existing tattoos. Previous reports suggest modest improvement with systemic or topical corticosteroids or other immunomodulating medications. Tetracyclines have anti-inflammatory properties and have been shown to be efficacious in non-tattoo associated cutaneous sarcoidosis. The pharmacology of minocycline suggests that its higher concentration in the skin may improve its efficacy in the treatment of cutaneous granulomas. We present a case of a 35-year-old man with a history of pulmonary sarcoidosis who developed raised plaques within tattoos present for over 10 years. Skin biopsy findings revealed non-caseating granulomas consistent with cutaneous sarcoidosis. The patient was started on minocycline 100mg twice daily and had resolution of pruritus in four days and improvement of sarcoidal plaques within one week. To our knowledge, this is the first report of cutaneous sarcoidosis in tattoos treated with minocycline. Our patient's rapid response to minocycline suggests that minocycline may be a quickly effective medication for cutaneous sarcoidosis and should be considered as a therapeutic option given its favorable side-effect profile.

  10. Alopecia: manifestação cutânea rara de sarcoidose Alopecia: an uncommon cutaneous manifestation of sarcoidosis

    Directory of Open Access Journals (Sweden)

    Fabiane Mulinari Brenner

    2008-10-01

    Full Text Available A sarcoidose é doença granulomatosa multissistêmica que geralmente compromete o trato respiratório e os linfonodos hilares. A pele é comumente afetada, mas raramente o couro cabeludo. Dois casos de sarcoidose com lesões no couro cabeludo são relatados: o primeiro, em paciente negra apresentando áreas de alopecia no couro cabeludo associada a outras lesões cutâneas; e o segundo, em paciente branca, portadora de sarcoidose pulmonar, com alopecia como manifestação cutânea isolada. A sarcoidose de couro cabeludo merece especial atenção, pois nos pacientes com essa forma de lesão cutânea existe alta incidência de acometimento sistêmico.Sarcoidosis is a multi-system granulomatous disease that generally affects the respiratory tract and hilar lymph nodes. The skin is also commonly involved, although cutaneous sarcoidosis on the scalp is rare. Two cases of scalp sarcoidosis are reported: the first presented with patchy alopecia, cutaneous sarcoidosis and also systemic disease in a black patient; the second case is related to an uncommon presentation with alopecia as the single cutaneous manifestation in a Caucasian patient with pulmonary sarcoidosis. Scalp sarcoidosis deserves special attention because there is a high incidence of other systemic lesions with this cutaneous manifestation, thus a careful investigation should be performed in these patients.

  11. Percutanous vertebroplasty for vertebral compression fracture in ...

    African Journals Online (AJOL)

    ; a report of two cases. ... Background: Osteoporotic vertebral fractures are common in the geriatric age group. ... Conclusion: Percutanous vertebroplasty offers effective, immediate and sustained pain relief in osteoporotic vertebral fractures.

  12. An invertebrate stomach's view on vertebrate ecology

    DEFF Research Database (Denmark)

    Calvignac-Spencer, Sébastien; Leendertz, Fabian H.; Gilbert, Tom

    2013-01-01

    Recent studies suggest that vertebrate genetic material ingested by invertebrates (iDNA) can be used to investigate vertebrate ecology. Given the ubiquity of invertebrates that feed on vertebrates across the globe, iDNA might qualify as a very powerful tool for 21st century population...

  13. Reflectance Demonstration.

    Science.gov (United States)

    Kowalski, Frank

    1993-01-01

    Presents a demonstration in which a mirror "disappears" upon rotation. The author has used the demonstration with students from fourth grade up through college. Suggestions are given for making the demonstration into a permanent hallway display. (MVL)

  14. Origin and evolution of retinoid isomerization machinery in vertebrate visual cycle: hint from jawless vertebrates.

    Directory of Open Access Journals (Sweden)

    Eugenia Poliakov

    Full Text Available In order to maintain visual sensitivity at all light levels, the vertebrate eye possesses a mechanism to regenerate the visual pigment chromophore 11-cis retinal in the dark enzymatically, unlike in all other taxa, which rely on photoisomerization. This mechanism is termed the visual cycle and is localized to the retinal pigment epithelium (RPE, a support layer of the neural retina. Speculation has long revolved around whether more primitive chordates, such as tunicates and cephalochordates, anticipated this feature. The two key enzymes of the visual cycle are RPE65, the visual cycle all-trans retinyl ester isomerohydrolase, and lecithin:retinol acyltransferase (LRAT, which generates RPE65's substrate. We hypothesized that the origin of the vertebrate visual cycle is directly connected to an ancestral carotenoid oxygenase acquiring a new retinyl ester isomerohydrolase function. Our phylogenetic analyses of the RPE65/BCMO and N1pC/P60 (LRAT superfamilies show that neither RPE65 nor LRAT orthologs occur in tunicates (Ciona or cephalochordates (Branchiostoma, but occur in Petromyzon marinus (Sea Lamprey, a jawless vertebrate. The closest homologs to RPE65 in Ciona and Branchiostoma lacked predicted functionally diverged residues found in all authentic RPE65s, but lamprey RPE65 contained all of them. We cloned RPE65 and LRATb cDNAs from lamprey RPE and demonstrated appropriate enzymatic activities. We show that Ciona ß-carotene monooxygenase a (BCMOa (previously annotated as an RPE65 has carotenoid oxygenase cleavage activity but not RPE65 activity. We verified the presence of RPE65 in lamprey RPE by immunofluorescence microscopy, immunoblot and mass spectrometry. On the basis of these data we conclude that the crucial transition from the typical carotenoid double bond cleavage functionality (BCMO to the isomerohydrolase functionality (RPE65, coupled with the origin of LRAT, occurred subsequent to divergence of the more primitive chordates

  15. Zygotic Genome Activation in Vertebrates.

    Science.gov (United States)

    Jukam, David; Shariati, S Ali M; Skotheim, Jan M

    2017-08-21

    The first major developmental transition in vertebrate embryos is the maternal-to-zygotic transition (MZT) when maternal mRNAs are degraded and zygotic transcription begins. During the MZT, the embryo takes charge of gene expression to control cell differentiation and further development. This spectacular organismal transition requires nuclear reprogramming and the initiation of RNAPII at thousands of promoters. Zygotic genome activation (ZGA) is mechanistically coordinated with other embryonic events, including changes in the cell cycle, chromatin state, and nuclear-to-cytoplasmic component ratios. Here, we review progress in understanding vertebrate ZGA dynamics in frogs, fish, mice, and humans to explore differences and emphasize common features. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Primary bone lymphoma with multiple vertebral involvement

    Directory of Open Access Journals (Sweden)

    Showkat Hussain Dar

    2013-01-01

    Full Text Available A 20-year-old student presented with 2 months history of fever and night sweats, 15 days history of low backache, progressive weakness of both limbs of 7 days duration, and urinary retention for last 24 h. Examination revealed a sensory level at D 10 dermatome and grade two power in both the lower limbs with absent reflexes. Examination of spine revealed a knuckle at T8 level, which was tender on palpation. MRI spine showed erosion of D11-12 and L1 in vertebral bodies with destruction of left pedicles, transverse processes and lamina, and a prominent psoas abscess. Post gadolinium study revealed ring-enhancing lesions in the D11-12 and L1 vertebrae as well as the dural sac. Fine needle aspiration cytology (FNAC and bone biopsy demonstrated a non-Hodgkin′s lymphoma (NHL, large cell high-grade of the spine (primary, which as per age is the youngest case of NHL ever reported in literature with multiple vertebral involvement.

  17. Vertebral development and amphibian evolution.

    Science.gov (United States)

    Carroll, R L; Kuntz, A; Albright, K

    1999-01-01

    Amphibians provide an unparalleled opportunity to integrate studies of development and evolution through the investigation of the fossil record of larval stages. The pattern of vertebral development in modern frogs strongly resembles that of Paleozoic labyrinthodonts in the great delay in the ossification of the vertebrae, with the centra forming much later than the neural arches. Slow ossification of the trunk vertebrae in frogs and the absence of ossification in the tail facilitate the rapid loss of the tail during metamorphosis, and may reflect retention of the pattern in their specific Paleozoic ancestors. Salamanders and caecilians ossify their centra at a much earlier stage than frogs, which resembles the condition in Paleozoic lepospondyls. The clearly distinct patterns and rates of vertebral development may indicate phylogenetic separation between the ultimate ancestors of frogs and those of salamanders and caecilians within the early radiation of ancestral tetrapods. This divergence may date from the Lower Carboniferous. Comparison with the molecular regulation of vertebral development described in modern mammals and birds suggests that the rapid chondrification of the centra in salamanders relative to that of frogs may result from the earlier migration of sclerotomal cells expressing Pax1 to the area surrounding the notochord.

  18. Initial clinical experience with a novel vertebral augmentation system for treatment of symptomatic vertebral compression fractures: A case series of 26 consecutive patients

    Directory of Open Access Journals (Sweden)

    Miller Larry E

    2011-09-01

    Full Text Available Abstract Background Minimally invasive vertebral augmentation procedures are widely used to treat vertebral compression fractures although procedural polymethylmethacrylate cement leakage remains common. We report herein our initial experience with a novel vertebral augmentation technique designed to treat symptomatic vertebral osteoporotic fractures and osteolytic metastases with minimal cement extravasation. Methods Forty-two vertebral fractures were identified in 26 consecutive patients (mean age 74 ± 9 years. All patients were treated with a novel percutaneous vertebral augmentation device (Kiva® VCF Treatment System, Benvenue Medical, Santa Clara, CA, USA. Indications for surgery included recent (≤ 3 months symptomatic osteoporotic vertebral fracture (n = 34 and pathologic vertebral fractures (e.g. metabolic bone disease, myeloma, metastasis (n = 8 located between T10 and S1. Patient outcomes were evaluated pre-treatment and at 2- and 6-month follow-up visits. Postoperative cement extravasation was assessed with computed tomography. Patient-reported back pain was quantified using an 11-point numeric scale. Back-specific functional disability was self-reported with the Oswestry Disability Index on a 0 to 100% scale. Results No cases of intraoperative hypotension, respiratory disturbance, neurological deterioration, infection, or death were observed. There were 2 (4.8% levels where anterior cement leakage was visible radiographically in patients with osteolyses. No intracanal leakage was observed. Back pain scores improved 71% (p Conclusions The initial clinical experience with the Kiva® System demonstrated significant improvements in back pain and function with minimal and clinically insignificant procedural cement leakage.

  19. A rare case report of bilateral testicular masses as an initial manifestation of systemic sarcoidosis

    Directory of Open Access Journals (Sweden)

    Hiren Patel

    2015-01-01

    Full Text Available Sarcoidosis is an idiopathic, systemic disease that rarely involves the genitourinary tract. Here, we present a case of a 40-year-old male presented with bilateral scrotal swelling. The scrotal ultrasound showed multiple echogenic masses bilaterally ranging between 3 mm and 15 mm involving both testicles. Enlarged retroperitoneal lymph nodes were detected on the abdominal computed tomography (CT. Surgical exploration of the testes with a frozen section analysis of the left testicular mass was carried out, and it revealed noncaseating granulomas. CT scan of the chest revealed the classic bilateral hilar and mediastinal lymphadenopathy with reticulonodular infiltrates. The final pathological diagnosis was systemic sarcoidosis with bilateral testicular involvement. Treatment with high-dose corticosteroids resulted in complete resolution of the testicular mass and a significant decrease in the size of the hilar, mediastinal, and retroperitoneal lymphadenopathy.

  20. Tissue is the issue-sarcoidosis following ABVD chemotherapy for Hodgkin's lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Subbiah Vivek

    2007-11-01

    Full Text Available Abstract Thirty two year old Caucasian female presented 2 months post partum with fevers, cough and shortness of breath. CT scan of the chest to rule out pulmonary embolism revealed mediastinal lymphadenopathy. Biopsy of the nodes revealed classic Hodgkin's lymphoma and she received ABVD chemotherapy. She was in remission as confirmed by a PET/CT scan. Five months later she had another PET/CT scan which showed areas of hypermetabolism indicating a possible relapse. Biopsy revealed sarcoidosis. She received steroids and 18 months later remained in clinical remission. This rare case of sarcoid following classic Hodgkin's lymphoma illustrates that clinical presentation, physical exam, lab investigations and even PET/CT scans may not be able to discriminate between Hodgkin's lymphoma and sarcoidosis. Tissue biopsy and pathological diagnosis remain the gold standard.

  1. Pancreatic involvement detected with (18)F-FDG PET/CT in disseminated sarcoidosis.

    Science.gov (United States)

    Delgado-Bolton, R C; Arias Navalón, J A; Rodríguez Alfonso, B; Delgado-Bolton, A N; Pérez-Castejón, M J; Sánchez-Escribano, R; Cabrera Martín, M N; Lapeña Gutiérrez, L; Carreras Delgado, J L

    2011-01-01

    La sarcoidosis is a granulomatous disease of unknown etiology. It may mimic malignancy and may affect multiple organs, with a variable clinical course. Pancreatic involvement is very rare. We present the case of a 78-year-old woman with systemic sarcoidosis whose (18)F-FDG PET/CT scan showed a pancreatic lesion and multiple lymphatic (thoracic and non-thoracic) and visceral lesions. The abnormal (18)F-FDG uptake in the pancreas was limited to the tail. A second (18)F-FDG PET/CT study performed 4 months and 3 weeks after the first PET/CT, following corticosteroid therapy during three and a half months, evidenced partial regression of the disease, with persistent (18)F-FDG pathological uptake in the pancreatic lesion. Copyright © 2010 Elsevier España, S.L. and SEMNIM. All rights reserved.

  2. Relapsing bronchiolitis obliterans organising pneumonia and chronic sarcoidosis in an atopic asthmatic patient.

    Science.gov (United States)

    Carbonelli, C; Roggeri, A; Cavazza, A; Zompatori, M; Zucchi, L

    2008-03-01

    Asthma is thought to be a Th2 disease while sarcoidosis is considered a Th1 granulomatous disorder. Organising pneumonia is a histologic pattern of lung injury. When it has no recognisable cause it is defined as cryptogenic organising pneumonia. We herein report the case of a patient with recurrent and steroid sensitive organising pneumonia associated with chronic sarcoidosis in an atopic, moderate persistent asthmatic patient. Each disease has been documented with transbronchial biopsies and recurrence of organising pneumonia was suggested by clinical features and by follow up HRCT which shows distinctive signs even in associated disease. Steroids are the mainstay of therapy for these disorders and especially for the consolidated processes typical of organising pneumonia but prognostic indices for relapse and progression are lacking.

  3. Exercise capacity, muscle strength, and fatigue in sarcoidosis: a follow-up study.

    Science.gov (United States)

    Marcellis, Rik G J; Lenssen, Antoine F; Kleynen, Stephan; De Vries, Jolanda; Drent, Marjolein

    2013-06-01

    The purpose of this study was to examine changes in the prevalence of exercise intolerance, reduced muscle strength, and fatigue and the changes in these parameters in individual patients during a 2-year follow-up study. Ninety sarcoidosis patients (62 males and 28 females; mean age: 46.0 ± 10.2 years) participated in a 2-year follow-up study. At the baseline and follow-up measurements, patients performed a 6-min walk test and elbow flexor muscle strength, quadriceps peak torque, and hamstrings peak torque tests. Maximal inspiratory pressure was recorded. All patients completed the Fatigue Assessment Scale. Both at baseline and follow-up, a substantial proportion of the patients showed a reduced 6-minute walk test (41.6 and 34.8 %, respectively), elbow flexor muscle strength (6.7 and 14.6 %), quadriceps peak torque (21.3 and 18 %), hamstrings peak torque (13.5 and 12.4 %), and maximal inspiratory pressure (45.9 and 48.6 %). The majority of the patients reported fatigue (86 and 77 %). These physical impairments remained stable during the follow-up period. The prevalence of these physical impairments in patients diagnosed with sarcoidosis muscle weakness, and fatigue are frequent problems in symptomatic sarcoidosis patients with a stable and persistent character. This study highlights that beyond medical treatment a rehabilitation program should be considered as adjunct therapy in the multidisciplinary management of sarcoidosis patients even though the achieved benefit needs future studies.

  4. Sarcoidosis en la infancia. Una rara enfermedad sistémica

    Directory of Open Access Journals (Sweden)

    Antonio Zamora-Chávez

    2016-03-01

    Conclusiones: Se resalta la importancia de considerar el diagnóstico de sarcoidosis en los pacientes con hepatomegalia, adenopatías, daño pulmonar difuso, eritema nodoso, masa testicular e hipercalcemia, así como la necesidad del abordaje multidisciplinario para valorar el compromiso orgánico múltiple y el inicio oportuno de la terapia con esteroides, con el fin de evitar la progresión de la enfermedad.

  5. Development of sarcoidosis 6-month post discontinuation of etanercept: coincidence or real association?

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-08-01

    There have been numerous reports of granulomatous diseases developing in patients receiving anti-tumour necrosis factor (TNF) therapy. Herein, we report a patient who developed sarcoidosis 6 months after discontinuation of etanercept. To date, all reported cases have occurred in patients undergoing ongoing treatment with TNF blockers with resolution on its discontinuation. A 47-year-old man was diagnosed with seropositive rheumatoid arthritis (RA) in 2003. He was initially treated with methotrexate and corticosteroids. In 2005, adalimumab was added due to ongoing disease activity. However, he had persistent low-grade synovitis of bilateral wrist joints and remained oral glucocorticoids dependent. In October 2008, adalimumab was switched to etanercept with marginal benefit; however, etanercept was continued until March 2009. Rituximab was discontinued due to an immediate allergic reaction. In September 2009, he developed bilateral ankle synovitis with erythema nodosum. Further investigations (chest X-ray and CT scan of thorax) revealed new development of bilateral hilar lymphadenopathy and interstitial nodular changes typical of sarcoidosis. His baseline therapy of methotrexate was continued. His recent repeat chest X-ray and CT scan of thorax (March 2010) has shown significant spontaneous resolution of his mediastinal lymphadenopathy and pulmonary nodules. Apart from the initial brief course of NSAIDs, his sarcoidosis resolved spontaneously without requiring any further therapy. For his rheumatoid arthritis, he has been recently commenced on abatacept and his baseline therapy of methotrexate has been continued. It remains speculative as to whether the concurrence of RA and sarcoidosis is purely serendipitous, or is related to an immunodysregulatory state attributable to TNF blockade.

  6. Acute arthritis as initial presentation of sarcoidosis: Significance of chest X-ray

    Directory of Open Access Journals (Sweden)

    Muhammed Emin Akkoyunlu

    2012-03-01

    Full Text Available Sarcoidosis is a multisystem granulomatous disease thatpredominantly affects the lungs. It usually presents withfatigue and respiratory findings. The rate of arthritis insarcoidosis is 15-25%. Arthritis as initial manifestation ofsarcoidosis has been reported rarely in the literature. Inpresent paper we highlighted the importance of sarcoidosisand conventional chest X-ray in differential diagnosisof acute arthritis. J Clin Exp Invest 2012; 3(1: 102-104

  7. Ventricular Tachycardia Storm in Cardiac Sarcoidosis: A 76-Day-ICU-Nightmare

    Directory of Open Access Journals (Sweden)

    Dr. Ajay M. Naik, MD, DM, DNB, FACC, FHRS

    2012-11-01

    Full Text Available Ventricular Tachycardia (VT is a life threatening complication in a patient with Cardiac Sarcoidosis. The management becomes extremely challenging when it is refractory to traditional anti-arrhythmic drugs. Herein, we describe a case where a 33-year-old patient with VT storm, with an implantable cardioverter defibrillator (ICD, was managed by medications, sedation, ventilator support and multiple Radio-Frequency (RF ablation procedures over 76- days ICU stay period.

  8. The Klatskin Tumor That Wasn?t: An Unusual Presentation of Sarcoidosis

    OpenAIRE

    Farooq, Priya D.; Potosky, Darryn R.

    2016-01-01

    We present the case of a patient who presented with signs and symptoms associated with a Klatskin tumor. After endoscopic retrograde cholangiopancreatography (ERCP) and biopsy, she was found instead to have granulomatous infiltration of the extrahepatic biliary tree consistent with biliary sarcoidosis. The patient was treated successfully with systemic corticosteroids and azathioprine. She later developed cutaneous, lymphatic, and pulmonary granulomatous disease. Isolated biliary disease is a...

  9. Diminished activity of tartrate resistant acid phosphatase in alveolar macrophages from patients with active sarcoidosis.

    OpenAIRE

    Barth, J.; Kreipe, H.; Kiemle-Kallee, J; Radzun, H.J.; Parwaresch, M R; Petermann, W.

    1988-01-01

    Alveolar macrophages differ from their percursors in blood, monocytes, by expressing strong activity of the tartrate resistant variant of acid phosphatase (TAcP). A study was carried out to analyse the expression of this enzyme cytochemical marker by alveolar macrophages from bronchoalveolar lavage cells from 34 patients with sarcoidosis and 12 control subjects. Alveolar macrophages from control subjects displayed a strong and homogeneous staining pattern and only 0.1% of cells were negative ...

  10. Post-9/11 sarcoidosis in WTC-exposed firefighters and emergency medical service workers.

    Science.gov (United States)

    Webber, Mayris P; Yip, Jennifer; Zeig-Owens, Rachel; Moir, William; Ungprasert, Patompong; Crowson, Cynthia S; Hall, Charles B; Jaber, Nadia; Weiden, Michael D; Matteson, Eric L; Prezant, David J

    2017-06-07

    The World Trade Center (WTC) disaster released a huge quantity and variety of toxicants into the environment. To-date, studies from each of the three major cohorts of WTC-exposed workers have suggested "greater than expected" numbers of post-9/11 cases in some workers. We undertook this study to estimate the incidence of post-9/11 sarcoidosis in ∼13,000 male firefighters and EMS workers enrolled in The Fire Department of the City of New York (FDNY) WTC Health Program; to compare FDNY incidence to rates from unexposed, demographically similar men in the Rochester Epidemiology Project (REP); and, to examine rates by level of WTC exposure. We calculated incidence of sarcoidosis diagnosed from 9/12/2001 to 9/11/2015, and generated expected sex- and age-specific rates based on REP rates. Standardized incidence ratios (SIR) based on REP rates, and 95% confidence intervals (95% CI) were estimated. Two sensitivity analyses limited cases to those with intra-thoracic symptoms or biopsy confirmation. We identified 68 post-9/11 cases in the FDNY cohort. Overall, FDNY rates were significantly higher than expected rates (SIR = 2.8; 95% CI = 2.2, 3.6). Including only symptomatic cases, the SIR decreased (SIR = 2.2; 95% CI = 1.5, 3.0), but remained significantly elevated. SIRs ranged from 2.7 (95% CI = 2.0, 3.5) in the lower WTC exposure group to 4.2 (95% CI = 1.9, 8.0) in the most highly exposed. We found excess incident post-9/11 sarcoidosis in WTC-exposed workers. Continued surveillance, particularly of those most highly exposed, is necessary to identify those with sarcoidosis and to follow them for possible adverse effects including functional impairments and organ damage. Copyright © 2017 Elsevier Ltd. All rights reserved.

  11. A CASE OF CARDIAC SARCOIDOSIS WITH SUSTAINED VENTRICULAR TACHYCARDIA SUCCESSFULLY TREATED BY METHYLPREDONISOLONE PULSE THERAPY

    OpenAIRE

    木田, 順富; 中嶋, 民夫; 山本, 広光; 坂口, 泰弘; 椎木, 英夫; 橋本, 俊雄; 土肥, 和紘; 藤本, 眞一

    2000-01-01

    A 63-year old woman was admitted because of ventricular tachycardia and congestive heart failure. A chest radiograph showed cardiomegaly and bilateral hilar lymphadenopathy. Echocardiogram showed diffuse left ventricular hypokinesis, dilated left ventricle and thinning of the interventricular septum. On gallium scintigram gallium was accumulated in the heart, especially in the left ventricle. Although endomyocardial biopsy revealed non-specific fibrosis, cardiac sarcoidosis was clinically sus...

  12. Steroid pulse therapy was effective for cardiac sarcoidosis with ventricular tachycardia and systolic dysfunction

    OpenAIRE

    Okabe, Toshitaka; Yakushiji, Tadayuki; Hiroe, Michiaki; Oyama, Yuji; Igawa, Wataru; Ono, Morio; Kido, Takehiko; Ebara, Seitaro; Yamashita, Kennosuke; Yamamoto, Myong Hwa; Saito, Shigeo; Hoshimoto, Koichi; Kisaki, Amemiya; Isomura, Naoei; Araki, Hiroshi

    2016-01-01

    Abstract A 32?year?old man presented with palpitation. He was diagnosed with pulmonary sarcoidosis by lung biopsy. The electrocardiogram showed first?degree atrioventricular block and complete right bundle branch block (CRBBB). We planned to examine laboratory data, echocardiography, Holter monitoring, and gallium?67 scintigraphy. Before he went through all these exams, he developed ventricular tachycardia. After defibrillation was performed, his electrocardiogram revealed complete atrioventr...

  13. Spectrum of lymphoid hyperplasia: colonic manifestations of sarcoidosis, infectious mononucleosis, and Crohn's disease

    Energy Technology Data Exchange (ETDEWEB)

    Ell, S.R.; Frank, P.H.

    1981-10-15

    The radiographic pattern of nodular lymphoid hyperplasia, perhaps better called the lymphoid follicular pattern, has variously been described as an indication of disease and as a normal variant in the adult, with current opinion favoring the latter. We report 3 cases wherein this pattern resulted from definite pathologic processes: sarcoidosis, infectious mononucleosis, and Crohn's disease. Although usually of no pathological significance, the benign follicular pattern may reflect a variety of diseases.

  14. Increased serum YKL-40 in patients with pulmonary sarcoidosis--a potential marker of disease activity?

    DEFF Research Database (Denmark)

    Johansen, Julia S; Milman, Nils; Hansen, Michael

    2005-01-01

    YKL-40, a growth factor for fibroblasts and vascular endothelial cells, is secreted by macrophages and neutrophils. Elevated serum YKL-40 is found in patients with diseases characterized by inflammation, tissue remodelling and ongoing fibrosis. The aim was to evaluate whether macrophages and giant...... cells in the granulomatous sarcoid lesions of patients with pulmonary sarcoidosis produce YKL-40 and to determine whether serum YKL-40 in these patients were associated with disease activity....

  15. Effects of molecular structural variants on serum Krebs von den Lungen-6 levels in sarcoidosis

    Directory of Open Access Journals (Sweden)

    Shigemura Masahiko

    2012-07-01

    Full Text Available Abstract Background Serum Krebs von den Lungen-6 (KL-6, which is classified as human mucin-1 (MUC1, is used as a marker of sarcoidosis and other interstitial lung diseases. However, there remain some limitations due to a lack of information on the factors contributing to increased levels of serum KL-6. This study was designed to investigate the factors contributing to increased levels of serum KL-6 by molecular analysis. Methods Western blot analysis using anti-KL-6 antibody was performed simultaneously on the bronchoalveolar lavage fluid (BALF and serum obtained from 128 subjects with sarcoidosis. Results KL-6/MUC1 in BALF showed three bands and five band patterns. These band patterns were associated with the MUC1 genotype and the KL-6 levels. KL-6/MUC1 band patterns in serum were dependent on molecular size class in BALF. Significantly increased levels of serum KL-6, serum/BALF KL-6 ratio and serum soluble interleukin 2 receptor were observed in the subjects with influx of high molecular size KL-6/MUC1 from the alveoli to blood circulation. The multivariate linear regression analysis involving potentially relevant variables such as age, gender, smoking status, lung parenchymal involvement based on radiographical stage and molecular size of KL-6/MUC1 in serum showed that the molecular size of KL-6/MUC1 in serum was significant independent determinant of serum KL-6 levels. Conclusions The molecular structural variants of KL-6/MUC1 and its leakage behavior affect serum levels of KL-6 in sarcoidosis. This information may assist in the interpretation of serum KL-6 levels in sarcoidosis.

  16. [Hemoptysis in sarcoidosis. Apropos of 6 cases including 4 with fatal outcome].

    Science.gov (United States)

    Lemay, V; Carette, M F; Parrot, A; Bazelly, B; Grivaux, M; Milleron, B

    1995-01-01

    Haemoptysis rarely occurs in sarcoidosis. Most cases usually occur in patients with advanced disease and major fibrosis. We observed 6 cases including 5 with fibrosis and 1 with inaugural unilateral lymph node involvement. Haemoptysis is a sign of gravity since it is the second most frequent cause of death described in the literature. In our series 4 of the 6 cases were fatal. Aspergillus colonization of a cavity is the most frequently found aggravating factor. Ideally, surgery is indicated but usually cannot be performed due to the patients respiratory function and the extent of the lesions. Oral drugs have little effect. Certain authors have had success with local, initially intrabronchic anti-aspergillus treatment. CT-guided application is often helpful. Embolization may stop the bleeding but in the long-term, a more or less voluminous haemoptysis often recurs. Other causes of bleeding are rare. Systemic hypervascularization of sarcoidosis lesions has been proposed as one mechanism other than infection. The cause may also be a simple granuloma. Symptomatic initial treatment by embolization is also proposed in these cases. Finally, massive haemoptysis can occur by erosion of the pulmonary artery due to a necrotic sarcoidosis lesion. In our series, surgery was impossible in three patients who died. In the three others, embolization was possible in 2 and the third underwent successful surgery.

  17. Patient confidence and quality of life in idiopathic pulmonary fibrosis and sarcoidosis.

    Science.gov (United States)

    Kotecha, Jalpa; Atkins, Christopher; Wilson, Andrew

    2016-12-23

    Idiopathic pulmonary fibrosis (IPF) and sarcoidosis impact significantly on health-related quality of life (HRQOL). There are few studies on the impact of patient confidence on HRQOL in these conditions. 1. To investigate whether patient confidence is associated with HRQOL, anxiety, depression, dyspnoea or fatigue. 2. To assess if patient confidence is associated with inpatient admissions, access to community healthcare and, for IPF patients, mortality and disease severity. Study participants self-completed seven questionnaires: Hospital Anxiety and Depression Scale, EuroQol 5D (EQ5D), King's Brief Interstitial Lung Disease questionnaire, St George's Respiratory Questionnaire, MRC dyspnoea scale, Fatigue Assessment Scale and a non-validated questionnaire assessing patient confidence, symptom duration and access to community healthcare. Lung function and follow-up data were collected from hospital electronic databases. Spearman's rank correlation coefficients were calculated to assess for correlation between patient confidence, questionnaire variables and inpatient admissions. Chi-square tests were performed to assess for association between patient confidence, mortality and disease severity. 75 IPF patients and 69 sarcoidosis patients were recruited to the study. Patient confidence in IPF was significantly negatively correlated with depression and fatigue, and significantly positively correlated with EQ5D scores, but not healthcare outcomes. No associations were found between confidence and any of the variables assessed in sarcoidosis. Lower levels of confidence in IPF patients are associated with higher levels of depression and fatigue and worse HRQOL. Efforts should be made to improve patient confidence to assess the impact on HRQOL.

  18. [Extensive interactions between eating and weight disorder, major depression, pain, and sarcoidosis - case 5/2012].

    Science.gov (United States)

    Schäflein, Eva; Wettach, Irmtraud; Smolka, Robert; Kuprion, Jürgen; Zipfel, Stephan; Teufel, Martin

    2012-06-01

    We report on a 41-year-old female patient suffering from obesity, binge eating more than twice a week with loss of control, eating rapidly and feeling guilty after eating, dyspnoea and chronic pain in the whole body, especially in her arms, legs and in both ankles. Furthermore, subdued mood, loss of interest and pleasure, fatigue and impaired concentration could be recognized. In the past, weight increase had been observed when corticosteroids were given against exacerbations of sarcoidosis. In the case of our patient, the beginning of sarcoidosis and increase of weight and pain correlated with augmentation of depression and psychosocial stress. Dysfunctional behavioral features and multiple interactions between diseases could be observed. We diagnosed obesity, binge eating disorder, major depression, chronic pain disease with somatic and psychical components and sarcoidosis. The patient was treated in a multimodal therapy program including psychotherapy, pharmacotherapy and psychopharmacotherapy, nutritionist advice and therapeutic exercise. A weight loss of 7.9 kg (5.9 %), well-balanced diet, reduction of binge eating and of pain intensity, mood stabilization as well as perception and expression of emotions and coping strategies in chronic diseases were achieved. Interdisciplinary treatment of patients suffering from psychosomatic, somatic and mental diseases is crucial for a good outcome. © Georg Thieme Verlag KG Stuttgart · New York.

  19. [Coexistence of sarcoidosis and primary Sjögren syndrome: a clinical analysis and literature review].

    Science.gov (United States)

    Song, X Y; Huang, H; Liu, Y Z; Zhao, Y Y; Li, S; Xu, Z J

    2017-05-01

    Four patients with coexistence of sarcoidosis and primary Sjögren syndrome (pSS) were retrospectively analyzed.All patients were female, who were referred to our department mainly because of respiratory symptoms.Positive antinuclear antibody(ANA) was detected in 2 patients and anti-Sjögrens syndrome A (SSA) antibody positive in 1 patient.All patients presented specific histologic patterns of both sarcoidosis and pSS.Publications related to coexistence of these two diseases were reviewed.Forty-one patients were finally included in the analysis, among whom 37 confirmed patients were from literature search.There were 37 women and 4 men.The main clinical features presentation were xerophthalmia in 40, xerostomia in 38, hilaradenopathies in 28, interstitial lung disease in 15, respiratory symptoms in 13.The main immunologic data were positive ANA in 23, SSA antibody in 19, anti-Sjögrens syndrome B antibody in 10 and rheumatoid factor in 12.All patients presented specific histologic patterns of both diseases.Patients with both sarcoidosis and pSS of ten represent multisystemic involvement and positive immunologic parameters, as well as the dual expression of specific histologic characteristics.

  20. MR characteristics of benign lymph node enlargement in sarcoidosis and Castleman's disease

    Energy Technology Data Exchange (ETDEWEB)

    Geer, G. de; Webb, W.R.; Sollitto, R.; Golden, J.

    1986-05-01

    The magnetic resonance characteristics of mediastinal and hilar lymphadenopathy were reviewed in five patients with sarcoidosis and one with Castleman's disease. Although the T1 values of 26 nodes measured in patients with sarcoidosis ranged from 390 to 786 msec (mean 549 msec; SD 88 msec), the mean values recorded for each patient were quite similar (range 501-616 msec). T2 values ranged from 40 to 68 msec (mean 49 msec; SD 7 msec). In the patient with Castleman's disease T1 and T2 values measured 838 msec and 45 msec respectively. In comparison to T1 and T2 values we have recently measured in patients with proven mediastinal lymph node metastases from bronchogenic carcinoma, lymph nodes in the patients with sarcoidosis have significantly lower T1 (p<.001) and T2 (p<.05) values. However, because of significant overlap between the values recorded in these two groups, their differentiation may be difficult in individual cases.

  1. Imaging of sarcoidosis of the airways and lung parenchyma and correlation with lung function.

    Science.gov (United States)

    Nunes, Hilario; Uzunhan, Yurdagul; Gille, Thomas; Lamberto, Christine; Valeyre, Dominique; Brillet, Pierre-Yves

    2012-09-01

    Imaging has a prominent role in the assessment of sarcoidosis diagnosis and outcome, which are extremely variable. Chest radiography staging helps predict the probability of spontaneous remission, and stage IV is associated with higher mortality. However, the reproducibility of reading is poor and changes in radiography and lung function are inconsistently correlated, which may be problematic for the monitoring of disease and treatment response. Chest computed tomography (CT) makes a great diagnostic contribution in difficult cases. Bilateral hilar lymphadenopathy with peri-lymphatic micronodular pattern is highly specific for sarcoidosis. CT is important for the investigation of pulmonary complications, including aspergilloma and pulmonary hypertension. CT improves the yield of bronchoscopy for obtaining a positive endobronchial or transbronchial biopsy. CT findings may also discriminate between active inflammation and irreversible fibrosis, with occasional influence on therapeutic decisions. Three CT patterns of fibrotic sarcoidosis are identified, with different functional profiles: predominant bronchial distortion is associated with obstruction; honeycombing is associated with restriction and lower diffusing capacity of the lung for carbon monoxide; whereas functional impairment is relatively minor with linear pattern. The clinical impact of correlations between CT severity scores and functional impairment is uncertain, except for its utility elucidating the mechanisms of airflow limitation, which include bronchial distortion, peribronchovascular thickening, air-trapping and bronchial compression by lymphadenopathy.

  2. Sarcoidosis cardíaca: descripción de tres casos

    Directory of Open Access Journals (Sweden)

    Mirta Diez

    2010-01-01

    Full Text Available RESUMENLa sarcoidosis es una enfermedad multisistémica de etiología desconocida que puede afectarcualquier órgano. Presenta considerables diferencias en su prevalencia según los distintospaíses y razas. Diversas comunicaciones sugieren que el compromiso cardíaco determinamal pronóstico. Existen pocas series clínicas de sarcoidosis cardíaca en Latinoamérica y nohay consenso claro acerca de los aspectos diagnósticos y terapéuticos de esta patología. Enesta presentación se describen tres casos de sarcoidosis cardíaca, sus características clínicas,electrocardiográficas, métodos de imágenes y, en dos de ellos, la anatomía patológica delmaterial obtenido mediante biopsia endomiocárdica. Se refiere, asimismo, la evolución finalde los pacientes durante el seguimiento.REV ARGENT CARDIOL 2010;78:361-363.

  3. Tested Demonstrations.

    Science.gov (United States)

    Gilbert, George L., Ed.

    1990-01-01

    Presented are two demonstrations; "Heat of Solution and Colligative Properties: An Illustration of Enthalpy and Entropy," and "A Vapor Pressure Demonstration." Included are lists of materials and experimental procedures. Apparatus needed are illustrated. (CW)

  4. High-throughput hyperdimensional vertebrate phenotyping.

    Science.gov (United States)

    Pardo-Martin, Carlos; Allalou, Amin; Medina, Jaime; Eimon, Peter M; Wählby, Carolina; Fatih Yanik, Mehmet

    2013-01-01

    Most gene mutations and biologically active molecules cause complex responses in animals that cannot be predicted by cell culture models. Yet animal studies remain too slow and their analyses are often limited to only a few readouts. Here we demonstrate high-throughput optical projection tomography with micrometre resolution and hyperdimensional screening of entire vertebrates in tens of seconds using a simple fluidic system. Hundreds of independent morphological features and complex phenotypes are automatically captured in three dimensions with unprecedented speed and detail in semitransparent zebrafish larvae. By clustering quantitative phenotypic signatures, we can detect and classify even subtle alterations in many biological processes simultaneously. We term our approach hyperdimensional in vivo phenotyping. To illustrate the power of hyperdimensional in vivo phenotyping, we have analysed the effects of several classes of teratogens on cartilage formation using 200 independent morphological measurements, and identified similarities and differences that correlate well with their known mechanisms of actions in mammals.

  5. {sup 18}F-FDG PET patterns and BAL cell profiles in pulmonary sarcoidosis

    Energy Technology Data Exchange (ETDEWEB)

    Keijsers, Ruth G.; Verzijlbergen, Fred J. [St Antonius Hospital, Department of Nuclear Medicine, Postbox 2500, Nieuwegein (Netherlands); Grutters, Jan C.; Bosch, Jules M. van den [St Antonius Hospital, Department of Pulmonology, Nieuwegein (Netherlands); Velzen-Blad, Heleen van [St Antonius Hospital, Medical Microbiology and Immunology, Nieuwegein (Netherlands); Oyen, Wim J. [Radboud University Nijmegen Medical Centre, Department of Nuclear Medicine, Nijmegen (Netherlands)

    2010-06-15

    Bronchoalveolar lavage (BAL) and {sup 18}F-fluorodeoxyglucose ({sup 18}F-FDG) PET can both demonstrate sarcoid activity. To assess whether metabolic activity imaged by {sup 18}F-FDG PET represents signs of disease activity as reflected by BAL, {sup 18}F-FDG PET patterns were compared with BAL cell profiles. In this retrospective analysis, 77 newly diagnosed pulmonary sarcoidosis patients underwent BAL and {sup 18}F-FDG PET. Based on {sup 18}F-FDG PET, patients were diagnosed with exclusively mediastinal/hilar activity (group A) and activity in the lung parenchyma (group B). Per group, BAL lymphocytes (%), CD4/CD8 ratio, CD103{sup +}CD4{sup +}/CD4{sup +} ratio and neutrophils (%) were compared with the extent of metabolic activity expressed as the maximum standardized uptake value (SUV{sub max}). Additionally, SUV{sub max} and BAL parameters per radiographic stage were analysed. Overall, the SUV{sub max} in the lung parenchyma correlated with neutrophils and SUV{sub max} of the mediastinum/hila correlated with the CD4/CD8 ratio. In both groups, a significant, negative correlation between the SUV{sub max} of the mediastinum/hila and the CD103{sup +}CD4{sup +}/CD4{sup +} ratio was found. In group B, the SUV{sub max} of the mediastinum/hila correlated with the CD4/CD8 ratio, while the SUV{sub max} in the lung parenchyma correlated with the CD103{sup +}CD4{sup +}/CD4{sup +} ratio and neutrophils. Significant differences were found in the SUV{sub max}, CD4/CD8 ratio, CD103{sup +}CD4{sup +}/CD4{sup +} ratio and neutrophils between the radiographic stages. The SUV{sub max} of the lung parenchyma was positively related to the radiographic stage, while the SUV{sub max} of the mediastinum/hila and CD4/CD8 ratio were inversely related. {sup 18}F-FDG PET correlates with the CD4/CD8 ratio and neutrophils, suggesting that {sup 18}F-FDG PET represents this specific cell profile in BAL. High SUV{sub max} values of the lung parenchyma may therefore correlate with more severe

  6. 18F-fluorodeoxyglucose positron emission tomography-positive sarcoidosis after chemoradiotherapy for Hodgkin’s disease: a case report

    Directory of Open Access Journals (Sweden)

    Pham Alan

    2011-06-01

    Full Text Available Abstract Introduction The occurrence of granulomatous disease in the setting of Hodgkin's disease is rare; however, when it occurs it can pose significant clinical and diagnostic challenges for physicians treating these patients. Case presentation We report the case of a 33-year-old Caucasian woman of Mediterranean descent with newly diagnosed 18F-fluorodeoxyglucose (18F-FDG positron emission tomography (PET/computed tomography (CT scan-positive, early-stage Hodgkin's disease involving the cervical nodes who, despite having an excellent clinical response to chemotherapy, had a persistent 18F-FDG PET scan-positive study, which was suggestive of residual or progressive disease. A subsequent biopsy of her post-chemotherapy PET-positive nodes demonstrated sarcoidosis with no evidence of Hodgkin's disease. Conclusion This case highlights the fact that abnormalities observed on posttherapy PET/CT scans in patients with Hodgkin's disease are not always due to residual or progressive disease. An association between Hodgkin's disease and/or its treatment with an increased incidence of granulomatous disease appears to exist. Certain patterns of 18F-FDG uptake observed on PET/CT scans may suggest other pathologies, such as granulomatous inflammation, and because of the significant differences in prognosis and management, clinicians should maintain a low threshold of confidence for basing their diagnosis on histopathological evaluations when PET/CT results appear to be incongruent with the patient's clinical response.

  7. Effect of the TNF-α inhibitor adalimumab in patients with recalcitrant sarcoidosis: a prospective observational study using FDG-PET

    DEFF Research Database (Denmark)

    Milman, Nils; Graudal, Niels; Loft, Annika

    2012-01-01

    -PET) in patients with recalcitrant sarcoidosis treated with adalimumab. Methods: Prospective 24-week observational study. Patients continued medication with steroids and antimetabolites and received adalimumab 40 mg subcutaneously every other week. Ten patients with biopsy-proven sarcoidosis (two men) were...

  8. Osteomielitis vertebral piógena Pyogenic vertebral osteomyelitis

    Directory of Open Access Journals (Sweden)

    Pedro P. Perrotti

    2009-10-01

    Full Text Available La osteomielitis vertebral piógena (OVP es una localización poco frecuente (2-7% Se confirma con el aislamiento de un microorganismo de una vértebra, disco intervertebral, absceso epidural o paravertebral. Se describe una serie de casos por la infrecuente presentación de esta enfermedad, que puede ser consulta inicial en los servicios de clínica médica y por su sintomatología inespecífica que supone una dificultad diagnóstica. Tanto la columna lumbar como la dorsal fueron los sitios más afectados. El dolor dorsolumbar y la paraparesia fueron los síntomas más frecuentes de presentación. En ocho pacientes se aislaron Staphylococcus aureus, en uno Escherichia coli y en el restante Haemophylus sp. Se observó leucocitosis sólo en tres pacientes, y en dos velocidad de sedimentación globular mayor de 100 mm/h. Los diez pacientes presentaron imágenes características de osteomielitis vertebral piógena en la resonancia nuclear magnética. Dentro de las complicaciones, los abscesos paravertebrales y epidurales fueron los más frecuentes (en cinco enfermos. Además, un paciente presentó empiema pleural. De los diez pacientes de esta serie, siete recibieron inicialmente tratamiento médico empírico y luego específico para el germen aislado. En los restantes el tratamiento fue guiado de acuerdo al antibiograma. A dos enfermos fue necesario realizarles laminectomía descompresiva por compromiso de partes blandas y a otros dos estabilización quirúrgica por inestabilidad espinal, observándose buena evolución en todos los casos. Esta serie demuestra que, ante un paciente con dolor dorsolumbar y síntomas neurológicos se deberá tener en cuenta esta entidad para evitar un retraso en el tratamiento.Pyogenic osteomyelitis seldom affects the spine (2-7%. It is diagnosed by the isolation of a bacterial agent in the vertebral body, the intervertebral disks or from paravertebral or epidural abscesses. We report a retrospective study of ten

  9. Perforin down-regulation and adhesion molecules activation in pulmonary sarcoidosis: an induced sputum and BAL study.

    Science.gov (United States)

    Antoniou, Katerina M; Tsiligianni, Ioanna; Kyriakou, Despina; Tzanakis, Nikolaos; Tzouvelekis, Argyris; Siafakas, Nikolaos M; Bouros, Demosthenes

    2006-06-01

    Sarcoidosis is thought to be a T-helper type 1 cytokine-mediated disorder. Sputum induction has been proposed as a useful noninvasive method mainly for the assessment of airway diseases. However, it is unknown whether the balance of T-cytotoxic (Tc1) type 1 and Tc2 cells is altered in sarcoidosis. The primary aim of this study was to characterize the CD8+ T lymphocyte subpopulations in induced sputum from sarcoidosis patients, and to compare these subpopulations to those found in BAL fluid (BALF) from sarcoidosis patients. To further investigate the mechanism of the cytotoxic activity of CD8+ lymphocytes, we measured their perforin expression. Additionally, two adhesion molecules (CD62 and CD71), which are expressed on CD8+ T cells and may serve as novel immunologic markers, were detected. Department of Thoracic Medicine, University of Crete, and Department of Pneumonology, Democritus University of Thrace, Alexandroupolis, Greece. We prospectively studied 22 patients with sarcoidosis (median age, 48 years; age range, 25 to 65 years) and 10 healthy subjects (5 female and 5 male; median age, 39 years; age range, 26 to 60 years). The stimulation of lymphocytes with phorbol 12-myristate 13-acetate was followed by the use of double immunocytochemical methods to identify CD8+ interferon (IFN)-gamma producing cells (ie, Tc1) and CD8+ interleukin-4 producing cells (ie, Tc2). We found a significant decrease in the prestimulation percentage of IFN-gamma-positive CD8+ T cells in the BALF (p = 0.001) and induced sputum (p = 0.001) of sarcoidosis patients compared to the number in samples from healthy control subjects. However, no significant difference was documented between lymphocyte subsets poststimulation. Decreased levels of perforin expression were found in BALF (p = 0.001) and induced sputum (p < 0.001) of sarcoidosis patients compared to those in control subjects. The adhesion molecules were significantly increased in both the BALF and induced sputum of the sarcoid

  10. Lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia, and sarcoidosis: more pathological findings in the same chest CT, or a single pathological pathway?

    Science.gov (United States)

    Di Marco, Fabiano; Palumbo, Giuseppina; Terraneo, Silvia; Imeri, Gianluca; Lesma, Elena; Sverzellati, Nicola; Peron, Angela; Gualandri, Lorenzo; Canevini, Maria Paola; Centanni, Stefano

    2017-07-28

    Autoimmune hepatitis/primary biliary cirrhosis overlap syndrome, lymphangioleiomyomatosis/tuberous sclerosis complex (LAM-TSC), and sarcoidosis are three rare diseases. Here we present, to the best of our knowledge, the first description of a patient with the coexistence of these three diseases. A 47-year-old woman affected by LAM-TSC and primary biliary cirrosis/autoimmune hepatitis overlap syndrome. During her follow up a high resolution chest CT scan (HRTC) confirmed the presence of both multiple cysts and micronodular opacities consistent with multifocal micronodular pneumocytes hyperlasia (MMPH), and revealed multiple hilar-mediastinal symmetrical lymphadenopathies suggestive of sarcoidosis. Simultaneously, subcutaneous nodules appeared on her forearm bilaterally. Cutaneous biopsy showed granulomatous dermatitis with sarcoid-like granulomas. A diagnosis of stage I pulmonary sarcoidosis was made. No treatment for sarcoidosis was initiated since the patient had neither systemic involvement, nor respiratory impairment. The presence of more than one rare disease should challenge the concept of a potential common underlying mechanism, since the a priori probability of the concomitant presence of different conditions with different pathogenic mechanisms - especially if rare diseases - is low. We speculate that the dysregulation of the pathway involving mTOR and MAPK and their interaction might play a role in the pathogenesis of other diseases, including sarcoidosis.

  11. [Clinical features of Blau syndrome and early-onset sarcoidosis and associating CARD15/NOD2 gene mutations].

    Science.gov (United States)

    Kanazawa, Nobuo

    2007-04-01

    Sarcoidosis is a systemic inflammatory disease clinically characterized by swelling of bilateral hilar lymph nodes and histologically defined by non-caseating epithelioid cell granulomas. Among child cases, a special subtype, called the early-onset sarcoidosis, is known to appear in children younger than 4 years of age and to be characterized by a distinct triad of skin, joint and eye disorders without pulmonary involvement. On the other hand, autosomal dominantly-transmitted disease with a characteristic features similar to those of early-onset sarcoidosis has been reported as Blau syndrome. By a linkage analysis, the responsible gene for Blau syndrome has been mapped close to the IBD (Inflammatory Bowel Disease) 1 locus. After CARD15 (NOD2), originally identified as the susceptibility gene for Crohn's disease, was also proved to be responsible for Blau syndrome, the same gene mutations have been found in sporadic early-onset sarcoidosis cases. Nod2 recognizes a signal from bacterial cell wall component in the cytoplasm of monocytic cells to activate NF-kappaB, and thus can work as an intracellular sensor of bacteria. While the loss-of-function mutations in its LRR domain are associated with Crohn's disease, Blau syndrome and early-onset sarcoidosis are autoinflammatory diseases that are caused by the gain-of-function mutations in its NOD domain.

  12. CIRSE Guidelines on Percutaneous Vertebral Augmentation

    Energy Technology Data Exchange (ETDEWEB)

    Tsoumakidou, Georgia, E-mail: gtsoumakidou@yahoo.com; Too, Chow Wei, E-mail: spyder55@gmail.com; Koch, Guillaume, E-mail: guillaume.koch@gmail.com; Caudrelier, Jean, E-mail: jean.caudrelier@chru-strasbourg.fr; Cazzato, Roberto Luigi, E-mail: gigicazzato@hotmail.it; Garnon, Julien, E-mail: juliengarnon@gmail.com; Gangi, Afshin, E-mail: gangi@unistra.fr [Strasbourg University Hospital, Interventional Radiology Department (France)

    2017-03-15

    Vertebral compression fracture (VCF) is an important cause of severe debilitating back pain, adversely affecting quality of life, physical function, psychosocial performance, mental health and survival. Different vertebral augmentation procedures (VAPs) are used in order to consolidate the VCFs, relief pain,and whenever posible achieve vertebral body height restoration. In the present review we give the indications, contraindications, safety profile and outcomes of the existing percutaneous VAPs.

  13. Evolutionary Specialization of Tactile Perception in Vertebrates

    Science.gov (United States)

    Schneider, Eve R.

    2016-01-01

    Evolution has endowed vertebrates with the remarkable tactile ability to explore the world through the perception of physical force. Yet the sense of touch remains one of the least well understood senses at the cellular and molecular level. Vertebrates specializing in tactile perception can highlight general principles of mechanotransduction. Here, we review cellular and molecular adaptations that underlie the sense of touch in typical and acutely mechanosensitive vertebrates. PMID:27053733

  14. Evolutionary Specialization of Tactile Perception in Vertebrates

    OpenAIRE

    Schneider, Eve R.; Gracheva, Elena O.; Bagriantsev, Slav N.

    2016-01-01

    Evolution has endowed vertebrates with the remarkable tactile ability to explore the world through the perception of physical force. Yet the sense of touch remains one of the least well understood senses at the cellular and molecular level. Vertebrates specializing in tactile perception can highlight general principles of mechanotransduction. Here, we review cellular and molecular adaptations that underlie the sense of touch in typical and acutely mechanosensitive vertebrates.

  15. Vertebral wedging characteristic changes in scoliotic spines.

    Science.gov (United States)

    Parent, Stefan; Labelle, Hubert; Skalli, Wafa; de Guise, Jacques

    2004-10-15

    A morphometric analysis of vertebral wedging in vertebrae from scoliotic specimens. To quantify the vertebral body changes in 30 anatomic specimens affected by a scoliotic deformity. Only a few studies have evaluated the exact changes occurring at the level of the vertebral body in scoliotic spines. Most are observational studies of rare scoliotic specimens presenting major curvatures. The orientation of vertebral wedging is important for the planning of corrective surgery, performing vertebral osteotomy, and the evaluation of possible growth modulation. Thirty scoliotic specimens with curves presenting various degrees of severity were studied using a three-dimensional digitizing protocol developed to create a precise three-dimensional reconstruction of the vertebrae. Every scoliotic specimen was then matched with a normal specimen, and comparisons were made on the vertebral body parameters both for thoracic and lumbar vertebrae. Analysis of variance and t test calculations were performed to identify significant differences with P = 0.05. A total of 471 vertebrae from scoliotic spines and 510 vertebrae from normal specimens were measured. Vertebral wedging increased progressively towards the apex of the curve and was maximal at the apex. Vertebral wedging was more prominent in the frontal plane, and there was minimal wedging in the sagittal plane. Vertebral heights were significantly different at T3 and T4 for the upper adjacent curve and at T6-T8 for a typical right thoracic curve, with smaller heights located on the concavity of the curve. No changes were observed on the convexity of the curve. Vertebral wedging is an essential component of the scoliotic deformity. The present study provides critical information for corrective surgery and vertebral osteotomy, as vertebral wedging occurs primarily in the frontal plane. Accurate knowledge of this deformity should also provide new insight into corrective surgical strategies aiming at growth modulation and more

  16. Tested Demonstrations.

    Science.gov (United States)

    Gilbert, George L., Ed.

    1985-01-01

    Two demonstrations are described. The first (useful as an introduction to kinetics) shows how the rate of a reaction is fast at first and then gradually decreases to zero when one reactant has been used up. The second is a gas density demonstration using 1,1,2-trichloro-1,2,2-trifluoro ethane. (JN)

  17. A case of traumatic intracranial vertebral artery injury presenting with life-threatening symptoms

    Directory of Open Access Journals (Sweden)

    Kishi S

    2012-04-01

    Full Text Available Seiji Kishi1, Kenji Kanaji2, Toshio Doi1, Tadashi Matsumura21Department of Nephrology, Tokushima University Hospital, Kuramoto-cho Tokushima, 2Department of General Internal Medicine, Rakuwakai Otowa Hospital, Otowachinji-cho Yamashina-ku Kyoto, JapanAbstract: Traumatic intracranial vertebral artery injury is a relatively rare but potentially fatal disease. We present a case of a 63-year-old man who presented with sudden onset of loss of consciousness after hitting his head. After immediate resuscitation, he showed quadriplegia and absence of spontaneous breathing. Brain and cervical spine magnetic resonance imaging revealed an atlantoaxial subluxation, fractured C2 odontoid process, left vertebral artery occlusion, and bilateral extensive ischemia in the medulla oblongata and high cervical spinal cord. Digital subtraction angiography demonstrated left vertebral artery dissection just below the level of vertebral body C2.Keywords: vertebral artery dissection, brainstem infarction, bilateral spinal cord infarction, neck trauma

  18. Ghrelin receptors in non-mammalian vertebrates

    Directory of Open Access Journals (Sweden)

    Hiroyuki eKaiya

    2013-07-01

    Full Text Available The growth hormone secretagogue-receptor (GHS-R was discovered in humans and pigs in 1996. The endogenous ligand, ghrelin, was discovered three years later, in 1999, and our understanding of the physiological significance of the ghrelin system in vertebrates has grown steadily since then. Although the ghrelin system in non-mammalian vertebrates is a subject of great interest, protein sequence data for the receptor in non-mammalian vertebrates has been limited until recently, and related biological information has not been well organized. In this review, we summarize current information related to the ghrelin receptor in non-mammalian vertebrates.

  19. CARD15 single nucleotide polymorphisms 8, 12 and 13 are not increased in ethnic Danes with sarcoidosis

    DEFF Research Database (Denmark)

    Milman, Nils; Nielsen, Ole Haagen; Hviid, Thomas Vauvert F

    2007-01-01

    and SNP13, respectively, were performed by capillary electrophoresis single-strand confirmation polymorphism in 53 patients with histologically verified sarcoidosis and in 103 healthy controls. RESULTS: The frequencies of CARD15 mutations in sarcoidosis patients were: SNP8, 4/106 chromosomes (3.8%); SNP12......, 2/106 chromosomes (1.9%); SNP13, 2/106 chromosomes (1.9%); SNP8+SNP12+SNP13, 8/106 chromosomes (7.6%). All 8 patients were heterozygous. The frequencies in controls were: SNP8, 9/206 chromosomes (4.4%); SNP12, 2/206 chromosomes (1.0%); SNP13, 4/206 chromosomes (1.9%); SNP8+SNP12+SNP13, 15......BACKGROUND: Mutations of the caspase-activating recruitment domain 15 (CARD15) gene on chromosome 16 are associated with chronic inflammatory granulomatous bowel disease (Crohn's disease). Sarcoidosis is a systemic granulomatous disease with unknown etiology, which shares histological features...

  20. Allergenicity of vertebrate tropomyosins: Challenging an immunological dogma.

    Science.gov (United States)

    González-Fernández, J; Daschner, A; Cuéllar, C

    With the exception of tilapia tropomyosin, other anecdotic reports of tropomyosin recognition of vertebrate origin are generally not accompanied by clinical significance and a dogmatic idea is generally accepted about the inexistence of allergenicity of vertebrate tropomyosins, based mainly on sequence similarity evaluations with human tropomyosins. Recently, a specific work-up of a tropomyosin sensitised patient with seafood allergy, demonstrated that the IgE-recognition of tropomyosin from different fish species can be clinically relevant. We hypothesise that some vertebrate tropomyosins could be relevant allergens. The hypothesis is based on the molecular evolution of the proteins and it was tested by in silico methods. Fish, which are primitive vertebrates, could have tropomyosins similar to those of invertebrates. If the hypothesis is confirmed, tropomyosin should be included in different allergy diagnosis tools to improve the medical protocols and management of patients with digestive or cutaneous symptoms after fish intake. Copyright © 2016 SEICAP. Published by Elsevier España, S.L.U. All rights reserved.