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Sample records for igg4-related systemic diseases

  1. Immunoglobulin G4-related cholangitis: a variant of IgG4-related systemic disease.

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    Novotný, Ivo; Dítě, Petr; Trna, Jan; Lata, Jan; Husová, Libuše; Geryk, Edvard

    2012-01-01

    IgG4-related sclerosing cholangitis as part of IgG4 systemic-related diseases is commonly associated with autoimmune pancreatitis. Major clinical manifestations of IgG4-related sclerosing diseases are apparent in the organs in which tissue fibrosis with obstructive phlebitis is pathologically induced. IgG4-related sclerosing cholangitis is included within the heterogeneous group of 'sclerosing cholangitis'. Sclerosing cholangitis may be associated with choledocholithiasis, infection or biliary malignancies. Sclerosing cholangitis of unknown etiology is called primary sclerosing cholangitis (PSC). Conservative therapy of PSC is usually unsuccessful, the disease involves extra- and/or intrahepatic biliary tree, and the end point of this disease is liver cirrhosis. Typically, PSC is identified at the age of 30 to 40 years, and the disease is frequently associated with inflammatory bowel diseases. On the other hand, IgG4-related sclerosing cholangitis is not associated with inflammatory bowel diseases. In patients with IgG4-related sclerosing cholangitis, a first symptom can be obstructive jaundice, whereas obstructive jaundice is rarely present in PSC. Clinically, patients with IgG4-related sclerosing cholangitis are older at diagnosis compared to patients with PSC. A typical diagnostic feature of IgG4-related sclerosing cholangitis is elevation of serum immunoglobulin G4. In patients with IgG4-related sclerosing cholangitis, response to steroid therapy is high; in patients with PSC corticosteroid therapy is unsuccessful. Histochemically abundant infiltration of IgG4-positive plasma cells is detected in the biliary duct wall. Histologically, we can identify dense lymphoplasmacytic infiltration of the bile duct wall, transmural fibrosis, lymphoplasmacytic infiltration and fibrosis in the periportal area of the liver - a typically obliterative phlebitis. The biliary epithelium is usually intact in contrast to PSC, where mucosal erosion is often present. Steroids are the

  2. Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease.

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    Kim, Seokhwi; Bae, Hyunsik; Choi, Misun; Kim, Binnari; Heo, Jin Seok; Kim, Ho Seong; Choi, Seung Hee; Jang, Kee-Taek

    2016-07-01

    IgG4-related disease (IgG4-RD) may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.

  3. Isolated Mass-Forming IgG4-Related Cholangitis as an Initial Clinical Presentation of Systemic IgG4-Related Disease

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    Seokhwi Kim

    2016-07-01

    Full Text Available IgG4-related disease (IgG4-RD may involve multiple organs. Although it usually presents as diffuse organ involvement, localized mass-forming lesions have been occasionally encountered in pancreas. However, the same pattern has been seldom reported in biliary tract. A 61-year-old male showed a hilar bile duct mass with multiple enlarged lymph nodes in imaging studies and he underwent trisectionectomy under impression of cholangiocarcinoma. Gross examination revealed a mass-like lesion around hilar bile duct. Histopathologically, dense lymphoplasmacytic infiltration and storiform fibrosis were identified without evidence of malignancy. Immunohistochemical stain demonstrated rich IgG4-positive plasma cell infiltration. Follow-up imaging studies disclosed multiple enlarged lymph nodes with involvement of pancreas and perisplenic soft tissue. The lesions have been significantly reduced after steroid treatment, which suggests multi-organ involvement of systemic IgG4-RD. Here, we report an unusual localized mass-forming IgG4-related cholangitis as an initial presentation of IgG4-RD, which was biliary manifestation of systemic IgG4-related autoimmune disease.

  4. IgG4-related sclerosing disease

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    Terumi Kamisawa; Atsutake Okamoto

    2008-01-01

    Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retroperitoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.

  5. IgG4-Related Disease Presenting as Recurrent Mastoiditis With Central Nervous System Involvement

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    April L. Barnado MD

    2013-09-01

    Full Text Available We report a case of a 43-year-old female who presented with right ear fullness and otorrhea. She was initially diagnosed with mastoiditis that was not responsive to multiple courses of antibiotics and steroids. She was then diagnosed with refractory inflammatory pseudotumor, and subsequent treatments included several mastoidectomies, further steroids, and radiation therapy. The patient went on to develop mastoiditis on the contralateral side as well as central nervous system involvement with headaches and right-sided facial paresthesias. Reexamination of the mastoid tissue revealed a significantly increased number of IgG4-positive cells, suggesting a diagnosis of IgG4-related disease. The patient improved clinically and radiographically with rituximab and was able to taper off azathioprine and prednisone. IgG4-related disease should be considered in patients with otologic symptoms and be on the differential diagnosis in patients with inflammatory pseudotumor. Staining for IgG and IgG4 is essential to ensure a prompt diagnosis and treatment.

  6. IgG4-Related Perineural Disease

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    Dai Inoue

    2012-01-01

    Full Text Available Aims. To elucidate characteristics of IgG4-related disease involving the peripheral nervous system. Methods. Retrospective review of 106 patients with IgG4-related disease identified 21 peripheral nerve lesions in 7 patients. Clinicopathological and radiological features were examined. Results. Peripheral nerve lesions were commonly identified in orbital or paravertebral area, involving orbital (=9, optic (=4, spinal (=7, and great auricular nerves (=1. The predominant radiological feature was a distinct perineural soft tissue mass, ranging 8 to 30 mm in diameter. Histologically, the epineurium was preferentially involved by massive lymphoplasmacytic infiltration rich in IgG4+ plasma cells. All lesions were neurologically asymptomatic and steroid-responsive at the first presentation, but one recurrent lesion around the optic nerve caused failing vision. Conclusion. IgG4-related disease of the peripheral nervous system is characterized by orbital or paravertebral localization, perineural mass formation, and rare neurologic symptoms. The term “IgG4-related perineural disease” seems appropriate to describe this entity.

  7. Utility of FDG PET/CT in IgG4-related systemic disease

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    Nakatani, K., E-mail: koyakn@kuhp.kyoto-u.ac.jp [Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto (Japan); Nakamoto, Y.; Togashi, K. [Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto (Japan)

    2012-04-15

    IgG4-related systemic disease (IgG4-RSD) is an emerging clinical entity about which much remains to be elucidated, in terms of its aetiology, pathogenesis, diagnosis, treatment and outcome. Autoimmune pancreatitis (AIP) and Mikulicz disease (MD) are the two major, well-studied constituents of IgG4-RSD. AIP and MD have common characteristics of forming tumour-mimicking lesions that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous immunoglobulin G4 (IgG4)-positive plasma cells, as well as various multi-organ manifestations of IgG4-RSD. 2-[{sup 18}F]-fluoro-2-deoxy-D-glucose positron-emission tomography/ computed tomography (FDG PET/CT) enables the acquisition of whole-body images and provides functional information about disease activity; as such it has a valuable role in staging extent of disease, guiding biopsy, and monitoring response to treatment. However, FDG PET/CT is likely to be only one component of the management strategy, and clinical, laboratory, imaging and histological findings are crucial in the overall diagnosis of the condition. At present FDG PET/CT does not have a well-established role in the assessment of patients with IgG4-RSD and future prospective studies are required to define the cost-effectiveness and clinical impact in this patient group more accurately.

  8. IgG4 Related Lung Disease

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    Mihir Patel

    2016-01-01

    Full Text Available IgG4 related disease is a poorly understood immune mediated condition. Lung involvement is rare and difficult to diagnose and can mimic primary lung malignancy on imaging. A patient who was found to have an incidental lung lesion with risk factors for primary pulmonary malignancy is reported.

  9. A case of tubulointerstitial nephritis in IgG4-related systemic disease with markedly enlarged kidneys.

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    Mise, Naobumi; Tomizawa, Yutaka; Fujii, Akiko; Yamaguchi, Yutaka; Sugimoto, Tokuichiro

    2009-06-01

    IgG4-related systemic disease, including autoimmune pancreatitis, is a multi-organ disorder characterized by elevated serum immunoglobulin G4 (IgG4) concentration and IgG4-positive plasma cell infiltration. We report the case of a 67-year-old man with IgG4-related tubulointerstitial nephritis, presenting with markedly enlarged kidneys and renal dysfunction. The serum IgG4 level was elevated with 4200 mg/dl and pathological examination revealed patchy, clearly fringed areas of IgG4-positive plasma cell infiltration and advanced fibrosis in the renal parenchyma, perirenal tissue and lymph nodes. With oral prednisolone at a dose of 60 mg daily, a contraction of the kidneys and an improvement of renal function were observed. No recurrence of the disease was observed during the reduction of prednisolone to 2 mg daily over 4 years.

  10. RARE DIAGNOSIS OF IGG4-RELATED SYSTEMIC DISEASE BY LIP BIOPSY IN AN INTERNATIONAL SJÖGREN SYNDROME REGISTRY

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    Baer, Alan N.; Gourin, Christine; Westra, William H.; Cox, Darren; Greenspan, John; Daniels, Troy E.

    2012-01-01

    IgG4-related disease has been recently defined as a distinct clinic-pathologic entity, characterized by dense IgG-4 plasmacytic infiltration of diverse organs, fibrosis, and tumefactive lesions. Salivary and lacrimal glands are a target of this disease and, when affected, may clinically resemble Küttner tumor, Mikulicz disease, or orbital inflammatory pseudotumor. In some patients, the disease is systemic, with metachronous involvement of multiple organs, including the pancreas, aorta, kidneys, and biliary tract. We report a 66-year old man who presented with salivary gland enlargement and severe salivary hypofunction and was diagnosed with IgG4-related disease on the basis of a labial salivary gland biopsy. Additional features of his illness included a marked peripheral eosinophilia, obstructive pulmonary disease, and lymphoplasmacytic aortitis. He was evaluated in the context of a research registry for Sjögren syndrome and was the only one of 2594 registrants with minor salivary gland histopathologic findings supportive of this diagnosis. PMID:23146570

  11. IgG4-Related Disease in a Urachal Tumor

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    Travis W. Dum

    2014-01-01

    Full Text Available IgG4-related disease is a newly recognized fibroinflammatory disorder that has the ability to affect nearly every organ system. It is characterized by tumefactive lesions and fibrosis and closely mimics neoplasms. Only one case of IgG4-related bladder mass has been reported in the literature, but there are no reports of IgG4-related disease in a urachal mass. Herein, we report a 26-year-old male who initially presented with symptoms of recurrent UTI. Work-up revealed a 6 cm urachal tumor, a 1.4 cm pulmonary lesion, and mediastinal lymphadenopathy; all metabolically active on PET scan and suspicious for urachal adenocarcinoma. Lung lesion fine needle aspiration and TURBT pathology revealed inflammation but no evidence of malignancy. The patient underwent a partial cystectomy and umbilectomy with pathology demonstrating dense plasmacytic cells, a high rate of immunohistochemistry staining positive for IgG4 plasma cells, a storiform pattern of fibrosis, and an obliterative phlebitis. Furthermore, the patient had an elevated serum IgG4 level of 227 mg/dL (range 2.4–121 mg/dL. IgG4-related disease is a newly recognized fibroinflammatory disorder that can mimic neoplastic processes and a high index of suspicion and accurate tissue pathology is necessary for an accurate diagnosis.

  12. IgG4-related disease: a systemic condition with characteristic microscopic features

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    Detlefsen, Sönke

    2013-01-01

    diseases on their own, others have been included under the umbrella of "multifocal fibrosclerosis". Biopsies or resection specimens from affected organs in IgG4-RD reveal several common microscopic features irrespective of the site of the lesion. Cellular and storiform fibrosis, lymphoplasmacytic...... on the microscopic features, diagnosis and differential diagnosis of the different organ manifestations of IgG4-RD, and the current concepts of its pathogenesis will also be addressed....

  13. [Raise awareness of IgG4 relative ocular disease].

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    Wei, Shihui; Li, Hongyang

    2015-12-01

    Purpose IgG4-related ocular disease is a chronic systemic disease with lymphocyte abnormal. The lacrimal glands, extraocular muscles and infraorbital nerve were often involved which was often the first symptom of systemic disease. While ophthalmologists did not know this disease well. They usually misdiagnosed it as idiopathic inflammatory pseudotumor, thyroid-associated ophthalmopathy etc, which resulted in delayed treatments. Here pathogenesis, clinical features and treatment methods of IgG4-relative ocular disease were described in order to improve awareness of this ocular disease, reduce clinical misdiagnosis, improve disease prognosis and standardized treatment. As the incidence of this disease increased in recent years, it is very necessary to improve awareness of the disease for ophthalmologists.

  14. IgG4-RELATED DISEASE. CLINICAL NOTES

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    Vladimir Ivanovich Vasilyev

    2013-01-01

    Full Text Available IgG4-related diseases are a new nosological entity that encompasses a few previously known diseases. IgG4-related systemic disease is diagnosed if two or more affected organs are detected. This group of diseases has two similar signs: serological (elevated serum IgG4 subclass concentrations and histological (organ and tissue infiltration from plasmo-cytes secreting IgG4, and eosinophils, and the development of fibrosclerosis and phlebitis obliterans. The paper describes two cases. In one case, a multisystemic disease was observed virtually at its onset whereas in the other this lesion was diagnosed several years after the natural course of the disease.

  15. Recent advances in the concept and pathogenesis of IgG4-related disease in the hepato-bilio-pancreatic system.

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    Okazaki, Kazuichi; Yanagawa, Masahito; Mitsuyama, Toshiyuki; Uchida, Kazushige

    2014-09-01

    Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of "biliary diseases with pancreatic counterparts." Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.

  16. Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

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    Cron Randy Q

    2011-01-01

    Full Text Available Abstract Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology.

  17. [IgG4-related kidney disease. Diagnosis and treatment].

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    Kawano, Mitsuhiro; Mizushima, Ichiro; Yamada, Kazunori; Taniguchi, Yoshinori; Saeki, Takako

    2015-01-01

    IgG4-related disease (IgG4-RD) is a systemic inflammatory disorder that can affect most organs/tissues like sarcoidosis. The kidney is one of the most frequently affected organs. While tubulointerstitial nephritis (TIN) with characteristic imaging findings is the representative lesion of IgG4-related kidney disease (IgG4-RKD), a variety of glomerular lesions, particularly membranous nephropathy, sometimes overlap on TIN. Clinically, either decreased renal function and/or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography are typical presenting features. Histologically, plasma cell (PC)-rich TIN accompanied by characteristic fibrosis called storiform fibrosis with dense IgG4-positive PC infiltration is a typical finding. Although a swift response to corticosteroid is a very important feature of IgG4-RKD, in cases with moderately to severely decreased renal function before therapy, only partial recovery of renal function is obtained. This review provides a comprehensive overview of IgG4-RKD from the clinical, laboratory, imaging, and histological aspects and also addresses some of the therapeutic issues concerning it.

  18. IgG4 related sclerosing mastitis: expanding the morphological spectrum of IgG4 related diseases.

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    Chougule, Abhijit; Bal, Amanjit; Das, Ashim; Singh, Gurpreet

    2015-01-01

    IgG4 related disease (IgG4RD) is a recently recognised condition characterised by mass forming lesions associated with storiform fibrosis, obliterative phlebitis, lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and elevated serum IgG4 levels. Although rare, mammary involvement has been reported as IgG4 related sclerosing mastitis, the morphological counterpart of a growing family of IgG4 related diseases. A total of 17 cases belonging to mass forming benign inflammatory breast lesions such as plasma cell mastitis, granulomatous lobular mastitis, non-specific mastitis and inflammatory pseudotumour were investigated as a possible member of IgG4 related sclerosing mastitis. Clinical, radiological, histopathological and immunohistochemistry findings were noted in all cases. Cases diagnosed as inflammatory pseudotumour showed all the histopathological features of IgG4RD along with increased number of IgG4 positive plasma cells and IgG4/IgG ratio >40%. However, only a few IgG4 positive cells were seen in plasma cell mastitis, granulomatous lobular mastitis and non-specific mastitis cases. These cases also did not fulfill the morphological criteria for the diagnosis of IgG4 related diseases. IgG4RD should be excluded in plasma cell rich lesions diagnosed on core biopsies by IgG4 immunostaining. This can avoid unnecessary surgery as IgG4 related diseases respond to simple and effective steroid treatment.

  19. IgG4-Related Disease Presenting as Isolated Scleritis

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    Arnon, Ella; Yaakobi, Alona; Cohen, Yuval; Tiosano, Beatrice

    2017-01-01

    A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms. This case highlights the significance of IgG4-RD in the differential diagnosis of scleritis and raises the question as to whether various organs affected by IgG4-RD may have different underlying pathophysiological mechanisms in which pathogenic T cells play a role. PMID:28149653

  20. IgG4-Related Disease Presenting as Isolated Scleritis

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    Eran Berkowitz

    2017-01-01

    Full Text Available A rare case of IgG4-related disease (IgG4-RD manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate led to significant improvement in signs and symptoms. This case highlights the significance of IgG4-RD in the differential diagnosis of scleritis and raises the question as to whether various organs affected by IgG4-RD may have different underlying pathophysiological mechanisms in which pathogenic T cells play a role.

  1. IgG4-related systemic disease of the pancreas with involvement of the lung: a case report and literature review

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    Hurley JR

    2013-08-01

    Full Text Available IgG-related systemic disease (ISD remains exceedingly rare and unfamiliar, particularly extrapancreatic disease. We report a patient with separate presentations of IgG4 pulmonary disease and recurring IgG4 related biliary sclerosis and pancreatitis. Because of the intricate and perplexing pathogenesis, overlapping organ systems and wide variation in disease presentation, ISD in its entirety remains undefined. Accurate identification of ISD is critical to avoid permanent organ damage especially since treatment is nearly always successful with corticosteroids. As recognition and awareness of this disease grows, development of standard diagnostic criteria and treatment plans are needed.

  2. IgG4-related disease and the kidney.

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    Cortazar, Frank B; Stone, John H

    2015-10-01

    IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that involves almost every organ system. In this Review, we summarize current knowledge of IgG4-RD and its most frequent manifestations in the kidney—IgG4-related tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy (MGN). Diagnosis of IgG4-RD relies on histopathology: the typical features are a dense lymphoplasmacytic infiltrate and storiform fibrosis. A high percentage of plasma cells observed within lesions stain positively for IgG4. IgG4-related TIN bears the hallmark pathological findings of IgG4-RD; distinctive radiographic characteristics are also frequently observed with use of contrast-enhanced CT. MGN secondary to IgG4-RD seems to be distinct from idiopathic MGN. Humoral and cell-mediated immunity seem to have roles in the pathophysiology of IgG4-RD, but the details of these roles remain unclear. The IgG4 molecule itself is unlikely to be the primary driver of inflammation; rather, it probably downregulates the immune response. Fibrosis might be caused by activation of innate immune cells by polarized CD4(+) T cells. Glucocorticoids are the standard initial treatment for IgG4-RD, but their long-term adverse effects and the high frequency of relapse and renal damage associated with use of this treatment has prompted a search for more effective options. B-cell depletion and the targeting of plasmablasts are both promising approaches.

  3. IgG4-related Disease of the Genitourinary Tract

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    Mukul K. Divatia

    2014-02-01

    Full Text Available IgG4-related disease (IgG4-RD is a recently established albeit well recognized fibro-inflammatory condition with distinctive features including a characteristic histopathological appearance; a propensity to develop tumefactive lesions in multiple body sites; and oft elevated serum IgG4 levels. The consensus statement on IgG-4 RD equips practicing pathologists with a set of working guidelines for the diagnosis of pathologic lesions identified in a host of different organ system affected with this disease. The diagnosis of IgG4-RD requires the combined presence of the characteristic histopathological appearance and increased numbers of IgG4-positive plasma cells. The essential histopathological features include a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. Tissue IgG4-positive plasma cell counts and IgG4: IgG ratios are significant ancillary aids in establishing the diagnosis. The spectrum of IgG4-RD continues to expand and involve multiple body sites. The genitourinary system comprising of the kidneys, ureters, urinary bladder, urethra, prostate gland, testes and penis is one of the multiple organ systems to be affected by IgG4-RD. This review describes the clinical and histopathologic patterns of involvement of the genitourinary system by IgG4-RD, in association with serologic and radiological features. [J Interdiscipl Histopathol 2014; 2(1.000: 3-18

  4. IgG4-Related Disease: A Multispecialty Condition

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    Iuri Usêda Santana

    2014-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases.

  5. IgG4-related mastitis, a rare disease, can radiologically and histologically mimic malignancy.

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    Yamada, Rin; Horiguchi, Shin-ichiro; Yamashita, Toshinari; Kamisawa, Terumi

    2016-03-23

    IgG4-related disease (IgG4-RD) is characterised by high serum concentrations of IgG4, dense lymphoplasmacytic infiltrates, storiform fibrosis and increased IgG4-positive plasma cells in tissues. This systemic disease occurs in various organs metachronously, but IgG4-related mastitis appears extremely rare. We report a case of IgG4-related mastitis, radiologically considered to represent breast cancer mainly composed of intraductal component and requiring histological differentiation from mucosa-associated lymphoid tissue (MALT) lymphoma. The breast mass disappeared with steroid therapy. When patients have a breast mass, regardless of the presence or absence of IgG4-RD, IgG4-related mastitis should be considered in addition to breast cancer. If histological findings show dense lymphoplasmacytic infiltrates, IgG4-related mastitis should be suspected in addition to malignant lymphoma, and lack of monoclonality should be confirmed. To avoid unnecessary surgery or chemotherapy, knowledge and accurate diagnosis of the entity of IgG4-related mastitis is necessary. 2016 BMJ Publishing Group Ltd.

  6. TREATMENT OF IgG4-RELATED DISEASE

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    E. V. Sokol

    2016-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a fibroinflammatory condition characterized by the occurrence of tumor-like foci in different organs with a unique histological pattern (moirо-like fibrosis, obvious lymphoplasmacytic infiltration with large numbers of IgG4+ plasma cells, and obliterating phlebitis and elevated serum IgG4 levels in the majority of patients. Its first-line therapy is glucocorticoids at a starting dose of 0.6 mg/kg/day (equivalent to prednisolone; however, this treatment entails a great number of adverse events and high recurrence rates. The paper provides a review of today's literature on the treatment of IgG4-RD; particular emphasis is laid on the description of therapy with glucocorticoids and rituximab.

  7. IgG4-Related Disease without Overexpression of IgG4: Pathogenesis Implications

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    Naoshi Nishina

    2012-01-01

    Full Text Available IgG4-related disease is a new disease group that affects multiple organs. It is characterized by high serum IgG4 and abundant infiltration of IgG4-bearing plasma cells in the affected organ. Here, we describe an intriguing case that suggested that IgG4-related disease might present without IgG4 overexpression or infiltration, at least during a relapse. A 47-year-old man had been diagnosed with systemic lupus erythematosus 15 years. He was admitted due to a pituitary mass, systemic lymphadenopathy, and multiple nodules in the lungs and kidneys. The serum IgG4 level was normal and histopathological examination of the pituitary mass showed abundant lymphocyte and plasma cell infiltration with very few IgG4-positive cells. When we examined specimens preserved from 15 years ago, we found high serum IgG4 levels and IgG4-bearing plasma cell infiltration. This resulted in a diagnosis of IgG4-related disease, and we considered the current episode to be a relapse without IgG4 overexpression. This case indicated that, to clarify the pathogenesis of IgG4-related disease, current cases should repeat specimen evaluations over the course of IgG4-related disease to define diagnostic markers.

  8. [IgG4-related kidney disease: what the nephrologist needs to know].

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    Galeano, Dario; Zanoli, Luca; Scarfia, Viviana Rosalia; L'Imperio, Vincenzo; Malatino, Lorenzo; Fatuzzo, Pasquale; Granata, Antonio

    2016-01-01

    IgG4 related disease is a systemic fibro-inflammatory disorder characterized by multiple organ and multiple tissue lesions. The real pathogenesis is currentlyactually unknown. For these reasons many authors compare IgG4 related disease to sarcoidosis. Lesions are often localized in the pancreas, salivary and lacrimal glands, biliary ducts, retroperitoneum and in many other organs. The diagnosisis difficult because of mild symptoms and the possibility of mimicking other severe diseases. Therefore, histopathology together with clinical and radiological typical findings are mandatory tools for diagnosis. Steroidtherapy usually enables disappearance of tumor like lesions and complete recovery. Kidney has an extensive organ involvement in the contextof IgG4-related disease. Historically, tubule - interstitial nephritis(TIN) is considered the main renal feature of renal lesions, however recent studies extend the spectrum of renal lesions also to glomerular tuft. These findings allow to introduce in the nosography the term of IgG4related kidney disease (IgG4 RKD). This review focuses on renal involvement in IgG4related disease, in order to help nephrologists to improve their clinical, diagnostic and therapeutic approach to this emerging pleiotropic clinical pattern.

  9. A small subgroup of Hashimoto's thyroiditis is associated with IgG4-related disease.

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    Jokisch, Friedrich; Kleinlein, Irene; Haller, Bernhard; Seehaus, Tanja; Fuerst, Heinrich; Kremer, Marcus

    2016-03-01

    IgG4-related disease is a newly identified syndrome characterized by high serum IgG4 levels and increased IgG4-positive plasma cells in involved organs. The incidence of IgG4-related thyroiditis in the Caucasian population of Europe is unknown. We investigated formalin-fixed thyroid gland samples of 216 patients (191 Hashimoto's thyroiditis, 5 Riedel's thyroiditis, and 20 goiters, as controls), morphologically, and immunohistochemically. Cases were divided into two groups: IgG4-related Hashimoto's thyroiditis (24 cases) together with Riedel thyroiditis (1 case) and 171 non-IgG4-related thyroiditis. Compared to the non-IgG4-related cases, IgG4-related thyroiditis showed a higher IgG4/IgG ratio (0.6 vs. 0.1, p Hashimoto's thyroiditis was diagnosed in 23 of the 24 IgG4-related cases (96 %) and in 13 of 167 (18 %, p > 0.001) non-IgG4-related cases. The single case of IgG4-related Riedel's thyroiditis also showed a higher median IgG4 plasma cell count (56.3 vs. 14.3) and a higher IgG4/IgG ratio (0.5 vs. 0.2) than the four cases of non-IgG4-related Riedel's thyroiditis. Our data suggests the incidence of IgG4-related disease (IgG4-RD) of the thyroid gland in Europe is considerably lower than that observed in other studies. A significant elevation of IgG4-positive plasma cells was only found in a small group of Hashimoto's thyroiditis and then accompanied by intense fibrosis, indicating an association with IgG4-RD. Morphologically, IgG4-RD of the thyroid gland differs from that in other organ systems, exhibiting a dense fibrosis without intense eosinophilia or obliterative phlebitis.

  10. Renal manifestations of IgG4-related systemic disease%IgG4相关系统性疾病的肾脏损害

    Institute of Scientific and Technical Information of China (English)

    黄倩

    2012-01-01

    IgG4相关系统性疾病(IgG4-related systemic disease,IgG4-RSD)是一组最近认识的临床综合征,可累及多个器官,胰腺最常受累,主要表现为血清IgG4升高,受累组织弥漫IgG4阳性浆细胞浸润.本文主要介绍IgG4-RSD累及肾脏时的临床表现、影像学、实验室检查、病理特点及治疗情况.%IgG4-related systemic disease represents a recently recognized group of multi-organ diseases characterized by a high level of serum IgG4 and dense infiltration of IgG4-positive cells into multiple organs. The most commonly involved organ was pancreas. In this article,the relevant literatures about the renal involvements of IgG4-related systemic disease will be reviewed.

  11. Prevalence of IgG4-Related Disease in Japan Based on Nationwide Survey in 2009

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    Kazushige Uchida

    2012-01-01

    Full Text Available The number of patients with autoimmune pancreatitis who visited hospitals in Japan in 2007 was approximately 2709 (95% confidence interval; range 2540–3040. Because IgG4-related disease is a new clinical entity, there are no data with regard to its prevalence. To estimate the number of patients with IgG4-related disease in Japan, we randomly selected hospitals using stratification and asked them how many patients they had with IgG4-related disease in 2009. The number of patients with Mikulicz’s disease, IgG4-related retroperitoneal fibrosis, IgG4-related renal disease, IgG4-related pulmonary disease, and IgG4-related lymphadenopathy who visited hospitals in Japan in 2009 was approximately 4304 (95% confidence interval; range 3360–5048, 272 (95% confidence interval; range 264–306, 57 (95% confidence interval; range 47–66, 354 (95% confidence interval; range 283–424, and 203 (95% confidence interval; range 187–240, respectively. The total number of patients with IgG4-related disease without autoimmune pancreatitis in Japan was approximately 5190 (95% confidence interval; range 4141–6084. The male : female ratio was 1 : 0.77, and the average of age of disease onset was 58.8 years. The total number of patients with IgG4-related disease in Japan in 2009, including autoimmune pancreatitis, was approximately 8000.

  12. IgG4-related Hashimoto's thyroiditis--a new variant of a well known disease.

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    Luiz, Henrique Vara; Gonçalves, Diogo; Silva, Tiago Nunes da; Nascimento, Isabel; Ribeiro, Ana; Mafra, Manuela; Manita, Isabel; Portugal, Jorge

    2014-11-01

    Hashimoto's thyroiditis (HT) has been characterized for many years as a well-defined clinicopathologic entity, but is now considered a heterogeneous disease. IgG4-related HT is a new subtype characterized by thyroid inflammation rich in IgG4-positive plasma cells and marked fibrosis. It may be part of the systemic IgG4-related disease. We report a case of a 56-year-old Portuguese man who presented with a one-month history of progressive neck swelling and dysphagia. Laboratory testing revealed increased inflammatory parameters, subclinical hypothyroidism and very high levels of thyroid autoantibodies. Cervical ultrasound (US) demonstrated an enlarged and heterogeneous thyroid gland and two hypoechoic nodules. US-guided fine needle aspiration cytology was consistent with lymphocytic thyroiditis. The patient was submitted to total thyroidectomy and microscopic examination identified typical findings of HT, marked fibrosis limited within the thyroid capsule and lymphoplasmacytic infiltration, with >50 IgG4-positive plasma cells per high-power field and an IgG4/IgG ratio of >40%. After surgery, serum IgG4 concentration was high-normal. Symptoms relief and reduction in laboratory inflammatory parameters were noticed. Thyroid function is controlled with levothyroxine. To our knowledge we report the first case of IgG4-related HT in a non-Asian patient. We also perform a review of the literature regarding IgG4-related disease and IgG4-related HT. Our case highlights this new variant of the well known HT, and helps physicians in recognizing its main clinical features, allowing for proper diagnosis and treatment.

  13. Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease.

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    Okazaki, Kazuichi; Uchida, Kazushige; Koyabu, Masanori; Miyoshi, Hideaki; Takaoka, Makoto

    2011-03-01

    Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more suggestive of abnormal immunity such as allergy or autoimmunity. Moreover, patients with type 1 AIP often have extrapancreatic lesions, such as sclerosing cholangitis, sclerosing sialadenitis, or retroperitoneal fibrosis, showing pathological features similar to those of the pancreatic lesions. Based on these findings, an international concept of and diagnostic criteria for AIP have been proposed recently. Of interest, many synonyms have been proposed for the conditions of AIP and extrapancreatic lesions associated with IgG4, such as "multifocal idiopathic fibrosclerosis," "IgG4-related autoimmune disease," "IgG4-related sclerosing disease," "systemic IgG4-related plasmacytic syndrome (SIPS)," and "IgG4-related multiorgan lymphoproliferative syndrome," all of which may refer to the same conditions. Therefore, the Japanese Research Committee for "Systemic IgG4-Related Sclerosing Disease" proposed a disease concept and clinical diagnostic criteria based on the concept of multifocal fibrosclerosing disease, in 2009, in which the term "IgG4-related disease" was agreed upon as a minimal consensus to cover these conditions. Although the significance of IgG4 in the development of "IgG4-related disease" remains unclear, we have proposed a hypothesis for the development of type 1 AIP, one of the IgG4-related diseases. The concept and diagnostic criteria of "IgG4-related disease" will be changed in accordance with future studies.

  14. A Case of IgG4-Related Lung Disease Presenting as Interstitial Lung Disease.

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    Ahn, Jee Hwan; Hong, Sun In; Cho, Dong Hui; Chae, Eun Jin; Song, Joon Seon; Song, Jin Woo

    2014-08-01

    Intrathoracic involvement of immunoglobulin G4 (IgG4)-related disease has recently been reported. However, a subset of the disease presenting as interstitial lung disease is rare. Here, we report a case of a 35-year-old man with IgG4-related lung disease with manifestations similar to those of interstitial lung disease. Chest computed tomography showed diffuse ground glass opacities and rapidly progressive pleural and subpleural fibrosis in both upper lobes. Histological findings showed diffuse interstitial lymphoplasmacytic infiltration with an increased number of IgG4-positive plasma cells. Serum levels of IgG and IgG4 were also increased. The patient was diagnosed with IgG4-related lung disease, treated with anti-inflammatory agents, and showed improvement. Lung involvement of IgG4-related disease can present as interstitial lung disease and, therefore, should be differentiated when evaluating interstitial lung disease.

  15. IgG4-related disease and other causes of inflammatory meningeal disease.

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    Carruthers, Robert; Carruthers, Mollie; Della-Torre, Emanuel

    2014-09-01

    Immunoglobulin-4 (IgG4-) related disease is a newly described treatable condition that has recently expanded the differential diagnosis of inflammatory meningeal disorders. This review will discuss the main clinical and pathophysiological features of IgG4-related meningeal disease in the context of meningeal inflammatory disorders in general. Particular attention will be dedicated to the differential diagnosis and the different therapeutic approaches.

  16. Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes.

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    Sato, Yasuharu; Inoue, Dai; Asano, Naoko; Takata, Katsuyoshi; Asaoku, Hideki; Maeda, Yoshinobu; Morito, Toshiaki; Okumura, Hirokazu; Ishizawa, Shin; Matsui, Shoko; Miyazono, Takayoshi; Takeuchi, Tamotsu; Kuroda, Naoto; Orita, Yorihisa; Takagawa, Kiyoshi; Kojima, Masaru; Yoshino, Tadashi

    2012-07-01

    Progressively transformed germinal centers is a benign condition of unknown pathogenesis characterized by a distinctive variant form of reactive follicular hyperplasia in lymph nodes. We recently reported Ig G4-related disease in progressively transformed germinal centers. However, no large case series has been reported and clinicopathologic findings remain unclear. Here, we report 40 Japanese patients (28 men, 12 women; median age, 56 years) with progressively transformed germinal centers of the lymph nodes who fulfilled the histological diagnostic criteria for IgG4-related disease (IgG4(+) progressively transformed germinal centers), with asymptomatic localized lymphadenopathy involving the submandibular nodes in 24, submandibular and cervical nodes in 14, cervical nodes only in 1, and cervical and supraclavicular nodes in 1. In all, 16 (52%) of 31 examined patients had allergic disease. Histologically, the lymph nodes demonstrated uniform histological findings, namely marked follicular hyperplasia with progressively transformed germinal centers, and localization of the majority of IgG4(+) plasma cells in the germinal centers. Serum IgG4, serum IgE and peripheral blood eosinophils were elevated in 87%, 92% and 53% of examined patients, respectively. Eighteen patients subsequently developed extranodal lesions (including five who developed systemic disease), which on histological examination were consistent with IgG4-related disease. IgG4(+) progressively transformed germinal centers presents with uniform clinicopathological features of asymptomatic localized submandibular lymphadenopathy, which persists and/or relapses, and sometimes progresses to extranodal lesions or systemic disease. Nine patients were administered steroid therapy when the lesions progressed, to which all responded well. We suggest that IgG4(+) progressively transformed germinal centers should be included in the IgG4-related disease spectrum.

  17. IgG4-related disease presenting as a lung mass and weight loss: Case report and review of the literature

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    Kevin Grewal

    2016-01-01

    Full Text Available We describe a case of IgG4-related lung disease presenting as a lung mass with associated weight loss. IgG4-related disease is a systemic sclerosing disorder that causes fibrotic, often tumor-like manifestations that variably effect different organ systems. The clinical presentation of IgG4-related disease is protean. Timely recognition and diagnosis requires awareness on the part of clinicians and pathologists to the variable manifestations of this newly recognized disorder. We offer a concise review of the pulmonary manifestations, diagnosis and treatment of IgG4-related lung disease.

  18. A case of non-lacrimal immunoglobulin G4 (IgG4)-related orbital disease with mastitis.

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    Farooq, Tahir Ali; Mudhar, Hardeep; Sandramouli, S

    2016-01-01

    IgG4-related orbital disease is a recognised cause for orbital inflammation. As its awareness increases and diagnostic accuracy improves there will be an increased number of cases being identified. This unique case demonstrates for the first time, with histological evidence, a case of a non-lacrimal IgG4-related orbital disease with concurrent IgG4-related mastitis. We describe a 47 year old who presented with a supraorbital swelling and mass. This was initially successfully treated with oral steroids and was later excised on recurrence. Immunohistochemical and blood serum analysis confirmed IgG4-related orbital disease. On systemic enquiry she was found to have a mass of the breast, which was shown to be IgG4-related mastitis. She is currently asymptomatic with no sign of recurrence and is under long-term surveillance. This case highlights the importance of systemic work up in patients presenting with orbital foci of IgG4 disease.

  19. [Two cases of IgG4-related disease with pleural effusion].

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    Suzuki, Nobuaki; Saeki, Takako; Shimaoka, Yuichi; Kuriyama, Hideyuki; Nishibori, Takeaki; Ebe, Yusuke; Sato, Kazuhiro

    2011-02-01

    We describe 2 cases of IgG4-related disease with pleural effusion in elderly men. Both patients had elevated serum IgG4 levels, and the characteristics of their pleural effusion were similar. Patient 1 had pericardial effusion and retroperitoneal fibrosis, and a biopsy specimen from the pericardium showed infiltration of abundant IgG4-positive plasma cells with fibrosis. Because his pleurisy, pericarditis and retroperitoneal fibrosis responded to steroid therapy, we diagnosed pleurisy associated with IgG4-related disease. Patient 2 had been treated with steroids because of IgG4-related sialadenitis and interstitial pneumonitis, but pleural effusion developed. Although histopathological examination of the pleura showed infiltration of abundant IgG4-positive plasma cells with fibrosis, Mycobacterium tuberculosis was cultured from the pleural effusion, and histologic examination also showed epithelioid granuloma. Chemotherapy for tuberculosis was effective for the pleurisy, and we diagnosed tuberculous pleurisy as a complication of IgG4-related disease. In cases of IgG4-related disease associated with pleural effusion, the clinical course should be considered together with the serum IgG4 levels and pleural histology.

  20. Acute tubulointerstitial nephritis with severe renal impairment associated with multisystem IgG4-related disease

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    Rafael Coimbra Ferreira Beltrame

    Full Text Available Abstract The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%. The patient was treated with prednisone at a dose of 60 mg/day, followed by mycophenolate mofetil, and showed clinical and renal function improvement at 6 months of follow-up. The high index of suspicion of IgG4-related disease with multisystem involvement and the early treatment of this condition are essential to improve the prognosis of affected patients.

  1. Steroid-responsive IgG4-related disease with isolated prostatic involvement: An unusual presentation with elevated serum PSA

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    Vikas Jain

    2016-01-01

    Full Text Available Autoimmune prostatitis is known to occur as a part of multisystem fibro-inflammatory disorder known as IgG4 related disease (IgG4 RD. The usual presentation is with symptoms of gastro-intestinal disease with prostatic involvement presenting as lower urinary tract symptoms. The disease responds to corticosteroids. We report an asymptomatic young man who was diagnosed to have IgG4 related prostatitis on TRUS-guided prostate biopsy done for elevated serum PSA, in the absence of any other systemic involvement. The treatment with steroid resulted in normalization of S PSA levels.

  2. Serum Immunoglobulin Free Light Chain Assessment in IgG4-Related Disease

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    Aurélie Grados

    2013-01-01

    Full Text Available Immunoglobulin free light chains are produced in excess during normal antibody synthesis. Their evaluation is commonly used in case of a monoclonal gammopathy. In polyclonal hypergammaglobulinemia related to the Sjögren syndrome or systemic lupus, erythematosus serum free light chain levels are increased and could correlate with disease activity. We show here that the κ ( and λ ( free light chains and the κ : λ ratio ( are increased in sixteen patients with IgG4-related disease when compared to healthy controls. The increase of κ and λ free light chains probably reflects the marked polyclonal B cell activation of the disease. We could not assess in this small cohort of patients a significative correlation of serum free light chain levels and disease activity or extension.

  3. IgG4-Related Autoimmune Prostatitis: Is It an Unusual or Underdiagnosed Manifestation of IgG4-Related Disease?

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    María T. Bourlon

    2013-01-01

    Full Text Available IgG4-related disease (IgG4-RD encompasses a wide range of extrapancreatic manifestations. Albeit some are relatively well known, others such as autoimmune prostatitis remain poorly described. We present a 61-year-old Latin-American male with autoimmune pancreatitis (AIP who presented with lower urinary tract symptoms (LUTS, normal prostate specific antigen (PSA test, and prostate enlargement attributed to benign prostatic hyperplasia (BPH. He underwent a transurethral resection of the prostate (TURP after which symptoms were resolved. On histopathology, prostatic stroma had a dense inflammatory infiltrate rich in plasma cells and lymphocytes; immunohistochemical morphometric assessment showed >10 IgG4-positive plasma cells/high power field (HPF. The diagnosis of IgG4-related prostatitis was postoperatively. We compared the patient characteristics with those of previous reports on Asian patients. Shared findings included prostate enlargement, LUTS (symptoms that can be confused with BPH, and PSA within normal limits or mild elevations. IgG4-related prostatitis is rarely considered as a preprocedural diagnosis, even in patients with evidence of IgG4-RD. Involved prostate zones include mainly central and transitional zones and less frequently the peripheral. Currently, there is insufficient data about the natural history and outcome. Whether steroids, transurethral resection, or both are the treatment of choice needs to be elucidated.

  4. The changing faces of IgG4-related disease: Clinical manifestations and pathogenesis.

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    Islam, Arshia Duza; Selmi, Carlo; Datta-Mitra, Ananya; Sonu, Rebecca; Chen, Mingyi; Gershwin, M Eric; Raychaudhuri, Siba P

    2015-10-01

    Since the earliest reports in 2001, immunoglobulin G4 (IgG4)-related disease has been defined as an autoimmune systemic disease characterized by the lymphoplasmacytic infiltration of affected tissues leading to fibrosis and obliterative phlebitis along with elevated serum IgG4 levels. Prior to this unifying hypothesis, a plethora of clinical manifestations were considered as separate entities despite the similar laboratory profile. The pathology can be observed in virtually all organs and may thus be a challenging diagnosis, especially when the adequate clinical suspicion is not present or when obtaining a tissue biopsy is not feasible. Nonetheless, the most frequently involved organs are the pancreas and exocrine glands but these may be spared. Immunosuppressants lead to a prompt clinical response in virtually all cases and prevent histological sequelae and, as a consequence, an early differential diagnosis from other conditions, particularly infections and cancer, as well as an early treatment should be pursued. We describe herein two cases in which atypical disease manifestations were observed, i.e., one with recurrent neck lymph node enlargement and proptosis, and one with jaundice. Our understanding of the pathogenesis of IgG4-related disease is largely incomplete but data support a significant role for Th2 cytokines with the contribution of innate immunity factors such as Toll-like receptors, macrophages and basophils. Further, macrophages activated by IL4 overexpress B cell activating factors and contribute to chronic inflammation and the development of fibrosis. We cannot rule out the possibility that the largely variable disease phenotypes reflect different pathogenetic mechanisms and the tissue microenvironment may then contribute to the organ involvement.

  5. IgG4-related disease simulating Hodgkin lymphoma in a child

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    D. Eric Ewing, MD

    2016-06-01

    Full Text Available Immunoglobulin (Ig G4-related disease is a recently described syndrome characterized by mass forming lymphoplasmacytic tissue infiltration and elevated serum IgG4 concentrations usually affecting middle-aged or older individuals. Lymphadenopathy is frequently observed and is sometimes the first or only manifestation of the disease. We report a case of IgG4-related disease mimicking Hodgkin lymphoma in a 13-year-old girl. The patient presented with progressive unilateral cervical lymphadenopathy of several months duration. Biopsy showed follicular hyperplasia with progressive transformation of germinal centers. Interfollicular areas were expanded by small lymphocytes, histiocytes, eosinophils and fibrosis with occasional CD30 positive cells initially concerning for interfollicular Hodgkin lymphoma. Immunohistochemical analysis revealed an intrafollicular plasmacytosis with an IgG4-positive/IgG-positive plasma cell ratio of 50% supporting a diagnosis of IgG4-related lymphadenopathy, progressively transformed germinal centers type. Laboratory studies were supportive with elevated serum IgG4 (178 mg/dL and IgE (30.40 kU/L levels along with an elevated serum IgG4/IgG ratio (0.16. Very few cases of IgG4-related disease have been described in children. Within this age group, there is considerable clinical overlap between IgG4-related disease associated lymphadenopathy and Hodgkin lymphoma. In addition, lymphadenopathy secondary to IgG4-related disease demonstrates substantial histologic diversity with the potential to simulate the inflammatory background and fibrosis of Hodgkin lymphoma. The importance of accurate diagnosis is underscored by the prognostic implications considering the marked response of the syndrome to steroid therapy. In addition, appropriate follow up is critical to monitor for relapse and additional organ involvement.

  6. Bilateral Vision Loss Secondary to Pachymeningitis in a Patient with IgG4-Related Disease

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    Lucas eRamirez

    2014-10-01

    Full Text Available IgG4-Related Disease is a recently recognized fibroinflammatory condition associated with disease in nearly every organ, including the meninges. A proportion of Idiopathic Hypertrophic Pachymeningitis cases may involve a component of meningeal IgG4-RD. We present a patient with severe bilateral vision loss found to have thickening of the dura mater on MRI, and subsequently diagnosed with IgG4-RD after dural biopsy.

  7. Topiramate-induced maculopathy in IgG4-related disease

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    DaCosta J

    2016-06-01

    Full Text Available Joanna DaCosta,1,2 Saad Younis1 1Ophthalmology Department, Imperial College Healthcare NHS Trust, Western Eye Hospital, 2Barts Health NHS Trust, Whipps Cross University Hospital, London, UK Abstract: This report describes a case of reversible topiramate-induced maculopathy in a 32-year-old female patient with IgG4-related disease. The patient presented with decreased vision associated with anterior uveitis and cystoid macula edema, which was unresponsive to oral and topical steroids. Following topiramate cessation, both cystoid macula edema and vision improved. The ocular side effects of topiramate and putative pharmacological mechanisms for topiramate-induced maculopathy in the context of IgG4-related disease are discussed. This report highlights that neurologists and ophthalmologists should be aware that patients presenting with topiramate-associated maculopathy should be advised to discontinue topiramate promptly to prevent irreversible loss of vision. Keywords: IgG4 disease, maculopathy, topiramate

  8. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease.

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    Della Torre, Emanuel; Mattoo, Hamid; Mahajan, Vinay S; Carruthers, Mollie; Pillai, Shiv; Stone, John H

    2014-02-01

    IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder that can affect virtually every organ system. T-helper type 2 responses have been presumed to be pathogenic in this disease, and a high proportion of patients with IgG4-RD are reported to have longstanding allergies, peripheral blood eosinophilia, and serum IgE elevation. It has therefore been proposed that allergic mechanisms drive IgG4-RD. However, no epidemiological assessment of atopy, peripheral blood eosinophilia, and serum IgE concentrations has ever been undertaken in patients with IgG4-RD. In this study, we evaluated these parameters in a large cohort of patients with IgG4-RD in whom a wide range of organs were affected by disease. Our results demonstrate that the majority of patients with IgG4-RD are nonatopic. Nevertheless, a subset of nonatopic subjects exhibit peripheral blood eosinophilia and elevated IgE, suggesting that processes inherent to IgG4-RD itself rather than atopy per se contribute to the eosinophilia and IgE elevation observed in the absence of atopy. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  9. Acute tubulointerstitial nephritis with severe renal impairment associated with multisystem IgG4-related disease.

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    Beltrame, Rafael Coimbra Ferreira; Friderichs, Maurício; Fior, Bárbara Rayanne; Schaefer, Pedro Guilherme; Thomé, Gustavo Gomes; Silva, Dirceu Reis da; Barros, Elvino José Guardão; Seligman, Renato; Veronese, Francisco Veríssimo

    2016-01-01

    The IgG4-related disease has a wide clinical spectrum where multiple organs can be affected, and the diagnosis depends on typical histopathological findings and an elevated IgG4 expression in plasma cells in the affected tissue. We describe the clinical presentation and evolution of a patient with acute tubulointerstitial nephritis, severe kidney failure and systemic manifestations such as lymphadenomegaly and chronic pancreatitis. The diagnosis was confirmed by the clinical picture and kidney and lymph node histopathology, in which immunohistochemistry of the lymphoid tissue showed policlonality and increased expression of IgG4, with a IgG4/total IgG ratio > 80%. The patient was treated with prednisone at a dose of 60 mg/day, followed by mycophenolate mofetil, and showed clinical and renal function improvement at 6 months of follow-up. The high index of suspicion of IgG4-related disease with multisystem involvement and the early treatment of this condition are essential to improve the prognosis of affected patients. Resumo A doença relacionada à IgG4 tem um espectro clínico amplo em que múltiplos órgãos podem ser afetados, e o diagnóstico depende de achados histopatológicos típicos e elevada expressão de IgG4 em plasmócitos no tecido afetado. Descrevemos o quadro clínico e a evolução de um paciente com nefrite túbulo-intersticial aguda, insuficiência renal grave e manifestações sistêmicas como linfoadenomegalias e pancreatite crônica. O diagnóstico foi confirmado pelas características clínicas e pela histopatologia renal e de linfonodo, na qual a imunohistoquímica mostrou tecido linfoide com policlonalidade e expressão aumentada de IgG4, com uma relação IgG4/IgG total > 80%. O paciente foi tratado com prednisona na dose de 60 mg/dia, seguido de micofenolato mofetil, e apresentou melhora clínica e da função renal depois de 6 meses de tratamento. O alto índice de suspeição da doença relacionada ao IgG4 com comprometimento multissist

  10. IgG4-related epididymo-orchitis associated with bladder cancer: possible involvement of BAFF/BAFF-R interaction in IgG4-related urogenital disease.

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    Migita, Kiyoshi; Miyashita, Taiichiro; Mizuno, Aya; Jiuchi, Yuka; Ito, Masahiro; Matsuo, Manabu; Izumi, Yasumori; Takeoka, Atsushi; Nishino, Ayako; Hayashi, Mikio

    2014-01-01

    We describe herein a patient who presented with immunoglobulin G4-related disease (IgG4-RD) involving the testis and prostate as well as the submandibular glands. Massive infiltration of IgG4-expressing plasma cells was observed in testis and prostate tissues. Serum concentrations of B cell activating factor belonging to the tumor necrosis factor family (BAFF) were elevated in parallel with serum IgG4 concentrations, and infiltration of BAFF-receptor (BAFF-R)-expressing B cells and BAFF-expressing lymphoid cells was observed around the ectopic lymphoid foci in the affected urogenital tissues. To date, testicular involvement in a patient diagnosed with IgG4-RD had not been reported, making this the first reported case of IgG4-related epididymo-orchitis. These findings suggest that the immune mechanism underlying ectopic lymphoneogenesis in IgG4-RD may involve enhanced BAFF/BAFF-R interactions among lymphoid cells.

  11. IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century.

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    Masaki, Yasufumi; Kurose, Nozomu; Umehara, Hisanori

    2011-01-01

    IgG4-related disease is a novel lymphoproliferative disorder that shows hyper-IgG4-γ-globulinemia and IgG4-producing plasma cell expansion in affected organs with fibrotic or sclerotic changes. Patients show systemic inflammatory conditions and various symptoms depending on the affected organ. Since the first report of patients with elevated serum IgG4 in sclerosing pancreatitis in 2001, various systemic disorders described by many names have been reported. Despite similarities in the organs involved in IgG4-related Mikulicz's disease and Sjögren's syndrome, there are marked clinical and pathological differences between these conditions. Most patients diagnosed with autoimmune pancreatitis in Japan have IgG4-related pancreatitis [Type 1 autoimmune pancreatitis (AIP), lymphoplasmacytic sclerosing pancreatitis (LPSP)], a disease distinct from some of the western type [Type 2 AIP, idiopathic duct-centric chronic pancreatitis (IDCP), autoimmune pancreatitis with granulocytic epithelial lesions (GEL)]. Diagnosis of IgG4-related disease is characterized by both elevated serum IgG4 (>135 mg/dL) and histopathological features including lymphocyte and IgG4(+) plasma cell infiltration (IgG4(+) plasma cells/IgG(+) plasma cells>40%). Differential diagnosis from other distinct disorders, such as sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, cancer, and other existing conditions associated with high serum IgG4 level or abundant IgG4-bearing plasma cells in tissues is necessary. We have begun a clinical prospective study to establish a treatment strategy (Phase II prospective treatment study for IgG4-multiorgan lymphoproliferative syndrome: UMIN R000002311).

  12. Sjögren’s syndrome versus IgG4-related diseases – classification difficulties and treatment progress

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    Anna Nowakowska-Płaza

    2014-09-01

    Full Text Available Sjögren’s syndrome (SS is a chronic autoimmune disorder characterized by lymphocytic infiltration in exocrine glands mainly salivary and lacrimal which affects impairment of their functions. Some patients develop extraglandular symptoms such as chronic fatigue, arthralgia, or lung, renal, central or peripheral nervous system involvement. Recent decades have brought understanding of some pathogenetic mechanisms and offered new therapeutic options by depleting B cells. Furthermore, the American College of Rheumatology proposed a new set of classification criteria based on objective symptoms. IgG4-related diseases are new nosological entities. The clinical course similarities of SS to Mikulicz’s disease (a subtype of IgG4-related disease result in diagnostic difficulties. Typical conditions of them are: an increased IgG4 level and infiltrations of parenchymal organs by plasmatic cells. This review summarizes classification difficulties, pathogenesis and treatment strategies of SS and IgG4-related diseases.

  13. Mitochondrial-dependent Autoimmunity in Membranous Nephropathy of IgG4-related Disease

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    Simona Buelli

    2015-05-01

    Full Text Available The pathophysiology of glomerular lesions of membranous nephropathy (MN, including seldom-reported IgG4-related disease, is still elusive. Unlike in idiopathic MN where IgG4 prevails, in this patient IgG3 was predominant in glomerular deposits in the absence of circulating anti-phospholipase A2 receptor antibodies, suggesting a distinct pathologic process. Here we documented that IgG4 retrieved from the serum of our propositus reacted against carbonic anhydrase II (CAII at the podocyte surface. In patient's biopsy, glomerular CAII staining increased and co-localized with subepithelial IgG4 deposits along the capillary walls. Patient's IgG4 caused a drop in cell pH followed by mitochondrial dysfunction, excessive ROS production and cytoskeletal reorganization in cultured podocytes. These events promoted mitochondrial superoxide-dismutase-2 (SOD2 externalization on the plasma membrane, becoming recognizable by complement-binding IgG3 anti-SOD2. Among patients with IgG4-related disease only sera of those with IgG4 anti-CAII antibodies caused low intracellular pH and mitochondrial alterations underlying SOD2 externalization. Circulating IgG4 anti-CAII can cause podocyte injury through processes of intracellular acidification, mitochondrial oxidative stress and neoantigen induction in patients with IgG4 related disease. The onset of MN in a subset of patients could be due to IgG4 antibodies recognizing CAII with consequent exposure of mitochondrial neoantigen in the context of multifactorial pathogenesis of disease.

  14. Prohibitin Is Involved in Patients with IgG4 Related Disease.

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    Hongwu Du

    Full Text Available IgG4-related disease (IgG4-RD is a chronic systemic disease involved in many organs and tissues. As only limited autoantigens have been found since the beginning of this century, the aim of this study was to reveal new candidate autoantigens of IgG4-RD.Multiple cell lines including HT-29, EA.hy926, HEK 293 and HepG2 were used to test the binding ability of circulating autoantibodies from IgG4-RD sera. The amino-acid sequence was then analyzed by matrix-assisted laser desorption/ionization time-of-flight tandem (MALDI-TOF/TOF mass spectrometry. After the cloning and expression of recombinant putative autoantigen in a bacterial expression system, the corresponding immuno assay was set up and utilized to observe the prevalence of serum autoantibodies in a large set of confirmed clinical samples.One positive autoantigen was identified as prohibitin. ELISA analysis showed that a majority of patients with IgG4-RD have antibodies against prohibitin. Anti-prohibitin antibodies were present in the sera of patients with definite autoimmune pancreatitis (25/34; 73.5%, Mikulicz's disease (8/15; 53.3%, retroperitoneal fibrosis (6/11; 54.5%, other probable IgG4-RD (26/29; 89.7% and Sjögren's syndrome (4/30; 13.3% but not in apparently healthy donors (1/70; 1.4%.An association between prohibitin and patients with some IgG4-RD was observed, although the results were quite heterogeneous among different individuals within autoimmune pancreatitis, Mikulicz's disease and retroperitoneal fibrosis.

  15. Idiopathic granulomatous orchitis: morphology and evaluation of its relationship to IgG4 related disease.

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    Karram, Sarah; Kao, Chia-Sui; Osunkoya, Adeboye O; Ulbright, Thomas M; Epstein, Jonathan I

    2014-04-01

    Idiopathic granulomatous orchitis (IGO) is rare, thought to result from an autoimmune reaction to spermatogenic elements. Its relationship to IgG4-related disease (IgG4-RD) has not been evaluated. Sixteen orchiectomy specimens (1984-2012) with a prominent intratubular granulomatous reaction were reviewed: IGO (n = 6); intratubular germ cell neoplasia unclassified (IGCNU) with a granulomatous reaction and associated seminoma (GS, n = 6); and unclassified intratubular granulomatous orchitis not fitting into a specific entity (UGO, n = 4). Men with IGO were 32 to 86 years old, presenting with a mass suspicious for malignancy. Only one patient had a history of an inflammatory disease. Clinical follow-up was available for 2 patients with IGO, and both had no evidence of systemic IgG4-RD. All IGO cases had an epithelioid granulomatous reaction confined to seminiferous tubules, an extensive interstitial lymphoplasmacytic inflammation, 3 of 6 had prominent interstitial fibrosis, and 3 of 6 cases had plasma cells with an IgG4+/IgG+ ratio >40%. In GS, 10% to 100% of tubules with IGCNU had a granulomatous reaction, which in 3 cases replaced IGCNU cells. In contrast to IGO, GS had more intratubular multinucleated giant cells, more peritubular sclerosis, fewer interstitial plasma cells, and no interstitial fibrosis. Of the 4 UGO cases, most had predominantly interstitial with less intratubular granulomatous inflammation. Only 1 non-IGO case had elevated tissue IgG4 (GS case). It is critical and sometimes difficult to distinguish GS from IGO. IGO shares some features with IgG4-RD, yet current evidence does not support its classification as a localized manifestation of IgG4-RD occurring in the testis.

  16. Perivascular fibrosis and IgG4-related disease: a case report

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    S. Monti

    2014-11-01

    Full Text Available Immunoglobulin G4-related disease (IgG4-RD is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.

  17. Normal pressure hydrocephalus, a possible complication in IgG4-related disease

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    Hiroshi Kobayashi

    2016-09-01

    Full Text Available We report a 75-year-old man with glucocorticoid-responsive normal pressure hydrocephalus (NPH complicated with tubulointerstitial renal disease, protein-losing gastroenteropathy (PLG and elevated serum IgG4, which might be a possible subset of IgG4-related disease (IgG4-RD. Although either PLG or NPH, especially in combination, has rarely been reported in IgG4-RD, the glucocorticoid-responsive nature of every abnormality observed in the patient supports the diagnosis of IgG4-RD. Of course, pathological confirmation is essential to fulfill the recently raised diagnostic criteria, however, such invasive procedure might not always be indicated in high-risk patients. Our report illustrates that IgG4-RD might be considered as one of the underlying causes and/or the aggravating factors of NPH before shunt operation.

  18. Distinct histopathological features of Hashimoto's thyroiditis with respect to IgG4-related disease.

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    Li, Yaqiong; Zhou, Gengyin; Ozaki, Takashi; Nishihara, Eijun; Matsuzuka, Fumio; Bai, Yanhua; Liu, Zhiyan; Taniguchi, Emiko; Miyauchi, Akira; Kakudo, Kennichi

    2012-08-01

    A form of Hashimoto's thyroiditis with lymphoplasmacytic sclerosing changes and increased numbers of IgG4-positive plasma cells has recently been reported in the literature. These histopathological features suggest that this subtype of Hashimoto's thyroiditis may be closely related to IgG4-related disease. Therefore, this unique form of IgG4-related Hashimoto's thyroiditis, which is referred to as IgG4 thyroiditis, has its own clinical, serological, and sonographic features that are distinct from those associated with non-IgG4 thyroiditis. IgG4 thyroiditis shares similarities with the well-known fibrous variant of Hashimoto's thyroiditis; however, the detailed histopathological features of IgG4 thyroiditis have not been well established. Based on immunostaining results, 105 patients with Hashimoto's thyroiditis were divided into an IgG4 thyroiditis group (n=28) and a non-IgG4 thyroiditis group (n=77). As in our previous reports, IgG4 thyroiditis was associated with a patient population of a younger age, a lower female-to-male ratio, rapid progression, higher levels of thyroid autoantibodies, subclinical hypothyroidism, and diffuse sonographic echogenicity. Histopathologically, this group revealed severe lymphoplasmacytic infiltration, dense stromal fibrosis, marked follicular cell degeneration, numerous micro-follicles, and notable giant cell/histiocyte infiltration. Importantly, the IgG4-related group did not completely overlap with fibrous variant of Hashimoto's thyroiditis. Four cases (14%) in the IgG4 thyroiditis group presented only mild fibrosis in the stroma, whereas 29 cases (38%) in the non-IgG4 thyroiditis group met the diagnostic criteria for fibrous variant of Hashimoto's thyroiditis. Furthermore, we observed three patterns of stromal fibrosis in Hashimoto's thyroiditis: interfollicular fibrosis, interlobular fibrosis, and scar fibrosis. The IgG4 thyroiditis group was significantly associated with the presence of predominant interfollicular fibrosis. In

  19. Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease

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    Teresa Carbone

    2015-01-01

    Full Text Available IgG4-related disease (IgG4-RD encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.

  20. High Expression of Galectin-3 in Patients with IgG4-Related Disease: A Proteomic Approach

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    Adeeb Salah

    2017-01-01

    Full Text Available Objectives. Immunoglobulin G4-related disease (IgG4-RD is a multiorgan condition manifesting itself in different forms. This study aimed to investigate protein expression profiles and to find the possible biomarker for IgG4-RD by liquid chromatography mass spectrometry (LC-MS using tissue sections in IgG4-RD patients. Methods. Protein expression profiles in five IgG4-related pancreatitis and three normal pancreatic samples were compared using LC-MS and were validated by quantitative real-time PCR (qRT-PCR, immunoblotting, and immunohistochemistry. ELISA was employed in the serum of 20 patients with systemic IgG4-RD before and during steroid treatment. Results. LC-MS indicated that the levels of 17 proteins were significantly higher and 12 others were significantly lower in IgG4-related pancreatitis patients compared to controls. Among these proteins, galectin-3 levels were 13-fold higher in IgG4-related pancreatitis (P<0.01. These results were confirmed by immunoblotting and qRT-PCR. The average number of galectin-3 + cells in various organs of IgG4-RD patients, including salivary glands, lungs, and lymph nodes, was higher than in controls. Galectin-3 was detectable in macrophages, dendritic cells, and stromal myofibroblast-like cells, but not in lymphocytes by immunofluorescence staining. Serum galectin-3 levels were higher in patients with IgG4-RD compared with healthy donors and remained high during steroid therapy. Conclusion. Galectin-3 was overexpressed in IgG4-RD and the levels were indirectly related to clinical activity.

  1. IgG4-related multiorgan disease: report of the first autopsy case

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    Ochoa, Minerva Lazos; Gabiño López, Belem; Cabello, Raúl Romero; Feregrino, Raúl Romero

    2013-01-01

    IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. At present, several illnesses such as Mikulicz disease, Küttner tumour, multifocal fibrosclerosis, etc, are considered to be part of the IgG4RD spectrum. The symptoms of the disease tend to appear over months and years and diagnosis is achieved on average 13.5 months (4–60 months) after the onset. The purpose of this report was to provide information about a case that was sadly fatal but that permitted a complete histopathological study of the damaged tissues. PMID:23645656

  2. Increased RP105-Negative B Cells in IgG4-Related Disease.

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    Koarada, S; Tashiro, S; Nagao, N; Suematsu, R; Ohta, A; Tada, Y

    2013-01-01

    Four patients with IgG4-related disease (IgG4-RD) showed increased percentages of RP105-negative B cells in the peripheral blood. Case 1: A 66-year-old man having retroperitoneal fibrosis had 18.8% of RP105-negative B cells. Oral prednisolone improved the affected lesions and the percentage of RP105-negative B cells decreased (3.2%) after the treatment. Case 2: A 53-year-old man with retroperitoneal fibrosis had 27.9% of RP105-negative B cells. Case 3: A 38-year-old man with follicular hyperplasia showed increased percentage of RP105-negative B cells (8.3%). Case 4: A 60-year-old man with interstitial nephritis had 27.5% of RP105-negative B cells. The treatment decreased the numbers of RP105-negative B cells. Increased numbers of RP105-negatvie B cells is possibly associated with disease activity of IgG4-RD. Analysis of expression of RP105 on B cells may be helpful in evaluation of disease activity of IgG4-RD.

  3. IgG4-related systemic diseases: a report of eight cases%IgG4相关系统性疾病八例临床分析

    Institute of Scientific and Technical Information of China (English)

    郑可; 李雪梅; 蔡建芳; 文煜冰

    2012-01-01

    Objective To explore the clinical features of IgG4-related systemic diseases (IgG4-RSD)Methods A total of 8 inpatients with IgG4-RSD diagnosed at our hospital during August 2010 to December 2011 were recruited.We analyzed the clinical data,laboratory profiles,radiological and pathologic features and prognostic factors of these patients with IgG4-RSD.Results There were 5 males and 3 females with a mean onset age of 52.5 years.IgG4-related diseases were described in multiple organ systems:pancreas (n =5),biliary tree (n =3),kidneys (n =6),lungs (n =3),aorta and periaortic tissue,retroperitoneum (n =5) and lymph nodes (n =6).Hyperglobulinemia,elevated serum levels of IgG and IgG4,anemia(n =6),renal dysfunctions(n =5) and obstructive jaundice (n =2) were common laboratory findings.Lymphoplasmacytic infiltration and fibrosis were common pathologic findings.A diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field provided compelling evidences of IgG4-related disease.Patients with IgG4-RSD responded well to glucocorticoids.Conclusion With heterogeneous clinical characteristics,IgG4-RSD is found in various organ systems.The prominent histopathologic features of IgG4-RSD include a diffuse infiltration of plasma cells with over 30 IgG4-positive cells per high-power field.And the therapy of glucocorticoids is efficacious.%目的 总结IgG4相关系统性疾病(IgG4-RSD)的临床特点,提高对该疾病的认识.方法 回顾性分析符合IgG4-RSD诊断的8例患者的临床表现、实验室检查、影像学结果、病理表现和治疗及预后情况.结果 8例IgG4-RSD患者中,男5例,女3例,中位年龄52.5(36 ~72)岁;临床首发表现多样化;受累器官包括胰腺(5例)、胆道(3例)、肾脏(6例)、肺部(3例)、后腹膜和腹主动脉(5例)、淋巴结(6例)等;实验室检查中所有患者均存在高球蛋白血症,IgG及IgG4亚型水平升高,部分患者存在贫血(6例)、肾功能异常(5

  4. Histopathologic Overlap between Fibrosing Mediastinitis and IgG4-Related Disease

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    Tobias Peikert

    2012-01-01

    Full Text Available Fibrosing mediastinitis (FM and IgG4-related disease (IgG4-RD are two fibroinflammatory disorders with potentially overlapping clinical and radiological features. In this paper, we looked for histopathologic features of IgG4-RD and enumerated infiltrating IgG4-positive plasma cells within mediastinal tissue biopsies from FM patients. We identified 15 consecutive FM surgical mediastinal tissue biopsies between 1985 and 2006. All patients satisfied the clinical and radiological diagnostic criteria for FM. All patients had either serological or radiological evidence of prior histoplasmosis or granulomatous disease, respectively. Formalin-fixed paraffin-embedded tissue sections of all patients were stained for H&E, IgG, and IgG4. Three samples met the predefined diagnostic criteria for IgG4-RD. In addition, characteristic histopathologic changes of IgG4-RD in the absence of diagnostic numbers of tissue infiltrating IgG4-positive plasma cells were seen in a number of additional cases (storiform cell-rich fibrosis in 11 cases, lymphoplasmacytic infiltrate in 7 cases, and obliterative phlebitis/arteritis in 2 cases. We conclude that up to one-third of histoplasmosis or granulomatous-disease-associated FM cases demonstrate histopathological features of IgG4-RD spectrum. Whether these changes occur as the host immune response against Histoplasma or represent a manifestation of IgG4-RD remains to be determined. Studies to prospectively identify these cases and evaluate their therapeutic responses to glucocorticoids and/or other immunosuppressive agents such as rituximab are warranted.

  5. IgG4-related disease-experience of 100 consecutive cases from a specialist centre.

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    Bateman, Adrian C; Culver, Emma L

    2017-04-01

    To describe the features of 100 consecutive cases referred to a single UK institution in which a diagnosis of IgG4-related disease (IgG4-RD) was under consideration. The histological features were reviewed by a single histopathologist, and cases were categorized according to the 2012 Boston criteria: Category 1-histologically highly suggestive of IgG4-RD; Category 2-probable histopathological features of IgG4-RD; and Category 3-insufficient histopathological evidence of IgG4-RD. A 'global assessment' was performed with the available clinical information: Assessment group 1-'definite/very likely IgG4-RD'; Assessment group 2-'possible IgG4-RD'; Assessment group 3-'not IgG4-RD'; and Assessment group 4-insufficient information. The mean IgG4+ plasma cell count and IgG4+/IgG+ ratio were highest in Category 1 [134/high-power field (HPF); 57%] and Assessment group 1 (113/HPF; 52%), and lowest in Category 3 (11/HPF; 18%) and Assessment group 3 (43/HPF; 31%) (Category comparison of IgG4+ count and ratio, both P < 0.001; Assessment group comparison of IgG4+ count, P < 0.0002; and Assessment group comparison of ratio, P = 0.04). A non-IgG4-RD diagnosis was rare in Category 1 (7%) but common in Category 2 (60%) and Category 3 (47%). Stromal reactions to neoplasia and chronic oral ulceration were simulants of IgG4-RD. The Boston criteria are linked to the likelihood of IgG4-RD. Other conditions may show some histological features of IgG4-RD. The likelihood of IgG4-RD is much greater when the histological features reach the threshold for Category 1 than when they reach the thresholds for Categories 2 and 3. Despite the utility of the Boston criteria, this study highlights the crucial importance of careful clinicopathological correlation when a diagnosis of IgG4-RD is under consideration. © 2016 John Wiley & Sons Ltd.

  6. IgG4-related interstitial lung disease%IgG4相关间质性肺疾病

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    荣令; 李家树; 周新

    2010-01-01

    IgG4-related interstitial lung disease is proposed based on the findings of systemic IgG4-related sclerosing disease. The diagnosis of IgG4-related interstitial lung disease requires the combination of clinical-radiological-pathological features. The therapeutic response of IgG4-related interstitial lung disease to glucocorticoid is good,and follow-up is necessary for the detection of recurrence.%IgG4相关间质性肺疾病的提出是基于对系统性IgG4相关硬化性疾病的研究发现.IgG4相关间质性肺疾病的诊断需要临床医师结合临床-影像学-病理学特征疑及此病.IgG4相关间质性肺疾病对糖皮质激素治疗反应良好,监测随访对于发现复发是必要的.

  7. IgG4-Related Mikulicz's Disease Associated with Thyroiditis:a Case Report and Review of the Literature

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    Yujiao Zhang; Yi Du; Kaijun Li; Jianfeng He

    2014-01-01

    Purpose:To report an unusual case of IgG4-related Mikulicz's disease associated with thyroiditis. Case report:.We describe a 25-year-old Chinese man who presented with bilateral,.painless swellings of the lachrymal glands,.parotid glands,.and thyroid nodules..The patient un-derwent left-sided dacryoadenectomy and the diagnosis of IgG4-related Mikulicz's disease was pathologically confirmed. The size of the right-sided lachrymal gland and parotid glands recovered fundamentally after one month of glucocorticoid therapy. Conclusion:.IgG4-related Mikulicz's disease associated with thyroiditis should be considered in the differential diagnosis of bilateral swellings of lachrymal glands, salivary glands, and thyroid nodules. Surgical excision is recommended in order to treat the tumor and to ensure the pathological diagnosis. Glu-cocorticoid therapy should be considered in association with surgery after removal. (Eye Science 2014; 29:47-52).

  8. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia

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    Francesco Rapisarda

    2015-01-01

    Full Text Available The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm3 (or <20% of total T cells in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.

  9. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia.

    Science.gov (United States)

    Rapisarda, Francesco; Zanoli, Luca; Portale, Grazia; Scuto, Salvo; Castellino, Pietro

    2015-01-01

    The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm(3) (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.

  10. Is IgG4-Related Disease a Cause of Xerostomia? A Cohort Study of 60 Patients

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    M. Hermet

    2012-01-01

    Full Text Available Objective. Immunoglobulin-G4-(IgG4- related disease (IgG4 RD is a fibrosing process characterized by a significant infiltration of IgG4-secreting plasma cells. IgG4 RD can affect almost all organs including salivary glands. Whether IgG4 RD plays a role in the development of sicca syndrome and particularly dry mouth syndrome remains to be investigated. Methods. We conducted a monocentric cohort study for two years to search for IgG4 RD features in patients with dry mouth syndrome using immunostainings of labial salivary gland specimens with anti-IgG4 antibody. Results. Among 60 patients presenting with dry mouth syndrome who underwent labial salivary gland biopsy, 18 showed positive immunostaining with the anti-IgG4 antibody including 4 patients with typical systemic IgG4 RD. Five also fulfilled criteria for Sjögren's syndrome. Conclusion. These findings suggest that clinical forms of IgG4 RD salivary involvement without salivary swelling may occur. This salivary involvement is probably overlooked in everyday practice and could represent a mild form of IgG4 RD.

  11. Recent advances in understanding and managing IgG4-related disease [version 1; referees: 4 approved

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    Anna R. Wolfson

    2017-02-01

    Full Text Available IgG4-related disease was only recently discovered, so its description, management, and new discoveries related to its etiology are rapidly evolving. Because IgG4 itself is a unique antibody which is intimately related to the diagnosis of the disease, the role of plasmablasts in the pathophysiology remains an active area of discussion. Recent studies have uncovered a possible role for CD4-positive cytotoxic T lymphocytes, T follicular helper cells, and M2 macrophages. The clinical presentation is variable and can be vague, as this disease affects many organs and new presentations are continuing to be described. The diagnosis depends on clinical and histopathological assessment. The mainstay of treatment is with glucocorticoids, but rituximab has recently shown promise. Monitoring disease activity using imaging modalities (including positron emission tomography and serum markers is imperative, as relapses are common. IgG4-related disease spans many medical disciplines but is a treatable condition with which all clinicians should be familiar.

  12. [IgG4-related disease is a rare differential diagnosis of malignant and autoimmune diseases].

    Science.gov (United States)

    Storgaard, Anders; Detlefsen, Sönke

    2015-04-06

    Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory and fibrotic disease with the potential to produce diffuse enlargement, massforming lesions or stenoses in a wide range of organs. Elevation of serum IgG4 concentration and high levels of IgG4-positive cells in the inflamed tissue are common denominators. Type 1 autoimmune pancreatitis is one of the main manifestations, and its recognition preceded the definition of IgG4-RD as a novel clinical entity. The aetiology, pathophysiology, epidemiology and clinical long-term outcome of IgG4-RD are not fully elucidated. Steroids are effective in most patients, sometimes combined with other antiinflammatory drugs.

  13. Tubulointerstitial Nephritis Associated with IgG4-Related Systemic Disease: a Report of Two Cases and Literature Review%免疫球蛋白G4相关小管间质性肾炎2例报告及文献复习

    Institute of Scientific and Technical Information of China (English)

    刘红; 侯君; 蔡锋晴; 丁小强

    2011-01-01

    目的:通过个案报告及文献复习,认识一种新的肾脏疾病——免疫球蛋白G4(IgG4)相关小管间质性肾炎.方法:分析2例IgG4相关小管间质性肾炎的诊断、病理表现及治疗经过,并复习相关文献.结果:IgG4相关小管间质肾炎是IgG4相关性系统性疾病累及肾脏时最常见的病变,患者除有高球蛋白血症、高IgG4血症、低补体血症外,肾脏损伤表现主要为少量蛋白尿、偶见镜下血尿伴肾功能损伤.肾间质中大量IgG4阳性浆细胞浸润、间质纤维化为其特点.糖皮质激素治疗有效.结论:IgG4相关性系统性疾病可以导致肾小管间质炎症,值得临床重视.%Objective:To study a new renal disease: IgG4 -related tubulointerstial nephritis. Methods: Analyze clinical feature, laboratory examination, renal biopsy, treatment and follow-up data of two case of tubulointerstitial nephritis associated with IgG4 -related systemic disease and review the literature. Results: IgG4-related systemic disease was a protean disorder that covers a wide variety of lesions. Tubulointerstitial nephritis was the major finding when kidney affected by it. The injuries were associated with a high level of serum IgG4 and abundant IgG4-positive plasma cell infiltration into the renal interstitial with fi-brosis. Corticosteroids therapy is effective. Conclusions: We should pay more attention to IgG4-related autoimmune disease, which could be one cause of tubulointerstitial nephritis.

  14. IgG4-related sclerosing diseases%IgG4相关的硬化性疾病

    Institute of Scientific and Technical Information of China (English)

    孙希印; 燕丽; 刘萍; 李新功

    2016-01-01

    IgG4-related sclerosing diseases are newly recognized, which are considered to be related to au-toimmunity, including cellular and humoral immune abnormity. IgG4 levels in serum are higher than normal. Histopathological findings include fibrosis with significant inflammatory cells, mainly IgG4(+) plasma cells. Lesions can involve many internal organs. Skin lesions are mostly found in head and neck, presenting with erythema, patches or subcutaneous nodules accompanying itching. Glucocorticoid is effective in the treatment of IgG4-related skin diseases.%IgG4相关硬化性疾病是近年被认识的一种疾病,被认为与自身细胞、体液免疫反应异常有关。患者血清IgG4水平升高,病变器官纤维化,并有显著IgG4阳性浆细胞浸润。病变可以累及许多器官,部分病例伴有皮肤的病变。皮肤病变以头颈部多见,可为红斑、斑片或皮下结节,有痒感。 IgG4相关皮肤疾病糖皮质激素治疗有效。

  15. Solely lung-involved IgG4-related disease : a case report and review of the literature.

    Science.gov (United States)

    Zhang, Xiao-Qin; Chen, Guo-Ping; Wu, Sheng-Chang; Yu, Sa; Wang, Hong; Chen, Xuan-Yi; Ren, Zhuo-Chao

    2016-12-23

    By analyzing the clinical data of 1 case of IgG4-related lung disease(IgG4-RLD) and the review of literature, the author investigated the clinical characteristics of IgG4-RLD. IgG4-RLD is a rare disease characterized by significant elevation of serum IgG4 and infiltration of a large number of IgG4+ plasma cells. The clinical manifestations of the disease were nonspecific, and the imaging features were mixed with several types. The disease can only be involved in the lung, but also multiple organ involvement. Solely lung-involved IgG4-RD is not only extremely rare but also easily misdiagnosed as tuberculosis, lung cancer, lymphoma and other common pulmonary diseases. Histopathological examination is the key to the diagnosis of the disease. Corticosteroids are the first choice of treatment, and the overall prognosis is good.

  16. IgG4-related disease: current challenges and future prospects

    Directory of Open Access Journals (Sweden)

    Lang D

    2016-02-01

    Full Text Available David Lang,1 Jochen Zwerina,2 Herwig Pieringer1,3 1Second Department of Internal Medicine, Kepler University Clinic, Linz, 2First Department of Internal Medicine, Ludwig Boltzmann-Institute of Osteology, Hanusch Hospital, Vienna, 3Paracelsus Private Medical University, Salzburg, Austria Abstract: Immunoglobulin G4-related disease (IgG4-RD represents an immune-mediated fibroinflammatory condition with a characteristic histopathological appearance that can affect various organs. Although numerous single-organ manifestations have been described more than a century ago, its systemic nature and unique features were only discovered in the last 2 decades, when IgG4-RD emerged as a new entity of disease. IgG4-RD is usually considered a rare disease, but its true epidemiology has not yet been fully clarified. Also, despite recent advances in the identification of the underlying immunological processes, its pathophysiology is only incompletely understood till now. The diagnostic workup of IgG4-RD is complex and usually requires a combination of clinical examination, imaging, histological, and serological analyses. However, no finding alone is specific for IgG4-RD. Therefore, its diagnosis requires careful interpretation of examination results in context with the patient’s clinical appearance as well as the exclusion of a broad variety of differential diagnoses. The past years brought rapid advances concerning this novel disease entity: diagnostic criteria, further insights into the underlying immunological processes, new biomarkers, and novel therapeutic approaches were proposed and widened the knowledge in the field of IgG4-RD. Still, a greater number of questions remain unanswered, and many recent developments require further discussion and proof from clinical trials. This review should give an overview on current knowledge and future perspectives in epidemiology, pathophysiology, diagnosis, and therapy of IgG4-RD. Keywords: PET/CT, autoimmune

  17. Fibrosing variant of Hashimoto thyroiditis is an IgG4 related disease.

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    Deshpande, Vikram; Huck, Amelia; Ooi, Esther; Stone, John H; Faquin, William C; Nielsen, G Petur

    2012-08-01

    Hashimoto thyroiditis (HT) and the fibrosing variant of Hashimoto thyroiditis (FVHT) are immune-mediated tumefactive lesions of the thyroid. Immunoglobulin G4-related disease (IgG4-RD) is now a widely recognised multi-organ system disease characterised by elevated serum and tissue concentrations of IgG4. In this study, the authors address several unresolved questions pertaining to the relationship between HT and FVHT, and the association of each of these diseases with IgG4-RD. The authors evaluated 28 consecutive cases of HT and nine cases of FVHT. The clinical, demographic and serological data were recorded. The slides were stained immunohistochemically using antibodies to IgG4 and IgG and the quantitative analysis was recorded. Data on thyroid function tests were available on seven cases of FVHT and 14 cases of HT. Based on the availability of data, hypothyroidism was noted in 62% (9/14) of HT and 86% of FVHT (6/7). FVHT demonstrated an exaggerated lobular pattern with lobules separated by cellular storiform-type fibrosis, resembling fibrosis seen in other forms of IgG-RD. The median IgG4 counts per high power field (×40) in HT and FVHT were 2.3 and 22, respectively. The median IgG4:IgG ratios in HT and FVHT were 0.11 and 0.58, respectively. The authors propose that FVHT belongs to the spectrum of IgG4-RD. Although a proportion of cases of HT show elevated numbers of IgG4 positive plasma cells, these cases lack the histological features typically associated with IgG4-RD, and thus the relationship between HT and IgG4-RD remains unproven.

  18. Is MuSK myasthenia gravis linked to IgG4-related disease?

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    Raibagkar, Pooja; Ferry, Judith A; Stone, John H

    2017-04-15

    Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition that affects a wide variety of sites, including the nervous system, where it can involve the meninges or the pituitary gland, and cause perineural mass lesions. A large subset of acetylcholine receptor antibody (Ab)-negative myasthenia gravis (MG) patients has muscle-specific tyrosine kinase (MuSK) Abs, generally of the IgG4 subclass. There has not been any association found between IgG4-RD and MuSK MG yet. We report the first case of MuSK MG associated with lymphadenopathy with histopathology consistent with IgG4-RD. A 54-year-old woman with MuSK MG developed eight compression fractures related to steroid therapy. Eighteen months after initial presentation she was found to have retroperitoneal lymphadenopathy with biopsy findings consistent with IgG4-RD. She was started on rituximab with clinical improvement and ability to taper immunomodulatory agents for the first time. Our case raises number of questions regarding a potential link between MuSK MG and IgG4-RD which may shed further light on the pathophysiology and management of these diseases. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. Clinical significance of complement as a biomarker of disease activity in 4 cases of IgG4-related disease with retroperitoneal fibrosis.

    Science.gov (United States)

    Kihara, Mari; Sugihara, Takahiko; Hosoya, Tadashi; Miyasaka, Nobuyuki

    2013-01-01

    Hypocomplementaemia is frequently observed in IgG4-related diseases, however the clinical significance is unclear. We describe herein the clinical courses of 4 patients with IgG4-related disease with hypocomplementaemia. Our cases showed autoimmune pancreatitis, retroperitoneal fibrosis, Mikulicz's disease, interstitial lung disease, lymphadenopathy and mesenteric fibrosis around the aorta. A decrease in serum complement preceded deterioration of the disease and clinical improvement was observed in accordance with normalisation of serum complement. These clinical courses suggest that serum complement is a biomarker of the disease activity.

  20. Autoimmune Pancreatitis and IgG4 Related Disease in Three Children

    Science.gov (United States)

    Chong, Sze Yee; Coleman, Lee; MacGregor, Duncan; Hardikar, Winita; Oliver, Mark R.

    2016-01-01

    We report 3 children who presented with fever and abdominal pain, deranged liver function tests, and on abdominal ultrasound were found to have an enlarged pancreas, substantial abdominal lymphadenopathy, and extrahepatic biliary duct dilatation. After ruling out malignancy, probable immunoglobulin G4-related disease (IgG4RD) associated with autoimmune pancreatitis was considered. This condition was first described in the adults and often mimics pancreatic cancer. It can involve multiple organs, either synchronously or metachronously, and is rarely reported in children. The disorder mostly responds to corticosteroid therapy and other immune suppression. We highlight the difficulty in diagnosing autoimmune pancreatitis/IgG4-related disease in children and illustrate the difference between pediatric and adult presentation. PMID:27622194

  1. Overlapping Morphologic and Immunohistochemical Features of Hashimoto Thyroiditis and IgG4-Related Thyroid Disease.

    Science.gov (United States)

    Raess, Philipp W; Habashi, Arlette; El Rassi, Edward; Milas, Mira; Sauer, David A; Troxell, Megan L

    2015-05-01

    Immunoglobulin G4-related disease (IgG4-RD) is an emerging clinicopathologic entity characterized by both IgG4+ plasma cell infiltration and fibrosis in one or more organs, prototypically pancreas or salivary/lacrimal glands. IgG4-RD in the thyroid (IgG4-RTD) is an area of active study, and the relationship between IgG4-RTD and Hashimoto thyroiditis is not fully delineated due to their overlapping histologic features. Retrospective review was performed of all thyroidectomy cases demonstrating lymphocytic inflammation at a single institution over a 4-year period. Approximately half (23/38) of patients had a clinical diagnosis of Hashimoto thyroiditis (HT). Nine of the 38 patients had increased absolute and relative numbers of IgG4+ plasma cells. Patients with a clinical diagnosis of HT had increased lymphoplasmacytic inflammation, but the relative proportion of IgG4+ plasma cells was not increased compared to patients without HT. There was no correlation between IgG4 levels and the amount of fibrosis in patients with or without HT. Patients identified as having the fibrosing variant of HT were not more likely to have increased levels of IgG4+ plasma cells than those without. There is significant morphologic and immunohistochemical overlap between HT and IgG4-RTD. Future studies to identify specific characteristics of IgG4-RTD involving the thyroid are necessary to accurately define this entity.

  2. Severe IgG4-Related Disease in a Young Child: A Diagnosis Challenge

    Directory of Open Access Journals (Sweden)

    Susana Corujeira

    2015-01-01

    Full Text Available Immunoglobulin G4-related disease (IgG4-RD is an increasingly recognized syndrome that can appear with multiple organ involvement, typically with tumor-like swelling, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and elevated serum IgG4 concentrations. We report the case of a 22-month-old female child with failure to thrive and recurrent respiratory tract infections since 8 months of age. Physical examination was normal except for pulmonary auscultation with bilateral crackles and wheezes. Laboratory tests revealed elevated erythrocyte sedimentation rate, and elevated serum IgG and IgG4 with polyclonal hypergammaglobulinemia. Thoracic CT and MRI showed multiple mediastinal lymphadenopathies and a nodular posterior mediastinal mass in right paratracheal location with bronchial compression. Initial fine needle aspiration biopsy was compatible with reactive lymphadenopathy but after clinical worsening a thoracoscopic partial resection of the mass was performed and tissue biopsy revealed lymphoplasmacytic infiltrate and increased number of IgG4-positive plasma cells and a ratio of IgG4/IgG positive cells above 40%. Glucocorticoids therapy was started with symptomatic improvement, reduction in the size of the mass, and decrease of serum IgG4 levels after 6 weeks. There are very few reports of IgG4-RD in children. Long-term follow-up is necessary to monitor relapses and additional organ involvement.

  3. Clinicopathological features of Riedel's thyroiditis associated with IgG4-related disease in Japan.

    Science.gov (United States)

    Takeshima, Ken; Inaba, Hidefumi; Ariyasu, Hiroyuki; Furukawa, Yasushi; Doi, Asako; Nishi, Masahiro; Hirokawa, Mitsuyoshi; Yoshida, Akira; Imai, Ryoukichi; Akamizu, Takashi

    2015-01-01

    Riedel's thyroiditis (RT) is a rare chronic fibrosing disorder characterized by a hard, infiltrative lesion in the thyroid gland, which is often associated with multifocal fibrosclerosis. Immunoglobulin G4-related disease (IgG4-RD) is typified by infiltration of IgG4-positive plasma cells into multiple organs, resulting in tissue fibrosis and organ dysfunction. In order to evaluate the clinicopathological features of RT and its relationship with IgG4-RD, we performed a Japanese literature search using the keywords "Riedel" and "Riedel's thyroiditis." We used the electronic databases Medline and Igaku Chuo Zasshi, the latter of which is the largest medical literature database in Japan. The diagnosis of RT was based on the presence of a fibroinflammatory process with extension into surrounding tissues. Only 10 patients in Japan fulfilled RT diagnostic criteria during the 25-year period between 1988 and 2012. Two patients with confirmed IgG4/IgG immunohistochemical findings demonstrated 43 and 13 IgG4-positive plasma cells per high-power field, respectively, and the IgG4-positive/IgG-positive plasma cell ratios of 20% and less than 5%. Of the 10 patients with RT, two received glucocorticoids, one of whom experienced marked shrinkage of the thyroid lesion. One patient had extra-thyroid involvement in the form of retroperitoneal fibrosis. Although the clinicopathological features of RT suggest that IgG4-RD may be the underlying condition in some cases, further investigation is needed to clarify the etiology of RT in relation to IgG4-RD.

  4. Cutoff Values of Serum IgG4 and Histopathological IgG4+ Plasma Cells for Diagnosis of Patients with IgG4-Related Disease

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    Yasufumi Masaki

    2012-01-01

    Full Text Available IgG4-related disease is a new disease classification established in Japan in the 21st century. Patients with IgG4-related disease display hyper-IgG4-gammaglobulinemia, massive infiltration of IgG4+ plasma cells into tissue, and good response to glucocorticoids. Since IgG4 overexpression is also observed in other disorders, it is necessary to diagnose IgG4-related disease carefully and correctly. We therefore sought to determine cutoff values for serum IgG4 and IgG4/IgG and for IgG4+/IgG+ plasma cells in tissue diagnostic of IgG4-related disease. Patients and Methods. We retrospectively analyzed serum IgG4 concentrations and IgG4/IgG ratio and IgG4+/IgG+ plasma cell ratio in tissues of 132 patients with IgG4-related disease and 48 patients with other disorders. Result. Serum IgG4 >135  mg/dl demonstrated a sensitivity of 97.0% and a specificity of 79.6% in diagnosing IgG4-related disease, and serum IgG4/IgG ratios >8% had a sensitivity and specificity of 95.5% and 87.5%, respectively. IgG4+cell/IgG+ cell ratio in tissues >40% had a sensitivity and specificity of 94.4% and 85.7%, respectively. However, the number of IgG4+ cells was reduced in severely fibrotic parts of tissues. Conclusion. Although a recent unanimous consensus of all relevant researchers in Japan recently established the diagnostic criteria for IgG4-related disease, findings such as ours indicate that further discussion is needed.

  5. IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy

    Science.gov (United States)

    Li, Dujuan; Kan, Yunzhen; Fu, Fangfang; Wang, Shuhuan; Shi, Ligang; Liu, Jie; Kong, Lingfei

    2015-01-01

    Immunoglobulin G4-related disease (IgG4-RD) is a recently described inflammatory disease involving multiple organs. Prostate involvement with IgG4-RD is very rare. In this report, we describe a case of IgG4-related prostatitis progressed from localized IgG4-related lymphadenopathy. This patient was present with urine retention symptoms. MRI and CT examination revealed the prostatic enlargement and the multiple lymphadenopathy. Serum IgG4 levels were elevated. Prostatic tissue samples resected both this time and less than 1 year earlier showed the same histological type of prostatitis with histopathologic and immunohistochemical findings characteristic of IgG4-RD. The right submandibular lymph nodes excised 2 years earlier were eventually proven to be follicular hyperplasia-type IgG4-related lymphadenopathy. This is the first case of IgG4-RD that began as localized IgG4-related lymphadenopathy and progressed into a systemic disease involving prostate and multiple lymph nodes. This patient showed a good response to steroid therapy. This leads us to advocate a novel pathogenesis of prostatitis, and a novel therapeutic approach against prostatitis. Pathologists and urologists should consider this disease entity in the patients with elevated serum IgG4 levels and the symptoms of prostatic hyperplasia to avoid ineffective medical or unnecessary surgical treatment. PMID:26617921

  6. IgG4相关性疾病的发病机制及进展%Progress in pathogenesis of IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    苏玉莹; 王晨琼; 董凌莉

    2014-01-01

    IgG4-related disease, a newly recognized systemic entity, is characterized by elevated serum levels of IgG4 and a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells in involved tissues. Its complicated clinical manifestation and multidiscipline involvement make it easy to be misdiagnosed or miss diagnosed. In order to better understand this disease, this review elaborated on update in pathogenesis of IgG4-related disease including genetics, infection and molecular mimicry, immunology and so on.%IgG4相关性疾病是一种多器官、多系统受累的新被认识的疾病,其主要特征为:血清IgG4水平升高和病变组织IgG4+浆细胞浸润。IgG4相关性疾病临床表现复杂多样,可涉及多个学科,临床易漏诊和误诊。本篇综述将从基因遗传、感染和分子模拟、免疫学角度等多方面阐述IgG4相关性疾病的发病机制,以加深临床医师对该病的了解。

  7. 提高对IgG4相关性眼病的认识%Raise awareness of IgG4 relative ocular disease

    Institute of Scientific and Technical Information of China (English)

    魏世辉; 李红阳

    2015-01-01

    Purpose IgG4-related ocular disease is a chronic systemic disease with lymphocyte abnormal.The lacrimal glands, extraocular muscles and infraorbital nerve were often involved which was often the first symptom of systemic disease.While ophthalmologists did not know this disease well.They usually misdiagnosed it as idiopathic inflammatory pseudotumor, thyroid-associated ophthalmopathy etc,which resulted in delayed treatments.Here pathogenesis, clinical features and treatment methods of IgG4-relative ocular disease were described in order to improve awareness of this ocular disease, reduce clinical misdiagnosis, improve disease prognosis and standardized treatment.As the incidence of this disease increased in recent years, it is very necessary to improve awareness of the disease for ophthalmologists.%IgG4相关性眼病是一种与淋巴细胞密切相关的慢性系统性疾病,泪腺、眼外肌和眶下神经常常受累,常常为全身病变的首发症状.眼科医师对该疾病尚缺乏足够的认识,经常误诊为炎性假瘤、甲状腺相关性眼病等而延误治疗.本文通过对IgG4相关性眼病的发病机制、临床特点及治疗方法进行阐述,旨在提高临床医师对该眼病的认识,降低该眼病的临床误诊率,提升疾病标准化治疗和预后的质量.由于该疾病近年发病率有所提高,因此提高临床医师对该疾病的认识十分必要.

  8. Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome.

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    Danlos, François-Xavier; Rossi, Giovanni Maria; Blockmans, Daniel; Emmi, Giacomo; Kronbichler, Andreas; Durupt, Stéphane; Maynard, Claire; Luca, Luminita; Garrouste, Cyril; Lioger, Bertrand; Mourot-Cottet, Rachel; Dhote, Robin; Arlet, Jean-Benoit; Hanslik, Thomas; Rouvier, Philippe; Ebbo, Mikael; Puéchal, Xavier; Nochy, Dominique; Carlotti, Agnès; Mouthon, Luc; Guillevin, Loïc; Vaglio, Augusto; Terrier, Benjamin

    2017-10-01

    Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria. Eighteen patients were included (median age 55.5years, 13 men). AAV and IgG4-RD were diagnosed concomitantly in 13/18 (72%) patients; AAV preceded IgG4-RD in 3/18 (17%) while IgG4-RD preceded AAV in 2/18 (11%). AAV diagnoses included granulomatosis with polyangiitis in 14 (78%), microscopic polyangiitis in 3 (17%), and eosinophilic granulomatosis with polyangiitis in one case. IgG4-RD diagnosis included definite IgG4-RD in 5 (28%) cases, probable IgG4-RD in 5 (28%) and possible IgG4-RD in 8 (44%). IgG4-RD manifestations were chronic periaortitis in 9/18 (50%) patients, orbital mass and tubulointerstitial nephritis in 4 (22%) cases, prevertebral fibrosis in 3 (17%), pachymeningitis and autoimmune pancreatitis in 2 (11%) cases. Patients required median number of 2 (range 0-4) lines of immunosuppressants in combination with glucocorticoids. During the follow-up (median 49,8months, range 17,25-108months), AAV manifestations relapsed in 10/18 (56%) cases and IgG4-RD lesions in 5/18 (28%). When used, mainly for relapses, rituximab showed response in all cases. AAV and IgG4-RD may overlap. Clinicians should consider that atypical manifestations during AAV could be related to IgG4-RD rather than to refractory granulomatous or vasculitic lesions. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. IgG4-Related Disease Is Not Associated with Antibody to the Phospholipase A2 Receptor

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    Arezou Khosroshahi

    2012-01-01

    Full Text Available Patients with IgG4-related disease (IgG4-RD share histopathological characteristics that are similar across affected organs. The finding of infiltration with IgG4+ plasma cells in the proper clinical and histopathological contexts connects a large number of clinical entities that were viewed previously as separate conditions. The renal involvement in IgG4-RD is usually characterized by tubulointerstitial nephritis, but membranous nephropathy has also been reported to be one of the renal complications of IgG4-RD. The recent discovery that a high proportion of patients with idiopathic membranous nephropathy (IMN have IgG4 autoantibodies to the M-type phospholipase A2 receptor (PLA2R in the circulation and glomerular immune deposits, together with the profound IgG4 hypergammaglobulinemia and occasional reports of membranous nephropathy in IgG4-RD, raised the question of a common antigen. To assess the presence of anti-PLA2R antibody in patients with IgG4-RD, we screened sera from 28 IgG4-RD patients by immunoblot. None of the patients in this cohort had detectable circulating anti-PLA2R antibodies. This study suggests that despite some clinical and serological overlaps between IgG4-RD and IMN,anti-PLA2R antibodies do not play a role in the pathogenesis of IgG4-RD. Additional studies of IgG4-RD with evidence of membranous nephropathy are important to exclude any definite relationship.

  10. IgG4-related kidney disease: MRI findings with emphasis on the usefulness of diffusion-weighted imaging

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    Kim, Bohyun; Kim, Jin Hee, E-mail: kimjhrad@amc.seoul.kr; Byun, Jae Ho; Kim, Hyoung Jung; Lee, Seung Soo; Kim, So Yeon; Lee, Moon-Gyu

    2014-07-15

    Objectives: To investigate the imaging findings of immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) on magnetic resonance imaging (MRI) including diffusion-weighted imaging (DWI) and to evaluate the usefulness of DWI in lesion detection. Methods: This retrospective cohort study included 31 patients with IgG4-KD who underwent MRI covering both kidneys. Two radiologists reviewed in consensus the MR images to determine the distribution pattern (location, laterality, and multiplicity) and the visually assessed signal intensity (hypointense, isointense or hyperintense) of the renal lesions compared to the normal renal parenchyma on each sequence. Per-patient sensitivity for detecting IgG4-KD and the number of detectable lesions were compared in T2-weighted images, DWI, and dynamic contrast-enhanced images. Results: IgG4-KD typically manifested as bilateral (83.9%), multiple (93.5%), and renal parenchymal (87.1%) nodules appearing isointense (93.5%) on T1-weighted images, hypointense (77.4%) on T2-weighted images, hyperintense (100%) on DWI (b = 1000), and hypointense (83.3%) in the arterial phase and with a progressive enhancement pattern on dynamic contrast-enhanced images. The sensitivity of DWI for detecting IgG4-KD was significantly higher than that of T2-weighted images (100% vs. 77.4%, P = 0.034). The median number of detectable lesions was significantly greater in DWI (n = 9) than in T2-weighted images (n = 2) and dynamic contrast-enhanced images (n = 5) (P ≤ 0.008). Conclusions: The characteristic MRI findings of IgG4-KD were bilateral, multiple, renal parenchymal nodules with T2 hypointensity, diffusion restriction, and a progressive enhancement pattern. As DWI was useful in the detection of IgG4-KD, adding DWI to conventional MRI for patients suspected of having IgG4-KD may enhance the diagnosis.

  11. Detection of serum IgG4 levels in patients with IgG4-related disease and other disorders.

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    Yuying Su

    Full Text Available Elevated serum IgG4 levels are an important hallmark for diagnosing IgG4-related disease (IgG4-RD, but can also be observed in other diseases. This study aimed to compare two different testing methods for IgG4: ELISA and nephelometric assay. Both assays were used to measure serum IgG4 concentrations, and to assess the prevalence of high serum IgG4 levels in both IgG4-RD and non-IgG4-RD diseases.A total of 80 serum samples were tested using the nephelometric assay and ELISA method that we established. Serum IgG4 concentrations were determined by ELISA for 957 patients with distinct diseases, including 12 cases of IgG4-RD and 945 cases of non-IgG4-RD.IgG4 levels from 80 selected serum samples examined by ELISA were in agreement with those detected using the nephelometry assay. Meanwhile, the serum IgG4 concentrations measured by ELISA were also consistent with the clinical diagnoses of patients with IgG4-RD during the course of disease. The Elevated levels of serum IgG4 (>1.35 g/L were detected in all IgG4-RD (12/12 patients, and the prevalence of high IgG4 serum levels was 3.39% in non-IgG4-RD cases. Among them, the positive rates of serum IgG4 were 2.06% in patients with carcinoma and 6.3% in patients with other non-IgG4 autoimmune diseases.Our established ELISA method is a reliable and convenient technique, which could be extensively used in the clinic to measure serum IgG4 levels. High levels of IgG4 were observed in IgG4-RD. However, this phenomenon could also be observed in other diseases, such as carcinomas and other autoimmune diseases. Thus, a diagnosis of IgG4 disease cannot only be dependent on the detection of elevated serum IgG4 levels.

  12. Progress in diagnosis and treatment of IgG4-related hepatobiliary diseases%lgC4相关肝胆疾病诊疗进展

    Institute of Scientific and Technical Information of China (English)

    李萍; 李永哲

    2014-01-01

    IgG4相关性疾病是一类以淋巴浆细胞性炎症为主,伴血清和组织中IgG4增多并累及多器官或组织的慢性进行性全身性炎性疾病,累及肝胆胰腺系统时称之为IgG4相关肝胆疾病。简述了IgG4相关自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关自身免疫性肝炎的临床表现和实验室检查等方面。准确识别IgG4相关肝胆疾病的各个实验室结果,并正确诊断此类疾病,有助于避免不必要的手术和错误的治疗。%IgG4-related diseases are a class of chronic,progressive,systemic inflammatory disorders that are characterized by lymphoplas-macytic inflammation,as well as elevated IgG4 levels in serum and tissue,and may involve multiple organs or tissues.When involving the pancreas,liver,and biliary tracts,they are called IgG4-related hepatobiliary diseases.IgG4-related autoimmune pancreatitis,IgG4-re-lated sclerosing cholangitis,and IgG4-associated autoimmune hepatitis are reviewed in terms of their clinical manifestations and laboratory findings.Accurate identification of the laboratory results for IgG4-related hepatobiliary diseases and correct diagnosis of these diseases help to avoid unnecessary surgery and wrong treatment.

  13. Characterizing IgG4-related disease with {sup 18}F-FDG PET/CT: a prospective cohort study

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    Zhang, Jingjing; Ma, Yanru; Niu, Na; Wang, Xinwei; Li, Fang; Zhu, Zhaohui [Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Nuclear Medicine, Peking Union Medical College Hospital, Beijing (China); Chen, Hua; Lin, Wei; Zhang, Fengchun; Zhang, Wen [Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Rheumatology, Peking Union Medical College Hospital, Beijing (China); Xiao, Yu; Liang, Zhiyong [Chinese Academy of Medical Sciences and Peking Union Medical College, Department of Pathology, Peking Union Medical College Hospital, Beijing (China)

    2014-08-15

    IgG4-related disease (IgG4-RD) is an increasingly recognized clinicopathological disorder with immune-mediated inflammatory lesions mimicking malignancies. A cohort study was prospectively designed to investigate the value of {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in characterizing IgG4-RD. Thirty-five patients diagnosed with IgG4-RD according to the consensus criteria were enrolled with informed consent. All patients underwent baseline {sup 18}F-FDG PET/CT evaluation. Among them, 29 patients underwent a second {sup 18}F-FDG PET/CT scan after 2 to 4 weeks of steroid-based therapy. All 35 patients were found with {sup 18}F-FDG-avid hypermetabolic lesion(s); 97.1 % (34/35) of these patients showed multi-organ involvement. Among the 35 patients, 71.4 % (25/35) patients were found with more organ involvement on {sup 18}F-FDG PET/CT than conventional evaluations including physical examination, ultrasonography, and computed tomography (CT). {sup 18}F-FDG PET/CT demonstrated specific image characteristics and pattern of IgG4-RD, including diffusely elevated {sup 18}F-FDG uptake in the pancreas and salivary glands, patchy lesions in the retroperitoneal region and vascular wall, and multi-organ involvement that cannot be interpreted as metastasis. Comprehensive understanding of all involvement aided the biopsy-site selection in seven patients and the recanalization of ureteral obstruction in five patients. After 2 to 4 weeks of steroid-based therapy at 40 mg to 50 mg prednisone per day, 72.4 % (21/29) of the patients showed complete remission, whereas the others exhibited > 81.8 % decrease in {sup 18}F-FDG uptake. F-FDG PET/CT is a useful tool for assessing organ involvement, monitoring therapeutic response, and guiding interventional treatment of IgG4-RD. The image pattern is suggested to be updated into the consensus diagnostic criteria for IgG4-RD. (orig.)

  14. ¹⁸F-FDG PET-CT usefulness in extra-pancreatic involvement in IgG4 related diseases.

    Science.gov (United States)

    Cárdenas-Perilla, R; Monturiol-Duran, J; Simó-Perdigó, M; Barios-Profitós, M; Castell-Conesa, J

    2014-01-01

    IgG4-related diseases are a group of recently identified entities that include disorders that were previously known by other names, such as Mikulicz disease, Küttner's tumor, Riedel thyroiditis, among others, as well as some new ones described in the last years. These pathologies are a challenge for the medical community in terms of diagnosis and characterization due to their wide spectrum of clinical presentation. Functional imaging can provide a new approach to the comprehension of physiopathology, staging and targeting site of biopsy of IgG4-related diseases. In this clinical note, we describe five patients who underwent ¹⁸F-FDG PET-CT and correlate their findings with previous reports. Copyright © 2013 Elsevier España, S.L.U. and SEMNIM. All rights reserved.

  15. Graves' Disease Patients with Persistent Hyperthyroidism and Diffuse Lymphoplasmacytic Infiltration in the Thyroid Show No Histopathological Compatibility with IgG4-Related Disease.

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    Eijun Nishihara

    Full Text Available IgG4-related disease is a novel disease entity characterized by diffuse lymphoplasmacytic infiltration rich in IgG4-positive plasma cells and fibrosis into multiple organs. There is still controversy over whether some thyroid diseases are actually IgG4-related disease. The objective of this study was to elucidate the clinicopathological features of Graves' disease with diffuse lymphoplasmacytic infiltration in the thyroid.Among 1,484 Graves' disease patients who underwent thyroidectomy, we examined their histopathological findings including the degree of lymphoplasmacytic and fibrotic infiltration and levels of IgG4-positive plasma cells in the thyroid. Their clinical pictures were defined by laboratory and ultrasonographic evaluation.A total of 11 patients (0.74% showed diffuse lymphoplasmacytic infiltration in the stroma of the thyroid gland. Meanwhile, other patients showed variable lymphoid infiltration ranging from absent to focally dense but no aggregation of plasma cells in the thyroid gland. Based on the diagnostic criteria of IgG4-related disease, 5 of the 11 subjects had specifically increased levels of IgG4-positive plasma cells in the thyroid. Fibrotic infiltration was present in only 1 patient developing hypothyroidism after anti-thyroid drug treatment for 4 years, but not in the other 10 patients with persistent hyperthyroidism. Obliterative phlebitis was not identified in any of the 11 subjects. Thyroid ultrasound examination showed 1 patient developing hypothyroidism who had diffuse hypoechogenicity, but the other hyperthyroid patients had a coarse echo texture.In our study, Graves' disease patients with persistent hyperthyroidism who had diffuse lymphoplasmacytic infiltration rich in IgG4-positive plasma cells in the thyroid showed no concomitant fibrosis or obliterative phlebitis.

  16. Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

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    Kazuichi Okazaki

    2012-01-01

    Full Text Available Recent studies suggest simultaneous or metachronous lesions in multiorgans characterized by elevated serum levels of IgG4 and abundant infiltration of IgG4-positive plasma cells with various degrees of fibrosis. Two Japanese research committees for IgG4-RD, one from fibrosclerosis (Okazaki team and the other from lymph proliferation (Umehara team supported by the “Research Program for Intractable Disease” of the Ministry of Health, Labor, and Welfare of Japan, have agreed with the unified nomenclature as “IgG4-RD” and proposed the comprehensive diagnostic criteria (CDC for IgG4-RD. Validation of the CDC demonstrated satisfactory sensitivity for the practical use of general physicians and nonspecialists but low sensitivity in the organs to be difficult in taking biopsy specimens such as type1 autoimmune pancreatitis (IgG4-related AIP, compared with IgG4-related sialadenitis/dacryoadenitis (Mikulicz's disease and IgG4-related kidney disease. Although the diagnostic criteria covering all IgG4-RD are hard to be established, combination with the CDC and organ-specific diagnostic criteria should improve sensitivity.

  17. [IgG4-related disease and clonal B-cell lymphoid proliferation: Description of two clinical cases and a review of literature].

    Science.gov (United States)

    Sokol, E V; Vasilyev, V I; Kovrigina, A M; Safonova, T N; Nasonov, E L

    2015-01-01

    IgG4-related disease (IgG4-RD) is a systemic immune-related disease that may involve the pancreas, liver, retroperitoneal space, biliary tract, salivary and lacrimal glands, eye socket, lung, and kidney. In term of pathomorphogenesis, it is a fibroinflammatory disease manifesting as a tumor-like lesion of organs, elevated serum IgG4 levels, and a morphofunctional substrate - the development of marked fibrosis and lymphoplasmacytic infiltration in the tissues with the high content of IgG4-positive plasma cells. The detection of a tumor-like nodule frequently leads to that the patients with IgG4-RD undergo major traumatic surgery for presumed cancer. At the same time, a number of investigations show the association of IgG4-RD with the development of cancer and lymphoproliferative diseases. The paper describes two clinical cases: Russia's first diagnosis of MALT lymphoma of the lacrimal gland, IgG4-positive and IgG4-RD with a rare onset with a destruction focus in the cervical vertebrae, multiple organ dysfunction, B-cell clonality in salivary gland tissue and PIgMκ secretion. It also reviews world literature on the development of lymphoproliferative diseases in the presence of IgG4-RD.

  18. Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases.

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    Yamamoto, Motohisa; Tabeya, Tetsuya; Naishiro, Yasuyoshi; Yajima, Hidetaka; Ishigami, Keisuke; Shimizu, Yui; Obara, Mikiko; Suzuki, Chisako; Yamashita, Kentaro; Yamamoto, Hiroyuki; Hayashi, Toshiaki; Sasaki, Shigeru; Sugaya, Toshiaki; Ishida, Tadao; Takano, Ken-Ichi; Himi, Tetsuo; Suzuki, Yasuo; Nishimoto, Norihiro; Honda, Saho; Takahashi, Hiroki; Imai, Kohzoh; Shinomura, Yasuhisa

    2012-06-01

    IgG4-related disease (IgG4-RD) is a novel disease entity that includes Mikulicz's disease, autoimmune pancreatitis (AIP), and many other conditions. It is characterized by elevated serum IgG4 levels and abundant IgG4-bearing plasmacyte infiltration of involved organs. We postulated that high levels of serum IgG4 would comprise a useful diagnostic tool, but little information is available about IgG4 in conditions other than IgG4-RD, including rheumatic diseases. Several reports have described cutoff values for serum IgG4 when diagnosing IgG4-RD, but these studies mostly used 135 mg/dL in AIP to differentiate from pancreatic cancer instead of rheumatic and other common diseases. There is no evidence for a cutoff serum IgG4 level of 135 mg/dL for rheumatic diseases and common diseases that are often complicated with rheumatic diseases. The aim of this work was to re-evaluate the usual cutoff serum IgG4 value in AIP (135 mg/dL) that is used to diagnose whole IgG4-RD in the setting of a rheumatic clinic by measuring serum IgG4 levels in IgG4-RD and various disorders. We therefore constructed ROC curves of serum IgG4 levels in 418 patients who attended Sapporo Medical University Hospital due to IgG4-RD and various rheumatic and common disorders. The optimal cut-off value of serum IgG4 for a diagnosis of IgG4-RD was 144 mg/dL, and the sensitivity and specificity were 95.10 and 90.76%, respectively. Levels of serum IgG4 were elevated in IgG4-RD, Churg-Strauss syndrome, multicentric Castleman's disease, eosinophilic disorders, and in some patients with rheumatoid arthritis, systemic sclerosis, chronic hepatitis, and liver cirrhosis. The usual cut-off value of 135 mg/dL in AIP is useful for diagnosing whole IgG4-RD, but high levels of serum IgG4 are sometimes observed in not only IgG4-RD but also other rheumatic and common diseases.

  19. The research progress of IgG4-related kidney disease%IgG4相关性肾病研究进展

    Institute of Scientific and Technical Information of China (English)

    李正东; 杜晓刚

    2014-01-01

    IgG4-related kidney diseases is an autoimmune disease that was found in recent years, which can present with acute or chronic renal insufficiency and proteinuria and high level of serum IgG4. Iconography examination always shows a renal organic lesions. Tubulointerstitial nephritis with dense infiltration of IgG4-positive plasma cells is the classic pathological feature. This disease alway responds to steroid therapy with a good effect. This review will focus on the progress of clinical diagnose and treatment for IgG4-related kidney diseases.%IgG4相关性肾病(IgG4-RKD)是近年来发现的一种自身免疫性疾病,多表现为急性或慢性肾功能不全、蛋白尿,血清IgG4升高,影像学上可见肾实质损害,组织病理表现为小管间质性肾炎,以间质大量 IgG4阳性浆细胞浸润为主要特征,对糖皮质激素治疗反应较好。本文将对IgG4-RKD的临床诊治进展作一综述。

  20. Probable IgG4-related sclerosing disease presenting as a gastric submucosal tumor with an intense tracer uptake on PET/CT: a case report.

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    Otsuka, Ryota; Kano, Masayuki; Hayashi, Hideki; Hanari, Naoyuki; Gunji, Hisashi; Hayano, Koichi; Matsubara, Hisahiro

    2016-12-01

    A 44-year-old man consulted an internist because of abnormalities in an upper gastrointestinal series. It showed an elevated lesion with central depression in the greater curvature of the middle part of the stomach. Upper gastrointestinal endoscopy showed an elevated lesion with central depression, bridging hold, and no abnormalities of the gastric mucosa in the greater curvature of the middle part of the stomach. Endoscopic ultrasonography showed a submucosal tumor derived from the muscle layer of the stomach. Computed tomography showed a 22-mm tumor in the upper part of the stomach. Integrated position emission tomography/computed tomography (PET/CT) showed an intense tracer uptake by the tumor. Based on these findings, a gastrointestinal stromal tumor was suspected and laparoscopic endoscopic cooperative surgery was performed. A histopathological examination showed lymphoplasmacytic infiltration and fibrosis, and an immunohistochemical analysis showed the infiltration of IgG4-positive lymphoplasmacytic cells. The probable diagnosis was IgG4-related sclerosing disease of the stomach. We herein describe a rare case of probable IgG4-related sclerosing disease which presented as a gastric submucosal tumor. PET/CT is a useful imaging technique for the diagnosis and follow-up of this disease.

  1. Identification of relapse predictors in IgG4-related disease using multivariate analysis of clinical data at the first visit and initial treatment.

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    Yamamoto, Motohisa; Nojima, Masanori; Takahashi, Hiroki; Yokoyama, Yoshihiro; Ishigami, Keisuke; Yajima, Hidetaka; Shimizu, Yui; Tabeya, Tetsuya; Matsui, Mikiko; Suzuki, Chisako; Naishiro, Yasuyoshi; Takano, Ken-ichi; Himi, Tetsuo; Imai, Kohzoh; Shinomura, Yasuhisa

    2015-01-01

    Inducting clinical remission by glucocorticoid treatment is relatively easy in IgG4-related disease (IgG4-RD), but relapse also occurs easily with tapering of the steroid dose. The present study tried to analyse the cases to extract predictors of relapse present at the diagnosis of IgG4-RD. Subjects comprised 79 patients with IgG4-related dacryoadenitis and sialadenitis, known as Mikulicz's disease, who were diagnosed between April 1997 and October 2013 and followed-up for >2 years from the initial induction treatment. They were applied to Cox proportional hazard modelling, based on the outcome of interval to relapse. We performed multivariate analysis for the clinical factors of these cases and identified predictors of relapse. Identified factors were male sex and younger onset in cases without organ involvement at diagnosis and low levels of serum IgG4 in cases with organ dysfunction at diagnosis. Complication with autoimmune pancreatitis and low steroid dose at initial treatment also tended to be associated with recurrence. Follow-up is important in cases with recognized risk factors for relapse, including male sex and younger onset in cases without organ damage. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  2. IgG4-related pleuritis with chylothorax.

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    Kato, Eisuke; Takayanagi, Noboru; Ishiguro, Takashi; Kagiyama, Naho; Shimizu, Yoshihiko; Sugita, Yutaka

    2014-01-01

    Presently, 6 cases of IgG4-related pleuritis have been reported. We encountered a patient who developed chylothorax due to IgG4-related disease. To our knowledge, such patients have not been reported. This patient developed right-sided chylothorax and left-sided non-chylothorax lymphocyte-predominant pleuritis. Elevated serum and pleural IgG4 concentrations and histopathological analysis of pleural biopsy confirmed the diagnosis of IgG4-related pleuritis. Left-sided pleuritis improved with corticosteroid therapy, but right-sided chylothorax persists. IgG4-related disease can be one cause of chylothorax.

  3. IgG4 Related Sclerosing Cholangitis

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    D. Joshi

    2014-01-01

    Full Text Available IgG4 related disease (IgG4-RD is a multisystemic disorder which has only recently been recognized. IgG4 related sclerosing cholangitis (IgG4-SC is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC that need to be considered, namely, primary sclerosing cholangitis (PSC and cholangiocarcinoma, and provide a management algorithm. Finally, we highlight future directions and unanswered questions which will provide new insights into this exciting and evolving disease entity.

  4. Increased IgG4 responses to multiple food and animal antigens indicate a polyclonal expansion and differentiation of pre-existing B cells in IgG4-related disease

    OpenAIRE

    Culver, EL; Vermeulen, E.; Makuch, M; Leeuwen, A. van; Sadler, R.; Cargill, T; Klenerman, P; Aalberse, RC; van Ham, SM; Barnes, E; Rispens, T

    2015-01-01

    Background: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition, characterised by an elevated serum IgG4 concentration and abundant IgG4-positive plasma cells in the involved organs. An important question is whether the elevated IgG4 response is causal or a reflection of immune-regulatory mechanisms of the disease. Objectives: To investigate if the IgG4 response in IgG4-RD represents a generalised polyclonal amplification by examining the response to common environmental ...

  5. Clinical study on IgG4-related autoimmune pancreatitis

    Institute of Scientific and Technical Information of China (English)

    吴庆军

    2013-01-01

    Objective To investigate the clinical features of IgG4-related autoimmune pancreatitis(IgG4-related AIP). Methods A prospective cohort study on IgG4 related disease(IgG4-RD) was carried out in Peking Union Medical College Hospital during December 2010 to June

  6. Decreased Expression of Innate Immunity-Related Genes in Peripheral Blood Mononuclear Cells from Patients with IgG4-Related Disease.

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    Akio Nakajima

    Full Text Available IgG4-related disease (IgG4-RD is a new clinical entity of unknown etiology characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. Although aberrancies in acquired immune system functions, including increases in Th2 and Treg cytokines observed in patients with IgG4-RD, its true etiology remains unclear. To investigate the pathogenesis of IgG4-RD, this study compared the expression of genes related to innate immunity in patients with IgG4-RD and healthy controls.Peripheral blood mononuclear cells (PBMCs were obtained from patients with IgG4-RD before and after steroid therapy and from healthy controls. Total RNA was extracted and DNA microarray analysis was performed in two IgG4-RD patients to screen for genes showing changes in expression. Candidate genes were validated by real-time RT-PCR in 27 patients with IgG4-RD and 13 healthy controls.DNA microarray analysis identified 21 genes that showed a greater than 3-fold difference in expression between IgG4-RD patients and healthy controls and 30 genes that showed a greater than 3-fold change in IgG4-RD patients following steroid therapy. Candidate genes related to innate immunity, including those encoding Charcot-Leyden crystal protein (CLC, membrane-spanning 4-domain subfamily A member 3 (MS4A3, defensin alpha (DEFA 3 and 4, and interleukin-8 receptors (IL8R, were validated by real-time RT-PCR. Expression of all genes was significantly lower in IgG4-RD patients than in healthy controls. Steroid therapy significantly increased the expression of DEFA3, DEFA4 and MS4A3, but had no effect on the expression of CLC, IL8RA and IL8RB.The expression of genes related to allergy or innate immunity, including CLC, MS4A3, DEFA3, DEFA4, IL8RA and IL8RB, was lower in PBMCs from patients with IgG4-RD than from healthy controls. Although there is the limitation in the number of patients applied in DNA microarray, impaired expression of genes related to innate immunity may be

  7. Assessment of pathologically diagnosed patients with Castleman's disease associated with diffuse parenchymal lung involvement using the diagnostic criteria for IgG4-related disease.

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    Ogoshi, Takaaki; Kido, Takashi; Yatera, Kazuhiro; Oda, Keishi; Kawanami, Toshinori; Ishimoto, Hiroshi; Sakamoto, Noriho; Sano, Arisa; Yoshii, Chiharu; Shimajiri, Shohei; Mukae, Hiroshi

    2013-12-01

    IgG4-related disease (IgG4RD) is a recently recognized disease entity. Differentiating IgG4RD from plasma cell type Castleman's disease (PCD) is important but also difficult using only pathological findings. In addition, little is known about the association between these two diseases with diffuse parenchymal lung involvement. We analyzed the serum IgG4 levels and the ratio of IgG4/IgG-positive plasmacytes in the lung and lymph node specimens of eight patients previously pathologically diagnosed of PCD with diffuse parenchymal lung involvement (DL-PCD). We also compared the clinical and laboratory findings observed in these patients. Six of the eight patients exhibited abundant IgG4-positive plasmacytes in the lung and lymph node tissues and elevated serum IgG4 levels, thereby fulfilling the diagnostic criteria of IgG4RD with DL (DL-IgG4RD) in addition to having obstructive phlebitis and massive lymphoplasmacytic infiltration with fibrosis. However, three of these six patients exhibited higher levels of serum interleukin-6 and were still diagnosed with DL-PCD. Accordingly, three of these eight patients were considered as IgG4RD with DL (DL-IgG4RD), and the other five patients were ultimately given a diagnosis of DL-PCD. These two diseases have different characteristics in terms of age, symptoms, serum levels of C-reactive protein, and IgA, complicating allergic disorders, response to corticosteroids, and prognosis. This is the first report to show a high prevalence of DL-IgG4RD in DL-PCD patients, although additional large investigations are necessary. Clinical and laboratory findings are important for distinguishing between these two diseases in other organs, as previously described.

  8. Immunohistochemical analysis of IgA expression differentiates IgG4-related disease from plasma cell-type Castleman disease.

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    Manabe, Akihiro; Igawa, Takuro; Takeuchi, Mai; Gion, Yuka; Yoshino, Tadashi; Sato, Yasuharu

    2017-03-01

    Plasma cell-type Castleman disease (PCD) is often encountered when differentiating IgG4-related disease (IgG4-RD). Given that serum IgA is often elevated in Castleman disease, we investigated whether IgA expression levels in histological specimens can be used to differentiate between the two diseases. Lymph node lesions obtained from 12 IgG4-RD and 11 PCD patients were analysed by immunohistochemistry with anti-IgG, -IgG4, and -IgA antibodies. In addition to all 12 cases of IgG4-RD, 8/11 cases (72.7 %) of PCD also met the diagnostic criteria of IgG4-RD (serum IgG4 ≥135 mg/dl and IgG4/IgG-positive cells ≥40 %). IgA-positive cells were sparsely and densely distributed in IgG4-RD and PCD cases, respectively. The median number of IgA-positive cells ± SD in all 12 cases of IgG4-RD was 31 ± 37 cells per three high-powered fields (3HPFs) (range 4-118 cells/3HPFs). In contrast, the median number of IgA-positive cells, which was significantly higher in all 11 cases of PCD, was 303 ± 238 cells/3HPFs (range 74-737 cells/3HPFs) (P IgA immunostaining can be used for differential diagnosis of IgG4-RD.

  9. IgG4-related inflammatory pseudotumor of the renal pelvis involving renal parenchyma, mimicking malignancy.

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    Park, Ho Gyun; Kim, Kyoung Min

    2016-01-22

    IgG4-related disease is a recently recognized systemic disease characterized by storiform fibrosis with infiltration of IgG4-positive plasma cells. In rare incidences, IgG4-related renal disease can present as a solitary mass lesion at renal pelvis and can pose a diagnostic challenge since these lesions mimic malignancy. Herein, we present a rare case of IgG4-related disease presenting as inflammatory pseudotumor lesion, involving the renal pelvis and also neighboring renal parenchyma. A 75-year-old man with no history of IgG4-related disease underwent computed tomography (CT) scan for evaluation of prostatic cancer. The CT scan incidentally revealed a mass lesion located at the right renal pelvis. Radiologic findings were highly suggestive of malignancy. Therefore, the patient underwent right nephroureterectomy. Microscopically, the mass lesion showed storiform fibrosis with diffuse and intense inflammatory cell infiltration. Infiltrating cells were mainly histiocytes and plasma cells. Tubulointerstitium adjacent to the lesion also showed fibrosis with abundant plasmacytic infiltration. Immunohistochemical staining revealed the presence of IgG4-positive plasma cells in both the mass lesion and tubulointerstitium (mean of 94/HPF per field). Considering these findings, we diagnosed the mass lesion as IgG4-related inflammatory pseudotumor of the renal pelvis. In patients with renal pelvic masses, IgG4-related inflammatory pesudotumor should be considered in the differential diagnosis to avoid unnecessary surgical intervention.

  10. Analysis of clinical feature of IgG4 related disease%IgG4相关性疾病临床特征分析

    Institute of Scientific and Technical Information of China (English)

    李燕明; 王和; 方芳; 张旻; 居阳; 孙铁英

    2015-01-01

    Objective To summarize the clinical characteristics of IgG 4 related disease.Methods A retrospective analysis of clinical manifestation , radiography and pathological information of patients diagnosed with IgG4 related disease in Beijing Hospital.Results Patients recruited in this study were 7 males and 7 females at the average age of (64.3 ±10.0) years old.Equal or greater than two organs were suffered in 10 patients.In these involved organs , pancreas was the most suffered organ with 10 times, bile duct and salivary gland was suffered 5 times respectively , lung was suffered 4 times and lacrimal gland was suffered 2 times.Laboratory examination of these 14 cases showed that 5 of these cases were with increased level of plasma IgG and all of the cases were with increased level of IgG 4, 7 patients were with increased level of eosinophil count and 9 patients in 12 cases were with the increased level of IgE.All of the diagnosed patients were eusemia and 13 of them received the treatment of glucocorticoid.After usage of glucocorticoid , the clinical symptom and radiography findings were at great improvement , and the levels of IgG4 were decreased , but only two cases were down to normal level.Conclusions IgG4 related disease is a kind of disease with various manifestation and multiple organs are commonly suffered.The increase of IgG , IgE levels and eosinophil count could be considered as diagnosis clues.%目的 分析IgG4相关性疾病的临床特征.方法 回顾分析2011年12月至2015年1月北京医院确诊的14例IgG4相关性疾病患者的临床表现、影像和病理学资料.结果 14例患者中男、女各7例,年龄(64.3 ±10.0)岁.10例存在≥2个器官受累,受累器官包括胰腺10例次,胆管和涎腺各5例次,肺4例次,泪腺2例次.14例患者中5例血清IgG水平升高,14例血清IgG4水平均升高,7例血嗜酸粒细胞绝对值升高,12例检查IgE的患者中9例升高.14例患者预后均良好,其中有13例应用糖皮质激

  11. IgG4-Related Nasal Pseudotumor

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    L. K. Døsen

    2015-01-01

    Full Text Available IgG4-related disease is recognized as one form of autoimmune pancreatitis. During the last ten years, it has also been described in several other organs. We present two patients with lesions showing a histological picture of fibrosis and lymphoplasmacytic infiltrations with abundant IgG4 positive plasma cells at hitherto unreported symmetrical nasal locations. The symmetrical complex consisted of one central lesion in the anterior nasal septum and the two others in each of the lateral nasal walls. The lesions extended from the anterior part of the inferior concha into the vestibulum and caused severe nasal obstruction.

  12. Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign

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    Soussan, J. Ben; Sadik, J.C.; Savatovsky, J.; Heran, F.; Lecler, A. [Fondation Ophtalmologique Adolphe de Rothschild, Department of Radiology, Paris (France); Deschamps, R. [Fondation Ophtalmologique Adolphe de Rothschild, Department of Neurology, Paris (France); Deschamps, L. [Bichat Hospital, APHP, Department of Pathology, Paris (France); Puttermann, M. [Necker-Enfants Malades Hospital, APHP, Department of Pathology, Paris (France); Zmuda, M.; Galatoire, O. [Fondation Ophtalmologique Adolphe de Rothschild, Department of Orbitopalpebral Surgery, Paris (France); Picard, H. [Fondation Ophtalmologique Adolphe de Rothschild, Clinical Research Unit, Paris (France)

    2017-04-15

    To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD). From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section. IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal. In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients. (orig.)

  13. Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease

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    Sing Yun Chang

    2012-01-01

    Full Text Available Granulomatosis with polyangiitis (Wegener’s (GPA may mimic IgG4-related disease (IgG4-RD on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31% biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio. The IgG4+ cells and IgG4+/IgG+ ratio ranged 37–137/hpf and 44–83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal (n=4 or orbital/periorbital (n=4 sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.

  14. T Cell Polarization toward TH2/TFH2 and TH17/TFH17 in Patients with IgG4-Related Disease

    Science.gov (United States)

    Grados, Aurélie; Ebbo, Mikael; Piperoglou, Christelle; Groh, Matthieu; Regent, Alexis; Samson, Maxime; Terrier, Benjamin; Loundou, Anderson; Morel, Nathalie; Audia, Sylvain; Maurier, François; Graveleau, Julie; Hamidou, Mohamed; Forestier, Amandine; Palat, Sylvain; Bernit, Emmanuelle; Bonotte, Bernard; Farnarier, Catherine; Harlé, Jean-Robert; Costedoat-Chalumeau, Nathalie; Vély, Frédéric; Schleinitz, Nicolas

    2017-01-01

    IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder involving virtually every organ with a risk of organ dysfunction. Despite recent studies regarding B cell and T cell compartments, the disease’s pathophysiology remains poorly understood. We examined and characterized subsets of circulating lymphocytes in untreated patients with active IgG4-RD. Twenty-eight consecutive patients with biopsy-proven IgG4-RD were included in a prospective, multicentric study. Lymphocytes’ subsets were analyzed by flow cytometry, with analysis of TH1/TH2/TH17, TFH cells, and cytokine release by peripheral blood mononuclear cells. Results were compared to healthy controls and to patients with primary Sjögren’s syndrome. Patients with IgG4-RD showed an increase of circulating T regulatory, TH2, TH17, and CD4+CXCR5+PD1+ TFH cell subsets. Accordingly, increased levels of IL-10 and IL-4 were measured in IgG-RD patients. TFH increase was characterized by the specific expansion of TFH2 (CCR6−CXCR3−), and to a lesser extent of TFH17 (CCR6+CXCR3−) cells. Interestingly, CD4+CXCR5+PD1+ TFH cells normalized under treatment. IgG4-RD is characterized by a shift of circulating T cells toward a TH2/TFH2 and TH17/TFH17 polarization. This immunological imbalance might be implicated in the disease’s pathophysiology. Treatment regimens targeting such T cells warrant further evaluation. PMID:28348556

  15. Interleukin-33 produced by M2 macrophages and other immune cells contributes to Th2 immune reaction of IgG4-related disease

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    Furukawa, Sachiko; Moriyama, Masafumi; Miyake, Kensuke; Nakashima, Hitoshi; Tanaka, Akihiko; Maehara, Takashi; Iizuka-Koga, Mana; Tsuboi, Hiroto; Hayashida, Jun-Nosuke; Ishiguro, Noriko; Yamauchi, Masaki; Sumida, Takayuki; Nakamura, Seiji

    2017-01-01

    IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren’s syndrome (SS) and controls. Expression of IL-33 and its receptor (ST2) was strongly detected around ectopic germinal centers (GCs) in the SGs from patients with IgG4-RD, whereas IL-33 was expressed only in epithelial cells in patients with SS and controls. Moreover, IL-33 and CD68+/CD163+ macrophages were mainly distributed around ectopic GCs in patients with IgG4-RD. Double immunofluorescence staining showed that IL-33 expression co-localized with CD68+/CD163+ macrophages. Finally, mRNA expression levels of IL-33 showed a positive correlation to those of Th2 cytokines (IL-4 and IL-13) in patients with IgG4-RD. Our data suggest that IL-33 produced by M2 macrophages might contribute to the pathogenesis of IgG4-RD via aberrant activation of Th2 immune responses. PMID:28205524

  16. IgG4-Related Kidney Disease in a Patient With History of Breast Cancer: Findings on 18F-FDG PET/CT.

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    Krebs, Simone; Monti, Serena; Seshan, Surya; Fox, Josef; Mannelli, Lorenzo

    2016-08-01

    A 64-year-old African American woman with history of stage III breast carcinoma, 3 years after complete response to therapy, presents with progressive fatigue, increasing arthralgia, and unintentional weight loss of 15 lb in 3 months. An F-FDG PET demonstrated new diffuse FDG avidity of the renal parenchyma, new FDG-avid foci in pancreas and lungs, and new FDG-avid lymph nodes above and below the diaphragm. While a retroperitoneal lymph node biopsy was inconclusive, a kidney biopsy resulted in diagnosis of diffuse, severe, IgG4-related tubulointerstitial nephritis. Treatment with corticosteroid led to complete resolution of the symptoms and PET findings.

  17. Clinical analysis of 49 cases with IgG4-related disease%IgG4相关性疾病49例分析

    Institute of Scientific and Technical Information of China (English)

    马玲瑛; 马莉莉; 纪宗斐; 纪元; 侯英勇; 姜林娣

    2015-01-01

    Objective To investigate the clinical manifestations,radiographic presentations,histopathological features,treatment and clinical follow-up of IgG4 related disease (IgG4-RD).Methods Forty-nine cases in our hospital were enrolled from 2009 to 2012 and were followed up.Results Of the 49 patients with IgG4-RD,the male to female ratio was 2.1∶1,the mean age was (53±15) years,and the serum IgG4 ranged between 0.39 to 20.8 g/L.The most commonly affected organ was pancreas.Two or more organs were involved in 15 patients.Histopathological findings included tissue infiltration by lymphocytes,IgG+ plasma cells and IgG4+ plasma cells and diffuse fibrosis among 45 patients.The IgG4+ plasma cells were over 10/high power fields (HPF).Responses to glucocorticoid in combination with immunosuppressants were good except 3 patients suffered relapses.Conclusion IgG4-RD is an autoimmune disease affecting multiple organs,and the increased IgG4 positive plasma cells is characteristic.This disease can be effectively alleviated with prompt use of glucocorticoid.%目的 总结IgG4相关性疾病(IgG4-RD)的临床特点、影像学特征、组织病理学表现及治疗预后.方法 回顾性收集2009-2012年在我院诊治的49例患者临床资料,并进行随访.结果 49例IgG4-RD平均年龄(53±15)岁,其中男女比例为2.1∶1,血清IgG4水平在0.39~20.8 g/L之间.胰腺受累患者最多,15例患者伴有多部位受累.共有45例患者进行组织病理学检查,主要表现为淋巴浆细胞浸润及纤维化形成,IgG4+细胞在每高倍镜视野下>10个.43例患者进行糖皮质激素联合免疫抑制剂治疗,激素减量后,3例患者疾病复发.结论 IgG4-RD是多器官受累的自身免疫病,受累脏器IgG4阳性浆细胞浸润是其主要特点,及时应用糖皮质激素可有效缓解病情.

  18. Diagnostic Value of Serum IgG4 for IgG4-Related Disease: A PRISMA-compliant Systematic Review and Meta-analysis.

    Science.gov (United States)

    Hao, Mingju; Liu, Min; Fan, Gaowei; Yang, Xin; Li, Jinming

    2016-05-01

    Many studies about serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD) have been reported. However, these studies had relatively small sample sizes and the diagnostic accuracy values varied much between them.The aim of this study was to perform a meta-analysis to evaluate the diagnostic value of serum IgG4 for IgG4-RD.We conducted a search of relevant articles using MEDLINE, EMBASE, Web of Science, SCOPUS, and Cochrane Library databases published before December 2015.Studies those assessed the diagnostic accuracy of serum IgG4 for IgG4-RD and those provided the cut-off value for serum IgG4 were included.Data were synthesized using the random-effect model. Statistical analysis was performed using STATA with the MIDAS module and Meta-DiSc 1.4 software.A total of 9 case-control studies were analyzed, which included 1235 patients with IgG4-RD and 5696 overall controls. The pooled estimate, for a cut-off value ranged from 135 to 144 mg/dL, produced a sensitivity of 87.2% (95% CI, 85.2-89.0%) and a specificity of 82.6% (95% CI, 81.6-83.6%). The positive likelihood ratio (PLR), negative likelihood ratio (NLR), and diagnostic odds ratio (DOR) were 6.48 (95% CI, 3.98-10.57), 0.14 (95% CI, 0.09-0.21), and 45.15 (95% CI, 23.41-87.06), respectively. The area under the curve (AUC) of the summary receiver operating characteristic curve (SROC) was 0.94 (0.92-0.96). When a cut-off value of 2-fold the upper limit of normal was used (ranged from 270 to 280 mg/dL), the pooled sensitivity was 63% (95% CI, 60.0-66.0%), and the specificity was 94.8% (95% CI, 94.1-95.4%). The PLR, NLR, and DOR were 13.3 (95% CI, 7.39-24.0), 0.41 (95% CI, 0.29-0.58) and 33.42 (95% CI, 13.88-80.43), respectively. The AUC of the SROC was 0.92 (0.90-0.94).Only a relatively small number of studies were included, and significant heterogeneity was observed in this meta-analysis.Serum IgG4 is a modestly effective marker to diagnose IgG4-RD. Doubling the cut-off value for IgG4 could not improve the

  19. Paying attention to the research on IgG4 related orbital disease%重视IgG4相关性眼眶疾病的研究

    Institute of Scientific and Technical Information of China (English)

    马建民; 李静

    2015-01-01

    IgG4 related disease (IgG4-RD) is a systemic disease which was named and accepted in recent years.IgG4-RD is a multiorgan-involved disease, mainly including pancrease, parotid gland, bile duct, liver, etc.The study on the relationship between IgG4 and orbital disease is relatively few.In recent years, our study group aimed directly to the relationship between IgG4 and common orbital disease, including benign lymphoepithelial lesion of lacrimal gland,idiopathic orbital inflammatory pseudotumor, orbital lymphoma, etc.We found that IgG4 was associated with the pathogenesis and progression of these diseases.This article addressed systematically the pathogenesis of IgG4 related orbital disease and evaluated the values of IgG4 in diagnosis,differential diagnosis,treatment and montoring of these orbital diseases based on related literature and reseaching results from our study group,in order to deepen the recognition of IgG4 related orbital diseases in ophthalmologists.%IgG4相关性疾病是近年被国际医学界逐渐认可并接受的一种全身性疾病的统称,其可累及全身多个组织器官,常见的受累器官为胰腺、腮腺、胆管和肝脏等,但目前有关IgG4与眼眶疾病之间关系的研究较为少见.近年来我们课题组主要针对IgG4与泪腺良性淋巴上皮病变、眼眶炎性假瘤、眼眶淋巴瘤等常见眼眶疾病之间关系进行研究,发现上述疾病的发生及发展与IgG4之间存在一定联系.在结合相关文献及我们课题组研究结果的基础上,本文将系统阐述IgG4相关性眼眶疾病的发病机制以及IgG4在IgG4相关性眼眶疾病诊断、鉴别诊断、治疗和预后中的作用,以期提高眼科医师对IgG4相关性眼眶疾病的认识.

  20. Colovesical fistula caused by glucocorticoid therapy for IgG4-related intrapelvic mass

    OpenAIRE

    2015-01-01

    IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can occur in almost all systemic organs and generally responds to corticosteroid treatment. We report a rare case of an IgG4-related intrapelvic mass lesion that responded to steroid therapy but caused a fistula between the sigmoid colon and bladder. A 71-year-old man was followed after treatment for hepatocellular carcinoma. Follow-up computed tomography (CT) incidentally depicted left hydronephrosis with an...

  1. IgG4-related pleural disease diagnosed by a re-evaluation of chronic bilateral pleuritis in a patient who experienced occasional acute left bacterial pleuritis.

    Science.gov (United States)

    Yamamoto, Hiroshi; Suzuki, Toshiro; Yasuo, Masanori; Kobayashi, Orie; Tsushima, Kenji; Ito, Michiko; Urushihata, Kazuhisa; Yamazaki, Yoshitaka; Hanaoka, Masayuki; Koizumi, Tomonobu; Uehara, Takeshi; Kawakami, Satoshi; Hamano, Hideaki; Kawa, Shigeyuki; Kubo, Keishi

    2011-01-01

    A 78-year-old man with cryptogenic chronic bilateral lymphoplasmacytic pleuritis, diagnosed based on left parietal pleural biopsy specimens obtained by pleuroscopy, developed acute left bacterial pleuritis. The left pleural effusion was neutrophil dominant, however, the right pleural effusion showed lymphoplasmacytic infiltration. Laboratory examinations revealed that his serum IgG4 concentration was increased, with a higher level of IgG4 in the right pleural effusion. Re-evaluation of the previous biopsy specimens using an immunostaining method revealed numerous IgG4-positive plasma cell infiltrations with IgG4-positive/IgG-positive plasma cells at 85.4%. Accordingly, the new diagnosis of this patient was considered to be chronic bilateral IgG4-related pleuritis.

  2. 累及肾和淋巴结的IgG4相关性疾病病理特点%Pathologic features of IgG4 related diseases involving kidney and lymph node

    Institute of Scientific and Technical Information of China (English)

    何同梅; 曲利娟; 谢飞来; 郑智勇

    2013-01-01

    Objective To explore the clinical and pathological features,differential diagnosis,treatment and prognosis of IgG4-related disease.Methods One case of IgG4-related diseases involving the kidneys and lymph node was studied by light microscopy,electron microscopy and immunohistochemistry,and review of literature to explore clinical pathological features of the disease.Results In the kidney biopsy a large number of plasma cells were noted in the renal interstitial infiltration,and these plasma cells mainly were IgG4 positive cells (> 10/HPF) ; increasing giomerular capillary wall became diffusely thick,and segments of double-track formation were observed.Axillary lymph node biopsy showed lymphoid follicles scattered,some follicles was shrinking; interfollicular zone expanded significantly with a large number of plasma cells in the interfollicular region,and mixed with more small lymphocytes and a small amount of immunoblasts.Immunohistochemistry showed that most of plasma cells were IgG positive (> 50/HPF),of which > 50% of the cells were IgG4-positive.Conclusions IgG4-related disease is a chronic systemic disease entity with unknown pathogenesis,multiple organ involvement,and extensive infiltration of IgG4-positive cells.The diagnosis relies on clinical,pathological and serological examination.There is no effective treatment,but the response to steroid therapy is better,and it can recur after treatment.%目的 探讨IgG4相关性疾病的临床病理学特点、鉴别诊断、治疗及预后.方法 对1例累及肾和淋巴结的IgG4相关性疾病病理活检标本进行光镜、电镜及免疫组化染色(采用EliVision法),并结合文献探讨该病的临床病理学特点.结果 患者肾穿刺活检见肾间质中大量浆细胞浸润,主要为IgG阳性细胞,其中IgG4阳性细胞(>10个/HPF);肾小球毛细血管壁弥漫增厚,并节段双轨形成.腋下淋巴结活检显示,淋巴滤泡散在分布,部分滤泡呈萎缩状态,滤泡间区明

  3. An IgG4-related sclerosing mediastinitis in posterior mediastinum: CT findings

    Directory of Open Access Journals (Sweden)

    Xiaolong Liu

    2015-03-01

    Full Text Available Immunoglobulin G4 (IgG4-related sclerosing disease, an uncommon disease entity, is known to involve various organs. To our knowledge, few reports have been presented on IgG4-related sclerosing diseases involving the mediastinum, especially the posterior mediastinum. We present a case of IgG4-related sclerosing disease of the posterior mediastinum with imaging findings on computed tomography (CT.

  4. IgG4相关硬化性疾病30例病理特点%Pathological Features of 30 Cases IgG4 Related Sclerosing Disease

    Institute of Scientific and Technical Information of China (English)

    陈志忠; 陈小岩

    2014-01-01

    Objective To explore the IgG4 related to the pathological features of disease, to improve the recognition of clinical and pathologic doctor for this disease. Methods Collect the pathological diagnosis of IgG4 related pathological 30 cases, disease changes to the histological and immunohistochemical characteristics and so on carries on the observation and analysis, and review the relevant literature. Results 30 cases of IgG4 related mean age, 52.0 years, patients with sclerosing disease than for men and women:3:2, af ected organs including nasopharyngeal 4 cases, 5 cases of submandibular gland, sublingual gland in 1 case, 1 cases of parotid gland, tear ducts or surrounding tissue in 3 cases, 1 case of spleen, lung, 4 cases, 2 cases of liver, lymph nodes in 1 case, 1 case of abdominal wall, 1 cases of retroperitoneal, 1 cases of pelvic floor, 2 cases and left temporal maxil ary neoplasm, knee, back the mass in 1 case;Histological changes of the af ected tissue lymphocytic and plasma cell infiltrates in great quantities, part with lymphoid fol icular formation, fibrous tissue hyperplasia, some cases visible inherent gland atrophy, infringement of vascular, hyperplasia of vascular occlusive vasculitis and existence;Immunohistochemical staining showed IgG4 + 30 ~ 100 / HPF plasma cellcounts, IgG4 + / IgG ratio > 40%. Conclusion IgG4 related sclerosing disease is a rare autoimmune disease, etiology is unknown and involving multiple systems, lack of characteristic clinical manifestations, diagnosis combined with clinical manifestation, laboratory examination and histopathology of comprehensive analysis, among them, the laboratory examination, tissue morphological features and immunohistochemical expression is an important diagnostic basis.%目的探讨IgG4相关硬化性疾病的病理特征,提高临床和病理医生对本病的认识。方法收集病理诊断为IgG4相关硬化性疾病的病理30例,对其组织学改变及免疫组化特点等进

  5. Concomitant occurrence of IgG4-related pleuritis and periaortitis: a case report with review of the literature.

    Science.gov (United States)

    Ishida, Mitsuaki; Hodohara, Keiko; Furuya, Aya; Fujishiro, Aya; Okuno, Hiroko; Yoshii, Miyuki; Horinouchi, Akiko; Shirakawa, Ayaka; Harada, Ayumi; Iwai, Muneo; Yoshida, Keiko; Kagotani, Akiko; Yoshida, Takashi; Okabe, Hidetoshi

    2014-01-01

    IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Some recent reports have demonstrated that this disease can occur in the respiratory system including the pleura. Herein, we describe the first documented case of concomitant occurrence of IgG4-related pleuritis and periaortitis. A 71-year-old Japanese female with a history of essential thrombocythemia presented with persistent cough and difficulty in breathing. Computed tomography demonstrated thickening of the right parietal pleura, pericardium, and periaortic tissue and pleural and cardiac effusions. Histopathological study of the surgical biopsy specimen of the parietal pleura revealed marked fibrous thickening with lymphoplasmacytic infiltration. Phlebitis was noted, however, only a few eosinophils had infiltrated. Immunohistochemical study revealed abundant IgG4-positive plasma cell infiltration and high ratio of IgG4-/IgG-positive plasma cells (84%). Therefore, a diagnosis of IgG4-related pleuritis was made with consideration of the elevated serum IgG4 level (684 mg/dL). Recently, the spectrum of IgG4-related sclerosing disease has expanded, and this disease can occur in the pleura, pericardium, and periaortic tissue. Although histopathological analysis of the pericardium and periaortic tissue was not performed in the present case, it was suspected that thickening of the pericardium and periaortic tissue was clinically due to IgG4-related sclerosing disease. Our clinicopathological analyses of IgG4-related pleuritis and pericarditis reveal that this disease can present as dyspnea and pleural and pericardial effusion as seen in the present case, therefore, it is important to recognize that IgG4-related sclerosing disease can occur in these organs for accurate diagnosis and treatment.

  6. Report on one case of IgG4-related retroperitoneal fibrosis and literatures review for IgG4-related disease%IgG4相关性腹膜后纤维化一例及IgG4相关性疾病文献复习

    Institute of Scientific and Technical Information of China (English)

    牛强; 丁倩倩; 陈勤奋

    2013-01-01

    目的 探讨IgG4相关性腹膜后纤维化(IgG4-RRPF)和IgG4相关性疾病(IgG4-RD)的临床表现、诊断和治疗方法.方法 对1例IgG4-RRPF患者的临床资料进行回顾性分析,复习相关文献并介绍一个新的临床病种IgG4-RD.结果 患者,52岁,男性,以左下腹痛和左肾积液为表现,影像学显示左侧腰大肌内侧异常软组织影,PET-CT显示该软组织影脱氧葡萄糖代谢摄取增高,经肿块切取活检术,病理诊断符合IgG4-RD,诊断为IgG4-RRPF,予泼尼松治疗后症状缓解,肿块缩小.复习最新文献发现IgG4-RRPF即以往所称的特发性腹膜后纤维化(IRF).结论 IgG4-RRPF是IgG4-RD谱中的一种罕见类型,以腹膜后组织慢性炎症和显著纤维化为特征,常包绕输尿管或其他腹部脏器,血液学检查显示血清IgG4浓度升高(≥1.35 g/L),组织病理学检查显示明显淋巴细胞和IgG4+浆细胞浸润伴纤维化.一线治疗给予糖皮质激素.%Objective To explore the clinical manifestation,diagnosis and treatment of IgG4-related retroperitoneal fibrosis (IgG4-RRPF) and IgG4-related disease (IgG4-RD).Methods The clinical data of a case of IgG4-RRPF was analyzed retrospectively,the related literatures were reviewed,and a novel clinical entity IgG4-RD was introduced.Results The patient was a 52-year-old man with the complaint of left lower abdominal pain due to left hydronephrosis.CT and MRI revealed a soft tissue mass at the left psoas major muscle.PET-CT showed increased FDG uptake.After biopsy,the pathological diagnosis was consistent with IgG4-RD.The patient was diagnosed as IgG4-RRPF and treated with prednisone.The symptoms improved and retroperitonea] mass reduced.According to review the latest literatures,it is found that IgG4-RRPF was socalled idiopathic retroperitoneal fibrosis (IRF) formerly.Conclusion IgG4-RRPF is a rare disease in IgG4-RD spectrum,characterised by the presence of a retroperitoneal tissue,consisting of chronic inflammation and marked

  7. Analysis of clinicopathological characters for IgG4-related disease%IgG4相关性疾病临床病理学特征分析

    Institute of Scientific and Technical Information of China (English)

    钟卫祥; 孙柯; 滕晓东

    2015-01-01

    目的:探讨IgG4相关性疾病的病理学形态、免疫表型特征。方法观察12例IgG4相关性疾病的镜下特点,结合免疫组化EnVision两步法染色检测IgG、IgG4、CD138、CD34的表达,分析IgG4相关性疾病的病理学形态特征。结果 IgG4相关性疾病主要表现为组织弥漫性纤维化;伴大量淋巴细胞、浆细胞浸润,围绕血管神经分布;闭塞性静脉炎形成,免疫组化EnVision两步法染色IgG4阳性浆细胞与IgG阳性浆细胞比例>40%。结论 IgG4相关性疾病临床特点和影像学无特异性,易误诊为肿瘤,术前血清IgG4检测可作为疑似病例的首选方法。%Purpose To study the clinicopathological features and immunohistochemical characteristics of IgG4-related disease. Meth-ods The microscopic characteristics and immunohistochemical staining (EnVision) of IgG, IgG4, CD138 and CD34 have been per-formed on 12 cases of IgG4-related disease. Results IgG4-related disease were characterized by diffuse fibrosis, accompanied with in-filtrating of dense lymphocytes and plasma cells surrounding neurovascular and occlusive phlebitis. Immunohistochemical staining re-sults showed the ratio of IgG4+/IgG+ cells were over 40%. Conclusions IgG4-related disease is absent of characteristic clinical and radiographic features and is easily misdiagnosed as tumor. Preoperative serum IgG4 detection could be used as the prior examina-tion for the suspected cases.

  8. CT,MRI imaging features of IgG4-related disease in the chest and abdominal%IgG4相关性疾病胸腹部 CT、MRI 影像表现

    Institute of Scientific and Technical Information of China (English)

    梁璐; 蒋涛; 彭朋; 李敏; 于洋; 高帅

    2015-01-01

    目的:分析 IgG4相关性疾病(IgG4-RD)的影像特征,提高对 IgG4-RD 的影像诊断水平。方法回顾性分析临床诊治的11例 IgG4-RD 的患者影像资料,包括 CT 增强扫描5例,MRI 增强扫描9例,胸部高分辨 CT 7例,MRCP 4例。结果(1)胰腺受累11例:8例胰腺弥漫性肿大,2例胰头局限性肿大,1例萎缩,4例有封套征,5例胰管呈弥漫性不规则狭窄;(2)胰腺外受累包括:胆管受累6例;肺间质改变2例;双肾受累1例,腹膜后纤维化1例;腹膜后淋巴结肿大1例,胰周淋巴结肿大2例,肝门区淋巴结肿大1例,纵隔淋巴结肿大2例。结论认识 IgG4-RD 多器官受累的影像表现,有助于早期诊断。%Objective To analyse image features of IgG4-related disease and improve its diagnostic accuracy. Methods Retro-spectively review imaging data in 1 1 cases of clinical diagnosed of IgG4-related diseases,including 5 enhanced CT scannings,9 en-hanced MR scannings,7 High-Resolution CT scannings,and 4 MRCPs.Results (1)All the 1 1 patients showed pancreas damage, in which 8 had diffuse enlargement pancreas,2 enlargement of head of pancreas,1 atrophy,4 envelope syndrome,5 irregular stric-ture of pancreatic duct.(2)The imaging findings of extrapancreatic lesions included:6 IgG4-related sclerosis cholangitis,2 pulmona-ry interstitial change,1 both-kidney damage,1 retroperitoneal fibrosis,1 retroperitoneal lymph nodes enlargement,2 peripancreatic lymph nodes enlargement,1 hilar lymph nodes enlargement,2 mediastinal lymph nodes enlargement.Conclusion IgG4-related dis-ease often had multiple organ damage.Recognize the imaging features can help us make diagnosis early.

  9. [Central nervous system in IgG4-related disease: case report and literature review].

    Science.gov (United States)

    Vanegas-Garcia, A L; Calle-Lopez, Y; Zapata, C H; Alvarez-Espinal, D M; Saavedra-Gonzalez, Y A; Arango-Viana, J C

    2016-08-01

    Introduccion. La enfermedad relacionada con IgG4 es una entidad clinica multisistemica recientemente descrita y que se presenta con diferentes manifestaciones clinicas. Los organos que estan afectados con mayor frecuencia son el pancreas, la via biliar y las glandulas salivales, y es menos frecuente la afeccion del sistema nervioso central. Caso clinico. Mujer de 33 años con alteraciones cognitivas, alucinaciones, cefalea, sindrome convulsivo, sinusitis maxilar con afeccion osea y evidencia de paquimeningitis y panhipopituitarismo, con biopsia meningea que confirmo una enfermedad relacionada con IgG4, tras haberse descartado causas secundarias. Se inicio tratamiento con glucocorticoides y azatioprina, sin recaidas despues de 12 meses de seguimiento. Conclusiones. Se debe considerar el diagnostico de enfermedad relacionada con IgG4 en casos de paquimeningitis hipertrofica e hipofisitis, incluso sin que se acompañen de otras manifestaciones sistemicas, siempre que se hayan descartado otras causas mas frecuentes. El tratamiento de eleccion son los glucocorticoides, y puede ser necesario añadir otro inmunosupresor como ahorrador de esteroides y para evitar las recaidas. Se necesitan estudios prospectivos para evaluar las diferentes manifestaciones clinicas y paraclinicas y establecer los resultados del tratamiento a largo plazo.

  10. Increased IgG4 responses to multiple food and animal antigens indicate a polyclonal expansion and differentiation of pre-existing B cells in IgG4-related disease

    Science.gov (United States)

    Culver, Emma L; Vermeulen, Ellen; Makuch, Mateusz; van Leeuwen, Astrid; Sadler, Ross; Cargill, Tamsin; Klenerman, Paul; Aalberse, Rob C; van Ham, S Marieke; Barnes, Eleanor; Rispens, Theo

    2015-01-01

    Background IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition, characterised by an elevated serum IgG4 concentration and abundant IgG4-positive plasma cells in the involved organs. An important question is whether the elevated IgG4 response is causal or a reflection of immune-regulatory mechanisms of the disease. Objectives To investigate if the IgG4 response in IgG4-RD represents a generalised polyclonal amplification by examining the response to common environmental antigens. Methods Serum from 24 patients with IgG4-RD (14 treatment-naive, 10 treatment-experienced), 9 patients with primary sclerosing cholangitis and an elevated serum IgG4 (PSC-high IgG4), and 18 healthy controls were tested against egg white and yolk, milk, banana, cat, peanut, rice and wheat antigens by radioimmunoassay. Results We demonstrated an elevated polyclonal IgG4 response to multiple antigens in patients with IgG4-RD and in PSC-high IgG4, compared with healthy controls. There was a strong correlation between serum IgG4 and antigen-specific responses. Responses to antigens were higher in treatment-naive compared with treatment-experienced patients with IgG4-RD. Serum electrophoresis and immunofixation demonstrated polyclonality. Conclusions This is the first study to show enhanced levels of polyclonal IgG4 to multiple antigens in IgG4-RD. This supports that elevated IgG4 levels reflect an aberrant immunological regulation of the overall IgG4 response, but does not exclude that causality of disease could be antigen-driven. PMID:25646372

  11. IgG4检测在IgG4相关性疾病的新进展%Value of serum IgG4 in IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    荆红运; 关秀茹

    2016-01-01

    Immunoglobulin G4(IgG4)-related disease (IgG4-RD) is a new systemic and chronic autoimmune disease.It is characterized by increased serum levels of IgG 4 and infiltration of abundant IgG 4-positive cells in various organs and tissues .It is needed to distinguish IgG 4-RD and other diseases which will induce high level of serum IgG4 and infiltration of abundant IgG4-positive cells in tissues.This study described the value of serum IgG4 in IgG4-RD.%IgG4相关性疾病( IgG4-RD)是一种新的系统性、慢性自身免疫性疾病。临床表现为IgG4阳性浆细胞及淋巴细胞浸润多种器官和组织,并伴有血清IgG4水平升高。此外,一些其他疾病也会出现血清IgG4升高或组织中IgG4阳性浆细胞浸润,因此需要进行鉴别诊断。本文论述了血清IgG4对IgG4-RD诊断及鉴别诊断的价值。(中华检验医学杂志,2016,39:817-819)

  12. Histologically confirmed isolated IgG4-related hypophysitis: two case reports in young women

    OpenAIRE

    Sosa, Gabriela Alejandra; Bell, Soledad; Christiansen, Silvia Beatriz; Pietrani, Marcelo; Glerean, Mariela; Loto, Monica; Lovazzano,Soledad; Carrizo, Antonio; Ajler, Pablo; Fainstein Day, Patricia

    2014-01-01

    Summary IgG4-related hypophysitis is a recently described entity belonging to the group of IgG4-related diseases. Many other organs can also be affected, and it is more common in older men. To date, 32 cases of IgG4-related hypophysitis have been reported in the literature, 11 of which included confirmatory tissue biopsy and the majority affecting multiple organs. The aim of this report is to present two cases of biopsy-proven IgG4-related hypophysitis occurring in two young female patients w...

  13. Unusual IgG4-related hypophysitis: one case report and analysis of clinicopathological characteristics

    Directory of Open Access Journals (Sweden)

    Zhen-qi LI

    2014-10-01

    Full Text Available Background Immunoglobulin G4 (IgG4-related disease is a recently characterized autoimmune disease entity marked by elevated serum IgG4 levels and tissue infiltration by IgG4-positive plasma cells in multiple involved organs. Hypophysitis is a rare inflammatory disorder and IgG4-related sclerosing disease involving the ituitary alone is especially rare. Imaging studies may reveal a mass lesion in the sellar area or a thickening of pituitary stalk, mimicking a pituitary tumor. Due to its rarity and non-specific appearance in radiological examination, it is a diagnostic challenge for clinicians and histopathologists to differentiate solitary IgG4-related hypophysitis from other pituitary lesions. The aim of this study is to summarize the clinicopathological features of unusual IgG4-related hypophysitis and discuss the differential diagnosis of histologically similar inflammatory lesions in pituitary. Methods The clinical manifestation of a patient with solitary IgG4-related hypophysitis was presented retrospectively. Resected mass was routinely paraffin-embedded and stained with Hematoxylin and Eosin. Dako EnVision immunohistochemical staining system was used to detect the tumor antigen expressions, including vimentin (Vim, S-100 protein (S-100, pan cytokeratin (PCK, epithelial membrane antigen (EMA, CD3, CD20, CD68, CD1a, κ-light chain, λ-light chain and progestrone receptor (PR.  Results A 47-year-old male patient presented with 1-year history of mild limb weakness and hyposexuality. Laboratory examination revealed hypopituitarism with low levels of serum testosterone, cortisol, luteinizing hormone (LH and follicle stimulating hormone (FSH, although his serum IgG4 level was high. MRI of the pituitary gland revealed a mass lesion in the sellar area with T1WI mild hyperintense and homogeneous enhancement after gadolinium administration. The patient underwent a transsphenoidal mass resection of the pituitary gland. Histological examination

  14. Diagnosis and treatment advances of IgG4-related disease%IgG4相关性疾病的诊治及进展

    Institute of Scientific and Technical Information of China (English)

    高丽霞

    2016-01-01

    IgG4相关性疾病(IgG4 related disease,IgG4-RD)是一类病因不明的慢性系统性自身免疫性疾病.IgG4-RD临床谱广泛,常累及全身多部位腺体、腹膜后组织、肾脏、垂体及淋巴结.IgG4-RD的主要临床特征是受累器官肿胀、纤维化和硬化,血清IgG4水平明显升高,受累组织和器官中大量淋巴细胞和IgG4阳性的浆细胞浸润.IgG4-RD的确诊主要依赖组织病理学活检,该疾病的组织病理学特征为:大量淋巴细胞和浆细胞浸润、组织席纹状纤维化和硬化以及闭塞性静脉炎.糖皮质激素是诱导缓解的首选药物,有些患者需要联合免疫抑制剂或生物制剂.%IgG4 related disease (IgG4-RD)is a kind of chronic systemic autoimmune disease,whose etiology is unknown.The clinical spectrum of IgG4-RD is wide. The disease always involves systemic glandular organs, retroperitoneum,kidney,pituitary gland and lymph nodes.IgG4-RD is characterized by swelling and fibrosclerosing change of affected organs,elevated serum IgG4 level,a large number of lymphocytes and IgG4 positive plasma cells infiltration in involved organ and tissue.The diagnosis is confirmed by biopsy,the histopathological features include:dense lymphocytes and plasmacytes infiltration,storiform fibrosis and sclerosis,obliterative phlebitis.Glucocorticoids are the first-line agent for remission induction,some IgG4-RD patients require the use of immunosuppressive drugs or biological agents.

  15. IgG4-related Sclerosing Mesenteritis in a 7-year-old Saudi Girl

    OpenAIRE

    Hasosah, Mohammed Y.; Satti, Mohamed B.; Yousef, Yasmin A.; Alzahrani, Daifullah M.; Almutairi, Sajdi A.; Ashraf F Alsahafi; Sukkar, Ghassan A.; Alzaben, Abdullah A.

    2014-01-01

    Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently ...

  16. IgG4相关性肺疾病1例及文献复习%A review of 1 case of IgG4-related lung disease

    Institute of Scientific and Technical Information of China (English)

    李燕; 黄妹; 蔡后荣; 孙琦; 苗立云; 孟凡青; 肖永龙

    2016-01-01

    目的:提高对IgG4相关性肺疾病的临床特征、胸部影像学和病理组织学的认识。方法对1例经病理证实的IgG4相关性肺疾病的临床资料进行分析,并结合文献进行回顾总结。结果患者男,62岁,以胸腔积液起病,在外院先后行胸膜活检、胸腔镜肺活检等均未能明确诊断,后至我院行CT引导下经皮肺穿刺活检术,结果示活检组织内纤维组织增生伴淋巴细胞、浆细胞等炎症细胞浸润,免疫组化示大量浆细胞(+),IgG4阳性,浆细胞最密集计数约为40个/高倍视野,血清IgG4浓度示4.07 g/L(0.03 g/L~2 g/L)。诊断IgG4相关性肺疾病,给予糖皮质激素治疗,2个月后复查胸部CT示肺部病灶较前局部吸收、好转。结论IgG4相关性疾病是一种累及多器官、以血清IgG4水平升高、组织IgG4阳性浆细胞浸润为特点的淋巴浆细胞病。目前国内IgG4相关性肺疾病的报道很少,报道这一病例并进行文献复习有助于提高对IgG4相关性肺疾病的认识。%Objective To describe the clinical characteristics of IgG4-related lung disease. Methods A case with pathologically diagnosed as IgG4-related lung disease was reviewed. The clinical manifestation, laboratory examination results, chest computed tomography ( CT) characteristics, histopathological features, and treatment of IgG4-related lung disease were summarized through literature reviewing. Results The initial clinical manifestation of a 62-year-old man was pleural effusion. He received pleural biopsy and thoracoscopic lung biopsy successively in oth-er two hospitals where failed to get definite diagnosis. He was admitted to our department afterwards and received per-cutaneous lung puncture biopsy guided by CT. The pathological examination of the biopsy tissue found proliferation of fibrous tissue, with lymphoplasmacytic infiltration. The immunohistochemical staining of lung tissues revealed pre-dominant plasma cells. The highest number of IgG4

  17. IgG4-related cholecystitis presenting as biliary malignancy: report of three cases.

    Science.gov (United States)

    Feely, Michael M; Gonzalo, David H; Corbera, Montserrat; Hughes, Steven J; Trevino, Jose G

    2014-09-01

    An increased awareness of IgG4-related diseases has led to an escalation in the number of sites known to be involved by this fibroinflammatory disease. We report three cases of IgG4-related cholecystitis which were thought to represent biliary malignancies both clinically and radiographically. All three cases underwent surgery tailored towards presumed malignant neoplasms. Only following pathologic examination was the true nature of the disease identified. Recognition of the clinical, radiographic, and pathologic presentation of IgG4-related cholecystitis is essential for the consideration of this disease process prior to surgical management for suspected gallbladder malignancies. However, the pre-operative diagnosis remains challenging and extensive surgical intervention is often necessary given the distressing presentation of IgG4-related cholecystitis.

  18. IgG4-related lung disease: a case report and review of the literature%IgG4相关性肺疾病一例并文献复习

    Institute of Scientific and Technical Information of China (English)

    刘涌; 孙永昌; 冯瑞娥; 刘晓芳; 刘广杰; 刘红刚

    2012-01-01

    目的 分析1例IgG4相关性肺疾病的临床资料并进行文献复习,探讨该病的诊断及治疗经验.方法 分析北京同仁医院呼吸科收治的1例IgG4相关性肺疾病患者的临床表现、实验室检查结果、影像学及组织病理学资料.以“IgG4相关疾病”和“肺”为关键词,自Pubmed共计检索到IgG4相关性肺疾病65例,对呼吸系统症状、肺外器官受累情况、血清IgG4水平、影像学表现及组织标本中IgG4阳性浆细胞数量以及糖皮质激素治疗反应等进行分析.结果 患者41岁,男,体检时X线朐片发现肺部阴影,入院后查胸部CT显示以双侧中肺野中内带为主的磨玻璃影,伴有纤维条索影及蜂窝样改变.经胸腔镜肺活检病理显示肺泡间隔及支气管血管束周围大量炎症细胞浸润,主要为淋巴浆细胞,并伴有胶原纤维沉积,肺泡间隔增厚,可见闭塞性静脉炎,免疫组织化学染色可见大量IgG4阳性浆细胞.血清IgG4明显升高(3.07 g/L).诊断IgG4相关性肺疾病,给予泼尼松口服治疗,4个月后复查胸部CT示磨玻璃影基本吸收,仪残余纤维条索影及蜂窝样改变,IgG4水平降至1.99 g/L.文献检索到IgG4相关性肺疾病共65例,其中单纯肺部受累者27例,合并肺外表现者38例,其中累及胰腺者21例.36例测定血清IgG4水平,其中34例高于正常.影像学分类显示实性结节型占55.4% (36/65),肺泡间质型占26.2%(17/65),支气管血管束型占13.8% (9/65),圆形磨玻璃影型占4.6%(3/65).应用糖皮质激素治疗者23例,疗效良好,仅1例治疗失败.结论 IgG4相关性肺疾病是一少见疾病,临床易于忽视,可与其他硬化性疾病并发,也可单发,诊断有赖于血清IgG4水平测定及组织病理学检查,糖皮质激素治疗有效,预后良好.%Objective To describe the clinical features of IgG4-related lung disease.Method The clinical symptoms,laboratory tests,radiographic patterns,histopathological features and

  19. Clinical analysis of primary orbital IgG4-related disease%原发眼眶IgG4相关性疾病的临床分析

    Institute of Scientific and Technical Information of China (English)

    俞丹洋; 魏锐利; 蔡季平; 吴联群; 程金伟

    2015-01-01

    目的 总结原发眼眶IgG4相关性疾病的临床症状、体征、诊断、治疗和预后.方法 回顾2013年1月至2014年1月在上海长征医院眼科就诊为原发眼眶IgG4相关性疾病患者9例,对其临床症状、眼部体征、实验室及影像学检查、诊断及治疗进行分析.结果 该病好发于中老年人,男性多见.临床主要表现为眼球突出伴眶组织肿胀,可伴眼球运动受限.影像学检查多表现泪腺组织病变,可累及眼周边附属器组织.实验室检查结果显示血清IgG4> 1.35 g/L.术中见8例瘤体无明显包膜,1例包膜完整.术后组织病理学检查见淋巴细胞、浆细胞浸润及纤维化;IgG4浆细胞浸润,且IgG4细胞超过10个/HPF.随访8~20个月有2例复发.结论 眼眶IgG4病易发生于老年人,多侵犯泪腺及其周边组织,血清学及组织病理检查诊断可以明确.%Objective To analyze the clinical manifestations of disease, diagnosis, treatment and prognosis of primary orbital IgG4-related disease.Methods Aretrospective study of 9 cases with primary orbital IG4 related diseases, and the clinical data, including clinical symptoms and signs, laboratory and radiological examination results, and treatment were analyzed.Results The disease usually occurred in elder men.The main clinical manifestations were proptosis and eye lid swelling.Imaging often showed enlarged lacrimal gland tissue lesions, partly may involve ocular adnexal surrounding tissue.Lesions from 8 cases had no obvious capsules, but one had.Recurrences were observed in 2 cases during the 8-20 months follow-up time.Conclusions IgG4-related diseases are prone to occur in the elderly, usually involve the lacrimal gland and its surrounding tissue.Serological and histopathological examinations are necessary to make the final diagnosis.

  20. IgG4-related disease:a retrospective analysis of 20 cases%IgG4相关性疾病20例临床特征分析

    Institute of Scientific and Technical Information of China (English)

    李坤鹏; 朱剑; 赵伟; 邓小虎; 张江林; 黄烽

    2012-01-01

    目的 总结IgG4相关性疾病临床特征及预后,提高认识,做到早期诊断及合理治疗.方法 对在我院住院明确诊断的20例IgG4相关性疾病患者资料进行回顾性分析.结果 20例患者中男性14例,女性6例,平均年龄58岁,胰腺和胆管为最常见受累器官,17例患者(85%)有≥2个器官受累.所有患者血清IgG4均明显升高(>1350 mg/L).12例患者行组织病理学检查,主要表现为组织弥漫性纤维化,伴有大量淋巴细胞、浆细胞浸润,免疫组织化学可见CD38阳性浆细胞浸润,IgG4阳性浆细胞浸润,IgG4阳性浆细胞占IgG阳性浆细胞比例均>40%.20例患者中,除1例患者死于急性化脓性胆管炎及1例未治疗,其余患者病情均明显缓解.结论 IgG4相关性疾病临床症状无特异性,血清IgG4可作为疑似病例的首选检查方法,及时应用糖皮质激素可迅速缓解病情.%Objective To understand the features of immunoglobulin G4-related diseases and to improve its diagnosis and treatment.Methods The clinical data of 20 cases discharged from our hospital were analyzed retrospectively.Results Twenty cases with IgG4 related diseases were reviewed retrospectively.IgG4-related diseasesis more prevalent in male than in female (14∶6),and the mean age was 58 years.Pancreas and bile ducts were the most commonly affected organs,2 and more than 2 organs were involved in 17 patients.All patients had significantly elevated serum immunoglobulin G4.Histopathological findings included diffuse tissue fibrosis with extensive lymphoplasmacytes infiltration.On immunohistochemical staining,the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was higher than 40%.One patient deceased and one patient was left untreated,other patients responded well to the treatment.Conclusion There is no characteristic clinical symptoms for IgG4-related diseases,and serum immunoglobulin G4 is the essential diagnostic method in suspected cases.IgG4-related

  1. IgG4-Related Tubulointerstitial Nephritis Pattern in 18F-FDG PET/CT.

    Science.gov (United States)

    Bélissant, Ophélie; Guernou, Mohamed; Rouvier, Philippe; Compain, Caroline; Bonardel, Gérald

    2015-10-01

    A 17-year-old adolescent girl was admitted with chronic arthralgia, Raynaud phenomenon, pericarditis, and evidences of chronic diffuse inflammation. F-FDG PET/CT scan was performed to search systemic vasculitis and showed diffuse moderate uptake in the kidneys. We suggested the existence of a nephritis, but the ultrasonography result was normal, and no treatment was introduced. Another F-FDG PET/CT scan was performed 7 months later to explore abdominal pain. It showed again diffuse intense uptake in both kidneys. A proteinuria was highlighted, and renal biopsy allowed to diagnose IgG4-related disease.

  2. Colovesical fistula caused by glucocorticoid therapy for IgG4-related intrapelvic mass.

    Science.gov (United States)

    Yabuuchi, Yohei; Matsubayashi, Hiroyuki; Matsuzaki, Masato; Shiomi, Akio; Moriguchi, Michihisa; Kawamura, Ichiro; Ito, Ichiro; Ono, Hiroyuki

    2015-12-16

    IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can occur in almost all systemic organs and generally responds to corticosteroid treatment. We report a rare case of an IgG4-related intrapelvic mass lesion that responded to steroid therapy but caused a fistula between the sigmoid colon and bladder. A 71-year-old man was followed after treatment for hepatocellular carcinoma. Follow-up computed tomography (CT) incidentally depicted left hydronephrosis with an ill-demarcated intrapelvic mass lesion. This lesion was histologically diagnosed as IgG4-RD by open biopsy, and peroral steroid therapy was initiated. One month after starting steroids, a colovesical fistula was detected by follow-up CT. A colostomy and urethral catheterization were emergently performed. The patient recovered and the mass lesion was drastically minimized by the initiation of glucocorticoids; however, he still needs urethral catheterization. IgG4-RD develops in various systemic organs and generally responds well to steroids. Clinicians must be watchful for the complications of responses to corticosteroids, such as fistulization, when the mass lesion of IgG4-RD is adjacent to multiple luminal organs.

  3. A case of IgG4-related lymphadenopathy, pericarditis, coronary artery periarteritis and luminal stenosis.

    Science.gov (United States)

    Hourai, Ryoto; Miyamura, Masatoshi; Tasaki, Ryunosuke; Iwata, Akiko; Takeda, Yoshihiro; Morita, Hideaki; Hanaoka, Nobuharu; Tanigawa, Jun; Shibata, Kensaku; Takeshita, Atsushi; Kawano, Mitsuhiro; Sato, Yasuharu; Hirose, Yoshinobu; Ishizaka, Nobukazu

    2016-10-01

    Immunoglobulin G4 (IgG4)-related disease is an emerging new clinicopathological disorder that is characterized by elevation of serum IgG4 levels and histological findings of IgG4-positive plasmacytic infiltration. IgG4-related disease may appear synchronously or metachronously in a wide variety of organs. The current patient was found to have pericardial effusion and retroperitoneal fibrosis. He was subsequently diagnosed with coronary artery stenosis. (18)F-FDG positron emission tomography showed enhanced FDG uptake in lymph nodes as well as pericardial and peri-aortic tissue. Histopathology of the mediastinal lymph node showed the infiltration of numerous IgG4-positive cells, leading to the diagnosis of IgG4-related lymphadenopathy with pericardial and periarterial involvement.

  4. IgG4-related diseases and the common medical imaging diagnosis for IgG4-related pancreatitis%IgG4相关疾病概述及IgG4相关性胰腺炎的常见医学影像学诊断

    Institute of Scientific and Technical Information of China (English)

    王宝华

    2015-01-01

    IgG4相关疾病(IgG4-related disease,IgG4-RD)是一组2010年首次命名的系统性炎症纤维化疾病,已受到国内外学者的广泛关注,是一组原因不明的慢性进行性自身免疫性疾病.其受累器官广泛,几乎可累计全身各个器官,多器官同时或相继受累,也可只累及某一器官.影像学检查是IgG4-RD诊断与鉴别诊断的重要手段之一,为使影像医师掌握该疾病的相关知识和影像学表现,结合国内外有关文献现对其进行论述.

  5. 肺受累的IgG4相关硬化性疾病1例%A case report of IgG4-related sclerosing disease with lung involvement

    Institute of Scientific and Technical Information of China (English)

    张颖健; 赵金霞; 刘蕊; 刘湘源

    2012-01-01

    IgG4-related sclerosing disease ( IgG4-RSD) is a kind of lymphoplasmacytic disease with multi-organ involvement and is characterized by serum IgG4 elevation and tissue IgG4 positive plasma cell infiltration. Autoimmune pancreatitis, sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis and lymphadenopathy make up its main clinical manifestations. This difficult case was a middle-aged female with onset as muiltiple lymph nodes and glands enlargement, including lacrimal gland, salivary glands and pancreas. Meanwhile, repeated examinations of auto-antibodies and serum IgG4 were all negative. The patient didn' t respond well to glucocorticoid therapy, and further progressed to rare lung involvement presenting as lung nodule. This complex entity was eventually diagnosed as IgG4-RSD by the support of histopathology evidence of IgG4 immunohistochemistry stain. Though IgG4-RSD has been known for years, it is still underappreciated in China and case reports are scarce. The case report here with literature review is just to enhance the recognition of this disease regarding its pathogenesis, various clinical manifestations, diagnosis and therapy.%IgG4相关硬化性疾病(IgG4 related sclerosing disease,IgG4-RSD)是一种累及多器官、以血清IgG4升高、组织IgG4阳性浆细胞浸润为特点的淋巴浆细胞病,主要表现为自身免疫性胰腺炎、硬化性胆管炎、硬化性涎腺炎、腹膜后纤维化和淋巴结病.本例患者以泪腺、唾液腺、淋巴结肿大起病,多种自身抗体阴性,外院应用激素效果不佳,多次查血清IgG4阴性,为诊断带来困难,患者在此基础上出现以肺内结节为主要表现的肺受累更为罕见,最终通过组织活检IgG4免疫组织化学染色确诊.IgG4-RSD作为一种新的临床疾病逐渐被广泛认识,但国内报道仍较少.报道这一病例并进行相关文献复习旨在提高对IgG4-RSD多种临床表现的认识并对其诊疗及发病机制进行探讨.

  6. [An IgG4-related pancreatitis mimicking an adenocarcinoma: A case report].

    Science.gov (United States)

    Courcet, Emilie; Beltjens, Françoise; Charon-Barra, Céline; Guy, France; Orry, David; Ghiringhelli, François; Arnould, Laurent

    2015-12-01

    Type 1 auto-immune pancreatitis (type 1 AIP) is the pancreatic manifestation of IgG4-related systemic disease (IgG4-RD). This disease has recently been individualized and is characterized by elevated serum IgG4 levels and extrapancreatic lesions with common histologic characteristic: dense infiltration of lymphocytes, IgG4-positive plasma cells and storiforme fibrosis. Obliterative phlebitis is frequently detected. The pancreas is frequently involved in this disease. As approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological and histopathological features. In pseudotumoral cases, AIP can be misdiagnosed as pancreatic cancer. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection. We report here a case of a patient who underwent surgery for presumed pancreatic cancer. The final diagnosis was type 1 AIP.

  7. Clinical analysis of 36 cases of IgG4-related disease%36例IgG4相关性疾病的临床分析

    Institute of Scientific and Technical Information of China (English)

    张伟; 薛峰; 刘孟春; 李红; 时维平; 邵乐平; 刘丽秋

    2016-01-01

    目的 总结IgG4相关性疾病(IgG4-RD)的临床特点,提高认识,做到早期诊断及合理治疗.方法 对在本院住院期间通过病理及临床特征确诊的36例IgG4-RD患者的临床资料进行回顾性分析,并使用IgG4-RD应答指数(RI)进行评估.结果 36例患者中男性30例,女性6例,平均发病年龄65.1岁.36例患者均存在活动性病变,其中颌下腺、淋巴结、腹膜后组织是本组病例最常见受累器官.77.7%患者血清IgG4明显升高,44.4%患者血清补体C3明显降低.血清IgG4升高组与正常组相比,RI值更高、受累器官更多(P<0.01).血清IgG4浓度与RI明显相关(r=0.737,P<0.01).累及肾脏患者低补体血症发生率高于累及其他器官患者(P<0.01).28例患者使用糖皮质激素治疗,治疗后RI值、IgG4浓度均明显降低(P<0.05).结论 IgG4-RD RI和IgG4浓度均可作为IgG4-RD病情活动度和治疗疗效的评估指标;IgG4-RD的诊断应以病理为基础,联合临床特点综合分析.%Objective To explore the clinical characteristics of IgG4-related disease (IgG4-RD) in Chinese by detailed clinicopathological and laboratory assessments.Methods The baseline features of 36 patients with biopsy-proven disease were reviewed.The diagnosis was confirmed by pathology review according to consensus diagnostic criteria and clinicopathologic correlation.Disease activity and damage were assessed by the IgG4-RD responder index (RI).Results Thirty (83.3%) of the patients were male,while six were female,and the average age of onset was 65.1 years.All of the 36 patients had active disease,in which submandibular gland,lymph nodes,retroperitoneal tissue were the most common affected organs in this group of patients.Among 36 patients,77.7% had elevated serum IgG4 concentrations and 44.4% had hypocomplementemia.Patients with elevated serum IgG4 had a higher RI,a greater number of organs involved (P < 0.01 for all comparisons).The correlation between serum IgG4 level

  8. Three Cases of Bronchial Asthma Preceding IgG4-Related Autoimmune Pancreatitis

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    Satoru Ito

    2012-01-01

    Discussion: It is possible that atopy and increased Th2 cell activity are related to a higher coincidence of IgG4-related diseases such as type 1 autoimmune pancreatitis. Because the present cases are few in number, further studies are necessary.

  9. 眼眶IgG4相关疾病的临床病理观察%Clinical pathology observation on orbit IgG4 related disease

    Institute of Scientific and Technical Information of China (English)

    郭继华; 田艳明; 马敏丽; 刘莹; 高晓唯

    2015-01-01

    AIM:To discuss clinical pathological features of orbit IgG4 related disease ( IgG4-RD) . METHODS: The clinical pathological materials of 23 patients ( 35 eyes ) with orbit IgG4-RD were collected. They were observed in terms of histology and immunohistochemistry, and its clinical and pathologic characteristics were summarized. RESULTS:There were 23 patients (35 eyes) with orbit IgG4-RD (8 male patients, 9 eyes;15 female patients, 26 eyes), with an average age of 52. 1 year-old (from age 28 to 72). 19 patients (30 eyes) occured in lacrimal gland and 4 cases (5 eyes) in other places, and they went to hospital for lacrimal gland cyst or exophthalmos. There were 11 cases in one side and 12 cases in both sides. The disease lasted from 1mo to 10a, averaging 27mo. It recureded in one patient (1 eye) after 1mo. In general inspection: Gray nodular goiter, thin fibrous coat wrapping around the lacrimal gland could be observed. Histologic characteristics: lacrimal gland bubble and catheter group shrinked or even disappeared, substituted by lymphocyte, plasma cells and lymphoid follicle and accompanied with fibrosis. Immunohistochemical staining:IgG4 positive plasma cells of 23 cases (35 eyes) was >50/HPF, and IgG4/IgG ratio of positive plasma cells was >40%. CONCLUSION: Orbit IgG4 - RD mainly occures in lacrimal gland tissue, and expression of IgG4 can be detected through histologic characteristics and immunohistochemical staining. IgG4 - RD should be screened, prevented and treated in the early phase.%目的:探讨眼眶 IgG4相关疾病( IgG4-RD )的临床病理特点。  方法:收集整理23例35眼眼眶IgG4-RD患者的临床病理资料,对其进行组织学和免疫组织化学观察,总结其临床和病理特点。  结果:眼眶IgG4-RD患者23例35眼,其中男8例9眼,女15例26眼,年龄28~72(平均52.1)岁。19例30眼来源于泪腺,4例5眼来源于眶内其他部位。以泪腺区肿胀或眼球突出就诊。单侧11例,双侧12例。病程1 mo

  10. IgG4-related sclerosing mesenteritis in a 7-year-old Saudi Girl

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    Mohammed Y Hasosah

    2014-01-01

    Full Text Available Sclerosing mesenteritis (SM is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes.

  11. IgG4-related sclerosing mesenteritis in a 7-year-old Saudi girl.

    Science.gov (United States)

    Hasosah, Mohammed Y; Satti, Mohamed B; Yousef, Yasmin A; Alzahrani, Daifullah M; Almutairi, Sajdi A; Alsahafi, Ashraf F; Sukkar, Ghassan A; Alzaben, Abdullah A

    2014-01-01

    Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes.

  12. Localized IgG4-related Cholecystitis Mimicking Gallbladder Cancer.

    Science.gov (United States)

    Inoue, Tadahisa; Okumura, Fumihiro; Mizushima, Takashi; Nishie, Hirotada; Iwasaki, Hiroyasu; Anbe, Kaiki; Ozeki, Takanori; Kachi, Kenta; Fukusada, Shigeki; Suzuki, Yuta; Watanabe, Kazuko; Sano, Hitoshi

    2015-01-01

    We encountered a case of localized IgG4-cholecystitis mimicking gallbladder cancer with focal/segmental type1 autoimmune pancreatitis (AIP). In this case, we were unable to exclude a diagnosis of gallbladder cancer and thus performed radical cholecystectomy. Type1 AIP is often associated with gallbladder lesions, accompanied by generally diffuse, circumferential thickening of the gallbladder wall. Although localized IgG4-related cholecystitis is extremely rare, differentiating this condition from gallbladder cancer is often very difficult.

  13. 头颈部IgG4相关性疾病的临床及影像学诊断%Clinical and imaging diagnosis of IgG4-related disease in the head and neck

    Institute of Scientific and Technical Information of China (English)

    余长亮; 刘斌; 余永强

    2013-01-01

    头颈部IgG4相关性疾病是新认识的一种多器官系统病变,临床上以血清IgG4升高、广泛的IgG4阳性淋巴浆细胞组织浸润、组织纤维化及皮质类固醇激素治疗有效为特征.在CT和MRI上也可呈现一些相对特征的表现,CT和MRI不仅能为头颈部IgG4相关性疾病的诊断及鉴别诊断提供有价值的信息,而且能为临床治疗方案的选择及其疗效、预后的评估提供帮助.%IgG4-related disease in the head and neck is a newly recognized multi-organ system disease characterized by elevated serum IgG4,infiltration of numerous IgG4-positive plasma cells,tissue fibrosis,and dramatic response to corticosteroid treatment.IgG4-related disease of the head and neck has some relative characteristics on CT and MRI,which can provide valuable information for the diagnosis and differential diagnosis,and are helpful for the clinical treatment,evaluation of therapeutic effects and prediction of prognosis.

  14. Treatment of IgG4-related disease with assistant of corticosteroid:one case report%肾上腺皮质激素助减剂治疗 IgG4相关性疾病一例

    Institute of Scientific and Technical Information of China (English)

    杨彩红; 李雪; 吴恒莲; 杨岫岩

    2014-01-01

    IgG4-related disease is a newly recognized clinicopathological entity characterized by in-creased serum IgG4 and abundant IgG4-bearing plasma cell infiltration involving a variety of organs and tissues. The most commonly involved organs or tissues include the pancreas,lacrimal gland,salivary glands,retroper-itoneal space,biliary tract,kidney,thyroid and pituitary,etc,presenting with diffuse swelling induced by chronic inflammation accompanied with progressive fibrosis.In this article,we reported the diagnosis and treatment of one case of IgG4-related disease that was effectively treated with disease-modifying anti-rheumatic drugs (DMARDs),providing physicians with deep understanding and clinical evidence of IgG4-related dis-ease.%IgG4相关性疾病(IgG4-RD)是新近被认识和关注的一类疾病。该类疾病以血清IgG4水平升高以及 IgG4阳性浆细胞浸润多种器官和组织为特征,常见受累器官包括胰腺、泪腺、唾液腺、后腹膜、胆道、肾脏、甲状腺及垂体等,累及的器官或组织由于慢性炎症及纤维化进程可导致弥漫性肿大。IgG4-RD 作为一种新的临床疾病逐渐被广泛认识,但国内报道仍较少。该文报道1例IgG4-RD 患者的诊治经过,为临床医生认识、诊治该疾病提供参考。

  15. A case of progressively transformed germinal center-type IgG4-related lymphadenopathy.

    Science.gov (United States)

    Seki, Nobuhiko; Yamazaki, Norikazu; Koizumi, Jun-ichi; Takano, Ken-ichi; Abe, Ayumi; Ikeda, Tatsuru; Noguchi, Hiroko; Himi, Tetsuo

    2015-08-01

    Progressively transformed germinal centers (PTGC), a lymph node process unfamiliar to most otolaryngologists, is a morphological variant of reactive lymphofollicular hyperplasia of lymph nodes. Immunoglobulin (Ig)G4-related disease (IgG4-RD) is a newly identified condition, characterized by hyper-IgG4-γ-globulinemia and mass-forming or hypertrophic lesions associated with infiltration of IgG4(+) plasma cells in the affected organs. Recently, a case study of PTGC was reported that fulfilled the diagnostic criteria of IgG4-RD (IgG4(+) PTGC) [1]. A 68-year-old male was referred to our hospital with swelling in the left submandibular region. Palpation revealed swollen lymph nodes, the largest of which measured 5cm in diameter. (18)F-fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography identified lymphadenopathy with high (18)F-FDG uptake in the left submandibular region. We strongly suspected malignant lymphoma, and excisional biopsy of the submandibular lymph node was performed under general anesthesia. Pathological findings were consistent with IgG4(+) PTGC, and serological examination demonstrated elevated levels of IgG4. These findings were consistent with IgG4-RD. The patient did not have systemic lesions; therefore, he has not undergone corticosteroid therapy. IgG4(+) PTGC should be considered as a differential diagnosis for cervical lymphadenopathy by otolaryngologists as well as pathologists.

  16. Importance should be attached to IgG4-related autoimmune diseases%应重视IgG4相关性自身免疫性疾病

    Institute of Scientific and Technical Information of China (English)

    李坤鹏; 黄烽

    2012-01-01

    @@ IgG4相关性疾病(IgG4-related disease)是新近被重新认识的一种自身免疫性、纤维炎症性疾病,特征包括肿瘤样增生、席纹状纤维化、大量IgG4阳性淋巴细胞浆细胞浸润、血清IgG4 水平显著升高以及糖皮质激素治疗反应良好.既往认为独立的疾病都可能是IgG4相关性疾病中某一器官的表现,如米库利兹综合征、慢性硬化唾液腺炎、Riedel甲状腺炎都有可能划分至IgG4 相关性疾病谱中.

  17. Persistent Lymphadenopathy due to IgG4-Related Disease

    Science.gov (United States)

    2012-10-01

    Aggarwal, and S. McDonald-Pinkett, “A woman with rheumatoid arthritis , Sjögren’s syndrome, leg ulcer, and significant weight loss,” Arthritis Care & Research, vol. 64, no. 5, pp. 785–792, 2012. ...and an antibiotic. When initial laboratory workup was unrevealing for an obvious etiology and symptoms persisted, computed tomography (CT) of the neck...resection that initially revealed only nonspecific B cell proliferation and did not support a specific etiology (Figure 2). With lymphoma a concern, she

  18. 眼眶IgG4相关疾病的临床病理学特点及其鉴别诊断%A clinicopathological study of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular disorders

    Institute of Scientific and Technical Information of China (English)

    吴霞; 罗清礼; 李甘地

    2016-01-01

    目的 探讨眼眶IgG4相关疾病(IgG4-ROD)的临床及组织病理学特点及其与眼眶非IgG4相关疾病(Non-IgG4-ROD)的鉴别诊断.方法 回顾性系列病例研究.收集2008年11月至2014年8月四川大学华西医院病理科的43例患者的临床资料.分析其临床表现、实验室检查、影像学结果、治疗情况及组织病理学特点.根据2014年IgG4-ROD的诊断标准和相关文献报道,本组研究病例分为2组:IgG4-ROD(18例,23份眼眶标本)和Non-IgG4-ROD(25例,31份眼眶标本).男女构成、淋巴滤泡的形成、导管及血管周围的纤维化、眼眶的病变范围采用Pearson x2检验,年龄、免疫组织化学IgG4+/HPF浆细胞数、IgG+/HPF浆细胞数、IgG4+/IgG+浆细胞比值采用两样本比较的t检验,病变累及的结构组织及形态学指标嗜酸性粒细胞和淋巴细胞和浆细胞的浸润采用配对设计的符号秩和检验.结果 两组病例在以下参数中的差异具有统计学意义:IgG4-ROD组每个高倍视野IgG4+浆细胞数(>40个/HPF)(t=12.17,P<0.01),IgG+浆细胞数(>100个/HPF) (t=2.91,P<0.01),IgG4+/IgG+浆细胞比值(≥40%)(t=16.98,P<0.01),淋巴细胞和浆细胞的浸润(F=3.62,P=0.03),导管周围的纤维化(F=4.71,P=0.03),眼眶病变范围(t=1.11,P=0.01).但是在平均年龄(t=1.17,P=0.28),男女构成(t=1.09,P=0.58)的差异无统计学意义.IgG4-ROD需要与眼眶非特异性的炎性病变、淋巴瘤等鉴别.结论 眼眶IgG4相关疾病主要发生在中老年男性,双侧眼眶无痛性肿胀为眼部常见症状,病变组织特点为大量IgG4+浆细胞浸润,并可以累及眼眶多种结构组织,以泪腺为主,但未见典型的闭塞性静脉炎改变.充分结合临床表现、影像学发现、实验室检查和组织病理学改变有助于与Non-IgG4-ROD及其他疾病进行鉴别诊断.%Objective To study the clinicopathologic features of IgG4-related ocular disease and differentiated diagnosis with non-IgG4-related ocular

  19. IgG4-related intraocular inflammation masquerading as ciliary body melanoma in a young girl

    Directory of Open Access Journals (Sweden)

    Dipankar Das

    2016-01-01

    Full Text Available Immunoglobulin G4 (IgG4-related diseases affects various tissues and organs of the human body. Orbital, adnexal, and scleral inflammations were already reported in the medical literature. To the best of our knowledge, we report the first case of intraocular IgG4-associated inflammatory mass in the ciliary body mimicking as a melanoma in a 23-year-old female from Northeast India. Characteristic histopathology, immunohistochemistry in the tissue, protein chemistry, and raised serum IgG4 were supportive for the diagnosis. As this newly diagnosed disease has multi-organ affection and little is known about its pathogenesis particularly in eye and adnexa, the present case will open many challenges in clinico-pathological diagnosis and research in the future.

  20. Immunohistochemical Characteristics of IgG4-Related Tubulointerstitial Nephritis: Detailed Analysis of 20 Japanese Cases

    Directory of Open Access Journals (Sweden)

    Mitsuhiro Kawano

    2012-01-01

    Full Text Available Although tubulointerstitial nephritis with IgG4+ plasma cell (PC infiltration is a hallmark of IgG4-related kidney disease (IgG4-RKD, only a few studies are available about the minimum number of IgG4+ PC needed for diagnosis along with IgG4+/IgG+ PC ratio in the kidney. In addition, the significance of the deposition of IgG or complement as a reflection of humoral immunity involvement is still uncertain. In this study, we analyzed 20 Japanese patients with IgG4-RKD to evaluate the number of IgG4+ PCs along with IgG4+/IgG+ PC ratio and involvement of humoral immunity. The average number of IgG4+ PCs was 43.8/hpf and the average IgG4+/IgG+ or IgG4+/CD138+ ratio was 53%. IgG and C3 granular deposits on the tubular basement membrane (TBM were detected by immunofluorescence microscopy in 13% and 47% of patients, respectively. Nine patients had a variety of glomerular lesions, and 7 of them had immunoglobulin or complement deposition in the glomerulus. In conclusion, we confirmed that infiltrating IgG4+ PCs > 10/hpf and/or IgG4/IgG (CD138+ PCs > 40% was appropriate as an item of the diagnostic criteria for IgG4-RKD. A relatively high frequency of diverse glomerular lesions with immunoglobulin or complement deposits and deposits in TBM may be evidence of immune complex involvement in IgG4-related disease.

  1. Immunogenetics of IgG4-Related AIP.

    Science.gov (United States)

    Ota, Masao; Umemura, Takeji; Kawa, Shigeyuki

    2017-01-01

    Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis characterized by high serum IgG4 concentration and a variety of complicating extra-pancreatic lesions. AIP has the features of a complex disease that is caused by multifactorial genes. However, the genetic factors underlying AIP have not been elucidated conclusively. Association studies by the candidate-gene approach and genome-wide association studies (GWAS) have revealed several susceptibility genes for AIP, including HLA DRB1*04:05-DQB1*04:01, FCRL3, CTLA4, and KCNA3, albeit in small-scale analyses. Thus, GWAS of large sample sizes and multinational collaborative meta-analyses are needed to identify the precise genetic variants that are associated with AIP onset. Systems genetics approaches that integrate DNA sequencing, expression quantitative trait locus (eQTL) mapping, proteomics, and metabolomics will also be useful in clarifying the pathogenesis of AIP.

  2. Occurrence of anaplastic large cell lymphoma following IgG4-related autoimmune pancreatitis and cholecystitis and diffuse large B-cell lymphoma.

    Science.gov (United States)

    Ishida, Mitsuaki; Hodohara, Keiko; Yoshida, Keiko; Kagotani, Akiko; Iwai, Muneo; Yoshii, Miyuki; Okuno, Hiroko; Horinouchi, Akiko; Nakanishi, Ryota; Harada, Ayumi; Yoshida, Takashi; Okabe, Hidetoshi

    2013-01-01

    IgG4-related sclerosing disease is an established disease entity with characteristic clinicopathological features. Recently, the association between IgG4-related sclerosing disease and the risk of malignancies has been suggested. IgG4-related autoimmune pancreatitis with pancreatic cancer has been reported. Further, a few cases of extraocular malignant lymphoma in patients with IgG4-related sclerosing disease have also been documented. Herein, we describe the first documented case of anaplastic large cell lymphoma (ALCL) following IgG4-related autoimmune pancreatitis and cholecystitis and diffuse large B-cell lymphoma (DLBCL). A 61-year-old Japanese male, with a past history of DLBCL, was detected with swelling of the pancreas and tumorous lesions in the gallbladder. Histopathological study of the resected gallbladder specimen revealed diffuse lymphoplasmacytic infiltration with fibrosclerosis in the entire gallbladder wall. Eosinophilic infiltration and obliterative phlebitis were also noted. Immunohistochemically, many IgG4-positive plasma cells had infiltrated into the lesion, and the ratio of IgG4/IgG-positive plasma cells was 71.6%. Accordingly, a diagnosis of IgG4-related cholecystitis was made. Seven months later, he presented with a painful tumor in his left parotid gland. Histopathological study demonstrated diffuse or cohesive sheet-like proliferation of large-sized lymphoid cells with rich slightly eosinophilic cytoplasm and irregular-shaped large nuclei. These lymphoid cells were positive for CD30, CD4, and cytotoxic markers, but negative for CD3 and ALK. Therefore, a diagnosis of ALK-negative ALCL was made. It has been suggested that the incidence of malignant lymphoma may be high in patients with IgG4-related sclerosing disease, therefore, intense medical follow-up is important in patients with this disorder.

  3. IgG4-Related Hypophysitis: Case Report and Literature Review.

    Science.gov (United States)

    Decker, Lauren; Crawford, Angela M; Lorenzo, Gamaliel; Stippler, Martina; Konstantinov, Konstantin N; SantaCruz, Karen

    2016-12-01

    IgG4-related hypophysitis is a rare, inflammatory process of the pituitary that mimics more commonly seen pituitary tumors. We report a case of IgG4-related hypophysitis in a 16-year-old female with diabetes insipidus who was found to have IgG4-related hypophysitis based on tissue diagnosis. This entity has not been previously described in a pediatric patient. Recognition of certain inflammatory processes of the pituitary may lead to alternative means of diagnosis and medical management without a biopsy.

  4. Renal cell carcinoma mimicking IgG4-related pseudotumor in autoimmune pancreatitis.

    Science.gov (United States)

    Khan, Muhammad Ali; Kamal, Sehrish; Ahmad, Usman; Chowdhury, Mohammed Andaleeb; Nawras, Ali

    2014-09-28

    Autoimmune pancreatitis is classified into two distinct clinical profiles. Type 1 autoimmune pancreatitis (AIP) is considered to be a manifestation of a novel clinicopathological entity called IgG4 related sclerosing disease, diagnosed using the Mayo Clinic HISORt criteria. Extra-pancreatic manifestations can include involvement of bile ducts, salivary gland, lung nodules, thyroiditis, tubulointerstitial nephritis, renal masses, and retroperitoneal fibrosis. Type 2 autoimmune pancreatitis on the other hand is confirmed by histologically seen duct centric pancreatitis without elevation of IgG4 or involvement of other organs. In type 1 autoimmune pancreatitis, extrapancreatic manifestations like bile duct strictures, tubulointerstitial nephritis, renal nodules, retroperitoneal fibrosis respond to steroid therapy. We present a case of type 1 autoimmune pancreatitis in which the renal mass did not respond to steroid therapy and was later on found to be renal cell carcinoma. To the best of our knowledge this is only the third reported case of autoimmune pancreatitis in which the patient had renal cell carcinoma. Our case highlights the importance of close follow up of lesions that do not respond to steroid treatment which in this case proved to be renal cell cancer.

  5. An IgG4-Related Salivary Gland Disorder: A Case Series Presenting with a Different Clinical Setting

    Directory of Open Access Journals (Sweden)

    Masayuki Ishida

    2011-01-01

    Full Text Available Küttner tumor is a chronic inflammatory disease that presents with a firm swelling of the submandibular gland and often mimics a neoplasm. Recently evidence suggests that Küttner tumor may be a type of disorder characterized by IgG4-related inflammations. Herein, we report 3 cases of submandibular gland swellings with severe fibrosis, inflammation with marked lymphoplasmacytic infiltration; this pathology mimics clinical manifestation of a malignant tumor in 18-fluorodeoxyglucose positron emission tomography (FDG-PET findings.

  6. Isolated IgG4-related cholecystitis mimicking gallbladder cancer: a case report.

    Science.gov (United States)

    Shin, Sang-Wook; Kim, Yongsoo; Jeong, Woo Kyoung; Kim, Jinoo; Kim, Min Yeong; Oh, Young Ha; Pyo, Ju Yeon

    2013-01-01

    A 58-year-old man with right upper quadrant pain was referred to the radiology department. The patient underwent computed tomography and magnetic resonance imaging, which suggested the diagnosis of gallbladder cancer invading the liver. After surgical removal of the gallbladder, and the adjacent liver parenchyma was performed, the histologic diagnosis of IgG4-related cholecystitis was made.

  7. The clinical significance of serum IgG4 in the diagnosis of IgG4-related diseases%血清IgG4检测在IgG4相关性疾病诊断中的临床意义

    Institute of Scientific and Technical Information of China (English)

    李萍; 陈华; 吴庆军; 吴子燕; 张蜀澜; 张文; 张奉春; 李永哲

    2014-01-01

    Objective To evaluate the clinical applications of serum IgG4 for the diagnosis of IgG4-related disease (IgG4-RD).Methods In this retrospective study,160 adult patients with IgG4-RD who in their first time visit in Peiking Union Medical College Hospital between 2011 to 2013 were reviewed.All patients had detailed clinical reference and final clear diagnosis.Meantime we selected 126 patients with other non-IgG4-related immune disease and 125 healthy subjects as controls from Peiking Union Medical College Hospital physical checkup center,IgG4 was detected by nephelometry,takeing the first testing result for analysis.Statistical analysis was performed using SPSS13.0 software.Results Serum IgG4 levels higher than 1 350 mg/L were seen in 82.5% of the patients (132/160) with IgG4-RD and 20.6% of the patients (26/126) with other diseases respectively.The serum concentrations of IgG4 in IgG4-RD group were significantly higher than other disease group and healthy control group (x2 =110.8,158.6,P < 0.05).There was no significant difference between other disease group and the healthy control group(x2 =1.964,P =0.161 > 0.05).The optimal diagnostic cut-off values for IgG4-RD was 1575mg / L.Conclusion The best diagnostic cut valuse of serum IgG4 for Chinese IgG4-RD is different from the current international standard,and realize that to establish reference vange of Chinese for diagnosis of IgG4-RD has very important significance.%目的 评估血清IgG4检测对诊断IgG4相关性疾病(IgG4-RD)的临床意义.方法 回顾性研究2011年2月至2013年3月首次在北京协和医院就诊的成年人IgG4-RD患者160例,均有详细的临床资料和最终明确诊断,同时选取126例患者为其他非血清IgG4相关的免疫性疾病对照组,125名来自北京协和医院体检中心的健康体检者作为健康对照组.采用散射比浊法测定血清IgG4,160例患者的血清IgG4结果以首次就诊时检测结果为准.所有数据采用SPSS13.0统计学

  8. Hypermethylation of MST1 in IgG4-related autoimmune pancreatitis and rheumatoid arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Fukuhara, Takataro; Tomiyama, Takashi [Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Yasuda, Kaneki [Department of Urology and Andrology, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Ueda, Yoshihiro [Department of Molecular Genetics, Institute of Biomedical Science, and JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Ozaki, Yoshio; Son, Yonsu; Nomura, Shosaku [Department of the First Department of Internal Medicine, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Uchida, Kazushige; Okazaki, Kazuichi [Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan); Kinashi, Tatsuo, E-mail: kinashi@takii.kmu.ac.jp [Department of Molecular Genetics, Institute of Biomedical Science, and JST CREST, Kansai Medical University, 2-5-1 Shin-machi, Hirakata, Osaka 573-1010 (Japan)

    2015-08-07

    The serine/threonine kinase Mst1 plays important roles in the control of immune cell trafficking, proliferation, and differentiation. Previously, we reported that Mst1 was required for thymocyte selection and regulatory T-cell functions, thereby the prevention of autoimmunity in mice. In humans, MST1 null mutations cause T-cell immunodeficiency and hypergammaglobulinemia with autoantibody production. RASSF5C(RAPL) is an activator of MST1 and it is frequently methylated in some tumors. Herein, we investigated methylation of the promoter regions of MST1 and RASSF5C(RAPL) in leukocytes from patients with IgG4-related autoimmune pancreatitis (AIP) and rheumatoid arthritis (RA). Increased number of CpG methylation in the 5′ region of MST1 was detected in AIP patients with extrapancreatic lesions, whereas AIP patients without extrapancreatic lesions were similar to controls. In RA patients, we detected a slight increased CpG methylation in MST1, although the overall number of methylation sites was lower than that of AIP patients with extrapancreatic lesions. There were no significant changes of the methylation levels of the CpG islands in the 5′ region of RASSF5C(RAPL) in leukocytes from AIP and RA patients. Consistently, we found a significantly down-regulated expression of MST1 in regulatory T cells of AIP patients. Our results suggest that the decreased expression of MST1 in regulatory T cells due to hypermethylation of the promoter contributes to the pathogenesis of IgG4-related AIP. - Highlights: • Mst1 controls immune cells trafficking, cell proliferation and differentiation. • Autoimmune pancreatitis (AIP) is an idiopathic pancreatitis affecting multiple organs. • Decreased MST1 expression and increased CpG methylation of promoter of MST1 in AIP. • Slight increased CpG methylation of MST1 in rheumatoid arthritis patients. • MST1 contributes pathogenesis of IgG4-related AIP.

  9. Advances in the diagnosis and treatment of IgG4-related sclerosing cholangitis:a review

    Institute of Scientific and Technical Information of China (English)

    Shunda Du; Gang Liu; Yilei Mao

    2015-01-01

    Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is an IgG4-related disease characterized by bile duct fibroinflammatory wal-thickening and stenosis, resulting in obstruction jaundice, weight loss. Dif-ferent regions of the bile duct can be involved, with the distal region being the most common. IgG4-SC can also have other organ involvement, such as the pancreas, urinary tract, salivary glands and lacrimal glands. In clinical practice, the manifestation of IgG4-SC is very similar to cholangiocarcinoma (CC) and primary sclerosing cholangitis (PSC), as wel as pancreatic malignancies, while the treatment and prognosis are totaly diferent. Japanese researchers ever established the clinical diagnostic criteria in 2012: (1) charac-teristic biliary imaging findings; (2) elevated serum IgG4 concentrations; (3) the coexistence of IgG4-related diseases except those of the biliary tract; and (4) characteristic histopathological features. According to our observations, IgG4-SC can be distinguished from CC with 100% specificity only at a cutof of six times the upper normal limit. Imaging findings have low specificity for diagnosis, with the exception of intraductal ultrasonography, which can reflect the lesion with relatively high specificity. IgG4 plasma cel infiltration can be found in bile duct biopsy tissue, although this procedure is dificult. According to recent studies, the treatment of IgG4-SC relies mainly on corticosteroids. Folowing steroid treatment, most IgG4-SC patients can recover and their symptoms are resolved although a few patients relapse after steroid withdrawal. Maintenance of steroid therapy or alternative drugs is necessary in such cases. There is, however, no strong evidence for malignant transformation in IgG4-SC.

  10. [Hashimoto's thyroiditis(chronic thyroiditis), IgG4-related thyroiditis].

    Science.gov (United States)

    Itoh, Mitsuyasu

    2012-11-01

    Hashimoto's thyroiditis emerges in patients who have genetic preponderance such as SNPs of CTLA-4 and risk factors such as excess intake of iodine, pregnancy or postpartum period, and smoking. Such risk factors also affect the entire clinical course. One of the major outcomes in Hashimoto's thyroiditis appears to be increased in cardio-vascular risks through subclinical hypothyroidism and concomitant metabolic syndrome, but in most cases, treatment with L-T4 has little effects on cardio-vascular benefit or quality of life. The pregnant women also have risks for obstetric complications and postpartum thyroid dysfunction. The women who have anti-TPO antibodies, type 1 diabetes, or previous history of post-partum thyroid dysfunction are recommended to be measured their TSH. It is noteworthy that Hashimoto's thyroiditis is sometimes complicated with encephalopathy, papillary carcinoma, or IgG4-related thyroiditis. IgG4-related thyroiditis is partly similar but partly discerned from a variant of Hashimoto's thyroiditis. The pathogenetic roles of this variant on autoimmune-based thyroiditis remain unclear.

  11. Clinicopathological features of IgG4-related lymphadenopathy%IgG4相关性淋巴结病的临床病理特点

    Institute of Scientific and Technical Information of China (English)

    张卉; 冯瑞娥

    2012-01-01

    Immunoglobulin G4-related disease (lgG4-RD) is an emerging disease entity of unknown etiology with multiorgan involvement. IgG4-related lymphadenopathy may be easily confused with multicentric Castleman's disease, follicular hyperplasia, or malignant lymphoma due to its lack of special clinicopathological features. Although IgG4-related lymphadenopathy shows a dramatic response to steroid therapy, some cases may be potentially associated with the development of lymphoma. Thus it is desirable to improve our knowledge of IgG4 related lymphadenopathy. In this review, we summarize the clinicopathological characteristics of IgG4-related lymphadenopathy.%IgG4相关性疾病作为一种全身系统性疾病,可累及多个器官及组织.发生于淋巴结的IgG4相关性淋巴结病由于其临床病理特点缺乏特异性,需与多种病变鉴别,如Castleman's病样、淋巴滤泡反应性增生、淋巴瘤等.尽管IgG4相关性疾病经激素治疗效果显著,但是某些患者可发展成淋巴瘤.因此有必要提高对IgG4相关性淋巴结病的认识.本文就IgG4相关性淋巴结病的临床及病理组织学等特点进行综述.

  12. 血清IgG4水平在IgG4相关性疾病及与风湿免疫性疾病鉴别诊断中的应用价值%Value of serum IgG4 in diagnosis of IgG4-related disease and in differentiation from rheumatic diseases

    Institute of Scientific and Technical Information of China (English)

    贺琤雯; 唐古生; 鲁琼; 沈茜

    2012-01-01

    Objective To investigate the value of serum IgG4 in diagnosis of IgG4-RD and in differentiation from rheumatic diseases.Methods Total of 23 patients with IgG4-RD and 502 patients with rheumatic diseases were enrolled,who presented at Changhai Hospital in 2010 to 2011.In the study,rheumatic diseases were categorized into groups of Sj(o)gren syndrome (n =26),ankylosing spondylitis (n-50),systemic sclerosis (n =3),rhcumatoid arthritis (RA,n =125),mixed connective tissue disease (n =15),systemic lupus erythematosus (SLE,n =212),adult onset still disease (n =20),Behcet syndrome (n =17),polymyositis (n =12),dermatomyositis (n =12),polymyalgiarheumatica (n =10).Serum IgG and IgG4 levels were measured by a rate nephelometer assay.The ROC curves were constructed to identify the optimal serum IgG4 cutoff value for diagnosing IgG4-RD and evaluate its sensitivity and specificity.Results The mean levels of serum lgG4 in the group with IgG4-RD were 11.4(5.0-14.8) g/L.In about 95.6% IgG4-RD patients,the serum IgG4 level was higher than > 1.4 g/L and other rheumatic diseases (U values were 6.0,21.0,0,58.5,0,9.0,3.0,4.0,0,3.0,3.5,P <0.01).The levels of serum IgG4 with RA was 0.6(0.3-1.2) g/L,the levels of serum IgG4 with SLE was 0.2 (0.1-0.4) g/L.There were statistical differences between RA and SLE (U value was 5847,P < 0.01).At the same time,some patients with other rheumatic diseases were found serum IgG4 level higher than > 1.4 g/L,which was about 10% in the patients whith RA,ankylosing spondylitis,adult onset still disease and polymyalgiarheumatica.According to the ROC constructed the cut off value in present study was 2.2 g/L,and sensitivity and specificity were 95.7% and 97.4%,respectively.Area under the cerve (AUC) was 0.995.There were no significant differences between the sensitivity and specificity values obtained with a cutoff value of 2.2 g/L.In patients with other rheumatic diseases,the ratio of high serum IgG4 level (> 2.2 g/L) were declined

  13. Renal Cell Carcinoma Mimicking Igg4-Related Pseudotumor in Autoimmune Pancreatitis

    Directory of Open Access Journals (Sweden)

    Muhammad Ali Khan

    2014-09-01

    Full Text Available Context Autoimmune pancreatitis is classified into two distinct clinical profiles. Care report Type 1 autoimmunepancreatitis (AIP is considered to be a manifestation of a novel clinicopathological entity called IgG4 related sclerosingdisease, diagnosed using the Mayo Clinic HISORt criteria. Extra-pancreatic manifestations can include involvement of bileducts, salivary gland, lung nodules, thyroiditis, tubulointerstitial nephritis, renal masses, and retroperitoneal fibrosis. Type2 autoimmune pancreatitis on the other hand is confirmed by histologically seen duct centric pancreatitis without elevationof IgG4 or involvement of other organs. In type 1 autoimmune pancreatitis, extrapancreatic manifestations like bile ductstrictures, tubulointerstitial nephritis, renal nodules, retroperitoneal fibrosis respond to steroid therapy. Conclusion Wepresent a case of type 1 autoimmune pancreatitis in which the renal mass did not respond to steroid therapy and was later on found to be renal cell carcinoma. To the best of our knowledge this is only the third reported case of autoimmune pancreatitis in which the patient had renal cell carcinoma. Our case highlights the importance of close follow up of lesions that do not respond to steroid treatment which in this case proved to be renal cell cancer.

  14. Plasmacytoid Dendritic Cell Activation and IFN-α Production Are Prominent Features of Murine Autoimmune Pancreatitis and Human IgG4-Related Autoimmune Pancreatitis.

    Science.gov (United States)

    Arai, Yasuyuki; Yamashita, Kouhei; Kuriyama, Katsutoshi; Shiokawa, Masahiro; Kodama, Yuzo; Sakurai, Toshiharu; Mizugishi, Kiyomi; Uchida, Kazushige; Kadowaki, Norimitsu; Takaori-Kondo, Akifumi; Kudo, Masatoshi; Okazaki, Kazuichi; Strober, Warren; Chiba, Tsutomu; Watanabe, Tomohiro

    2015-10-01

    The abnormal immune response accompanying IgG4-related autoimmune pancreatitis (AIP) is presently unclear. In this study, we examined the role of plasmacytoid dendritic cell (pDC) activation and IFN-α production in this disease as well as in a murine model of AIP (MRL/Mp mice treated with polyinosinic-polycytidylic acid). We found that the development of AIP in treated MRL/Mp mice occurred in parallel with pancreatic accumulation of pDCs producing IFN-α, and with pDC depletion and IFN-α-blocking studies, we showed that such accumulation was necessary for AIP induction. In addition, we found that the pancreas of treated MRL/Mp mice contained neutrophil extracellular traps (NETs) shown previously to stimulate pDCs to produce IFN-α. Consistent with these findings, we found that patients with IgG4-related AIP also exhibited pancreatic tissue localization of IFN-α-expressing pDCs and had significantly higher serum IFN-α levels than healthy controls. In addition, the inflamed pancreas of these patients but not controls also contained NETs that were shown to be capable of pDC activation. More importantly, patient pDCs cultured in the presence of NETs produced greatly increased levels of IFN-α and induced control B cells to produce IgG4 (but not IgG1) as compared with control pDCs. These data suggest that pDC activation and production of IFN-α is a major cause of murine AIP; in addition, the increased pDC production of IFN-α and its relation to IgG4 production observed in IgG4-related AIP suggest that this mechanism also plays a role in the human disease.

  15. Clinicopathological features of IgG4-related sialadenitis%IgG4相关性涎腺炎的临床病理特点

    Institute of Scientific and Technical Information of China (English)

    葛莉

    2013-01-01

    Immunoglobubin G4-related disease (IgG4-RD) is an emerging disease with unknown etiology and multiorgan involvement.IgG4-related sialadenitis is easily to be misdiagnosed as Sj(o)gren's syndrome,chronic nonspecific sialadenitis,or lymphoma.Thus many patients cannot be treated timely and even undergo many unnecessary surgery.It is important to improve our knowledge on IgG4-related sialadenitis.In this review,we summarized the clinicopathological characteristics of IgG4-related sialadenitis.%IgG4相关性疾病作为一种全身系统性疾,可累及多个器官及组织.发生于涎腺的IgG4相关性涎腺炎容易与干燥综合征、慢性非特异性涎腺炎、淋巴瘤等多种疾病混淆,延误治疗并给患者带来诸多不必要的手术负担.因此有必要提高对IgG4相关性涎腺炎的认识.本文就IgG4相关性涎腺炎的临床及病理组织学等特点进行综述.

  16. IgG4-related disease%IgG4相关疾病

    Institute of Scientific and Technical Information of China (English)

    蒲佳曦

    2014-01-01

    IgG4相关疾病(IgG4-RD)是一种新发现的累及全身多器官损伤的少见临床疾病.IgG4-RD的发病机制尚未明确,尤其IgG4是直接介导组织损伤还是旁观效应仍不清楚.IgG4-RD表现为各种器官同时或非同时的器官肿大或结节/增生病变,相关检查主要包括血清学,病理学和影像学.IgG4-RD综合诊断标准的诊断算法是用综合诊断标准结合器官特异性标准而制定的.治疗方面虽无指南可循但仍然推荐首选激素治疗,必要时考虑运用免疫抑制剂和生物治疗.

  17. Clinical and Pathological Features of IgG4-related Sclerosing Pachymeningitis%IgG4相关性硬化性脑膜炎临床及病理特点(附8例报告)

    Institute of Scientific and Technical Information of China (English)

    袁松英; 朱德茂; 文秋元

    2011-01-01

    [目的]探讨IgG4相关性硬化性脑膜炎的临床及病理特点.[方法]对8例IgG4相关性硬化性脑膜炎患者的临床资料结合形态特点以及IgG4表达阳性细胞进行分析.[结果]8例均表现头痛,各种脑神经受累6例、癫痫3例、呕吐2例.CT及MRI显示病变部位脑膜增厚,增强明显强化.血清学IgG4增高(2/2).病理表现为淋巴-浆细胞炎症反应,纤维化和闭塞性静脉炎,病变可弥漫或局限形成局部肿块.本组病例中3例IgG4阳性(IgG4+)浆细胞数20~30个/HPF,5例高于50个/HPF,IgG4+浆细胞数占IgG+细胞总数的40%以上.[结论]IgG4相关性硬化性脑膜炎是一种与自身免疫相关的疾病,可单发或伴有其他全身性病变.诊断有赖于病理组织学检查.%[Objective] To explore the clinical and pathological features of IgG4-related sclerosing pachymenin-gitis. [Methods]The clinical data, morphology features and IgG4-positive cells in 8 cases of IgG4-related sclerosing pachymeningitis were analyzed. [Results] All 8 patients had headache. There were 6 cases of various kinds of cerebral nerve involvement, 3 cases of epilepsy and 2 cases of vomiting. CT and MRI revealed meningeal thickening and obvious enhancement in lesions. Serum IgG4 increased. Pathological manifestation was lymphoplasmacytic inflammatory response, fibrosis and obstructive phlebitis. The lesions were suffused or localized into local mass. The number of IgG4 + plasma cells in 3 cases was 20 - 30/HPF and that in 5 cases was more than 50/HPF. The number of IgG4 + plasma cells occupied more than 40% of IgG+ cells. [Conclusion] IgG4 -related sclerosing pachymeningitis is an autoimmune-associated disease, which may be single or complicated with other systemic diseases. The diagnosis of IgG4 -related sclerosing pachymeningitis depends on histopathologic examination.

  18. IgG4相关性腹膜后纤维化CT诊断%Diagnosis of IgG4-related retroperitoneal fibrosis

    Institute of Scientific and Technical Information of China (English)

    田芳; 朴雪梅; 周自明; 常时新; 丁永生

    2012-01-01

    Objective: To study and summarize the clinical features and the imaging manifestations of IgG4-related retroperitoneal fibrosis and to improve the understanding of IgG4-related sclerosing disease. Methods: A case of IgG4-related retroperitoneal fibrosis was reported,including clinical features,laboratory reports,imaging manifestations and pathological results,and relative literatures were reviewed. Results: The disease had no distinctive clinical features. Symptoms include vague pain in the lower back or abdomen,anorexia,fatigue,fever and weight loss. The imaging manifestation was soft tissue mass. The level of serum IgG (especially IgG4) was strikingly high. The histological manifestation in the involved organs showed diffuse infiltration of lymphoplasma cells and fibrosis. Abundant IgG4-positive plasma cells could be found and the treatment with glucocorticoid was effective. Conclusion:IgG4-related retroperitoneal fibrosis lacks specifity. It is essential to improve its understanding to avoid misdiagnosis.%目的:总结IgG4相关腹膜后纤维化的临床表现及影像特点,提高时IgG4相关的硬化性疾病的认识.方法:报告1例IgC4相关腹膜后纤维化的临床表现、实验室检查、影像学资料、病理结果等,并复习相关文献.结果:该病临床症状无明显特异性,可有腹痛、背痛、疲乏、食欲下降等,影像表现为腹膜后肿块,该病与自身免疫性胰腺炎关系密切,血清IgG尤其是IgG4显著升高;受累脏器组织学表现为弥漫性淋巴浆细胞漫润和纤维化,大量IgG4阳性浆细胞;糖皮质激素治疗有效.结论:IgG4相关腹膜后纤维化临床症状缺乏特异性,极易误诊或漏诊,需提高对该病的认识.

  19. A Retrospectively Diagnosed Case of IgG4-Related Tubulointerstitial Nephritis Showing Good Renal Outcome and Pathological Progress

    Directory of Open Access Journals (Sweden)

    Qiong Wu

    2013-01-01

    Full Text Available A 74-year-old man was hospitalized for diabetic nephropathy evaluation and assessment of the effect of treatment on his tubulointerstitial nephritis (TIN. When he was 62 years old, he developed polyarthralgia and had superficial lymph node swelling, mildly increased serum creatinine concentration, hypergammaglobulinemia, hypocomplementemia, high serum IL-2R level, and positive titer of antinuclear antibody. Several tissues were biopsied. Mild chronic sialadenitis and reactive lymphadenitis were identified. Renal specimen showed mild glomerular ischemia, extensive storiform fibrosis, and abundant infiltrating monocytes and plasma cells. He was treated with oral prednisolone and cyclophosphamide. After the treatment, most of his clinical parameters quickly returned to within the reference range. However, he developed diabetes mellitus soon after steroid therapy. At the time of rebiopsy, a high level of serum IgG4 was detected. The second renal biopsy showed diabetic nephropathy without any tubulointerstitial damage. The first biopsied tissues were retrospectively investigated. Large numbers of IgG4-positive plasma cells were detected in the kidneys and lymph nodes. A retrospective diagnosis of IgG4-related TIN with lymph node involvement was made. In conclusion, this paper describes a retrospectively diagnosed case of IgG4-related TIN with lymph node involvement, showing good clinical and pathological prognosis.

  20. Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations*

    Science.gov (United States)

    Fernandes, Daniel Alvarenga; Kido, Ricardo Yoshio Zanetti; Barros, Ricardo Hoelz de Oliveira; Martins, Daniel Lahan; Penachim, Thiago José; Caserta, Nelson Marcio Gomes

    2016-01-01

    We present a case of immunoglobulin G4 (IgG4)-related disease with pancreatic and extrapancreatic involvement, including the biliary and renal systems. Given the importance of imaging methods for the diagnosis of IgG4-related disease and its differentiation from pancreatic adenocarcinoma, we emphasize important abdominal computed tomography and magnetic resonance imaging findings related to this recently recognized systemic autoimmune disease. PMID:27141136

  1. Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations

    Directory of Open Access Journals (Sweden)

    Daniel Alvarenga Fernandes

    2016-04-01

    Full Text Available Abstract We present a case of immunoglobulin G4 (IgG4-related disease with pancreatic and extrapancreatic involvement, including the biliary and renal systems. Given the importance of imaging methods for the diagnosis of IgG4-related disease and its differentiation from pancreatic adenocarcinoma, we emphasize important abdominal computed tomography and magnetic resonance imaging findings related to this recently recognized systemic autoimmune disease.

  2. Tumefactive immunoglobulin G4-related disease involving the dura mater: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jeong Hoon; Lee, Ji Hoon; Ko, Yong; Paik, Seoung Sam; Lee, Young Jun [Dept. of Radiology, Hanyang University Hospital, Seoul (Korea, Republic of); Park, Dong Woo [Dept. of Radiology, Hanyang University Hospital, Seoul (Korea, Republic of)

    2015-08-15

    Immunoglobulin G4 (IgG4)-related disease is a well-known disorder characterized by an inflammatory reaction with an increase in the number of IgG4-positive plasma cells associated with sclerosis. IgG4-related disease often affects the dura mater with a pattern of diffuse thickening when the central nervous system is involved. However, some nodular dural thickening requires discrimination from tumors because of obviously different treatment options. We report of a case of IgG4-related disease with tumefactive dural involvement.

  3. IgG4-related retroperitoneal fibrosis: A case report and literature review%IgG4相关性腹膜后纤维化病例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    王聪; 赵忺; 田建立; 巩路

    2015-01-01

    Clinical data of one patient diagnosed as IgG4-related retroperitoneal fibrosis was analyzed."IgG4" and "retroperitoneal fibrosis" were used as the key words to search literatures from PubMed and Wanfang Database.Clinical data of 27 cases with IgG4 retroperitoneal fibrosis,including clinical manifestation,diagnostic basis,therapeutic method and prognosis,which were retrieved through literatures,were analyzed.A 82-year-old male patient presented with urinary tract obstruction and renal insufficiency.Imaging examination revealed a retroperitoneal irregular lump shadow,and showed chronic inflammation of soft tissues in posterior peritoneum with fibrosis by biopsy pathology.The IgG4-positive plasmocyte infiltration was found by immunohistochemical method.The serum level of IgG4 was significantly increased,which was in accord with IgG4-related retroperitoneal fibrosis.After treatment with methylprednisolone,retroperitoneal lump shadow was obviously dissipated.The serum level of IgG4 became normal.27 cases with IgG4-related retroperitoneal fibrosis were searched through literature retrieval,with 19 confirmed cases,3 suspected cases,and 5 suspicious cases.Male accounted for 92.6% (25/27),and the average age was 67.6 years old.The cases with the involvement of extraperitoneal organs accounted for 37.0% (10/27).The misdiagnosis rate was 55.6% (15/27).23 cases took effective therapy with glucocorticoids.IgG4-related retroperitoneal fibrosis is an orphan disease,which can be misdiagnosed easily.The diagnosis is made with histopathological examination and determination on the serum level of IgG4.The therapy with glucocorticoids is effective.

  4. IgG4-related disease: description of a case with pulmonary lesions, mediastinal lymphadenopathies and rapidly progressive renal failure.

    Science.gov (United States)

    Fernández Lorente, Loreto; Álvarez, Dolores López; López, Virginia García; Kollros, Vesna Abujder; Ariza, Aurelio; Gálvez, Alejandro; Bonet, Josep

    2015-01-01

    This is a case report of a 73-year-old man with new-onset acute renal failure while being investigated for pulmonary infiltrates and mediastinal lymphadenopathies. Urine tests showed tubular range proteinuria with no microhaematuria. Immunology tests showed elevated serum IgG and hypocomplementaemia (classical pathway activation). Renal biopsy and clinical-pathological correlation were crucial in this case, reinforcing their important role in the final diagnosis of acute kidney injury. Copyright © 2015. Published by Elsevier España, S.L.U.

  5. [Hypertrophic pachymeningitis secondary to IgG4-related disease: case report and review of the literature].

    Science.gov (United States)

    Rodríguez-Castro, Emilio; Fernández-Lebrero, Aida; López-Dequidt, Iria A; Rodríguez-Osorio, Xiana; López-González, Francisco J; Suárez-Peñaranda, José M; Arias, Manuel

    2015-10-01

    Introduccion. La paquimeningitis hipertrofica es un trastorno infrecuente que produce un engrosamiento focal o difuso de la duramadre. Puede ser idiopatica o secundaria a procesos infecciosos, autoinmunes o neoplasicos. La recientemente descrita 'enfermedad relacionada con IgG4' podria ser la causa de bastantes cuadros considerados criptogenicos. Caso clinico. Mujer de 54 años, con historia de asma bronquial, que consulto por cefalea, vertigo y perdida de audicion por su oido izquierdo. En la resonancia magnetica cerebral con gadolinio se objetivo engrosamiento y realce dural, que se extendia desde la pared lateral del seno cavernoso izquierdo y la parte medial del lobulo temporal al angulo pontocerebeloso y parte del tentorio homolaterales. El liquido cefalorraquideo presentaba 10 leucocitos/µL (90% mononucleares), con 1 g/L de proteinas y sin consumo de glucosa. El estudio anatomopatologico mostro fibrosis y un infiltrado linfoplasmocitario, con 16 celulas plasmaticas IgG4+ por campo de gran aumento. El resto de estudios analiticos y microbiologicos resultaron normales o negativos. La tasa plasmatica de IgG4 estaba dentro de los limites normales. Tratada con esteroides, se produjo mejoria clinica acompañada de la practica desaparicion de las alteraciones detectadas en la neuroimagen. Conclusiones. La paquimeningitis hipertrofica como manifestacion de la enfermedad relacionada con IgG4 puede diagnosticarse basandose en los hallazgos de la resonancia magnetica si la IgG4 plasmatica esta elevada. En casos dudosos, habra que recurrir a la biopsia meningea. La corticoterapia suele ser eficaz y representa la primera linea terapeutica.

  6. IgG4-related disease: description of a case with pulmonary lesions, mediastinal lymphadenopathies and rapidly progressive renal failure

    Directory of Open Access Journals (Sweden)

    Loreto Fernández Lorente

    2015-03-01

    Full Text Available This is a case report of a 73-year-old man with new-onset acute renal failure while being investigated for pulmonary infiltrates and mediastinal lymphadenopathies. Urine tests showed tubular range proteinuria with no microhaematuria. Immunology tests showed elevated serum IgG and hypocomplementaemia (classical pathway activation. Renal biopsy and clinical-pathological correlation were crucial in this case, reinforcing their important role in the final diagnosis of acute kidney injury.

  7. IgG4-related sclerosing diseases%IgG4相关的硬化性疾病

    Institute of Scientific and Technical Information of China (English)

    黄文斌; 周晓军

    2008-01-01

    IgG4相关的硬化性疾病(IgG4-related sclerosing disease),又称IgG4相关的自身免疫性疾病(IsG4-related autoimmune disease)或高IgG4疾病(hyper-ISG4 disease),是新近认识的一种疾病,其名称尚未统一。该疾病主要累及胰腺,但也可累及胰腺外其他器官,有时胰腺可无病变。ISG4相关的硬化性疾病多与自身免疫有关,且对类固醇激素治疗有效。由于IgG4相关的硬化性疾病国内文献报道相对较少,人们对其尚未引起足够的重视,临床症状缺乏特异性,极易误诊或漏诊。本文主要介绍IgG4相关的硬化性疾病的临床特征、各器官的组织病理学特征、鉴别诊断和治疗。

  8. IgG4相关唾液腺炎研究热点概述%Research hotspot in IgG4-related sialadenitis

    Institute of Scientific and Technical Information of China (English)

    王琼; 平飞云; 潘恒标

    2016-01-01

    IgG4相关疾病累及唾液腺称为IgG4相关唾液腺炎,中老年好发,男女性均可发病.IgG4相关唾液腺炎以唾液腺无痛性肿胀、血清IgG4升高、组织内大量特征性IgG4阳性浆细胞浸润伴纤维化为主要特点,糖皮质激素治疗反应良好.通过复习国内外文献,着重于该病近年来的研究热点阐述并分析多种免疫细胞和细胞因子、上皮间质转化参与的致病过程、正确看待血清IgG4浓度升高和组织IgG4阳性浆细胞浸润的临床病理意义及其诊断价值、与唾液腺癌和淋巴瘤的相关性、正电子发射型计算机断层摄影(positron emission tomography,PET)-CT的应用、唇腺活检对病理诊断的意义及治疗预后等问题.%IgG4-related disease is a novel clinical entity which can affect single or multiple organs.IgG4-related sialadenitis is referred to the salivary gland involvement of IgG4-related disease, with or without other organ involvement.IgG4-related sialadenitis is characterized by painless swelling or enlargement of salivary glands, high serum IgG4 level, abundant IgG4+ plasma cells infiltration with fibrosis histologically, and good response to glucocorticoids.With review of related articles, highlight and provide an overview of the most recent and focused findings and concepts of this disease, including the most significant pathogenic process based on kinds of immunocytes, cytokines, as well as participation of epithelial-mesenchymal transition, the clinical value of elevated serum IgG4 concentration and pathological role of infiltrated IgG4+ plasma cells, the potential relationship with salivary gland malignant tumor, the applying and usefulness of positron emission tomography-CT, the diagnostic utility of lip biopsy, treatment, prognosis, and also future perspectives.

  9. THE PROGRESS OF IGG4-RELATED CHRONIC SCLEROSING SIALADENITIS%IgG4相关性慢性硬化性颌下腺炎的研究现状

    Institute of Scientific and Technical Information of China (English)

    高永珍; 王晓磊

    2016-01-01

    IgG4相关性疾病是近年来新认识的一种疾病,是由免疫介导的累及身体多个器官或组织的慢性自身免疫系统疾病。本文就IgG4相关性慢性硬化性颌下腺炎的临床表现、组织病理学、影像学表现、治疗方式等最新进展做一综述。%In recent years,IgG4-related disease has been knowed as a new disease. It is a chronic immune-mediated autoimmune diseases involving multiple body organs or tissues. This article,we will review thelatest developments of IgG4 - related Chronic Sclerosing Sialadenitis, through itchiest pathology,imaging,treatment and so on.

  10. The clinicopathological analysis of 4 cases of IgG4-related nonspecific interstitial pneumonia%IgG4相关非特异性间质性肺炎四例临床病理分析

    Institute of Scientific and Technical Information of China (English)

    张卉; 施举红; 冯瑞娥; 田欣伦; 徐作军; 许文兵; 刘鸿瑞; 刘彤华

    2012-01-01

    Objectives To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP.Methods Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010.The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin.Sections were cut for HE and immunohistochemical stain.According to the diagnostic criteria for IgG4-related disease,4 cases were confirmed to be IgG4-related NSIP.The clinicopathological features including clinical history,laboratory examination,and pathologic evaluation were studied.Results The 4 patients with IgG4-related NSIP included 1 man and 3 women,with a median age of 48 years (range,44-56 years).The presenting symptoms were dry cough or shortness of breath.One patient (1/4,25.0%)was found to have a positive autoantibody but no cases showed positive RF in serum.The histological finding of the 4 cases was characterized by inflammatory cell infiltration in interstitium with fibrosis,and 1 case showed obliterative arteritis.The numbers of IgG4-positive plasma cells in the 4 cases were 42/hpf,22/hpf,11/hpf,and 33/hpf respectively,while the percentages of IgG4-positive to IgG-positive plasma cells were 70%,71%,57%,43% respectively.Conclusions IgG4-related interstitial pulmonary disease can be characterized as the NSIP pattern.The pathological features of IgG4-related NSIP include infiltration of lymphoplasmacytes and eosinophils in interstitium with fibrosis,and lymphoid follicles are frequently identified in the area of lymphocyte aggregation,but obliterative arteritis is infrequently identified in the lesion.Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease.%目的 观察非特异性间质性

  11. Overlap of Post-obstructive Diuresis and Unmasked Diabetes Insipidus in a Case of IgG4-related Retroperitoneal Fibrosis and Tuberoinfundibular Hypophysitis: A Case Report and Review of the Literature.

    Science.gov (United States)

    Sasaki Yatabe, Midori; Watanabe, Kimio; Hayashi, Yoshimitsu; Yatabe, Junichi; Morimoto, Satoshi; Ichihara, Atsuhiro; Nakayama, Masaaki; Watanabe, Tsuyoshi

    The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated. The pituitary swelling recurred seven months later. In patients with IgG4-RD, the manifestation of polyuria after steroid therapy should prompt suspicion of post-obstructive diuresis and the unmasking of central diabetes insipidus.

  12. Overlap of Post-obstructive Diuresis and Unmasked Diabetes Insipidus in a Case of IgG4-related Retroperitoneal Fibrosis and Tuberoinfundibular Hypophysitis: A Case Report and Review of the Literature

    Science.gov (United States)

    Sasaki Yatabe, Midori; Watanabe, Kimio; Hayashi, Yoshimitsu; Yatabe, Junichi; Morimoto, Satoshi; Ichihara, Atsuhiro; Nakayama, Masaaki; Watanabe, Tsuyoshi

    2017-01-01

    The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated. The pituitary swelling recurred seven months later. In patients with IgG4-RD, the manifestation of polyuria after steroid therapy should prompt suspicion of post-obstructive diuresis and the unmasking of central diabetes insipidus. PMID:28049999

  13. A case of immunoglobulin G-4 related sclerosing disease mimicking lung cancer

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Soo Hee; Lee, Young Kyung; Shim, Mi Suk; Lee, Hyang Im [Seoul Medical Center, Seoul (Korea, Republic of)

    2013-07-15

    Immunoglobulin (Ig) G4-related sclerosing disease is a recently described systemic fibro-inflammatory disease associated with an elevated circulating level of IgG4 and extensive IgG4-positive lymphoplasmacytic infiltration, resulting in sclerosing inflammation involving various body organs. We experienced one case where surgery confirmed IgG4-related sclerosing disease as a solitary lung mass mimicking lung cancer. We report radiologic findings including chest computed tomography and positron emission tomography computed tomography, with clinical manifestations of IgG4-related sclerosing disease.

  14. Immunoglobulin G4-Related Sclerosing Disease Involving the Urethra: Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Jin Woo; KIm, Sang Youn; Cho, Jeong Yeon; Kim, Seung Hyup [Dept. of Radiology, Seoul National University College of Medicine, and Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul (Korea, Republic of); Moon, Kyung Chul [Dept. of Pathology, Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2012-11-15

    Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.

  15. Clinico-pathological Characteristics of IgG4 related Sialadenitis of Submandibular Gland with Local Regional Lymphadenopathy%IgG4相关下颌下腺炎伴局部淋巴结病变的临床与病理特点分析

    Institute of Scientific and Technical Information of China (English)

    王木; 周炼; 赵继志; 林润台; 张韬; 余立江

    2016-01-01

    Objective: To investigate the clinical and pathological features of IgG4 related submandibular gland sialadenitis with local regional lymphadenopathy and discuss the differential diagnosis and therapic measures. Methods:14 cases of IgG4 related submandibular gland sialadenitis, including 5 male and 9 female, age ranging from 16 to 75 were reviewed, and their clinical characteristics, radiological and pathological features were analyzed. Results: IgG4 related submandibular gland sialadenitis can occurred in different ages. Symtoms and signs consisted of enlargement of submandibular gland, xerophthalmia and lacrimal gland swelling. Laboratory finding revealed the serum IgG4 levels elevated to 3 380~21 600mg/L. Enhanced CT showed homogeneous enhancement of the gland with clear boundary. Pathology of the gland showed destruction of lobe structure, IgG4 positive plasma cell infiltration, follicular formation, storiform-type fibrosis and obliterative phlebitis appeared. Local regional lymphadenopathy presented amounts of follicular and active germinal centers. Conclusion: Immunoglobulin G4 (IgG4)-related disease is a fibro-inflammatory disease of unknown etiology,presenting a distinctive histopathological appearance. Differential diagnosis is required for local regional lymphadenopathy. General condition evaluation to excludes other organs involvement is suggested for those patients confirmed IgG4 related disease by biopsy.%目的:分析IgG4相关下颌下腺炎伴局部淋巴结病变的临床和病理特点,并深入探讨其鉴别诊断与治疗方法。方法:回顾2007-09—2015-08期间,诊断为IgG4相关下颌下腺炎性病变患者14例,其中男性5例,女性9例,年龄范围16~75岁,分析临床及影像学表现和病理特点。结果:IgG4相关下颌下腺炎可发生于多个年龄段,下颌下腺肿大可伴口干、眼干及泪腺肿大症状,血清IgG4分类水平3380~21600mg/L。增强CT表现为弥漫性肿大,均匀强化,

  16. The Digestive Involvement of IgG4-related Disease%消化内镜与IgG4相关消化系统疾病

    Institute of Scientific and Technical Information of China (English)

    杨爱明; 于航

    2016-01-01

    IgG4相关疾病(IgG4-RD)是一种慢性炎症硬化性疾病,特点为大量IgG4阳性浆细胞浸润、致密纤维化、闭塞性静脉炎及血清IgG4水平升高等.IgG4-RD可以累积于多种脏器,其常见的消化系统受累表现包括自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关自身免疫性肝炎、IgG4相关硬化性胆囊炎、自身免疫性胰腺炎相关性胃炎等.消化内镜,包括超声内镜、ERCP、ERCP结合IDUS、EUS-FNA在IgG4相关消化系统疾病的诊断,尤其是胰腺、胆道系统方面都有重要作用.IgG4-RD对激素治疗敏感,但容易复发,对疾病复发的患者可再次激素治疗或联合免疫调节治疗.

  17. Clinical analysis of patients with IgG4-related disease%IgG4相关性疾病临床特征分析

    Institute of Scientific and Technical Information of China (English)

    姚春容; 崔贝贝; 林辉; 薛丽佳; 朱利君; 刘毅

    2016-01-01

    目的 针对IgG4相关性疾病(IgG4-RD)的临床特点、影像学特征、组织病理学表现及预后进行分析,为IgG4-RD的诊断及治疗提供依据.方法 回顾性收集2011~2015年在四川大学华西医院诊治的48例诊断为IgG4相关性疾病患者临床资料,统计分析该病患者症状,血清IgG和IgG4、肿瘤血清学标志物及自身抗体表达,总结该病患者CT、MRI、超声等影像学特点,随访疾病预后.结果 48例IgG4-RD平均年龄(50±16)岁,其中男女比例为1.5∶1,血清IgG4水平在0.05~80.5 g/L之间.淋巴结受累患者最多,14例患者伴有多部位受累.共有47例患者进行组织病理学检查,主要表现为淋巴浆细胞浸润及纤维化形成,IgG4+细胞>10个高倍视野.38例患者进行糖皮质激素联合免疫抑制剂治疗,激素减量后,2例患者疾病复发.结论 IgG4-RD是多器官受累的自身免疫病,受累脏器IgG4阳性浆细胞浸润是其主要特点,及时应用糖皮质激素及免疫抑制剂可有效缓解病情.

  18. IgG4相关性胃病临床分析%Clinical Analysis of IgG4-related Gastric Disease

    Institute of Scientific and Technical Information of China (English)

    郑松柏; 李小雯; 肖立; 沈达明; 季大年

    2015-01-01

    背景:IgG4相关性胃病较罕见,临床上因对该病认识不足,易致错误诊断和治疗.目的:提高IgG4相关性胃病的诊治水平.方法:结合复旦大学附属华东医院近期1例IgG4相关性胃病与既往文献报道的8例病例,对该病作分析、归纳和讨论.结果:IgG4相关性胃病患者以中老年居多,主要临床表现为腹部不适、纳差、恶心、呕吐、贫血、体质量下降等,少数患者可伴有其他器官免疫性疾病.胃镜下表现可分为增生型和溃疡型,组织病理学特征为大量IgG4阳性淋巴细胞、浆细胞浸润,淋巴滤泡形成,可累及胃壁各层、神经周围间隙、小血管壁、区域淋巴结,基本无胃外器官受累.诊断可参考日本IgG4相关性疾病综合诊断标准,但多数病例血清IgG4正常.治疗以手术为主,包括局部切除、胃部分切除和全胃切除,患者预后良好.结论:有限的病例报道显示IgG4相关性胃病的诊治并不十分困难,关键是提高对该病的警惕性和认识并积累更多病例,以深入探讨其发病机制和临床特点.

  19. Appendiceal immunoglobulin G4-related disease mimicking appendiceal tumor or appendicitis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Soo; Kang, Won Kyung; Chung, Dong Jin [Yeouido St. Mary' s Hospital, The Catholic University of Korea, Seoul (Korea, Republic of)

    2016-02-15

    Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.

  20. 血清IgG4对IgG4相关性疾病的诊断价值及该病的眼部表现%Value of serum IgG4 for the diagnosis of IgG4-related disease and the ocular manifestations of IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    杨慧敏; 王晓栋; 叶霜; 华静; 王琴; 李劲

    2015-01-01

    目的 评价血清IgG4浓度对IgG4相关性疾病的鉴别诊断价值,并探讨IgG4相关性疾病的眼部表现.方法 收集IgG4相关性疾病患者29例,血清IgG4浓度升高的其他疾病患者161例(其中自身免疫性肝病47例,急性胰腺炎15例,原发性肾小球病及肾功能不全61例,炎症性肠病38例),正常对照190例;以免疫散射比浊法测定血清IgG4和IgG浓度,比较各疾病组患者的检测结果.以所有患者的血清IgG4浓度绘制受试者工作特征(ROC)曲线,评价血清IgG4浓度的诊断价值,并确定其诊断IgG4相关性疾病的最佳临界值.同时对IgG4相关性疾病患者进行眼科检查,评估其眼部表现.结果 IgG4相关性疾病组血清IgG4浓度与原发性肾小球病及肾功能不全组、炎症性肠病组、自身免疫性肝病组、急性胰腺炎组比较,差异有统计学意义(P<0.01).经ROC曲线分析,当IgG4为2.37 g/L时,Youden指数最大,该诊断界值的敏感度为93.1%,特异度为84.3%,曲线下面积为0.939.29例IgG4相关性疾病患者中,2例有眼眶占位性病变,其余患者眼部表现无特异性.结论 血清IgG4浓度对于诊断IgG4相关性疾病具有较高的敏感度和特异度,但仅能作为诊断IgG4相关性疾病的依据之一,提示IgG4相关性疾病的可能;该病眼部表现无特异性.

  1. IgG4-related idiopathic hypertrophic pachymeningitis: one case report and review of the literature%IgG4相关特发性肥厚性硬脑膜炎:一例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    倪海春; 朴月善; 卢德宏; 马晓丽

    2011-01-01

    Objective To study the clinical, neuroimaging and histopathological features of IgC4-related idiopathic hypertrophic pachymeningitis (IHP). Methods One case of pathologically confirmed IgG4 - related IHP was reported, and related literature of the clinical, radiological and pathological characteristics of this disease was reviewed. Postoperative specimen was sent to Department of Pathology, Xuanwu Hospital. It was sliced and then stained with HE method and examined by immunohistochemistrical study (S-100, Ki-67, CD20, CD3, IgG, IgG4). Results The case was a 55-year-old male patient with IHP. The clinical manifestations of IHP were variable. The patient firstly presented seizures of epilepsy showing left limb convulsions. Brain contrast - enhanced MRI showed that the pachymenx was partly thickened with line - like or nodular enhancement. Microscopically, meningeal biopsies showed dura mater encephali was thickened and hardened. Under microscope, meningeal biopsies showed abnormal thickening of the pachymenx with fibrous connective tissue hyperplasia and infiltration of many inflammatory cells. Inflammatory cells were mainly composed of lymphocytes and plasmocytes with a small amount of eosinophils. Immunohistochemistrical study showed a large number of lymphocytes with expression of IgG, and a small part with expression of IgG4. The treatment of IgG4 - related IHP with hormones and immunosuppressive agents was effective. Conclusion IHP is a class of unknown etiologic diseases, with dural fibrous inflammatory lesion, and part of the IHP patients may be suffering from IgG4-related IHP.%目的 探讨IgG4相关特发性肥厚性硬脑膜炎的临床表现、影像学及组织病理学特点.方法 回顾分析1例IgG4表达阳性的特发性肥厚性硬脑膜炎患者的临床表现、组织学特征,并复习相关文献.结果 男性患者,55岁.以癫痫发作起病,主要表现为发作性左侧肢体抽搐.MRI增强扫描显示局部硬脑膜增厚,并呈线样或

  2. Recognizing Immunoglobulin G4-Related Overlap Syndromes in Patients with Pancreatic and Hepatobiliary Diseases

    Directory of Open Access Journals (Sweden)

    Aldo J Montano-Loza

    2008-01-01

    Full Text Available The first description of autoimmune pancreatitis and elevated serum immunoglobulin-G4 (IgG4 in 2001 heralded further reports of several related autoimmune diseases with raised IgG4 levels. It is now recognized that a spectrum of overlap syndromes associated with increased IgG4 and biopsy evidence of IgG4-producing plasma cells, which has now been convincingly linked with cholangitis, autoimmune hepatitis, Sjögren’s syndrome, nephritis and retroperitoneal fibrosis. Collectively, this disease cluster is referred to as IgG4-related systemic disease. The importance of making the correct diagnosis is underscored by the management of individuals with IgG4-related systemic disease. In the first instance, patients generally have a dramatic response to immunosuppressive therapy, whereas patients with other forms of cholangitis and pancreatitis do not. Also, surgical management of pancreatic malignancy can be avoided once the correct diagnosis of IgG4-related disease has been made. In the present review, an overview of the current information regarding the role of IgG4 and IgG4-positive cells affecting the biliary system, pancreas and liver is provided.

  3. [Immunoglobulin-G4-related disease: a challenging diagnosis].

    Science.gov (United States)

    Banc, Ana; Stan, Cristina

    2014-01-01

    Immunglobulin G4 (IgG4)-related disease was recently described and represents a systemic lymphoproliferative disorder. The orbital form of the disease manifests as chronic lid swelling and proptosis. Visual disturbances may occur due to apical orbital lesions. A 65-year old pacient presents with the impossibility of maintaining his right eye open due to a progressive swelling of the upper lid. The general clinical examination shows adenopathy at 3 lymph node stations of the head. On ophthalmologic examination, a large tumor of the upper lid is observed in the right eye and proptosis and central retinal vein occlusion are noted in the left eye. The serum levels of the IgG are very high. A lymph node biopsy was performed. The differential diagnosis between the IgG4-related orbital disease and non-Hodgkin lymphoma is discussed. CONCLUSION The diagnosis criteria for IgG4-related disease are both the high serum levels of IgG4 and the specific immunohistochemistry stains.

  4. IgG4相关自身免疫性胰腺炎的临床特点%Clinical features of IgG4 related autoimmune pancreatitis

    Institute of Scientific and Technical Information of China (English)

    张锐; 曾弘; 余先焕; 唐启彬; 王捷; 刘超

    2014-01-01

    目的:探讨IgG4相关自身免疫性胰腺炎(AIP)的临床特点。方法回顾性研究2003年1月至2012年12月在中山大学孙逸仙纪念医院肝胆胰外科接受诊治并经病理学确诊的12例IgG4相关AIP患者临床资料。所有患者均签署知情同意书,符合医学伦理学规定。患者均为男性,平均年龄为(54±13)岁,均被误诊为胰头癌行胰头十二指肠切除术。收集患者临床表现及实验室、影像学、病理学检查等资料。患者术后接受随访,观察治疗情况及疗效。结果12例患者中腹痛7例、黄疸7例、消瘦6例、无明显症状1例,合并糖尿病3例、慢性下颌下腺炎1例。患者血、尿淀粉酶均正常,血GGT升高12例、糖链抗原19-9(CA19-9)轻度升高9例、癌抗原(CA)125升高3例、癌胚抗原(CEA)升高2例。增强CT示胰头局部肿大11例,伴胰管轻度扩张3例。MRI示胰体呈“腊肠样”改变4例,胰周呈“包鞘样”改变5例。磁共振胰胆管成像(MRCP)示胰头段胰管及胆总管下段狭窄,其中4例远端胰管轻度扩张。影像学检查未发现局部侵犯。病理学检查表现为淋巴浆细胞硬化性胰腺炎,免疫组织化学方法(免疫组化法)染色IgG4阳性。12例术后患者间断出现腹痛,其中7例需服用止痛药。1例患者经泼尼松治疗后症状缓解。结论 IgG4相关AIP临床表现与胰腺癌相似,易误诊为胰腺癌。其主要临床特点为血CA19-9轻度升高;影像学检查见胰头局部肿大、胰体呈“腊肠样”、胰周呈“包鞘样”改变,且无局部侵犯;病理学检查为淋巴浆细胞硬化性胰腺炎,免疫组化法染色IgG4阳性;肾上腺皮质激素治疗有效。%Objective To investigate the clinical features of IgG4 related autoimmune pancreatitis (AIP). Methods Clinical data of 12 patients pathologically diagnosed as IgG4 related AIP in Department of Hepatopancreatobiliary Surgery, Sun Yat-sen Memorial Hospital of Sun Yat-sen University from January 2003 to December 2012 were retrospectively analyzed. The informed consents of all patients were obtained and the ethical committee approval was received. All the patients were males with a mean age of (54±13) years old. All were misdiagnosed as pancreatic head carcinoma and received pancreatoduodenectomy. Data of the clinical manifestations, laboratory, imaging and pathological examinations were collected. The patients were followed up after operations, the treatment and outcome were studied. Results The clinical manifestations of the 12 patients were abdominal pain (n=7), jaundice(n=7), emaciation(n=6) and no obvious symptom (n=1). Three cases were combined with diabetes, 1 case with chronic sialadenitis of submandibular gland. The patient's blood and urine amylase were normal. Blood gamma-glutamyl transpeptidase (GGT) increase was observed in 12 cases. Mild increase of carbohydrate antigen 19-9 (CA19-9) was observed in 9 cases. Cancer antigen (CA) 125 increase was observed in 3 cases, and carcinoembryonic antigen (CEA) increase in 2 cases. Through contrast-enhanced CT, partial pancreatic head enlargement was observed in 11 cases, in which 3 cases were observed combining with mild expansion of pancreatic duct. Through magnetic resonance imaging (MRI), sausage-like change of pancreas body was observed in 4 cases, peripancreatic sheath-like change in 5 cases. Through magnetic resonance cholangiopancreatography (MRCP), stenoses in the pancreatic duct of pancreatic head segment and distal common bile duct were observed including 4 cases of mild expansion in the distal pancreatic duct. Local invasion was not observed by imaging examinations. Lymphoplasmacytic sclerosing pancreatitis was determined by pathological examination. Positive expression of IgG4 was observed by immunohistochemistry. Twelve cases suffered from discontinuous abdominal pain after operations, in which 7 cases needed acesodyne. One case relieved after using prednisone. Conclusions Clinical manifestations of IgG4 related AIP are similar to pancreatic carcinoma and can be misdiagnosed as pancreatic carcinoma easily. The main clinical features are mild increase of blood CA19-9. Partial pancreatic head enlargement, pancreas body sausage-like change, peripancreatic sheath-like change, and no local invasion are observed by imaging examinations. Lymphoplasmacytic sclerosing pancreatitis is determined by pathological examination. Positive expression of IgG4 is observed by immunohistochemistry. Adrenocortical hormone treatment is effective.

  5. IgG4相关肺炎性假瘤临床病理学特征及与CD4+T细胞和白介素10的关系分析%Clinicopathologic features of IgG4-related pulmonary inflammatory pseudo-tumor and its correlations with CD4+T lymphocytes and interleukin-10 expres-sion

    Institute of Scientific and Technical Information of China (English)

    黄玉钿; 谭云山; 纪元; 曾海英

    2015-01-01

    目的:研究IgG4相关肺炎性假瘤( IRPIP )临床病理学特征以及病变与CD4+T细胞和白介素10 ( IL-10 )的关系,探讨IgG4相关肺炎性假瘤中存在的免疫病理因素.方法:应用免疫组化法检测17例IgG4相关肺炎性假瘤和15例IgG4非相关肺炎性假瘤( INPIP)组织中IL-10、CD3、CD20、CD4、CD8的表达,分析这些指标在IgG4相关肺炎性假瘤和IgG4非相关肺炎性假瘤中的差异.结果:IRPIP组中CD4阳性细胞数高于INPIP组,差异有统计学意义(P=0.006). IRPIP组中CD4阳性细胞数/CD8阳性细胞数比值高于INPIP组,差异有统计学意义( P=0.023 ). IRPIP组中CD3、CD8、IL-10阳性细胞数高于INPIP组,差异无统计学意义(P>0.05). IRPIP组中CD20阳性细胞数低于INPIP组,差异无统计学意义(P>0.05). IgG4相关性肺炎性假瘤组中IgG4阳性浆细胞数与IL-10阳性细胞数之间呈正相关(r=0.517),相关性具有统计学意义(P=0.033).结论:IgG4相关肺炎性假瘤除了IgG4阳性浆细胞数量升高,同时伴随着病变背景中CD4+T数量的增加,且IgG4阳性浆细胞数与淋巴细胞IL-10的表达呈正相关,提示Th2细胞及细胞因子IL-10的分泌是参与IgG4相关肺炎性假瘤病变的免疫病理因素.%Objective:To study the clinicopathologic features of IgG4-related pulmonary inflammatory pseudotumor (IRPIP), and its correlations with CD4+T lymphocytes and interleukin-10 (IL-10) expression,and to investigate the immunopathological factors in the disease of IgG4-related pulmonary inflammatory pseudotumor .Methods: Expressions of IL-10, CD3, CD20, CD4, CD8 were examined by immunohistochemistry in 17 cases of IgG4-related pulmonary inflammatory pseudotumor tissues and 15 cases of IgG4-nonrelated pulmonary inflammatory pseudotumor tissues ( INPIP) .To analyze the differences of these indexes expression between IRPIP and INPIP.Results: In group of IRPIP, the CD4+T lymphocytes number and the ratio of CD 4+T lymphocytes number/CD8+T lymphocytes number

  6. Immunoglobulin G4-Related Disease Mimicking Asthma

    Directory of Open Access Journals (Sweden)

    Hiroshi Sekiguchi

    2013-01-01

    Full Text Available Immunoglobulin (Ig G4-related disease (also known as ‘IgG4-related sclerosing disease’, ‘IgG4-related systemic disease’ or ‘hyper-IgG4-disease’ is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

  7. 基于IgG4分类的桥本甲状腺炎临床意义探讨%Clinical significance of IgG4 related Hashimoto thyroiditis

    Institute of Scientific and Technical Information of China (English)

    钱伟; 杜婷婷; 吴汉妮

    2015-01-01

    目的:探讨IgG4相关性桥本甲状腺炎( HT)患者血清IgG4水平及其临床意义。方法:酶联免疫吸附法测定129例HT患者血清IgG4水平,并根据IgG4水平将患者分为IgG4组(IgG4≥135 mg/dl)和非IgG4组(IgG4<135 mg/dl);电化学发光法测定血清甲状腺激素、甲状腺过氧化物酶抗体( TPOAb)、甲状腺球蛋白抗体( TgAb);甲状腺超声影像检查。结果:HT患者血清TPOAb与同时测定的IgG4水平(r=0.4371,P=0.0127)和IgG4/IgG比值(r=0.3962,P=0.0235)呈显著正相关。与非IgG4组(97例)比较,IgG4组(32例):①平均患病年龄趋年轻化(P=0.0293);②较高的血清TPOAb(P=0.0021)、TgAb(P=0.0128)水平;③超声影像:更易形成甲状腺结节(P=0.0226);④多因素Logistic回归分析结果提示,血清IgG4、TPOAb水平是患甲状腺结节危险因素,OR值分别为1.672(P=0.021)、2.549(P=0.014)。结论:IgG4相关性HT患者存在相应的临床特征。对于血清中IgG4水平明显升高的HT患者,应更加密切监测甲状腺功能及形态变化。%Objective:To explore the serum IgG4 level in patients with IgG4-related Hashimoto thyroiditis(IgG4 HT),and its clinical implications.Methods:The serum IgG4 was determined in 129 patients with HT using enzyme-linked immunosorbent assays and classified into two subgroups based on IgG4 level:IgG4 HT group(IgG4≥135 mg/dl)and non-IgG4 HT group(IgG4<135 mg/dl).And the levels of serum thyroid hormone and thyroid peroxidase antibodies(TPOAb)and thyroglobulin antibodies(TgAb)were measured by electro-chemiluminescence immunoassay.Ultrasonic imaging of the thyroid gland were detected.Results:The TPOAb levels correlated significantly with both serum IgG4 levels(r=0.437 1,P=0.012 7)and IgG4/IgG ratios(r=0.396 2,P=0.023 5)in the patients with HT.Compared with that of non-IgG4 HT group(n=97),IgG4 HT group(n=32):①The mean age was lower(P=0.029 3);②Higher levels of serum TPOAb(P=0.002 1)and TgAb(P=0.012 8);③Ultrasound imaging:the more obvious thyroid nodule(P=0.022 6);④Logistic regression analysis showed that serum IgG4 and TPOAb were the risk factor for thyroid nodules(OR=1.672,P=0.021;2.549,P=0.014 ).Conclusion: IgG4 HT patients existed corresponding clinical characteristics.For the HT-patients with elevated serum IgG4,thyroid function and morphology should were more closely monitored.

  8. Immunoglobulin G4-related Lung Disease: A Disease with Many Different Faces

    Directory of Open Access Journals (Sweden)

    Philip Hui

    2013-01-01

    Full Text Available Immunoglobulin (Ig G4-related lung disease is a fibroinflammatory entity that presents in protean ways. Diagnostically, IgG4-related lung disease requires a high clinical index of suspicion complemented by elevated serum IgG4 levels and/or biopsy that shows the characteristic pathological features. The disease is almost always responsive to systemic corticosteroids. However, relapse is common following their discontinuation. The authors present three cases to highlight the diverse clinical features, and to illustrate the diagnostic and therapeutic approaches to this disease.

  9. Recent advances in IgG4-related lung disease%免疫球蛋白G4相关性肺疾病研究进展

    Institute of Scientific and Technical Information of China (English)

    张卉; 冯瑞娥

    2011-01-01

    @@ 近年来随着对免疫球蛋白G4(immunoglobulin G4,IgG4)相关性硬化性疾病的关注,越来越多的研究结果表明,IgG4相关性硬化性疾病是一组系统性疾病,除胰腺外,还可累及多个器官及组织,关于肺的IgG4相关性疾病也逐渐出现相关报道.IgG4相关性疾病以血清IgG4浓度升高、IgG4阳性浆细胞浸润及不规则纤维化为特征.

  10. IgG4相关性胆胰疾病的诊断和治疗%Diagnosis and Treatment of IgG4-related Biliary and Pancreatic Diseases

    Institute of Scientific and Technical Information of China (English)

    白文元; 戴胜兰

    2014-01-01

    近年来发现,IgG4相关性疾病可累及全身多系统多器官,目前研究提示IgG4相关性胆胰疾病主要有IgG4相关性硬化性胆管炎和自身免疫性胰腺炎(AIP).前者是一种免疫发病机制未明、生化学特点以及胆管影像学表现与原发性硬化性胆管炎(PSC)相似,对糖皮质激素应答良好的疾病.后者临床表现为复发性胰腺炎、梗阻性黄疸,伴胆管、涎腺等其他器官受累.实验室检查血清和组织学IgG4明显升高.影像学表现为胰腺呈“腊肠样”外观,伴主胰管弥漫不规则狭窄,同样对激素治疗敏感.二者常同时受累、关系密切,易误诊为胆管癌、硬化性胆管炎或胰腺癌,本文将当前国内外研究现状做一介绍,供临床诊治参考.

  11. 累及肾盂的IgG4相关性疾病1例报告并文献复习%Renal pelvis involved IgG4-related disease (A case report and literature review)

    Institute of Scientific and Technical Information of China (English)

    吴兴成; 纪志刚; 严维刚; 毛全宗

    2016-01-01

    目的:分析1例累及肾盂的IgG4相关性疾病的临床资料并进行文献复习,探讨该病的诊断和治疗经验,以提高对IgG4相关性肾盂疾病的认识及诊治水平.方法:分析北京协和医院收治的1例IgG4相关性肾盂疾病患者的临床表现、实验室检查、影像学及组织病理学资料.以“IgG4相关性疾病”和“肾盂”为关键词,自Pubmed共计检索到累及肾盂的IgG4相关性疾病4例,对临床症状、肾盂外器官受累情况、血清IgG4水平、影像学表现和组织学以及治疗等进行分析.结果:患者女性,53岁,B超发现右肾盂占位,无临床不适,体重近半年减重3 kg.尿路CT成像示右肾盂及中下部肾盏团块样软组织密度影.逆行造影未见右肾盂内明确充盈缺损.PET/CT示右肾盂软组织密度影放射性摄取增高.行腹腔镜右肾输尿管全长切除术,病理符合IgG4相关性疾病.血清IgG4明显升高(3 240 mg/L),给以泼尼松口服治疗,3个月后复查IgG4水平降至1 890 mg/L.文献报道IgG4相关性肾盂疾病共4例,合并其他器官受累3例.2例行患侧肾输尿管全长切除,1例行肾切除.应用糖皮质激素治疗3例,疗效良好,1例因合并严重消化道出血死亡.结论:IgG4相关性疾病是一少见疾病,累及肾盂罕见,临床易误诊为肾盂癌而行不必要的手术切除,可同时累及其他器官,诊断有赖于血清IgG4水平测定及组织病理学检查,糖皮质激素治疗有效,预后良好.

  12. IgG4升高的肾病:IgG4相关性肾病与特发性膜性肾病的研究进展%Advances in Kidney Diseases with Elevated IgG4 Levels:IgG4 -related Kidney Disease and Idiopathic Membranous Nephropathy

    Institute of Scientific and Technical Information of China (English)

    申嫒文; 林鹭; 郁胜强

    2016-01-01

    IgG4相关性疾病是以血清IgG4升高、组织IgG4浆细胞浸润以及纤维性病变为特征的多系统疾病,可累及胰腺、肾脏、唾液腺等多种器官,累及肾脏时称为IgG4相关性肾病,主要表现为IgG4相关性肾小管间质性肾炎,累及肾小球时以膜性肾病多见。而特发性膜性肾病的免疫复合物沉积也以IgG4为主,在临床上区分IgG4相关性疾病继发的膜性肾病与特发性膜性肾病非常重要。本文从发病机制、临床表现、诊断及治疗等方面探讨IgG4相关性肾病与特发性膜性肾病的区别,为临床的诊治提供一定的理论基础。%IgG4 -related disease is a multi -system disease , which often manifests as elevated serum IgG 4 concentrations , IgG4-positive plasma cell infiltration and fibrosis lesions .IgG4-related disease can affect many organs , such as pancreas, kidney, and salivary glands.When kidney is involved, we call it IgG4-related kidney disease, and its most common renal manifestation is IgG4 -related tubulointerstitial nephritis.In addition, glomerulus involvement , most commonly membranous nephropathy (MN), could also be found.Meanwhile, idiopathic membranous nephropathy is associated with high IgG4.It is important to distinguish between the MN secondary to IgG 4-related disease and idiopathic membranous nephropathy , which is a separate disorder .In this paper , through the discussion from pathogenesis , clinical manifestation , diagnosis and treatment, we compared the differences between IgG 4-related kidney disease and idiopathic membranous nephropathy , in order to provide some theoretical foundations for clicinal diagnosis and treatment .

  13. Gastric calcifying fibrous tumor: A case of suspected immunoglobulin G4-related gastric disease

    Directory of Open Access Journals (Sweden)

    Hejun Zhang

    2015-01-01

    Full Text Available Gastrointestinal lesions resulting from immunoglobulin G4-related disease are classified into two types: One is a gastrointestinal lesion showing marked thickening of the wall, and the other is an IgG4-related pseudotumor. We report the case of a woman with gastric calcifying fibrous tumor undergoing endoscopic resection that contained 62 IgG4+ plasma cells per high-power field and an IgG4-to-IgG ratio of 41% in lesional plasma cells, which shared clinical and histopathological features associated with gastric IgG4-related pseudotumor. So, we postulate that calcifying fibrous tumor as part of the spectrum of IgG4-related disease might be the unifying concept with IgG4-related pseudotumor. Meanwhile, the patient had coexistent autoimmune diseases, including autoimmune atrophic gastritis, Hashimoto's thyroiditis, and possible primary biliary cirrhosis. The clinical follow-up evaluation was uneventful.

  14. An immunoglobulin G-4 related sclerosing disease of the small bowel: CT and small bowel series findings

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Young Hwan; Hwang, Dae Hyun; Min, Seon Jeong [Dept. of Radiology, Hallym University Dongtan Sacred Heart Hospital, Hwaseong (Korea, Republic of); Woo, Ji Young; Kim, Jeong Won; Hong, Hye Sook; Yang, Ik; Lee, Yul [Hallym University Kangnam Sacred Heart Hospital, Seoul (Korea, Republic of)

    2013-10-15

    Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.

  15. Association between autoimmune pancreatitis and systemic autoimmune diseases

    Institute of Scientific and Technical Information of China (English)

    Viktória Terzin; Imre F(o)ldesi; László Kovács; Gyula Pokorny; Tibor Wittmann; László Czakó

    2012-01-01

    AIM:To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4).METHODS:The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment.Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and,in some cases,by computer tomography (CT).RESULTS:Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients.10 patients had Sj(o)gren's syndrome (SS) (IgG4:590 ±232 mg/L),2 of them in association with Hashimoto's thyroiditis,and 7 patients (IgG4:1388 ± 985.5 mg/L)had systemic lupus erythematosus (SLE).The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L).Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level.CONCLUSION:The serum IgG4 level may be elevated in SAIDs without the presence of AIP.The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.

  16. Comparing the clinical characteristics of IgG4-related sclerosing cholangitis with primary sclerosing cholangitis%原发性硬化性与IgG4相关硬化性胆管炎临床特征比较

    Institute of Scientific and Technical Information of China (English)

    王鹏辉; 何小东; 曹建中; 刘卫; 张太平; 洪涛; 曲强

    2016-01-01

    Objective To analyze and compare the clinical characteristics of primary sclerosing cholangitis (PSC) with IgG4-related sclerosing cholangitis (IgG4-SC).Methods The clinical data of 32 PSC patients and 72 IgG4-SC patients who were hospitalized in Peking Union Medical College Hospital (PUMCH) from January 2004 to December 2014 were retrospectively analyzed.Results Of the 32 PSC patients,there were 16 male and 16 female.Of the 72 IgG4-SC patients,there were 61 male and 11 female,(ratio =5.5∶ 1).The average ages were 44.9 (11 ~ 77) and 59.8 (28 ~ 83) years,respectively (P <0.05).The most common symptoms of PSC and IgG4-SC were abdominal pain and jaundice,and the incidences of abdominal pain and jaundice were 50.0% and 68.1%,78.1% and 81.9%,respectively.The serum IgG4 level of the IgG4-SC patients was significantly higher than the PSC patients (P < 0.05).The total protein in serum of the IgG4-SC patients was higher than the PSC patients (P < 0.05).The rate of bile duct wall thickening as detected on endoscopic ultrasonography (EUS) was higher than by abdominal ultrasound and abdominal CT,which were 91.2%,11.5% and 33.3%,respectively (P <0.05).12 PSC patients were followed up for over 2 years,including 2 patients who underwent liver transplantation after failure of conservative treatment,5 patients who died from hepatic failure and infection,and 3 with stable condition.43 IgG4-SC patients were followed up for over 2 years,including 16 patients with relapse.The recurrence rate was 37.2% (16/43).The more the extrabiliary organs or bile duct segments were involved,the higher was the recurrence rate.Conclusions Both PSC and IgG4-SC are cholestatic diseases,and they have many similarities in clinical and imaging manifestations.However,they still have unique features.IgG4-SC is sensitive to glucocorticoids therapy and has good prognosis.Thus,it is important to differentiate PSC from IgG4-SC.%目的 比较IgG4相关性硬化性胆管炎(IgG4-SC)

  17. IgG4相关性疾病的研究进展%Immunoglobulin G4-related disease

    Institute of Scientific and Technical Information of China (English)

    张雪英; 乔建军

    2016-01-01

    IgG4相关性疾病是近年来提出的一种新病种,本病可累及人体任何组织和器官.IgG4相关性疾病的显著特征为血清IgG4水平升高以及多种器官和组织中IgG4阳性浆细胞浸润.近年来发现,IgG4相关性疾病可有皮肤改变,皮损可表现为多种皮肤病的症状,包括Mikulicz病、皮肤浆细胞增多症、假性淋巴瘤、血管淋巴样增生伴嗜酸细胞增多和木村病等.IgG4相关性疾病患者对系统糖皮质激素治疗反应较好.%Immunoglobulin G4 (IgG4)-related disease is a recently proposed entity, and can affect any tissue or organ in the human body.It is characterized by increased serum levels of IgG4 and infiltration of abundant IgG4-positive cells in various organs and tissues.Recent studies have found that IgG4-related disease may present with various skin manifestations, such as Mikulicz's disease, cutaneous plasmacytosis, pseudolymphoma, angiolymphoid hyperplasia with eosinophilia and Kimura's disease.Systemic glucocorticoids are effective for the treatment of IgG4-related disease.

  18. Periodontal Disease and Systemic Health

    Science.gov (United States)

    ... Gum Disease and Other Diseases Gum Disease and Diabetes Gum Disease and Heart Disease Gum Disease and Other Systemic ... Gum Disease and Other Diseases Gum Disease and Diabetes Gum Disease and Heart Disease Gum Disease and Other Systemic ...

  19. Immunoglobulin G4 sinusitis in association with aspirin-exacerbated respiratory disease.

    Science.gov (United States)

    Johal, Kirti; Welch, Kevin; Peters, Anju

    2017-09-01

    Immunoglobulin G4 (IgG4) related disease is a systemic inflammatory disease characterized by tumor-like tissue infiltration with IgG4 positive (IgG4+) plasma cells. Aspirin-exacerbated respiratory disease (AERD) is defined as asthma, chronic rhinosinusitis with nasal polyposis, and hypersensitivity to cyclooxygenase-1 inhibitors. We described a case of a non-smoking 61-year-old male with prior NSAID sensitivity who presented with a 1-year history of left eye proptosis associated with chronic nasal symptoms, ultimately identified as concurrent AERD and IgG4 sinusitis. The patient was evaluated in the clinic and diagnosed by using clinical, radiographic, and surgical biopsy findings. Although initial concern was greatest for malignancy, a biopsy specimen confirmed the presence of a dense lymphoplasmacytic infiltrate and storiform fibrosis, associated with increased IgG4+ plasma cells. Therefore, IgG4-related disease (RD) was identified in this patient with AERD. Shared type II inflammation may be responsible for the coexistence of IgG4-RD and AERD as observed in our patient. Health care workers must be cognizant of the simultaneous presentation of both IgG4-RD and AERD.

  20. Liver in systemic disease

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Potential causes of abnormal liver function tests include viral hepatitis, alcohol intake, nonalcoholic fatty liver disease, autoimmune liver diseases, hereditary diseases, hepatobiliary malignancies or infection, gallstones and drug-induced liver injury. Moreover, the liver may be involved in systemic diseases that mainly affect other organs. Therefore, in patients without etiology of liver injury by screening serology and diagnostic imaging, but who have systemic diseases, the abnormal liver function test results might be caused by the systemic disease. In most of these patients, the systemic disease should be treated primarily. However, some patients with systemic disease and severe liver injury or fulminant hepatic failure require intensive treatments of the liver.

  1. Loss of interleukin-21 leads to atrophic germinal centers in multicentric Castleman's disease.

    Science.gov (United States)

    Yajima, Hidetaka; Yamamoto, Motohisa; Shimizu, Yui; Sakurai, Nodoka; Suzuki, Chisako; Naishiro, Yasuyoshi; Imai, Kohzoh; Shinomura, Yasuhisa; Takahashi, Hiroki

    2016-01-01

    Both multicentric Castleman's disease (MCD) and immunoglobulin (Ig)G4-related disease (IgG4-RD) are systemic diseases, presenting with hypergammaglobulinemia and elevated serum levels of IgG4. However, with regard to histopathological findings, MCD shows atrophic germinal centers. On the other hand, expanded germinal centers are detected in IgG4-RD. We extracted germinal centers from specimens of each disorder by microdissection and analyzed the expression of mRNAs by real-time polymerase chain reaction to clarify the mechanisms underlying atrophied germinal centers in MCD. This analysis disclosed loss of interleukin (IL)-21 and B cell lymphoma (Bcl)-6 in the germinal centers of MCD. Loss of IL-21 is considered to be involved in the disappearance of Bcl-6 and leads to atrophied germinal centers in MCD.

  2. A case of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA positive/IgG4-related lung disease

    Directory of Open Access Journals (Sweden)

    Hirokazu Touge

    2017-01-01

    Full Text Available IgG4-related lung disease (IgG4-RLD is a rare and chronic progressive autoimmune disease. We report a case of IgG4-related inflammatory pseudo-tumor of the lung that was seropositive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA. A 61-year-old male had a mass lesion in the right lower lung field in chest X-ray. Transbronchial lung biopsy resulted in a pathological diagnosis of IgG4-RLD. The condition was improved by hormonal therapy.

  3. IgG4相关性疾病累及腹部的临床、病理与影像学表现%Clinical, pathologic and imaging features of abdominal organ involvement in IgG4-related disease

    Institute of Scientific and Technical Information of China (English)

    钟小梅; 梁长虹

    2012-01-01

    IgG4相关性疾病是最近认知的以血清IgG4升高及IgG4+浆细胞广泛浸润多器官或组织为特征的慢性进行性自身免疫性疾病.该病常累及腹部器官组织,以自身免疫性胰腺炎最常见,某些器官组织受累时会出现一些特征性的影像学表现.该疾病对糖皮质激素治疗反应良好.

  4. DISEASE MANAGEMENT INFORMATION SYSTEM

    OpenAIRE

    Bens Pardamean; Anindito; Anjela Djoeang; Nana Tobing

    2013-01-01

    The study designed an information system model for Disease Management (DisMan) that met the specifications and needs of a consumer electronics manufacturer. The diseases monitored by this study were diabetes, hypertension and tuberculosis. Data were collected through interviews with the companyâs human resources department and occupational health provider. As for the model, literature and online research were conducted to collect health standards and information system standards on existing D...

  5. Psoriasis, a Systemic Disease?

    Directory of Open Access Journals (Sweden)

    Nilgün Atakan

    2012-09-01

    Full Text Available Psoriasis is a chronic inflammatory disease which is characterized by plaques with shiny white desquamation on the skin. It affects 1 to 3% of different ethnic populations. The disease significantly lowers the quality of life for the patients as the lesions appear on visible regions such as the scalp, face and extremities causing pruritus and extensive use of topical agents with a poor rate of recovery and the disease has a recurrent course with frequent attacks. Psoriasis was previously assumed to be a cutaneous disease resulting from epidermal cell hyperproliferation for a long time. However, studies conducted on the etiopathogenesis of the disease revealed that psoriasis is a chronic autoinflammatory disease which is caused by immune system dysregulation. Recently, the frequent association of psoriasis with other autoinflammatory diseases, comorbidities and complications which indeed shorten life expectancy concluded that psoriasis is a systemic disease and created a major difference in its treatment and follow-up modalities. In this review, the comorbidities, which are shown to be related to systemic inflammation and which also share a common pathogenesis with psoriasis, will be discussed. (Turk J Dermatol 2012; 6: 119-22

  6. A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

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    Kazuhiko Higashioka

    2015-01-01

    Full Text Available We report a case of IgG4-related disease (IgG4-RD with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

  7. Gene Disease Diagnostic System

    Institute of Scientific and Technical Information of China (English)

    黄国亮; 张腾飞; 程京; 周玉祥; 刘诚迅; 金国藩; 邬敏贤; 严瑛白; 杨蓉

    2002-01-01

    Binary optics, where the optical element can be fabricated on a thin glass plate with micro-ion-etching film layer, has been widely applied in recent years. A novel optical scanning system for gene disease diagnostics described in this paper has four kinds of optical devices, including beam splitters, an array lens, an array filter and detection arrays. A software was developed to design the binary optics system using an iterative method. Two beam splitters were designed and fabricated, which can divide a beam into a 9×9 array or into a 13×13 array. The beam splitters have good diffraction efficiencies (>70%) and an even energy distribution. The gene disease diagnostic system is a portable biochip and binary optics technology. The binary optical devices in the non-confocal scanning system can raise the fluorescence detection sensitivity of the micro-array hybrid biochip.

  8. Systemic manifestations of oral diseases

    OpenAIRE

    N Chaitanya Babu; Andrea Joan Gomes

    2011-01-01

    The oral cavity is the site of much infectious and inflammatory disease which has been associated with systemic diseases such as diabetes, cardiovascular disease and pre-term low births. This article emphasizes on the oral-systemic disease connection which is now a rapidly advancing area of research. The possible systemic diseases which arise from oral microorganisms are hereby focused.

  9. Hands in Systemic Disease

    Science.gov (United States)

    ... Z Videos Infographics Symptom Picker Anatomy Bones Joints Muscles Nerves Vessels Tendons About Hand Surgery What is a Hand Surgeon? What is ... nerves, skin and skin-related tissues, bones, and ... a systemic diseases. The hands may show changes noticed by the patient or ...

  10. Association between periodontal diseases and systemic diseases.

    Science.gov (United States)

    Weidlich, Patrícia; Cimões, Renata; Pannuti, Claudio Mendes; Oppermann, Rui Vicente

    2008-01-01

    Current evidence suggests that periodontal disease may be associated with systemic diseases. This paper reviewed the published data about the relationship between periodontal disease and cardiovascular diseases, adverse pregnancy outcomes, diabetes and respiratory diseases, focusing on studies conducted in the Brazilian population. Only a few studies were found in the literature focusing on Brazilians (3 concerning cardiovascular disease, 7 about pregnancy outcomes, 9 about diabetes and one regarding pneumonia). Although the majority of them observed an association between periodontitis and systemic conditions, a causal relationship still needs to be demonstrated. Further studies, particularly interventional well-designed investigations, with larger sample sizes, need to be conducted in Brazilian populations.

  11. Association between periodontal diseases and systemic diseases

    Directory of Open Access Journals (Sweden)

    Patrícia Weidlich

    2008-08-01

    Full Text Available Current evidence suggests that periodontal disease may be associated with systemic diseases. This paper reviewed the published data about the relationship between periodontal disease and cardiovascular diseases, adverse pregnancy outcomes, diabetes and respiratory diseases, focusing on studies conducted in the Brazilian population. Only a few studies were found in the literature focusing on Brazilians (3 concerning cardiovascular disease, 7 about pregnancy outcomes, 9 about diabetes and one regarding pneumonia. Although the majority of them observed an association between periodontitis and systemic conditions, a causal relationship still needs to be demonstrated. Further studies, particularly interventional well-designed investigations, with larger sample sizes, need to be conducted in Brazilian populations.

  12. AGRICULTURE DISEASE MITIGATION SYSTEM

    Directory of Open Access Journals (Sweden)

    Sion Hannuna

    2011-06-01

    Full Text Available Around 52% of the population of India rely on farming for their livelihood which accounts for 17% of India’s GDP. Whilst most farmers are familiar with conventional farming practices, they are often ill positioned to promptly deal with diseases and plant infestations affecting their crops. Current advisory systems tend to be generic and are not tailored to specific plots or farms. This work comprises an agriculture advisory call center similar to a modern call center to provide an agriculture disease mitigation system. The information regarding an individual farm is collected using mobile phones. The image of diseased/infected crop is also captured using mobile phones and is made available to the expert to provide the advisory. To scale the advisory, an attempt is also made to automate the disease recognition process using image processing. Unfortunately, the photos taken will be sensitive to a number of factors including camera type and lighting incident on the scene. Ideally, the images would be processed in such a way as to provide the expert with a visual representation of the affected crops that reflects the true nature of the scene. We describe a framework for standardising the colour of plant images taken using both mobile phones and compact cameras within the context of the advisory system.

  13. Case report of Mikulicz`s disease: A modern concept of an old entity

    Directory of Open Access Journals (Sweden)

    Božić Ksenija

    2016-01-01

    Full Text Available Introduction. Modern knowlegde defines Mikulicz´s disease as a part of immunoglobulin G4-related disease. The main feature is the presence of lymphoplasmacytic infiltrates, immunoglobulin G4 plasma cells positivity, distinctive storiform fibrosis and moderate eosinophilia. Case report. A 59-years old male presented with a mild keratoconjuctivitis sicca and enlarged lacrimal and salivary glands during the last two years. Althought clinical presentation of the patient was typical, earlier testing did not pinpoint Mikulicz ´s disease. By typical clinical presentation, elevated serum immunoglobulin G4 level and histopathological finding of lacrimal glands tissue we diagnosed Mikulicz´s disease successfully treated with corticosteroid therapy. Conclusion. We reported the first case of IgG4-related Mikulicz´s disease in Serbia. Our report highlights IgG4-related Mikulicz` s disease as an important differential diagnosis with Sjögren`s syndrome and lymphoproliferative disease in rheumatological practice.

  14. Acne and systemic disease.

    Science.gov (United States)

    Lolis, Margarita S; Bowe, Whitney P; Shalita, Alan R

    2009-11-01

    Acne is the most common disease of the skin. It affects 85% of teenagers, 42.5% of men, and 50.9% of women between the ages of 20 and 30 years.96,97 The role of hormones, particularly as a trigger of sebum production and sebaceous growth and differentiation, is well known. Excess production of hormones, specifically androgens, GH, IGF-1, insulin, CRH, and glucocorticoids, is associated with increased rates of acne development. Acne may be a feature in many endocrine disorders, including polycystic ovary disease, Cushing syndrome, CAH, androgen-secreting tumors, and acromegaly. Other nonendocrine diseases associated with acne include Apert syndrome, SAPHO syndrome, Behçet syndrome and PAPA syndrome. Acne medicamentosa is the development of acne vulgaris or an acneiform eruption with the use of certain medications. These medications include testosterone, progesterone,steroids, lithium, phenytoin, isoniazid, vitamins B2, B6, and B12, halogens, and epidermal growth factor inhibitors. Management of acne medicamentosa includes standard acne therapy. Discontinuation of the offending drug may be necessary in recalcitrant cases. Basic therapeutic interventions for acne include topical therapy, systemic antibiotics,hormonal agents, isotretinoin, and physical treatments. Generally, the severity of acne lesions determines the type of acne regimen necessary. The emergence of drug-resistant P acnes and adverse side effects are current limitations to effective acne management.

  15. Mucormycosis in systemic autoimmune diseases.

    Science.gov (United States)

    Royer, Mathieu; Puéchal, Xavier

    2014-07-01

    Mucormycosis is an emerging infection in systemic autoimmune diseases. All published cases of systemic autoimmune diseases complicated by mucormycosis were reviewed. The clinical features, diagnostic procedures and the main principles of treatment were analyzed. Twenty-four cases of mucormycosis have been reported in systemic auto-immune diseases, of which 83% in systemic lupus erythematosus, all occurring during immunosuppressants. In most cases, the infection was disseminated or rhinocerebral and it had mimicked a flare of the underlying connective tissue disease. A fatal outcome was reported in 58.3% of these patients. In conclusion, mucormycosis often mimics a flare of the underlying systemic disease and is associated with a high mortality rate. Systemic lupus erythematosus is by far the most common associated systemic autoimmune disease. A high degree of awareness is warranted to rapidly rule out infection, of which mucormycosis, in immunocompromised patients with systemic autoimmune disease before a disease flare is conclusively diagnosed.

  16. Immunoglobulin G4-related pancreatic and biliary diseases

    OpenAIRE

    Hisham Al-Dhahab; Julia McNabb-Baltar; Said Al-Busafi; Alan N Barkun

    2013-01-01

    BACKGROUND: Autoimmune pancreatitis and autoimmune cholangitis are new clinical entities that are now recognized as the pancreaticobiliary manifestations of immunoglobulin (Ig) G4-related disease.OBJECTIVE: To summarize important clinical aspects of IgG4-related pancreatic and biliary diseases, and to review the role of IgG4 in the diagnosis of autoimmune pancreatitis (AIP) and autoimmune cholangitis (AIC).METHODS: A narrative review was performed using the PubMed database and the following k...

  17. First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia.

    Science.gov (United States)

    Masutani, Hironori; Okuwaki, Kosuke; Kida, Mitsuhiro; Yamauchi, Hiroshi; Imaizumi, Hiroshi; Miyazawa, Shiro; Iwai, Tomohisa; Takezawa, Miyoko; Koizumi, Wasaburo

    2014-07-14

    To our knowledge, patients with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) associated with autoimmune hemolytic anemia (AIHA) have not been reported previously. Many patients with IgG4-SC have autoimmune pancreatitis (AIP) and respond to steroid treatment. However, isolated cases of IgG4-SC are difficult to diagnose. We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis. The patient was a 73-year-old man who was being treated for dementia. Liver dysfunction was diagnosed on blood tests at another hospital. Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis, but a rapidly progressing anemia developed simultaneously. After the diagnosis of AIHA, steroid treatment was begun, and the biliary stricture improved. IgG4-SC without AIP was thus diagnosed.

  18. Systemic Cat Scratch Disease

    Directory of Open Access Journals (Sweden)

    Hui-Min Liao

    2006-01-01

    Full Text Available Systemic cat scratch disease (CSD is often associated with prolonged fever and microabscesses in the liver and/or spleen. We report a case of systemic CSD with hepatic, splenic and renal involvement in an aboriginal child in Taiwan. A previously healthy 9-year-old girl had an intermittent fever for about 17 days, and complained of abdominal pain, headache and weight loss. Abdominal computed tomography showed multiple tiny hypodense nodular lesions in the spleen and both kidneys. Laparotomy revealed multiple soft, whitishtan lesions on the surface of the liver and spleen. Histopathologic examination of a biopsy specimen of the spleen showed necrotizing granulomatous inflammation with central necrosis surrounded by epithelioid cells and occasional Langhans' giant cells, strongly suggestive of Bartonella henselae infection. History revealed close contact with a cat. B. henselae DNA was detected by polymerase chain reaction in the tissue specimen, and the single antibody titer against B. henselae was greater than 1:2048. These results confirmed the diagnosis of visceral CSD caused by B. henselae. The patient's symptoms resolved after treatment with rifampin and tetracycline. This case illustrates the need for inclusion of systemic CSD in patients with fever of unknown origin and abdominal pain.

  19. Neuromuscular disease classification system

    Science.gov (United States)

    Sáez, Aurora; Acha, Begoña; Montero-Sánchez, Adoración; Rivas, Eloy; Escudero, Luis M.; Serrano, Carmen

    2013-06-01

    Diagnosis of neuromuscular diseases is based on subjective visual assessment of biopsies from patients by the pathologist specialist. A system for objective analysis and classification of muscular dystrophies and neurogenic atrophies through muscle biopsy images of fluorescence microscopy is presented. The procedure starts with an accurate segmentation of the muscle fibers using mathematical morphology and a watershed transform. A feature extraction step is carried out in two parts: 24 features that pathologists take into account to diagnose the diseases and 58 structural features that the human eye cannot see, based on the assumption that the biopsy is considered as a graph, where the nodes are represented by each fiber, and two nodes are connected if two fibers are adjacent. A feature selection using sequential forward selection and sequential backward selection methods, a classification using a Fuzzy ARTMAP neural network, and a study of grading the severity are performed on these two sets of features. A database consisting of 91 images was used: 71 images for the training step and 20 as the test. A classification error of 0% was obtained. It is concluded that the addition of features undetectable by the human visual inspection improves the categorization of atrophic patterns.

  20. Systemic abnormalities in liver disease

    Institute of Scientific and Technical Information of China (English)

    Masami Minemura; Kazuto Tajiri; Yukihiro Shimizu

    2009-01-01

    Systemic abnormalities often occur in patients with liver disease. In particular, cardiopulmonary or renal diseases accompanied by advanced liver disease can be serious and may determine the quality of life and prognosis of patients. Therefore, both hepatologists and non-hepatologists should pay attention to such abnormalities in the management of patients with liver diseases.

  1. [Systemic sclerosis: a multisystem disease

    NARCIS (Netherlands)

    Berrevoets, M.A.; Markhorst, J.; Meek, I.; Ede, A.E. van; Vonk, M.C.

    2014-01-01

    Systemic sclerosis is a rare, systemic autoimmune disease, characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. The disease is associated with a significantly increased morbidity and mortality, and can be rapidly progressive. Interstitial lung disease, renal hype

  2. Immunoglobulin G4-related disease presenting with obstructive jaundice

    Directory of Open Access Journals (Sweden)

    Yuan-Rung Li

    2014-06-01

    Full Text Available A 48-year-old male presented with diffuse abdominal fullness for 1 month and tea-colored urine for 10 days. Abdominal computed tomography/magnetic resonance cholangiopancreatography revealed diffuse enlargement of the pancreas and unusual soft tissue density around the left ureter. Endoscopic retrograde cholangiopancreatography demonstrated lumen narrowing of the distal common bile duct and irregularity of the pancreatic duct. Markedly elevated serum immunoglobulin G4 (IgG4 was also noted. Biopsy of soft tissue from the area surrounding the left ureter identified lymphoplasmacytic infiltration with high concentrations of IgG4-positive plasma cells accompanied by obliterative phlebitis, compatible with IgG4-related disease. The patient was administered steroid therapy and his symptoms improved. Clinicians should be aware of possible IgG4-related disease in a patient presenting with obstructive jaundice and diffuse pancreatic enlargement because glucocorticoid administration can achieve good response.

  3. The cornea in systemic disease

    Directory of Open Access Journals (Sweden)

    W.D.H. Gillan

    2005-01-01

    Full Text Available Systemic  disease  often  results  in  corneal signs that are easily observable by means of bio-microscopy. Optometry is in a unique position to detect the corneal manifestations of systemic disease. This review presents corneal signs of various systemic diseases. The list is not com-plete but the review rather attempts to empha-size the importance of detection, and referral, of systemic diseases.

  4. Periodontal disease and systemic complications

    Directory of Open Access Journals (Sweden)

    Rui Vicente Oppermann

    2012-01-01

    Full Text Available Periodontal diseases comprise a number of infectious and inflammatory conditions brought about by the interaction between supragingival and subgingival biofilms and the host inflammatory response. Periodontal diseases should be considered systemic conditions. This means that they are both modulated by the body's systems and play a role as a risk factor for systemic derangements. The current evidence supports some of these interactions, such as smoking as a risk factor for periodontal disease and diabetes mellitus, as both influenced by and influencing inflammatory changes in the periodontal tissue. Other potential associations are still being researched, such as obesity, hormonal changes, cardiovascular disease, and adverse outcomes in pregnancy. These, and others, still require further investigation before the repercussions of periodontal disease can be fully elucidated. Nevertheless, at the present time, the treatment of periodontal diseases-and, most importantly, their prevention-enables adequate intervention as a means of ensuring periodontal health.

  5. Periodontal disease and systemic complications.

    Science.gov (United States)

    Oppermann, Rui Vicente; Weidlich, Patricia; Musskopf, Marta Liliana

    2012-01-01

    Periodontal diseases comprise a number of infectious and inflammatory conditions brought about by the interaction between supragingival and subgingival biofilms and the host inflammatory response. Periodontal diseases should be considered systemic conditions. This means that they are both modulated by the body's systems and play a role as a risk factor for systemic derangements. The current evidence supports some of these interactions, such as smoking as a risk factor for periodontal disease and diabetes mellitus, as both influenced by and influencing inflammatory changes in the periodontal tissue. Other potential associations are still being researched, such as obesity, hormonal changes, cardiovascular disease, and adverse outcomes in pregnancy. These, and others, still require further investigation before the repercussions of periodontal disease can be fully elucidated. Nevertheless, at the present time, the treatment of periodontal diseases-and, most importantly, their prevention-enables adequate intervention as a means of ensuring periodontal health.

  6. [Corneal manifestations in systemic diseases].

    Science.gov (United States)

    Zarranz Ventura, J; De Nova, E; Moreno-Montañés, J

    2008-01-01

    Systemic diseases affecting the cornea have a wide range of manifestations. The detailed study of all pathologies that cause corneal alteration is unapproachable, so we have centered our interest in the most prevalent or characteristic of them. In this paper we have divided these pathologies in sections to facilitate their study. Pulmonar and conective tissue (like colagen, rheumatologic and idiopathic inflamatory diseases), dermatologic, cardiovascular, hematologic, digestive and hepatopancreatic diseases with corneal alteration are described. Endocrine and metabolic diseases, malnutrition and carential states are also studied, as well as some otorhinolaryngologic and genetic diseases that affect the cornea. Finally, a brief report of ocular toxicity induced by drugs is referred.

  7. Kidney in systemic diseases

    Directory of Open Access Journals (Sweden)

    Marta Patricia Casanova González

    2007-06-01

    Full Text Available Renal function alterant, sodium and water retention with intravascular volume expansion, and augmentation of the cardiac preload are closely related to the main causes of mortality and morbidity in patients suffering from symptomatic cardiac insufficiency. A review of the renal function alterant in congestive cardiac insufficiency and chronic liver diseases is carried out in this bibliographical revision. Important therapeutic aspects of the patients suffering from cardiac insufficiency considering its renal function alterant are also presented.

  8. The complement system in systemic autoimmune disease

    NARCIS (Netherlands)

    Chen, Min; Daha, Mohamed R.; Kallenberg, Cees G. M.

    2010-01-01

    Complement is part of the innate immune system. Its major function is recognition and elimination of pathogens via direct killing and/or stimulation of phagocytosis. Activation of the complement system is, however, also involved in the pathogenesis of the systemic autoimmune diseases. Activation via

  9. Movement disorders in systemic diseases.

    Science.gov (United States)

    Poewe, Werner; Djamshidian-Tehrani, Atbin

    2015-02-01

    Movement disorders, classically involving dysfunction of the basal ganglia commonly occur in neurodegenerative and structural brain disorders. At times, however, movement disorders can be the initial manifestation of a systemic disease. In this article we discuss the most common movement disorders which may present in infectious, autoimmune, paraneoplastic, metabolic and endocrine diseases. Management often has to be multidisciplinary involving primary care physicians, neurologists, allied health professionals including nurses, occupational therapists and less frequently neurosurgeons. Recognizing and treating the underlying systemic disease is important in order to improve the neurological symptoms.

  10. [Autoimmune Diseases of Digestive System].

    Science.gov (United States)

    Ivashkinl, V T; Sheptulina, A F; Raĭkhelson, K L; Losik, E A; Ivashkin, K V; Okhlobystin, A V; Baranskaia, E K; Polouvektova, E A; Shifrin, O S

    2015-01-01

    Autoimmune diseases of digestive system refer to pathological conditions, caused by autoimmune mechanisms, and their etiology remains unknown. This is a group of relatively rare diseases, however, during the last years a marked tendency towards the raise in incidence andprevalence is observed, which led to an increase in number of clinical investigations on etiology, pathogenesis, and, accordingly, development of new diagnostic methods and therapies. Results of such trials shown, for example, that the pathogenesis of chronic cholestatic liver diseases is associated with nuclear receptors function, while the main etiological and pathogenic factor of inflammatory bowel diseases represents gut microbiota. Despite new achievements in autoinmune diseases of digestive system research, therapies are low effective and are accompanied by a huge number of adverse events. The fact that these diseases may lead to malignant tumors is also worth noting. For example, patients with primary sclerosing cholangitis have a 160 times higher risk of cholangiocellular carcinoma, while 10-14% ofpatients with celiac disease may develop malignancies of esophagus, small and large intestine. Thus, these diseases require further investigation with a purpose of more accurate diagnostic methods for the detection of disease at early stages and new effective and safe therapies development.

  11. Serum total IgG and IgG4 levels in thyroid eye disease

    Directory of Open Access Journals (Sweden)

    Sy A

    2016-10-01

    Full Text Available Aileen Sy, Rona Z Silkiss Department of Ophthalmology, California Pacific Medical Center, San Francisco, CA, USA Purpose: To investigate the relationship between immunoglobulin G (IgG4-related disease (IgG4-RD and thyroid eye disease (TED with respect to IgG levels. Patients and methods: A retrospective review of total IgG, IgG subclass, and thyroid stimulating immunoglobulin (TSI levels in 24 patients with TED. Results: Five patients (20.8% demonstrated serum IgG4 levels consistent with IgG4-RD without any additional systemic disease. Total IgG and IgG subclass levels were found to be an inadequate proxy for TSI elevation. Conclusion: There may be a subtype of TED patients with elevated IgG4 in the absence of IgG4-RD systemic findings. Keywords: thyroid eye disease, IgG subclass, IgG4, Graves’ disease, Graves’ ophthalmopathy, IgG4-RD

  12. Immunoglobulin G4 -related kidney disease: A comprehensive pictorial review of the imaging spectrum, Mimickers, and clinicopathological characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Ni Eun; Kim, Jin Hee; Byun, Jae Ho; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon Gyu [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2015-10-15

    Immunoglobulin G4 (IgG4)-related kidney disease (IgG4-KD) has recently been demonstrated to be an important part of IgG4-related sclerosing disease (IgG4-SD). However, since IgG4-KD is still relatively unfamiliar to radiologists and physicians as compared to IgG4-SD involving other organs, it could, therefore, be easily missed. In this article, we present a comprehensive pictorial review of IgG4-KD with regards to the imaging spectrum, mimickers, and clinicopathologic characteristics, based on our clinical experience with 48 patients during the past 13 years, as well as a literature review. Awareness of the broad imaging spectrum of IgG4-KD and differential diagnosis from its mimickers will thus facilitate its early diagnosis and treatment.

  13. Hyalinizing cholecystitis with features of immunoglobulin G4-related disease-coincidence or an unrecognized association? A case report.

    Science.gov (United States)

    Gupta, Rajib K; Patton, Kurt T

    2015-04-01

    Hyalinizing cholecystitis (HC) is a recently described rare subtype of chronic cholecystitis characterized by dense, paucicellular collagenous transmural fibrosis, which usually replaces the mucosa and muscularis propria. Immunoglobulin (Ig)G4-associated cholecystitis is also a newly described cholecystitis variant characterized by transmural or extramural lymphoplasmacytic inflammation, lymphoid follicles, storiform fibrosis, phlebitis, and increased tissue IgG4-positive plasma cells. We describe a case of cholecystitis in an elderly white man who harbored features of both HC and IgG4-associated cholecystitis. In retrospect, the patient also had a significantly elevated serum IgG4 level. To the best of our knowledge, an association between HC and IgG4-related disease has not been previously described in the literature. Although not entirely conclusive, our observations raise the possibility that some cases of HC represent the end stage of IgG4-related disease.

  14. Systemic diseases with cutaneous manifestations.

    Science.gov (United States)

    Merchant, S R; Taboada, J

    1995-07-01

    The purpose of this article is to briefly discuss the following cutaneous manifestations of selected systemic diseases: poxvirus; feline leukemia virus (FeLV); feline immunodeficiency virus (FIV); herpesvirus; calcivirus; pseudorabies; plague; tularemia; toxoplasmosis; leishmania; hypothyroidism; hyperthyroidism; hyperadrenocorticism; diabetes mellitus; acromegaly; thallium poisoning; pancreatic disease; hypereosinophilic syndrome; mucopolysaccharidosis; and pansteatitis. Recognition of these cutaneous signs may help alert the clinician to the possibility of an internal disorder so that the appropriate diagnostic tests can be considered.

  15. Complement system in lung disease.

    Science.gov (United States)

    Pandya, Pankita H; Wilkes, David S

    2014-10-01

    In addition to its established contribution to innate immunity, recent studies have suggested novel roles for the complement system in the development of various lung diseases. Several studies have demonstrated that complement may serve as a key link between innate and adaptive immunity in a variety of pulmonary conditions. However, the specific contributions of complement to lung diseases based on innate and adaptive immunity are just beginning to emerge. Elucidating the role of complement-mediated immune regulation in these diseases will help to identify new targets for therapeutic interventions.

  16. Systemic diseases and the elderly.

    LENUS (Irish Health Repository)

    McCreary, Christine

    2010-11-01

    Although systemic diseases can occur at any age, they are more common in older patients. Accurate and detailed medical and drug histories are important in dental practice as many conditions and medications can influence oral health and dental care in patients. Not only can these conditions influence patient care in the surgery and oral hygiene at home, but access to dental services may also be adversely affected. Clinical Relevance: The systemic diseases can impact upon oral care or can have oral manifestations. Many of the pharmacological interventions prescribed for chronic conditions can have multiple and diverse adverse effects on the oral environment.

  17. Dermatologic Manifestations of Systemic Diseases.

    Science.gov (United States)

    Valdez, Maryn Anne; Isamah, Nwamaka; Northway, Rebecca M

    2015-12-01

    Dermatologic complaints are encountered frequently by the primary care provider. Patients often are required as well as want to see their primary care provider before referral to a specialist. Therefore, primary care providers must be skilled in a variety of topics including dermatology. Certain dermatologic manifestations are associated with, or indicative of, systemic diseases. Primary care providers must be knowledgeable in diagnosis, evaluation, and treatment of dermatologic conditions, as well as when to appropriately refer. This article reviews common dermatologic manifestations of systemic diseases. Copyright © 2015 Elsevier Inc. All rights reserved.

  18. The complement system in systemic autoimmune disease.

    Science.gov (United States)

    Chen, Min; Daha, Mohamed R; Kallenberg, Cees G M

    2010-05-01

    Complement is part of the innate immune system. Its major function is recognition and elimination of pathogens via direct killing and/or stimulation of phagocytosis. Activation of the complement system is, however, also involved in the pathogenesis of the systemic autoimmune diseases. Activation via the classical pathway has long been recognized in immune complex-mediated diseases such as cryoglobulinemic vasculitis and systemic lupus erythematosus (SLE). In SLE, the role of complement is somewhat paradoxical. It is involved in autoantibody-initiated tissue damage on the one hand, but, on the other hand, it appears to have protective features as hereditary deficiencies of classical pathway components are associated with an increased risk for SLE. There is increasing evidence that the alternative pathway of complement, even more than the classical pathway, is involved in many systemic autoimmune diseases. This is true for IgA-dominant Henoch Schönlein Purpura, in which additional activation of the lectin pathway contributes to more severe disease. In anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis the complement system was considered not to be involved since immunoglobulin deposition is generally absent in the lesions. However, recent studies, both in human and animal models, demonstrated complement activation via the alternative pathway as a major pathogenic mechanism. Insight into the role of the various pathways of complement in the systemic autoimmune diseases including the vasculitides opens up new ways of treatment by blocking effector pathways of complement. This has been demonstrated for monoclonal antibodies to C5 or C5a in experimental anti-phospholipid antibody syndrome and ANCA-associated vasculitis.

  19. An aortoduodenal fistula as a complication of immunoglobulin G4-related disease

    Institute of Scientific and Technical Information of China (English)

    Momir Sarac; Ivan Marjanovic; Mihailo Bezmarevic; Uros Zoranovic; Stanko Petrovic; Miodrag Mihajlovic

    2012-01-01

    Most primary aortoduodenal fistulas occur in the presence of an aortic aneurysm,which can be part of immunoglobulin G4 (IgG4)-related sclerosing disease.We present a case who underwent endovascular grafting of an aortoduodenal fistula associated with a high serum IgG4 level.A 56-year-old male underwent urgent endovascular reconstruction of an aortoduodenal fistula.The patient received antibiotics and other supportive therapy,and the postoperative course was uneventful,however,elevated levels of serum IgG,IgG4 and C-reactive protein were noted,which normalized alter the introduction of steroid therapy.Control computed tomography angiography showed no endoleaks.The primary aortoduodenal fistula may have been associated with IgG4-related sclerosing disease as a possible complication of IgG4-related inflammatory aortic aneurysm.Endovascular grafting of a primary aortoduodenal fistula is an effective and minimally invasive alternative to standard surgical repair.

  20. Disease assessment in systemic vasculitis.

    Science.gov (United States)

    Luqmani, Raashid Ahmed

    2015-04-01

    The anti-neutrophil cytoplasm antibody-associated vasculitides are complex multi-system disorders with many overlapping clinical features. Their outcome has been transformed by effective immunosuppression, preventing death in over 70% of cases. The quality of survival is affected by the disease course, which is characterized by a significant likelihood of relapse in 38%, chronic effects from the disease and its treatment, as well as emerging or worsening comorbidity, all of which contribute to the patient's clinical condition and outcome. Whilst imaging and laboratory testing including histology are important aspects of diagnosis, they are of limited value in assessing response to therapy or subsequent disease course. We have developed standardized validated clinical methods to quantify disease activity and damage; we are developing effective measures of patient experience to complement these procedures. This approach provides a rational basis for clinical management as well as being essential in the conduct of clinical trials and studies in vasculitis, by providing reproducible definitions of relapse, remission and response to therapy for patients with systemic vasculitis. Clinical assessment remains the current gold standard for evaluating disease progress, but requires regular training to ensure standardization. The development of biomarkers in future may produce a more accurate description of disease and identify potential targets for therapy as well as predictors of response to drugs.

  1. Breast manifestations of systemic diseases

    Directory of Open Access Journals (Sweden)

    Dilaveri CA

    2012-02-01

    Full Text Available Christina A Dilaveri, Maire Brid Mac Bride, Nicole P Sandhu, Lonzetta Neal, Karthik Ghosh, Dietlind L Wahner-RoedlerDivision of General Internal Medicine, Mayo Clinic, Rochester, MN, USAAbstract: Although much emphasis has been placed on the primary presentations of breast cancer, little focus has been placed on how systemic illnesses may affect the breast. In this article, we discuss systemic illnesses that can manifest in the breast. We summarize the clinical features, imaging, histopathology, and treatment recommendations for endocrine, vascular, systemic inflammatory, infectious, and hematologic diseases, as well as for the extramammary malignancies that can present in the breast. Despite the rarity of these manifestations of systemic disease, knowledge of these conditions is critical to the appropriate evaluation and treatment of patients presenting with breast symptoms.Keywords: breast, endocrine, hematologic, infectious, vascular

  2. Parkinson's Disease and Systemic Inflammation

    Science.gov (United States)

    Ferrari, Carina C.; Tarelli, Rodolfo

    2011-01-01

    Peripheral inflammation triggers exacerbation in the central brain's ongoing damage in several neurodegenerative diseases. Systemic inflammatory stimulus induce a general response known as sickness behaviour, indicating that a peripheral stimulus can induce the synthesis of cytokines in the brain. In Parkinson's disease (PD), inflammation was mainly associated with microglia activation that can underlie the neurodegeneration of neurons in the substantia nigra (SN). Peripheral inflammation can transform the “primed” microglia into an “active” state, which can trigger stronger responses dealing with neurodegenerative processes. Numerous evidences show that systemic inflammatory processes exacerbate ongoing neurodegeneration in PD patient and animal models. Anti-inflammatory treatment in PD patients exerts a neuroprotective effect. In the present paper, we analyse the effect of peripheral infections in the etiology and progression in PD patients and animal models, suggesting that these peripheral immune challenges can exacerbate the symptoms in the disease. PMID:21403862

  3. The relation of periodontal diseases to systemic diseases

    Directory of Open Access Journals (Sweden)

    Melanie Sadono Djamil

    2008-12-01

    Full Text Available Background: The relationship between systemic disorders and periodontal disease has been studied extensively. With few exceptions, it is more accurate to consider systemic diseases to be contributing factors in the pathogenesis of periodontal disease rather than the primary etiologic factors. The development of periodontal disease cannot be separated from the weakening of immunologic and immunopathological responses. Periodontal disease may enhance susceptibility to certain systemic diseases in several ways. Lipopolysaccharide (LPS and Gram-positive bacteria in the biofilm and proinflammatory cytokines produced from inflamed periodontal tissues may enter the circulation system causing the development of certain systemic diseases. On the other hand, through immunologic mediators, certain systemic disease may enhance susceptibility to periodontal disease caused by the decrease of immune responses and the increase of proinflammatory cytokines. Purpose: The Purpose of this article is to review the immunologic aspect of two way relationship between systemic diseases and periodontal diseases. Review: This review studied the relationship between general health status, systemic diseases, and periodontal diseases through immunopathological responses and the weakening of the immune system in the periodontal tissue. Conclusion: there is a two-way relationship between periodontal diseases and systemic diseases.

  4. Relationship between chronic sclerosing dacryoadenitis with high level of IgG4 and Castleman disease

    Directory of Open Access Journals (Sweden)

    Toshiyuki Oshitari

    2010-12-01

    Full Text Available Toshiyuki Oshitari1, Jiro Yotsukura1, Kaoru Asahagi1, Takayuki Baba1, Takashi Kishimoto2, Shuichi Yamamoto11Department of Ophthalmology and Visual Science, 2Department of Molecular Pathology, Chiba University Graduate School of Medicine, Chiba, JapanAbstract: The purpose of this study is to present a case of chronic sclerosing dacryoadenitis with high level of IgG4 in a patient diagnosed earlier with Castleman disease. A 79-year-old man noticed a swelling of his lower left jaw that was first seen 8 years earlier. He was diagnosed with Castleman disease from the histopathological examination of a biopsy of the submandibular gland. Since then, the size of the gland had not changed, and he had no systemic inflammatory signs or symptoms. He developed diplopia a year earlier, and CT scans showed bilateral swelling of the lacrimal glands. He was referred to our hospital for further examinations. The patient underwent partial dacryoadenectomy. From the histopathological examinations, he was diagnosed with chronic sclerosing dacryoadenitis with high level of the serum IgG4. He underwent oral steroid therapy and the swollen lacrimal glands were significantly improved. The results suggest that there may be pathological links between IgG4-related dacryoadenitis and Castleman disease.Keywords: IgG4, dacryoadenitis, Castleman disease, steroid

  5. Idiopathic Mediastinal Fibrosis: a Systemic Immune-Mediated Disorder. A Case Series and a Review of the Literature.

    Science.gov (United States)

    Rossi, Giovanni M; Emmi, Giacomo; Corradi, Domenico; Urban, Maria L; Maritati, Federica; Landini, Federica; Galli, Paola; Palmisano, Alessandra; Vaglio, Augusto

    2017-06-01

    Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 2006-2016. The purposes of our literature search were to assess the frequency of the association between IMF and other immune-mediated disorders and to analyse which disorders most often coexist with IMF. Of our nine IMF cases, one was associated with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, one with large-vessel arteritis, three with idiopathic retroperitoneal fibrosis (one of which was IgG4-related), one with pancreatitis and one with IgG4-related seminal vesicle involvement. The remaining two cases, in which IMF was not associated with any other disease, were both classifiable as IgG4-related. The literature review showed that, of the 84 IMF cases identified, 27 (32 %) were associated with other idiopathic autoimmune or fibro-inflammatory disorders, particularly small-vessel vasculitis, Behçet disease, retroperitoneal fibrosis and other conditions belonging to the IgG4-related disease spectrum. Based on our own data and the literature review, we conclude that IMF is often associated with other autoimmune or fibro-inflammatory diseases; therefore, its clinical management requires an accurate screening of associated conditions. Immune-mediated mechanisms may be shared by these disorders.

  6. Automated Periodontal Diseases Classification System

    Directory of Open Access Journals (Sweden)

    Aliaa A. A. Youssif

    2012-01-01

    Full Text Available This paper presents an efficient and innovative system for automated classification of periodontal diseases, The strength of our technique lies in the fact that it incorporates knowledge from the patients' clinical data, along with the features automatically extracted from the Haematoxylin and Eosin (H&E stained microscopic images. Our system uses image processing techniques based on color deconvolution, morphological operations, and watershed transforms for epithelium & connective tissue segmentation, nuclear segmentation, and extraction of the microscopic immunohistochemical features for the nuclei, dilated blood vessels & collagen fibers. Also, Feedforward Backpropagation Artificial Neural Networks are used for the classification process. We report 100% classification accuracy in correctly identifying the different periodontal diseases observed in our 30 samples dataset.

  7. Rehabilitation in vestibular system diseases

    Directory of Open Access Journals (Sweden)

    Maksim Valeryevich Zamergrad

    2013-01-01

    Full Text Available Vestibular rehabilitation is an important component of combination treatment in a patient with vertigo. Vestibular rehabilitation is indicated for different diseases of the central or peripheral vestibular system. The goal of vestibular rehabilitation is to ensure gaze stabilization, to train postural stability, and to reduce subjective vertigo. Vestibular rehabilitation is based on the stimulation of vestibular adaptation, sensory substitution, and habituation. Vestibular suppressants, inadequate mobility, anxiety, and depression decelerate vestibular compensation whereas early activation, mobility, and betaserc use accelerate it.

  8. Ischemic heart disease in systemic inflammatory diseases. An appraisal.

    Science.gov (United States)

    Gargiulo, Paola; Marsico, Fabio; Parente, Antonio; Paolillo, Stefania; Cecere, Milena; Casaretti, Laura; Pellegrino, Angela Maria; Formisano, Tiziana; Fabiani, Irma; Soricelli, Andrea; Trimarco, Bruno; Perrone-Filardi, Pasquale

    2014-01-01

    Systemic inflammatory diseases are inflammatory syndromes that are associated with increased cardiovascular morbidity and mortality. The link between inflammatory and cardiovascular diseases can be attributed to coexistence of classical risk factors and of inflammatory mechanisms activated in systemic inflammatory diseases and involving the immune system. Yet, clinical implications of these findings are not entirely clear and deeper knowledge and awareness of cardiac involvement in inflammatory diseases are necessary. The aims of this review are to summarize cardiac involvement in systemic inflammatory diseases and to identify areas where evidence is currently lacking that deserve further investigation in the future.

  9. Serum IgG subclasses in autoimmune diseases.

    Science.gov (United States)

    Zhang, Haoze; Li, Ping; Wu, Di; Xu, Dong; Hou, Yong; Wang, Qian; Li, Mengtao; Li, Yongzhe; Zeng, Xiaofeng; Zhang, Fengchun; Shi, Qun

    2015-01-01

    To characterize serum IgG subclass levels in several autoimmune diseases, including primary Sjogren syndrome (pSS), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and primary biliary cirrhosis (PBC). We aimed to analyze serum IgG subclass distribution and to test whether serum IgG4 levels are elevated in these diseases. Serum IgG subclass levels from 102 pSS, 102 SSc, 100 SLE, and 59 PBC patients, as well as 40 healthy controls (HCs), were measured using the immunonephelometric assay. The distribution of IgG subclasses among these autoimmune diseases was analyzed. In this cross-sectional study, serum IgG1 (IgG1/IgG) and/or IgG3 (IgG3/IgG) were significantly increased, compared with those in HCs. Only 6.34% of patients had levels of serum IgG4 >135 mg/dL. There were no significant differences in the frequency of elevated serum IgG4 levels between patients and HC. In pSS, serum IgG1 levels were much higher than those in other disease groups, whereas serum IgG2 and IgG3 levels were most prominently increased in PBC. A strikingly different serum IgG subclass distribution was detected in patients with autoimmune diseases compared with HCs. Serum IgG subclass levels also showed distinct characteristics among different autoimmune diseases. Serum IgG4 levels in these patients were lower or not much higher than those in HCs, which differed from IgG4-related diseases.

  10. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis.

    Science.gov (United States)

    Kato, Takehiro; Komori, Atsumasa; Bae, Sung-Kwan; Migita, Kiyoshi; Ito, Masahiro; Motoyoshi, Yasuhide; Abiru, Seigo; Ishibashi, Hiromi

    2012-01-14

    Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4(+) plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4(+) plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

  11. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis

    Institute of Scientific and Technical Information of China (English)

    Takehiro Kato; Atsumasa Komori; Sung-Kwan Bae; Kiyoshi Migita; Masahiro Ito; Yasuhide Motoyoshi; Seigo Abiru; Hiromi Ishibashi

    2012-01-01

    Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-yearold Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4+ plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4+ plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

  12. TOWARDS MODELING DISEASE OUTBREAK NOTIFICATION SYSTEMS

    Directory of Open Access Journals (Sweden)

    Farag Azzedin

    2014-06-01

    Full Text Available Disease outbreak detection, monitoring and notification systems play an important role in assessing threats to public health since disease outbreaks are becoming increasingly common world-wide. There are several systems in use around the world, with coverage of national, international and global disease outbreaks. These systems use different taxonomies and classifications for the detection and prioritization of potential disease outbreaks. In this paper, we study and analyze the current disease outbreak systems. Subsequently, we extract features and functions of typical and generic disease outbreak systems. We then propose a generic model for disease outbreak notification systems. Our effort is directed towards standardizing the design process for typical disease outbreak systems.

  13. [Immunoglobulin G4-associated to multiorganic lymphoproliferative disease].

    Science.gov (United States)

    Bourlon, María T; Chapa, Mónica; Chablé Montero, Fredy; Hernández Calleros, Jorge

    2011-01-01

    We report a case of a woman with lymphoproliferative multiorganic immunoglobulin G4 (IgG4) related disease with extensive involvement showing dacryoadenitis, sialoadenitis, parotiditis, pancreatitis, pneumonitis, lymphadenopathy and immune thrombocytopenic purpura. Serum elevation of acute phase reactant, polyclonal hypergammaglobulinemia, positivity for antinuclear antibodies and rheumatoid factor was found. Hystologically plasma cell infiltration was demonstrated on glandular and lymphatic tissue and immunochemistry was positive for IgG4 in > 30%. Immunosuppressive treatment with steroids and azathioprine was given with an excellent clinical response, the marked radiologic evidence of improvement and the decrease in inflammatory makers that conducted to symptom remission are shown in the text.

  14. 1例IgG4相关硬化性疾病临床病理分析及文献复习%IgG4-related sclerosing disease:A case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    荣娜; 魏日胞; 王远大

    2011-01-01

    Objective To report a rare case of IgG4- related sclerosing disease involving multiple organs in order to improve our understanding of it. Methods A rare case of IgG4- related sclerosing disease admitted to our hospital in November 2010 with its diagnosis established by pathology was analyzed and its clinical manifestations, diagnosis, treatment, and prognosis were discussed according to its domestic and foreign literature. Results The patient's clinical manifestations included diabetes mellitus, impaired renal function, enlargement of lymph nodes in the neck and groin, high IgG and IgG4 level, and enlargement of pancreas. Abdominal CT showed enlargement of pancreas and interstitial pneumonia. Biopsy of lymph nodes revealed infiltration of IgG4 -positive plasma cells. Biopsy of kidney tissue displayed focal sclerosing glomerulonephritis. The renal function became normal and the size of pancreas and lymph nodes was decreased 3 weeks after treatment with corticosteroids. The serum IgG and IgE levels returned to normal and diabetes mellitus was well controlled 20 weeks after treatment with corticosteroids. Conclusion IgG4-related sclerosing disease is a very rare systemic disease, which can be controlled with prompt use of corticosteroids.%目的 报告1例罕见的IgG4相关硬化性疾病多器官受累病例,提高该病的认识水平.方法 分析我院2010年11月诊治并经病理学确诊的IgG4相关硬化性疾病1例,并结合国内外文献,对本病的临床表现、诊断、治疗及预后进行分析讨论.结果 患者临床表现为糖尿病、肾功能受损,颈部、腹股沟等全身淋巴结肿大;血清IgG、IgG4异常增高,CT检查胰腺肿大、间质性肺炎,淋巴结活检可见IgG4阳性浆细胞浸润,肾脏活检病理为局灶硬化性肾小球肾炎;激素治疗3周肾功能正常,胰腺及淋巴结形态明显缩小,20周血IgG、IgE正常,糖尿病控制良好.结论 IgG4相关硬化性疾病是一种非常罕见的全身系统

  15. Oral infections and systemic diseases

    DEFF Research Database (Denmark)

    Holmstrup, Palle; Poulsen, Anne Havemose; Andersen, Lone

    2003-01-01

    An association between periodontal infection and CVD has been revealed in some epidemiologic studies, whereas other studies were unable to demonstrate such an association. A link between the two diseases may be explained by shared established or nonestablished risk factors. Future studies with ex...... of a number of other diseases including DM and rheumatoid arthritis have been associated wish periodontitis, but more research is necessary to elucidate possible pathogenic interactions....

  16. Oral infections and systemic diseases

    DEFF Research Database (Denmark)

    Holmstrup, Palle; Poulsen, Anne Havemose; Andersen, Lone

    2003-01-01

    An association between periodontal infection and CVD has been revealed in some epidemiologic studies, whereas other studies were unable to demonstrate such an association. A link between the two diseases may be explained by shared established or nonestablished risk factors. Future studies...... of a number of other diseases including DM and rheumatoid arthritis have been associated wish periodontitis, but more research is necessary to elucidate possible pathogenic interactions....

  17. Expert System For Diagnosis Of Skin Diseases

    Directory of Open Access Journals (Sweden)

    A.A.L.C. Amarathunga

    2015-01-01

    Full Text Available Abstract Dermatology is a one of major session of medicine that concerned with the diagnosis and treatment of skin diseases. Skin diseases are the most common form of disease in humans. Recently many of researchers have advocated and developed the imaging of human vision or in the loop approach to visual object recognition. This research paper presents a development of a skin diseases diagnosis system which allows user to identify diseases of the human skin and to provide advises or medical treatments in a very short time period. For this purpose user will have to upload an image of skin disease to our system and answer questions based on their skin condition or symptoms. It will be used to detect diseases of the skin and offer a treatment recommendation. This system uses technologies such as image processing and data mining for the diagnosis of the disease of the skin. The image of skin disease is taken and it must be subjected to various preprocessing for noise eliminating and enhancement of the image. This image is immediately segmentation of images using threshold values. Finally data mining techniques are used to identify the skin disease and to suggest medical treatments or advice for users. This expert system exhibits disease identification accuracy of 85 for Eczema 95 for Impetigo and 85 for Melanoma.

  18. Disease severity scoring systems in dermatology

    Directory of Open Access Journals (Sweden)

    Cemal Bilaç

    2016-06-01

    Full Text Available Scoring systems have been developed to interpret the disease severity objectively by evaluating the parameters of the disease. Body surface area, visual analogue scale, and physician global assessment are the most frequently used scoring systems for evaluating the clinical severity of the dermatological diseases. Apart from these scoring systems, many specific scoring systems for many dermatological diseases, including acne (acne vulgaris, acne scars, alopecia (androgenetic alopecia, tractional alopecia, bullous diseases (autoimmune bullous diseases, toxic epidermal necrolysis, dermatitis (atopic dermatitis, contact dermatitis, dyshidrotic eczema, hidradenitis suppurativa, hirsutismus, connective tissue diseases (dermatomyositis, skin involvement of systemic lupus erythematosus (LE, discoid LE, scleroderma, lichen planoplaris, mastocytosis, melanocytic lesions, melasma, onychomycosis, oral lichen planus, pityriasis rosea, psoriasis (psoriasis vulgaris, psoriatic arthritis, nail psoriasis, sarcoidosis, urticaria, and vitiligo, have also been developed. Disease severity scoring methods are ever more extensively used in the field of dermatology for clinical practice to form an opinion about the prognosis by determining the disease severity; to decide on the most suitable treatment modality for the patient; to evaluate the efficacy of the applied medication; and to compare the efficiency of different treatment methods in clinical studies.

  19. The microbiome systemic diseases connection

    NARCIS (Netherlands)

    van der Meulen, T.A.; Harmsen, H J M; Bootsma, H; Spijkervet, F K L; Kroese, F G M; Vissink, A

    2016-01-01

    The human microbiome consists of all microorganisms occupying the skin, mucous membranes and intestinal tract of the human body. The contact of the mucosal immune system with the human microbiome is a balanced interplay between defence mechanisms of the immune system and symbiotic or pathogenic micr

  20. ORAL BACTERIA AND SYSTEMS DISEASES: A REVIEW

    OpenAIRE

    Moromi Nakata, Hilda; Profesor Principal de Microbiología, jefe de la sección de C. Dinámicas. D.A. Ciencia Básicas. Miembro permanente del Instituto de Investigaciones Estomatológicas de la Facultad de Odontología de la Universidad Nacional Mayor de San Marcos. Lima. Perú.

    2014-01-01

    In order to show a global vision of oral bacteria in systemic diseases, it is important to analyze the presence and consequences of these microorganisms in relation with: bacteremia, endocarditis, cardiovascular disease, cerebrovascular disease, bacterial pneumonia, neonatal weight, nefritis, arthritis, dermatitis and diabetes mellitus, reaching conclusions for each one of them. Con el objeto de presentar una visión general de la bacterias orales en los procesos sistémicos, se analiza la p...

  1. Disease Recording Systems and Herd Health Schemes for Production Diseases

    Directory of Open Access Journals (Sweden)

    Østerås O

    2001-03-01

    Full Text Available Disease recording of cattle is compulsory in Sweden and Norway. Sweden and Denmark also have mandatory disease recording for swine, whereas Finland and Norway only have compulsory recording of infectious diseases. Both compulsory and voluntary systems are databased, the first ones developed in the 1970's. Disease recording at pig slaughtering is somewhat older. The veterinary practitioner, and often also the farmer, can report treated cases as well as fertility disturbances to the systems. Disease recording at slaughter is carried out by veterinarians and inspection officers. The databases are handled by the veterinary authorities or the agricultural organisations in each country. Costs are defrayed by the authorities and/or the agricultural industry. The farmers receive periodic reports. Data are stored for three to ten years, often longer. Affiliation to animal health schemes for cattle or swine is voluntary. In Sweden and Denmark (cattle they are run within the scope of government regulations. Affiliation to animal health programmes may also be demanded by organisations within the agricultural industry. These organisations are also responsible for the administration of the programmes. Costs to take part in herd health schemes are covered by the farmers themselves. In certain cases, grants are received from agricultural organisations, authorities, or the European Union. Recording of diseases and the format of animal health schemes in the Nordic countries are described here in order to illustrate the possibilities to compare data between countries.

  2. Systemic mastocytosis associated with minimal change disease

    Directory of Open Access Journals (Sweden)

    Tony Amin

    2016-07-01

    Full Text Available Systemic Mastocytosis (SM has not previously been reported in association with minimal change disease (MCD. Mastocytosis is a rare myeloproliferative disease that is characterised by uncontrolled proliferation of aberrant mast cells. This can lead to a wide variety of symptoms and can present as either a cutaneous or a systemic disease. Systemic manifestations usually include bone marrow, intestine, liver and splenic infiltration of mast cells, with reports of renal manifestations being rare. This is a case report of a 70-year-old man who was known to have Systemic Mastocytosis and who presented with nephrotic range proteinuria. Renal biopsy diagnosed Minimal Change Disease with mast cell infiltration being an identified by C-kit staining. The patient was treated with steroids and is currently in remission of the proteinuria.

  3. Systemic lupus erythematosus: Is it one disease?

    Science.gov (United States)

    Rivas-Larrauri, Francisco; Yamazaki-Nakashimada, Marco Antonio

    2016-01-01

    Systemic lupus erythematosus (SLE) is a multisystemic disease with a variety of clinical presentations. Monogenic predisposing conditions to the development of this disease have been described. As examples, an impaired expression of interferon-α regulated genes or complement deficiencies have been reported in patients with SLE, with particular clinical presentations. Those defects present particular presentations and a different severity, making an argument that lupus is not a single disease but many. Treatment could be individualized depending on the underlying defect generating the subtype of the disease.

  4. Psoriasis, a Systemic Disease? - Expert Opinion

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    Nilgün Atakan

    2012-09-01

    Full Text Available Psoriasis is a chronic inflammatory disease which is characterized by plaques with shiny white desquamation on the skin. It affects 1 to 3% of different ethnic populations. The disease significantly lowers the quality of life for the patients as the lesions appear on visible regions such as the scalp, face and extremities causing pruritus and extensive use of topical agents with a poor rate of recovery and the disease has a recurrent course with frequent attacks. Psoriasis was previously assumed to be a cutaneous disease resulting from epidermal cell hyperproliferation for a long time. However, studies conducted on the etiopathogenesis of the disease revealed that psoriasis is a chronic autoinflammatory disease which is caused by immune system dysregulation. Recently, the frequent association of psoriasis with other autoinflammatory diseases, comorbidities and complications which indeed shorten life expectancy concluded that psoriasis is a systemic disease and created a major difference in its treatment and follow-up modalities. In this review, the comorbidities, which are shown to be related to systemic inflammation and which also share a common pathogenesis with psoriasis, will be discussed.

  5. Cutaneous necrotizing vasculitis. Relation to systemic disease.

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    Lotti, T M; Comacchi, C; Ghersetich, I

    1999-01-01

    Cutaneous necrotizing vasculitis (CNV) is a complex multisystem disease generally involving the skin and mucous membranes, often accompanied by renal, gastrointestinal, pericardial, neurological, and articular signs and symptoms. CNV may be idiopatical or occur in association with a drug, infection, or underlying disease. CNV has been shown in patients with chronic infections (viral, bacterial, protozoa, helminthic), serum sickness, a variety of collagen vascular diseases (systemic lupus erythematous, Sjögren's syndrome, rheumatoid arthritis, Behçet's disease) hyperglobulinemic states, cryoglobulinemia, bowel bypass syndrome, ulcerative colitis, cystic fibrosis, primary biliary cirrhosis and HIV infection. Association with malignancies is not frequent. Lymphoproliferative disorders (Hodgkin's disease, mycosis fungoides, lymphosarcoma, adult T-cell leukemia, multiple mieloma) and solid tumors (lung cancer, colon carcinoma, renal, prostate, head and neck cancer and breast cancer) may be associated with CNV. Whenever possible, treatment is directed at the elimination of the cause. In other cases after adequate laboratory screening local and systemic therapy are recommended.

  6. Plant disease management in organic farming systems.

    Science.gov (United States)

    van Bruggen, Ariena H C; Gamliel, Abraham; Finckh, Maria R

    2016-01-01

    Organic farming (OF) has significantly increased in importance in recent decades. Disease management in OF is largely based on the maintenance of biological diversity and soil health by balanced crop rotations, including nitrogen-fixing and cover crops, intercrops, additions of manure and compost and reductions in soil tillage. Most soil-borne diseases are naturally suppressed, while foliar diseases can sometimes be problematic. Only when a severe disease outbreak is expected are pesticides used that are approved for OF. A detailed overview is given of cultural and biological control measures. Attention is also given to regulated pesticides. We conclude that a systems approach to disease management is required, and that interdisciplinary research is needed to solve lingering disease problems, especially for OF in the tropics. Some of the organic regulations are in need of revision in close collaboration with various stakeholders.

  7. Recording and surveillance systems for periodontal diseases

    DEFF Research Database (Denmark)

    Beltrán-Aguilar, Eugenio D; Eke, Paul I; Thornton-Evans, Gina

    2012-01-01

    This paper describes tools used to measure periodontal diseases and the integration of these tools into surveillance systems. Tools to measure periodontal diseases at the surveillance level have focussed on current manifestations of disease (e.g. gingival inflammation) or disease sequelae (e.......g. periodontal pocket depth or loss of attachment). All tools reviewed in this paper were developed based on the state of the science of the pathophysiology of periodontal disease at the time of their design and the need to provide valid and reliable measurements of the presence and severity of periodontal...... diseases. Therefore, some of these tools are no longer valid. Others, such as loss of periodontal attachment, are the current de-facto tools but demand many resources to undertake periodical assessment of the periodontal health of populations. Less complex tools such as the Community Periodontal Index...

  8. Favorable outcome of Epstein-Barr virus-associated B-cell lymphoproliferative disorder complicated by immunoglobulin G4-related disease treated with rituximab-based therapy: a case report.

    Science.gov (United States)

    Ueda, Koki; Ikeda, Kazuhiko; Ogawa, Kazuei; Sukegawa, Masumi; Sano, Takahiro; Kimura, Satoshi; Suzuki, Osamu; Hashimoto, Yuko; Takeishi, Yasuchika

    2016-08-24

    After acute infection of Epstein-Barr virus, Epstein-Barr virus-infected B cells survive but usually do not show clonal proliferation. However, Epstein-Barr virus-infected B cells occasionally acquire a proliferative capacity that provokes clonal lymphoproliferative disorders. We herein present a case with Epstein-Barr virus-infected CD30+ B cell and immunoglobulin G4+ plasmacytoid cell proliferation in the lymph nodes, suggesting a pathological and clinical interaction between Epstein-Barr virus-associated B-cell lymphoproliferative disorders and immunoglobulin G4-related disease. Immunoglobulin G4-related disease has been recognized as a benign disease with proliferation of IgG4-related disease+ plasmacytoid cells. Several studies have recently reported the coexistence of immunoglobulin G4-related disease+ plasmacytoid cells with Epstein-Barr virus-infected B cells in lymph nodes in some immunoglobulin G4-related disease cases. However, the pathogenic role of the clonal proliferation of Epstein-Barr virus-infected B cells in immunoglobulin G4-related disease, as well as the treatments for patients with both Epstein-Barr virus-infected B cells and immunoglobulin G4-related disease, have never been discussed. A 50-year-old Japanese man was referred to us for persistent fatigue and lymphadenopathy. His blood examination showed elevated IgG4, and detected high levels of Epstein-Barr virus DNA. A lymph node biopsy revealed IgG4+ plasmacytoid cells and infiltration of large lymphoid cells, which were positive for CD20, CD30, Epstein-Barr virus-related late membrane protein 1, and Epstein-Barr virus-encoded RNA, and were negative for IgG4. Based on the diagnosis of both Epstein-Barr virus-associated B-cell lymphoproliferative disorder and IgG4-related disease, the patient received eight cycles of rituximab combined with cyclophosphamide and prednisolone, which resulted in the complete disappearance of lymphadenopathy. Moreover, his serum IgG4 level was significantly

  9. The Complement System in Liver Diseases

    Institute of Scientific and Technical Information of China (English)

    Xuebin Qin; Bin Gao

    2006-01-01

    The complement system plays an important role in mediating both acquired and innate responses to defend against microbial infection, and in disposing immunoglobins and apoptotic cells. The liver (mainly hepatocytes) is responsible for biosynthesis of about 80-90% of plasma complement components and expresses a variety of complement receptors.Recent evidence from several studies suggests that the complement system is also involved in the pathogenesis of a variety of liver disorders including liver injury and repair, fibrosis, viral hepatitis, alcoholic liver disease, and liver ischemia/reperfusion injury. In this review, we will discuss the potential role of the complement system in the pathogenesis of liver diseases.

  10. Visual system manifestations of Alzheimer's disease.

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    Kusne, Yael; Wolf, Andrew B; Townley, Kate; Conway, Mandi; Peyman, Gholam A

    2016-11-19

    Alzheimer's disease (AD) is an increasingly common disease with massive personal and economic costs. While it has long been known that AD impacts the visual system, there has recently been an increased focus on understanding both pathophysiological mechanisms that may be shared between the eye and brain and how related biomarkers could be useful for AD diagnosis. Here, were review pertinent cellular and molecular mechanisms of AD pathophysiology, the presence of AD pathology in the visual system, associated functional changes, and potential development of diagnostic tools based on the visual system. Additionally, we discuss links between AD and visual disorders, including possible pathophysiological mechanisms and their relevance for improving our understanding of AD.

  11. The complement system in human cardiometabolic disease.

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    Hertle, E; Stehouwer, C D A; van Greevenbroek, M M J

    2014-10-01

    The complement system has been implicated in obesity, fatty liver, diabetes and cardiovascular disease (CVD). Complement factors are produced in adipose tissue and appear to be involved in adipose tissue metabolism and local inflammation. Thereby complement links adipose tissue inflammation to systemic metabolic derangements, such as low-grade inflammation, insulin resistance and dyslipidaemia. Furthermore, complement has been implicated in pathophysiological mechanisms of diet- and alcohol induced liver damage, hyperglycaemia, endothelial dysfunction, atherosclerosis and fibrinolysis. In this review, we summarize current evidence on the role of the complement system in several processes of human cardiometabolic disease. C3 is the central component in complement activation, and has most widely been studied in humans. C3 concentrations are associated with insulin resistance, liver dysfunction, risk of the metabolic syndrome, type 2 diabetes and CVD. C3 can be activated by the classical, the lectin and the alternative pathway of complement activation; and downstream activation of C3 activates the terminal pathway. Complement may also be activated via extrinsic proteases of the coagulation, fibrinolysis and the kinin systems. Studies on the different complement activation pathways in human cardiometabolic disease are limited, but available evidence suggests that they may have distinct roles in processes underlying cardiometabolic disease. The lectin pathway appeared beneficial in some studies on type 2 diabetes and CVD, while factors of the classical and the alternative pathway were related to unfavourable cardiometabolic traits. The terminal complement pathway was also implicated in insulin resistance and liver disease, and appears to have a prominent role in acute and advanced CVD. The available human data suggest a complex and potentially causal role for the complement system in human cardiometabolic disease. Further, preferably longitudinal studies are needed to

  12. Thyroid disease and the cardiovascular system.

    Science.gov (United States)

    Danzi, Sara; Klein, Irwin

    2014-06-01

    Thyroid hormones, specifically triiodothyronine (T3), have significant effects on the heart and cardiovascular system. Hypothyroidism, hyperthyroidism, subclinical thyroid disease, and low T3 syndrome each cause cardiac and cardiovascular abnormalities through both genomic and nongenomic effects on cardiac myocytes and vascular smooth muscle cells. In compromised health, such as occurs in heart disease, alterations in thyroid hormone metabolism may further impair cardiac and cardiovascular function. Diagnosis and treatment of cardiac disease may benefit from including analysis of thyroid hormone status, including serum total T3 levels. Copyright © 2014 Elsevier Inc. All rights reserved.

  13. Nephrogenic Systemic Fibrosis Risk and Liver Disease

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    Robert F. Hanna

    2014-01-01

    Full Text Available Objective. Evaluate the incidence of nephrogenic systemic fibrosis (NSF in patients with liver disease in the peritransplant period. Materials and Methods. This IRB approved study retrospectively reviewed patients requiring transplantation for cirrhosis, hepatocellular carcinoma (HCC, or both from 2003 to 2013. Records were reviewed identifying those having gadolinium enhanced MRI within 1 year of posttransplantation to document degree of liver disease, renal disease, and evidence for NSF. Results. Gadolinium-enhanced MRI was performed on 312 of 837 patients, including 23 with severe renal failure (GFR 30. Two of 23 patients with renal failure developed NSF compared to zero NSF cases in 289 patients with GFR > 30 (0/289; P<0.003. High dose gadodiamide was used in the two NSF cases. There was no increased incidence of NSF with severe liver disease (1/71 compared to nonsevere liver disease (1/241; P=0.412. Conclusion. Renal disease is a risk factor for NSF, but in our small sample our evidence suggests liver disease is not an additional risk factor, especially if a low-risk gadolinium agent is used. Noting that not all patients received high-risk gadolinium, a larger study focusing on patients receiving high-risk gadolinium is needed to further evaluate NSF risk in liver disease in the peritransplant period.

  14. A comprehensive infectious disease management system.

    Science.gov (United States)

    Marcu, Alex; Farley, John D

    2009-01-01

    An efficient electronic management system is now an essential tool for the successful management and monitoring of those affected by communicable infectious diseases (Human Immunodeficiency Virus - HIV, hepatitis C - HEP C) during the course of the treatment. The current methods which depend heavily on manual collecting, compiling and disseminating treatment information are labor-intensive and time consuming. Clinics specialized in the treatment of infectious diseases use a mix of electronic systems that fail to interact with each other, result in data duplication, and do not support treatment of the patient as a whole. The purpose of the Infectious Disease Management System is to reduce the administrative overhead associated with data collection and analysis while providing correlation abilities and decision support in accordance with defined treatment guidelines. This Infectious Disease Management System was developed to: Ensure cost effectiveness by means of low software licensing costs, Introduce a centralized mechanism of collecting and monitoring all infectious disease management data, Automate electronic retrieval of laboratory findings, Introduce a decision support mechanism as per treatment guidelines, Seamlessly integrate of application modules, Provide comprehensive reporting capabilities, Maintain a high level of user friendliness.

  15. Untangling the Web of Systemic Autoinflammatory Diseases

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    Donato Rigante

    2014-01-01

    Full Text Available The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs, an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1β and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflammatory cytokines and subsequent multisite inflammatory symptoms depicting various conditions, such as hereditary periodic fevers, granulomatous disorders, and pyogenic diseases, collectively described in this review. Further research should enhance our understanding of the genetics behind SAIDs, unearth triggers of inflammatory attacks, and result in improvement for their diagnosis and treatment.

  16. Untangling the Web of Systemic Autoinflammatory Diseases

    Science.gov (United States)

    Rigante, Donato; Lopalco, Giuseppe; Vitale, Antonio; Lucherini, Orso Maria; Caso, Francesco; De Clemente, Caterina; Molinaro, Francesco; Messina, Mario; Costa, Luisa; Atteno, Mariangela; Laghi-Pasini, Franco; Lapadula, Giovanni; Galeazzi, Mauro; Iannone, Florenzo; Cantarini, Luca

    2014-01-01

    The innate immune system is involved in the pathophysiology of systemic autoinflammatory diseases (SAIDs), an enlarging group of disorders caused by dysregulated production of proinflammatory cytokines, such as interleukin-1β and tumor necrosis factor-α, in which autoreactive T-lymphocytes and autoantibodies are indeed absent. A widely deranged innate immunity leads to overactivity of proinflammatory cytokines and subsequent multisite inflammatory symptoms depicting various conditions, such as hereditary periodic fevers, granulomatous disorders, and pyogenic diseases, collectively described in this review. Further research should enhance our understanding of the genetics behind SAIDs, unearth triggers of inflammatory attacks, and result in improvement for their diagnosis and treatment. PMID:25132737

  17. Systemic Coronaviral Disease in 5 Ferrets

    Science.gov (United States)

    Autieri, Christopher R; Miller, Cassandra L; Scott, Kathleen E; Kilgore, Alexandra; Papscoe, Victoria A; Garner, Michael M; Haupt, Jennifer L; Bakthavatchalu, Vasudevan; Muthupalani, Sureshkumar; Fox, James G

    2015-01-01

    The prevalence of reported systemic coronaviral disease in ferrets (Mustela putorius furo), which resembles the dry form of feline infectious peritonitis, has been increasing in the literature since its initial diagnosis and characterization approximately 10 y ago. Here we describe the clinical signs, pathologic findings, and diagnosis by immunohistochemistry using an FIPV3-70 monoclonal antibody of systemic coronaviral disease in 5 ferrets, 2 of which were strictly laboratory-housed; the remaining 3 were referred from veterinary private practices. This case report illustrates the importance of considering FRSCV infection as a differential diagnosis in young, debilitated ferrets with abdominal masses and other supporting clinical signs. PMID:26678368

  18. Clinical Update in Aspects of the Management of Autoimmune Thyroid Diseases

    Science.gov (United States)

    2016-01-01

    Aspects of autoimmune thyroid disease updated in this review include: immunoglobulin G4 (IgG4)-related thyroid disease (Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, IgG4-related Hashimoto's thyroiditis, and Graves' disease with elevated IgG4 levels); recent epidemiological studies from China and Denmark indicating that excess iodine increases the incidence of Hashimoto's thyroiditis and hypothyroidism; immunomodulatory agents (ipilimumab, pembrolizumab, nivolumab) activate immune response by inhibiting T-cell surface receptors which down-regulate immune response, i.e., cytotoxic T-lymphocyte antigen 4 and programmed cell death protein 1 pathways; alemtuzumab is a humanised monoclonal antibody to CD52 which causes immune depletion and thyroid autoimmune disease especially Graves' hyperthyroidism; small molecule ligand (SML) agonists which activate receptors, SML neutral antagonists, which inhibit receptor activation by agonists, and SML inverse agonists which inhibit receptor activation by agonists and inhibit constitutive agonist independent signaling have been identified. SML antagonism of thyroid-stimulating hormone-receptor stimulatory antibody could treat Graves' hyperthyroidism and Graves' ophthalmopathy; and thyroxine treatment of subclinical hypothyroidism can produce iatrogenic subclinical hyperthyroidism with the risk of atrial fibrillation and osteoporosis. The increased risk of harm from subclinical hyperthyroidism may be stronger than the potential benefit from treatment of subclinical hypothyroidism. PMID:28029020

  19. Clinical Update in Aspects of the Management of Autoimmune Thyroid Diseases

    Directory of Open Access Journals (Sweden)

    Duncan J. Topliss

    2016-12-01

    Full Text Available Aspects of autoimmune thyroid disease updated in this review include: immunoglobulin G4 (IgG4-related thyroid disease (Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, IgG4-related Hashimoto's thyroiditis, and Graves' disease with elevated IgG4 levels; recent epidemiological studies from China and Denmark indicating that excess iodine increases the incidence of Hashimoto's thyroiditis and hypothyroidism; immunomodulatory agents (ipilimumab, pembrolizumab, nivolumab activate immune response by inhibiting T-cell surface receptors which down-regulate immune response, i.e., cytotoxic T-lymphocyte antigen 4 and programmed cell death protein 1 pathways; alemtuzumab is a humanised monoclonal antibody to CD52 which causes immune depletion and thyroid autoimmune disease especially Graves' hyperthyroidism; small molecule ligand (SML agonists which activate receptors, SML neutral antagonists, which inhibit receptor activation by agonists, and SML inverse agonists which inhibit receptor activation by agonists and inhibit constitutive agonist independent signaling have been identified. SML antagonism of thyroid-stimulating hormone-receptor stimulatory antibody could treat Graves' hyperthyroidism and Graves' ophthalmopathy; and thyroxine treatment of subclinical hypothyroidism can produce iatrogenic subclinical hyperthyroidism with the risk of atrial fibrillation and osteoporosis. The increased risk of harm from subclinical hyperthyroidism may be stronger than the potential benefit from treatment of subclinical hypothyroidism.

  20. Stepwise Approach to Myopathy in Systemic Disease

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    Jasvinder eChawla

    2011-08-01

    Full Text Available Muscle diseases can constitute a large variety of both acquired and hereditary disorders. Myopathies in systemic disease results from several different disease processes including endocrine, inflammatory, paraneoplastic, infectious, drug and toxin-induced, critical illness myopathy, metabolic and myopathies with other systemic disorders. Patients with systemic myopathies often present acutely or sub acutely. On the other hand, familial myopathies or dystrophies generally present in a chronic fashion with exceptions of metabolic myopathies where symptoms on occasion can be precipitated acutely. Most of the inflammatory myopathies can have a chance association with malignant lesions; the incidence appears to be specifically increased only in patients with dermatomyositis. In dealing with myopathies associated with systemic illnesses, the focus will be on the acquired causes. Management is beyond the scope of this chapter. Prognosis is based upon the underlying cause and, most of the time, carries a good prognosis. In order to approach a patient with suspected myopathy from systemic disease, a stepwise approach is utilized.

  1. Interstitial lung disease in systemic sclerosis.

    Science.gov (United States)

    Wells, Athol U

    2014-10-01

    Based on international collaborative data, interstitial lung disease is now the most frequent cause of death in systemic sclerosis (SSc), having supplanted renal crisis in that regard. Despite detailed explorations of candidate mediators, no primary pathway in the pathogenesis of interstitial lung disease associated with SSc (SSc-ILD) has been definitively identified and, therefore, treatment with current agents is only partially successful. However, as immunomodulatory agents do, on average, retard progression of lung disease, early identification of SSc-ILD, using thoracic high resolution computed tomography (HRCT), is highly desirable. The decision whether to introduce therapy immediately is often difficult as the balance of risk and benefit favours a strategy of careful observation when lung disease is very limited, especially in long-standing SSc. The threshold for initiating treatment is substantially reduced when lung disease is severe, systemic disease is short in duration or ongoing progression is evident, based on pulmonary function tests and symptoms. This review summarises epidemiology, pathogenesis, difficult clinical problems and management issues in SSc-ILD.

  2. Systemic lupus erythematosus one disease or many?

    Science.gov (United States)

    Agmon-Levin, N; Mosca, M; Petri, M; Shoenfeld, Y

    2012-06-01

    Systemic lupus erythematosus (SLE) characterizes by a variety of clinical manifestations and the presence of a wide profile of autoantibodies. This clinical and serological heterogeneity raised the question: is SLE a single disease with varied phenotypes, or a similar phenotype shared by different diseases with diverse pathogenic mechanisms? Herein we debate the clinical, genetic, hormonal and serological differences typically observed in SLE on the one hand, and the numerous similarities between subtypes of this disease on the other. Leading to the conclusion that SLE may be considered not as a single disease but rather as a single syndrome, which defines by a set of signs, symptoms, or phenomena that occur together and suggest a particular abnormality. Additionally, the accumulated knowledge on gene expression pathways, autoantibodies clusters, hormonal and environmental factors associated with SLE may allow a better classification of this syndrome and updating of SLE criteria. This may further allow targeted biologics and other therapies as well as "personalized medicine" to begin.

  3. [Parasitic diseases of the central nervous system].

    Science.gov (United States)

    Schmutzhard, E

    2010-02-01

    Central nervous system infections and infestations by protozoa and helminths constitute a problem of increasing importance throughout all of central European and northern/western countries. This is partially due to the globalisation of our society, tourists and business people being more frequently exposed to parasitic infection/infestation in tropical countries than in moderate climate countries. On top of that, migrants may import chronic infestations and infections with parasitic pathogens, eventually also--sometimes exclusively--involving the nervous system. Knowledge of epidemiology, initial clinical signs and symptoms, diagnostic procedures as well as specific chemotherapeutic therapies and adjunctive therapeutic strategies is of utmost important in all of these infections and infestations of the nervous systems, be it by protozoa or helminths. This review lists, mainly in the form of tables, all possible infections and infestations of the nervous systems by protozoa and by helminths. Besides differentiating parasitic diseases of the nervous system seen in migrants, tourists etc., it is very important to have in mind that disease-related (e.g. HIV) or iatrogenic immunosuppression has led to the increased occurrence of a wide variety of parasitic infections and infestations of the nervous system (e. g. babesiosis, Chagas disease, Strongyloides stercoralis infestation, toxoplasmosis, etc.).

  4. Electronic integrated disease surveillance system and pathogen asset control system.

    Science.gov (United States)

    Wahl, Tom G; Burdakov, Aleksey V; Oukharov, Andrey O; Zhilokov, Azamat K

    2012-06-20

    Electronic Integrated Disease Surveillance System (EIDSS) has been used to strengthen and support monitoring and prevention of dangerous diseases within One Health concept by integrating veterinary and human surveillance, passive and active approaches, case-based records including disease-specific clinical data based on standardised case definitions and aggregated data, laboratory data including sample tracking linked to each case and event with test results and epidemiological investigations. Information was collected and shared in secure way by different means: through the distributed nodes which are continuously synchronised amongst each other, through the web service, through the handheld devices. Electronic Integrated Disease Surveillance System provided near real time information flow that has been then disseminated to the appropriate organisations in a timely manner. It has been used for comprehensive analysis and visualisation capabilities including real time mapping of case events as these unfold enhancing decision making. Electronic Integrated Disease Surveillance System facilitated countries to comply with the IHR 2005 requirements through a data transfer module reporting diseases electronically to the World Health Organisation (WHO) data center as well as establish authorised data exchange with other electronic system using Open Architecture approach. Pathogen Asset Control System (PACS) has been used for accounting, management and control of biological agent stocks. Information on samples and strains of any kind throughout their entire lifecycle has been tracked in a comprehensive and flexible solution PACS.Both systems have been used in a combination and individually. Electronic Integrated Disease Surveillance System and PACS are currently deployed in the Republics of Kazakhstan, Georgia and Azerbaijan as a part of the Cooperative Biological Engagement Program (CBEP) sponsored by the US Defense Threat Reduction Agency (DTRA).

  5. Electronic Integrated Disease Surveillance System and Pathogen Asset Control System

    Directory of Open Access Journals (Sweden)

    Tom G. Wahl

    2012-06-01

    Full Text Available Electronic Integrated Disease Surveillance System (EIDSS has been used to strengthen and support monitoring and prevention of dangerous diseases within One Health concept by integrating veterinary and human surveillance, passive and active approaches, case-based records including disease-specific clinical data based on standardised case definitions and aggregated data, laboratory data including sample tracking linked to each case and event with test results and epidemiological investigations. Information was collected and shared in secure way by different means: through the distributed nodes which are continuously synchronised amongst each other, through the web service, through the handheld devices. Electronic Integrated Disease Surveillance System provided near real time information flow that has been then disseminated to the appropriate organisations in a timely manner. It has been used for comprehensive analysis and visualisation capabilities including real time mapping of case events as these unfold enhancing decision making. Electronic Integrated Disease Surveillance System facilitated countries to comply with the IHR 2005 requirements through a data transfer module reporting diseases electronically to the World Health Organisation (WHO data center as well as establish authorised data exchange with other electronic system using Open Architecture approach. Pathogen Asset Control System (PACS has been used for accounting, management and control of biological agent stocks. Information on samples and strains of any kind throughout their entire lifecycle has been tracked in a comprehensive and flexible solution PACS.Both systems have been used in a combination and individually. Electronic Integrated Disease Surveillance System and PACS are currently deployed in the Republics of Kazakhstan, Georgia and Azerbaijan as a part of the Cooperative Biological Engagement Program (CBEP sponsored by the US Defense Threat Reduction Agency (DTRA.

  6. Integrated Knowledge Based Expert System for Disease Diagnosis System

    Science.gov (United States)

    Arbaiy, Nureize; Sulaiman, Shafiza Eliza; Hassan, Norlida; Afizah Afip, Zehan

    2017-08-01

    The role and importance of healthcare systems to improve quality of life and social welfare in a society have been well recognized. Attention should be given to raise awareness and implementing appropriate measures to improve health care. Therefore, a computer based system is developed to serve as an alternative for people to self-diagnose their health status based on given symptoms. This strategy should be emphasized so that people can utilize the information correctly as a reference to enjoy healthier life. Hence, a Web-based Community Center for Healthcare Diagnosis system is developed based on expert system technique. Expert system reasoning technique is employed in the system to enable information about treatment and prevention of the diseases based on given symptoms. At present, three diseases are included which are arthritis, thalassemia and pneumococcal. Sets of rule and fact are managed in the knowledge based system. Web based technology is used as a platform to disseminate the information to users in order for them to optimize the information appropriately. This system will benefit people who wish to increase health awareness and seek expert knowledge on the diseases by performing self-diagnosis for early disease detection.

  7. Disease processes as hybrid dynamical systems

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    Pietro Liò

    2012-08-01

    Full Text Available We investigate the use of hybrid techniques in complex processes of infectious diseases. Since predictive disease models in biomedicine require a multiscale approach for understanding the molecule-cell-tissue-organ-body interactions, heterogeneous methodologies are often employed for describing the different biological scales. Hybrid models provide effective means for complex disease modelling where the action and dosage of a drug or a therapy could be meaningfully investigated: the infection dynamics can be classically described in a continuous fashion, while the scheduling of multiple treatment discretely. We define an algebraic language for specifying general disease processes and multiple treatments, from which a semantics in terms of hybrid dynamical system can be derived. Then, the application of control-theoretic tools is proposed in order to compute the optimal scheduling of multiple therapies. The potentialities of our approach are shown in the case study of the SIR epidemic model and we discuss its applicability on osteomyelitis, a bacterial infection affecting the bone remodelling system in a specific and multiscale manner. We report that formal languages are helpful in giving a general homogeneous formulation for the different scales involved in a multiscale disease process; and that the combination of hybrid modelling and control theory provides solid grounds for computational medicine.

  8. Salivary biomarkers for detection of systemic diseases.

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    Nilminie Rathnayake

    Full Text Available BACKGROUND AND OBJECTIVE: Analysis of inflammatory biomarkers in saliva could offer an attractive opportunity for the diagnosis of different systemic conditions specifically in epidemiological surveys. The aim of this study was to investigate if certain salivary biomarkers could be used for detection of common systemic diseases. MATERIALS AND METHODS: A randomly selected sample of 1000 adults living in Skåne, a county in the southern part of Sweden, was invited to participate in a clinical study of oral health. 451 individuals were enrolled in this investigation, 51% women. All participants were asked to fill out a questionnaire, history was taken, a clinical examination was made and stimulated saliva samples were collected. Salivary concentrations of IL-1β, -6, -8, TNF-α, lysozyme, MMP-8 and TIMP-1 were determined using ELISA, IFMA or Luminex assays. RESULTS: Salivary IL-8 concentration was found to be twice as high in subjects who had experience of tumour diseases. In addition, IL-8 levels were also elevated in patients with bowel disease. MMP-8 levels were elevated in saliva from patients after cardiac surgery or suffering from diabetes, and muscle and joint diseases. The levels of IL-1β, IL-8 and MMP-8, as well as the MMP-8/TIMP-1 ratio were higher in subjects with muscle and joint diseases. CONCLUSION: Biomarkers in saliva have the potential to be used for screening purposes in epidemiological studies. The relatively unspecific inflammatory markers used in this study can not be used for diagnosis of specific diseases but can be seen as markers for increased systemic inflammation.

  9. Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease.

    Science.gov (United States)

    Cornell, Lynn D; Chicano, Sonia L; Deshpande, Vikram; Collins, A Bernard; Selig, Martin K; Lauwers, Gregory Y; Barisoni, Laura; Colvin, Robert B

    2007-10-01

    Autoimmune pancreatitis (AIP) is a mass-forming chronic fibroinflammatory condition centered on the pancreatobiliary system and characterized by predominant immunoglobulin G4 (IgG4)-positive plasma cells. Recent reports have brought to light the multiorgan involvement of this disease. We describe a series of 5 cases of tubulointerstitial nephritis (TIN) associated with AIP and characterize the clinical, pathologic, ultrastructural, and immunopathologic features of TIN. The specimens consisted of 4 biopsies and 1 nephrectomy. The average patient age was 64 years (range 45 to 78) and the male to female ratio was 4:1. All had histologic and/or clinical and radiographic evidence of AIP, mass-forming sclerosing cholangitis, or both. The clinical impression in 4 patients was a renal mass or vasculitis. Two patients had renal insufficiency. Histologic preparations revealed a dense tubulointerstitial lymphoplasmacytic infiltrate. Eosinophils were often numerous. Tubulitis and tubular injury were present, along with tubular atrophy with focally thickened tubular basement membranes (TBMs). The histologic appearance ranged from a cellular, inflammatory pattern without tubular atrophy to a striking expansive interstitial fibrosis with tubular destruction. The nephrectomy specimen demonstrated a masslike nodular pattern of inflammation with normal renal tissue elsewhere. Glomeruli were uninvolved. By immunohistochemistry or immunofluorescence, numerous plasma cells in the infiltrate were positive for IgG4. TBM granular IgG deposits, predominantly of the IgG4 subclass, were detected in 4 of 5 cases by either immunofluorescence or immunohistochemistry. By electron microscopy, corresponding amorphous electron-dense deposits were present in the TBM in these cases. This type of TIN, typically characterized by a masslike lesion consisting of a lymphoplasmacytic infiltrate with eosinophils and prominent IgG4-positive plasma cells and immune-complex deposits in the TBM, may be part of

  10. The Utility of Serum IgG4 Concentrations as a Biomarker

    Directory of Open Access Journals (Sweden)

    Shigeyuki Kawa

    2012-01-01

    Full Text Available IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1 systemic involvement; (2 solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3 high serum IgG4 concentration >135 mg/dL; (4 abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5 a positive response to corticosteroid therapy; and (6 complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.

  11. CEMARA an information system for rare diseases.

    Science.gov (United States)

    Landais, Paul; Messiaen, Claude; Rath, Ana; Le Mignot, Loïc; Dufour, Eric; Ben Said, Mohamed; Jais, Jean-Philippe; Toubiana, Laurent; Baujat, Geneviève; Bourdon-Lanoy, Eva; Gérard-Blanluet, Marion; Bodemer, Christine; Salomon, Rémi; Aymé, Ségolène; Le Merrer, Martine; Verloes, Alain

    2010-01-01

    Rare diseases cover a group of conditions characterized by a low prevalence, affecting less than 1 in 2,000 people; 5000 to 7000 rare diseases have been currently identified in Europe. Most diseases do not have any curative treatment. They represent thus an important public health concern. CEMARA is based on a n-tier architecture. Its main objective is to collect continuous and complete records of patients with rare diseases, and their follow-up through a web-based Information System, and to analyse the epidemiological patterns. In France, 41 out of 131 labelled Reference Centres (RC) are sharing CEMARA. Presently 56,593 cases have been registered by more than 850 health care professionals belonging to 171 clinical sites. The national demand of care was explored in relation with the offer of care in order to reach an improved match. Within 2 years, CEMARA stimulated sharing a common platform, a common ontology with Orphanet and initiating new cohorts of rare diseases for improving patient care and research.

  12. Sustainable public health systems for rare diseases.

    Science.gov (United States)

    Ferrelli, Rita Maria; Gentile, Amalia Egle; De Santis, Marta; Taruscio, Domenica

    2017-01-01

    In the framework of the Joint Action for Rare Diseases (RD-ACTION), a specific task was defined to identify mechanisms influencing sustainability, equity and resilience of health systems for rare diseases (RDs). Literature narrative review on health systems sustainability and resilience for RDs. Years: 2000-2015. Databases: PubMed, Scopus, EBSCOHost, EMBAL, PASCAL, EMBASE, STN International and GoogleScholar. interpretive synthesis concept and thematic analysis (Dixon-Wood, et al.). 97 papers and 4 grey literature publications were identified. Two main topics stand out: economic evaluation and networks. The first topic did not identify widely accepted criterion to assign more weight to individuals with greater health needs. Healthcare network are identified as increasingly important for sustainability and resilience, in all of their aspects: professional "expertise", "experience" networks of users and carers; policy, learning, and interest networks. Possible mechanisms for ensuring sustainability can be identified in networking, patients' empowerment and reorienting healthcare towards integrated community and home care.

  13. Infectious disease modeling a hybrid system approach

    CERN Document Server

    Liu, Xinzhi

    2017-01-01

    This volume presents infectious diseases modeled mathematically, taking seasonality and changes in population behavior into account, using a switched and hybrid systems framework. The scope of coverage includes background on mathematical epidemiology, including classical formulations and results; a motivation for seasonal effects and changes in population behavior, an investigation into term-time forced epidemic models with switching parameters, and a detailed account of several different control strategies. The main goal is to study these models theoretically and to establish conditions under which eradication or persistence of the disease is guaranteed. In doing so, the long-term behavior of the models is determined through mathematical techniques from switched systems theory. Numerical simulations are also given to augment and illustrate the theoretical results and to help study the efficacy of the control schemes.

  14. Periodontitis and systemic diseases: A literature review

    OpenAIRE

    Arigbede, Abiodun O.; B Osagbemiro Babatope; M Kolude Bamidele

    2012-01-01

    Studies have revealed possible link between periodontitis and different systemic diseases. There is need to review this interesting subject. The aims are: to provide a comprehensive literature that can easily be consulted, on the subject; to draw the attention of health practitioners to the impact of oral health on the general well-being; and to emphasize the need for a deeper interaction between medical and dental training. The Medline database was searched for relevant literature by combini...

  15. Central Nervous System Involvement in Whipple Disease

    OpenAIRE

    Compain, Caroline; Sacre, Karim; Puéchal, Xavier; Klein, Isabelle; Vital-Durand, Denis; Houeto, Jean-Luc; De Broucker, Thomas; Raoult, Didier; Papo, Thomas

    2013-01-01

    Abstract Whipple disease (WD) is a rare multisystemic infection with a protean clinical presentation. The central nervous system (CNS) is involved in 3 situations: CNS involvement in classic WD, CNS relapse in previously treated WD, and isolated CNS infection. We retrospectively analyzed clinical features, diagnostic workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data in 18 patients with WD and CNS infection. Ten men and 8 women were included with a median ag...

  16. Increased lymphangiogenesis in Riedel thyroiditis (Immunoglobulin G4-related thyroid disease).

    Science.gov (United States)

    Cameselle-Teijeiro, José; Ladra, María Jesús; Abdulkader, Ihab; Eloy, Catarina; Soares, Paula; Barreiro, Francisco; Sobrinho-Simões, Manuel; Beiras-Iglesias, Andrés

    2014-09-01

    The present study describes in depth a case of Riedel thyroiditis (RT) to clarify its pathogenesis and its putative inclusion in the spectrum of IgG4-related disease. We report the clinicopathological, immunohistochemical, and ultrastructural features of a case of RT in a 39-year-old white Spanish woman, admitted with a hard goiter and cold nodule in the left thyroid lobe. This case represents 0.05 % of a series of 1,973 consecutive thyroidectomies performed in our hospital. More than 80 % of the left thyroid lobe was effaced by fibrosis and inflammation (lymphocytes, 57 IgG4+ plasma cells per 1 high-power field, an IgG4/IgG ratio of 0.67, and eosinophils) with extension into the surrounding tissues and occlusive phlebitis. Immunostaining for podoplanin (D2-40) detected signs of increased lymphangiogenesis in the fibroinflammatory areas that were confirmed by electron microscopy. A strong, diffuse stain for podoplanin and transforming growth factor ß1 was also detected in the same areas. The increased number of lymphatic vessels in RT is reported for the first time. Our findings support the inclusion of RT within the spectrum of IgG4-related thyroid disease (IgG4-RTD). Although the etiology and physiopathology of IgG4-RTD still remain elusive, the results obtained in the present case suggest the participation of lymphatic vessels in the pathogenesis of RT.

  17. Periodontal disease associated to systemic genetic disorders.

    Science.gov (United States)

    Nualart Grollmus, Zacy Carola; Morales Chávez, Mariana Carolina; Silvestre Donat, Francisco Javier

    2007-05-01

    A number of systemic disorders increase patient susceptibility to periodontal disease, which moreover evolves more rapidly and more aggressively. The underlying factors are mainly related to alterations in immune, endocrine and connective tissue status. These alterations are associated with different pathologies and syndromes that generate periodontal disease either as a primary manifestation or by aggravating a pre-existing condition attributable to local factors. This is where the role of bacterial plaque is subject to debate. In the presence of qualitative or quantitative cellular immune alterations, periodontal disease may manifest early on a severe localized or generalized basis--in some cases related to the presence of plaque and/or specific bacteria (severe congenital neutropenia or infantile genetic agranulocytosis, Chediak-Higiashi syndrome, Down syndrome and Papillon-Lefévre syndrome). In the presence of humoral immune alterations, periodontal damage may result indirectly as a consequence of alterations in other systems. In connective tissue disorders, bacterial plaque and alterations of the periodontal tissues increase patient susceptibility to gingival inflammation and alveolar resorption (Marfan syndrome and Ehler-Danlos syndrome). The management of periodontal disease focuses on the control of infection and bacterial plaque by means of mechanical and chemical methods. Periodontal surgery and even extraction of the most seriously affected teeth have also been suggested. There are variable degrees of consensus regarding the background systemic disorder, as in the case of Chediak-Higiashi syndrome, where antibiotic treatment proves ineffective; in severe congenital neutropenia or infantile genetic agranulocytosis, where antibiotic prophylaxis is suggested; and in Papillon-Lefévre syndrome, where an established treatment protocol is available.

  18. RDIS: The Rabies Disease Information System.

    Science.gov (United States)

    Dharmalingam, Baskeran; Jothi, Lydia

    2015-01-01

    Rabies is a deadly viral disease causing acute inflammation or encephalitis of the brain in human beings and other mammals. Therefore, it is of interest to collect information related to the disease from several sources including known literature databases for further analysis and interpretation. Hence, we describe the development of a database called the Rabies Disease Information System (RDIS) for this purpose. The online database describes the etiology, epidemiology, pathogenesis and pathology of the disease using diagrammatic representations. It provides information on several carriers of the rabies viruses like dog, bat, fox and civet, and their distributions around the world. Information related to the urban and sylvatic cycles of transmission of the virus is also made available. The database also contains information related to available diagnostic methods and vaccines for human and other animals. This information is of use to medical, veterinary and paramedical practitioners, students, researchers, pet owners, animal lovers, livestock handlers, travelers and many others. The database is available for free http://rabies.mscwbif.org/home.html.

  19. Pancreatic adenocarcinoma: treating a systemic disease with systemic therapy.

    Science.gov (United States)

    Sohal, Davendra P S; Walsh, R Matthew; Ramanathan, Ramesh K; Khorana, Alok A

    2014-03-01

    Pancreatic adenocarcinoma, even when resectable, remains highly lethal. Although surgical outcomes have improved considerably, median overall survival after surgery and adjuvant therapy such as single-agent gemcitabine remains less than 2 years. We discuss preclinical and clinical data supporting the contention that even early-stage pancreatic cancer is a systemic disease. Autopsy series reveal that 70% to 85% of patients die of systemic recurrence, rather than local disease, after pancreatic cancer resection. Preclinical studies using genomics and mouse models reveal evidence of metastatic spread even before histopathologic evidence of a pancreatic tumor. Analogous to breast cancer, we propose that the Halstedian approach of treating pancreatic cancer as a local, surgical problem should be replaced by Fisher's alternative hypothesis of cancer as a systemic disease. Newer multiagent chemotherapy regimens have shown meaningful response rates and improvement in overall survival in the metastatic setting and, for the first time, offer investigators an opportunity to use effective systemic therapy. We emphasize that a surgery-first approach is not resonant with our current understanding of pancreatic adenocarcinoma biology and that an upfront systemic approach for even resectable pancreatic cancer warrants testing in clinical trials.

  20. Autonomic Nervous System Dysfunction in Parkinson's Disease.

    Science.gov (United States)

    Zesiewicz, Theresa A.; Baker, Matthew J.; Wahba, Mervat; Hauser, Robert A.

    2003-03-01

    Autonomic nervous system (ANS) dysfunction is common in Parkinson's disease (PD), affects 70% to 80% of patients, and causes significant morbidity and discomfort. Autonomic nervous system dysfunction symptoms in PD include sexual dysfunction, swallowing and gastrointestinal disorders, bowel and bladder abnormalities, sleep disturbances, and derangements of cardiovascular regulation, particularly, orthostatic hypotension. Autonomic nervous system dysfunction in PD may be caused by an underlying degenerative process that affects the autonomic ganglia, brainstem nuclei, and hypothalamic nuclei. Anti-parkinsonian medications can cause or worsen symptoms of ANS dysfunction. The care of a PD patient with ANS dysfunction relies on its recognition and directed treatment, including coordinated care between the neurologist and appropriate subspecialist. Pharmacotherapy may be useful to treat orthostasis, gastrointestinal, urinary, and sexual dysfunction.

  1. Parasitic diseases of the central nervous system.

    Science.gov (United States)

    Chacko, Geeta

    2010-08-01

    Parasitic infections, though endemic to certain regions, have over time appeared in places far removed from their original sites of occurrence facilitated probably by the increase in world travel and the increasing migration of people from their native lands to other, often distant, countries. The frequency of occurrence of some of these diseases has also changed based on a variety of factors, including the presence of intermediate hosts, geographic locations, and climate. One factor that has significantly altered the epidemiology of parasitic diseases within the central nervous system (CNS) is the HIV pandemic. In this review of the pathology of parasitic infections that affect the CNS, each parasite is discussed in the sequence of epidemiology, life cycle, pathogenesis, and pathology.

  2. Lung involvement in systemic connective tissue diseases

    Directory of Open Access Journals (Sweden)

    Plavec Goran

    2008-01-01

    Full Text Available Background/Aim. Systemic connective tissue diseases (SCTD are chronic inflammatory autoimmune disorders of unknown cause that can involve different organs and systems. Their course and prognosis are different. All of them can, more or less, involve the respiratory system. The aim of this study was to find out the frequency of respiratory symptoms, lung function disorders, radiography and high-resolution computerized tomography (HRCT abnormalities, and their correlation with the duration of the disease and the applied treatment. Methods. In 47 non-randomized consecutive patients standard chest radiography, HRCT, and lung function tests were done. Results. Hypoxemia was present in nine of the patients with respiratory symptoms (20%. In all of them chest radiography was normal. In five of these patients lung fibrosis was established using HRCT. Half of all the patients with SCTD had symptoms of lung involvement. Lung function tests disorders of various degrees were found in 40% of the patients. The outcome and the degree of lung function disorders were neither in correlation with the duration of SCTD nor with therapy used (p > 0.05 Spearmans Ro. Conclusion. Pulmonary fibrosis occurs in about 10% of the patients with SCTD, and possibly not due to the applied treatment regimens. Hypoxemia could be a sing of existing pulmonary fibrosis in the absence of disorders on standard chest radiography.

  3. Effect of diseases on symbiotic systems.

    Science.gov (United States)

    Tiwari, Pankaj Kumar; Sasmal, Sourav Kumar; Sha, Amar; Venturino, Ezio; Chattopadhyay, Joydev

    2017-09-01

    There are many species living in symbiotic communities. In this study, we analyzed models in which populations are in the mutualism symbiotic relations subject to a disease spreading among one of the species. The main goal is the characterization of symbiotic relations of coexisting species through their mutual influences on their respective carrying capacities, taking into account that this influence can be quite strong. The functional dependence of the carrying capacities reflects the fact that the correlations between populations cannot be realized merely through direct interactions, as in the usual predator-prey Lotka-Volterra model, but also through the influence of each species on the carrying capacities of the other one. Equilibria are analyzed for feasibility and stability, substantiated via numerical simulations, and global sensitivity analysis identifies the important parameters having a significant impact on the model dynamics. The infective growth rate and the disease-related mortality rate may alter the stability behavior of the system. Our results show that introducing a symbiotic species is a plausible way to control the disease in the population. Copyright © 2017 Elsevier B.V. All rights reserved.

  4. Ocular Involvement in Systemic Autoimmune Diseases.

    Science.gov (United States)

    Generali, Elena; Cantarini, Luca; Selmi, Carlo

    2015-12-01

    Eye involvement represents a common finding in patients with systemic autoimmune diseases, particularly rheumatoid arthritis, Sjogren syndrome, seronegative spondyloarthropathy, and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The eye is a privileged immune site but commensal bacteria are found on the ocular surface. The eye injury may be inflammatory, vascular or infectious, as well as iatrogenic, as in the case of hydroxychloroquine, chloroquine, corticosteroids, and bisphosphonates. Manifestations may affect different components of the eye, with episcleritis involving the episclera, a thin layer of tissue covering the sclera; scleritis being an inflammation of the sclera potentially leading to blindness; keratitis, referring to corneal inflammation frequently associated with scleritis; and uveitis as the inflammation of the uvea, including the iris, ciliary body, and choroid, subdivided into anterior, posterior, or panuveitis. As blindness may result from the eye involvement, clinicians should be aware of the possible manifestations and their management also independent of the ophthalmologist opinion as the therapeutic approach generally points to the underlying diseases. In some cases, the eye involvement may have a diagnostic implication, as for episcleritis in rheumatoid arthritis, or acute anterior uveitis in seronegative spondyloarthritis. Nonetheless, some conditions lack specificity, as in the case of dry eye which affects nearly 30 % of the general population. The aim of this review is to elucidate to non-ophthalmologists the major ocular complications of rheumatic diseases and their specific management and treatment options.

  5. Accelerated atherosclerosis in patients with systemic autoimmune diseases

    NARCIS (Netherlands)

    De Leeuw, K.; Kallenberg, Cees; Bijl, Marc; Shoenfeld, Y.; Gershwin, M.E.; Shoenfeld, Y; Gershwin, ME

    2005-01-01

    Systemic autoimmune diseases such as systemic lupus erythematosus and Wegener's granulomatosis are associated with a significantly increased prevalence of cardiovascular disease (CVD) compared with age- and sex-matched controls. Many risk factors are involved in the pathogenesis of atherosclerosis,

  6. Advanced and controlled drug delivery systems in clinical disease management

    NARCIS (Netherlands)

    Brouwers, JRBJ

    1996-01-01

    Advanced and controlled drug delivery systems are important for clinical disease management. In this review the most important new systems which have reached clinical application are highlighted. Microbiologically controlled drug delivery is important for gastrointestinal diseases like ulcerative co

  7. [Diseases of musculo-skeletal system in railway personnel].

    Science.gov (United States)

    Shemetova, G N; Trifonova, E V

    2006-01-01

    Epidemiological and medical-social aspects of diseases of locomotor system and conjunctive tissue in railway personnel (1993-2004) are analyzed. The established negative trends in propagation of diseases locomotor system predetermine priority of preventive measures in organization of medical care for the patients of this category. Establishment of medical rooms for preventing diseases of locomotor system is recommended.

  8. Sclerosing lesions of the orbit: A review

    Directory of Open Access Journals (Sweden)

    Gautam Lokdarshi

    2015-01-01

    Full Text Available Orbital sclerosing inflammation is a distinct group of pathologies characterized by indolent growth with minimal or no signs of inflammation. However, contrary to earlier classifications, it should not be considered a chronic stage of acute inflammation. Although rare, orbital IgG4-related disease has been associated with systemic sclerosing pseudotumor-like lesions. Possible mechanisms include autoimmune and IgG4 related defective clonal proliferation. Currently, there is no specific treatment protocol for IgG4-related disease although the response to low dose steroid provides a good response as compared to non-IgG4 sclerosing pseudotumor. Specific sclerosing inflammations (e.g. Wegener's disease, sarcoidosis, Sjogren's syndrome and neoplasms (lymphoma, metastatic breast carcinoma should be ruled out before considering idiopathic sclerosing inflammation as a diagnosis.

  9. Sclerosing Lesions of the Orbit: A Review.

    Science.gov (United States)

    Lokdarshi, Gautam; Pushker, Neelam; Bajaj, Mandeep S

    2015-01-01

    Orbital sclerosing inflammation is a distinct group of pathologies characterized by indolent growth with minimal or no signs of inflammation. However, contrary to earlier classifications, it should not be considered a chronic stage of acute inflammation. Although rare, orbital IgG4-related disease has been associated with systemic sclerosing pseudotumor-like lesions. Possible mechanisms include autoimmune and IgG4 related defective clonal proliferation. Currently, there is no specific treatment protocol for IgG4-related disease although the response to low dose steroid provides a good response as compared to non-IgG4 sclerosing pseudotumor. Specific sclerosing inflammations (e.g. Wegener's disease, sarcoidosis, Sjogren's syndrome) and neoplasms (lymphoma, metastatic breast carcinoma) should be ruled out before considering idiopathic sclerosing inflammation as a diagnosis.

  10. Statistical analyses in disease surveillance systems.

    Science.gov (United States)

    Lescano, Andres G; Larasati, Ria Purwita; Sedyaningsih, Endang R; Bounlu, Khanthong; Araujo-Castillo, Roger V; Munayco-Escate, Cesar V; Soto, Giselle; Mundaca, C Cecilia; Blazes, David L

    2008-11-14

    The performance of disease surveillance systems is evaluated and monitored using a diverse set of statistical analyses throughout each stage of surveillance implementation. An overview of their main elements is presented, with a specific emphasis on syndromic surveillance directed to outbreak detection in resource-limited settings. Statistical analyses are proposed for three implementation stages: planning, early implementation, and consolidation. Data sources and collection procedures are described for each analysis.During the planning and pilot stages, we propose to estimate the average data collection, data entry and data distribution time. This information can be collected by surveillance systems themselves or through specially designed surveys. During the initial implementation stage, epidemiologists should study the completeness and timeliness of the reporting, and describe thoroughly the population surveyed and the epidemiology of the health events recorded. Additional data collection processes or external data streams are often necessary to assess reporting completeness and other indicators. Once data collection processes are operating in a timely and stable manner, analyses of surveillance data should expand to establish baseline rates and detect aberrations. External investigations can be used to evaluate whether abnormally increased case frequency corresponds to a true outbreak, and thereby establish the sensitivity and specificity of aberration detection algorithms.Statistical methods for disease surveillance have focused mainly on the performance of outbreak detection algorithms without sufficient attention to the data quality and representativeness, two factors that are especially important in developing countries. It is important to assess data quality at each state of implementation using a diverse mix of data sources and analytical methods. Careful, close monitoring of selected indicators is needed to evaluate whether systems are reaching their

  11. Expressions and Roles of Periostin in Otolaryngological Diseases

    Directory of Open Access Journals (Sweden)

    Nobuo Ohta

    2014-01-01

    Full Text Available Periostin is a 90-kDa member of the fasciclin-containing family; it functions as part of matricellular proteins, and its production by airway epithelial cells is induced by IL-4 and IL-13. Periostin is secreted by fibroblasts and upregulated in the airway epithelia of patients with bronchial asthma; it is considered to contribute to remodeling under this pathological condition. However, despite many studies in diverse research areas, our overall understanding of this intriguing molecule is still inadequate. Here, we integrate the available evidence on periostin expression and its roles in otolaryngological diseases, including allergic rhinitis, chronic rhinosinusitis with nasal polyps, aspirin-induced asthma, organized hematoma, eosinophilic otitis media, and IgG4-related disease. Periostin might be involved as an important structural mediator in pathological processes such as insult and injury, Th2-driven inflammation, extracellular matrix restructuring, fibrosclerosis, tumor angiogenesis, and tissue remodeling.

  12. A study on periodontal disease and systemic disease relationship a hospital based study in Bangalore

    Directory of Open Access Journals (Sweden)

    Sukhvinder Singh Oberoi

    2013-01-01

    Full Text Available Background: Periodontal deterioration has been reported to be associated with various systemic conditions like Cardiovascular disease, Diabetes, Respiratory disease, Liver cirrhosis, Bacterial Pneumonia, Nutritional deficiencies and adverse pregnancy outcomes. Aim: To assess the periodontal disease among patients with systemic disease/conditions. Materials and Method: A total of 500 patients with systemic disease/conditions (Diabetes, Cardiovascular disease, Respiratory disease and Renal disease and 500-age and gender matched controls without systemic disease/conditions were selected from the Government Hospitals in Bangalore City. The medical conditions were recorded and the periodontal status of the study population was assessed using the CPITN index. Results: The prevalence of CPITN Code 4 was found to be more among the patients with systemic disease/conditions (46.2%. The mean number of sextants with CPITN code 3 and 4 were more among the patients with systemic disease/conditions. The prevalence of CPITN code was found to be more among the patients with Respiratory disease whereas the mean number of sextants was found to be more among the patients with Diabetes, Cardiovascular and Renal disease. Conclusion: It may be concluded that the systemic diseases/conditions are associated with higher severity of periodontal disease.

  13. [Systemic inflammatory rheumatic diseases competence network].

    Science.gov (United States)

    Rufenach, C; Burmester, G-R; Zeidler, H; Radbruch, A

    2004-04-01

    The foundation of the competence network for rheumatology, which is funded by the "Bundesministerium für Bildung und Forschung" (BMBF) since 1999, succeeded to create a unique research structure in Germany: medical doctors and scientists from six university rheumatology centres (Berlin, Düsseldorf, Erlangen, Freiburg, Hannover und Lübeck/Bad Bramstedt) work closely together with scientists doing basic research at the Deutsches Rheuma-Forschungszentrum (DRFZ), with rheumatological hospitals, reha-clinics, and rheumatologists. Jointly they are searching for causes of systemic inflammatory rheumatic diseases and try to improve therapies-nationwide and with an interdisciplinary approach. The primary objective of this collaboration is to transfer new scientific insights more rapidly in order to improve methods for diagnosis and patients treatment.

  14. Poultry Diseases Expert System using Dempster-Shafer Theory

    CERN Document Server

    Maseleno, Andino

    2012-01-01

    Based on World Health Organization (WHO) fact sheet in the 2011, outbreaks of poultry diseases especially Avian Influenza in poultry may raise global public health concerns due to their effect on poultry populations, their potential to cause serious disease in people, and their pandemic potential. In this research, we built a Poultry Diseases Expert System using Dempster-Shafer Theory. In this Poultry Diseases Expert System We describe five symptoms which include depression, combs, wattle, bluish face region, swollen face region, narrowness of eyes, and balance disorders. The result of the research is that Poultry Diseases Expert System has been successfully identifying poultry diseases.

  15. Immunoglobin G4-related disease: Two case reports and review of the recent literature

    Directory of Open Access Journals (Sweden)

    Urmila Anandh

    2015-01-01

    Full Text Available IgG4-related disease (IgG4-RD is a new, multiorgan and constantly evolving disease characterized by IgG4-positive plasma cells in the affected organ. This disease often affects the elderly. The pancreas is the main target organ affected, with almost all organs in the body being affected. A large amount of studies have been conducted to understand the pathogenesis of the disease and its spectrum. For accurate diagnosis of the condition, an adequate knowledge of imaging findings, clinical presentations and laboratory reports is essential. We report two cases of the IgG4-RD and review the recent literature about this increasingly recognized entity.

  16. Animal models for diseases of respiratory system

    Directory of Open Access Journals (Sweden)

    R. Adil

    2012-07-01

    Full Text Available Latest trends in understanding of respiratory diseases in human beings can be derived from thorough clinical studies of these diseases occurring in man, but conducting such studies in man is difficult in terms of experimental manipulation. In the last 2 decades, various types of experimental respiratory disease models has been developed and utilized by investigators, which have contributed a lot to the understanding of respiratory diseases in man, but only little investigation has been done on the naturally occurring pulmonary diseases of animals as potential models which could have added to our knowledge. There are certain selected examples of spontaneous pulmonary disease in animals that may serve as exploitable models for human chronic bronchitis, bronchiectasis, emphysema, interstitial lung disease, hypersensitivity pneumonitis, hyaline membrane disease, and bronchial asthma.

  17. Systems genetics : From GWAS to disease pathways

    NARCIS (Netherlands)

    van der Sijde, Marijke R.; Ng, Aylwin; Fu, Jingyuan

    2014-01-01

    Most common diseases are complex, involving multiple genetic and environmental factors and their interactions. In the past decade, genome-wide association studies (GWAS) have successfully identified thousands of genetic variants underlying susceptibility to complex diseases. However, the results fro

  18. Cutaneous manifestations of systemic tropical parasitic diseases.

    Science.gov (United States)

    Fernandes, Neil F; Kovarik, Carrie L

    2009-01-01

    Tropical diseases continue to cause significant health problems in developing nations. An overview of illnesses with notable cutaneous findings caused by protozoans and helminthes is provided. The role of the health care provider in disease management is described.

  19. Spectacular manifestations of systemic diseases of the snake

    DEFF Research Database (Denmark)

    Da Silva, Mari-Ann Otkjær; Bertelsen, Mads Frost; Heegaard, Steffen

    2015-01-01

    This paper reports histopathological findings in the spectacles of four snakes diagnosed with systemic gout, inclusion body disease, disseminated lymphoma and myeloproliferative disease, respectively. Gout was characterised by urate ghost tophi in the stroma and outer epithelium of the spectacle....

  20. Prevalence of periodontal disease, its association with systemic diseases and prevention.

    Science.gov (United States)

    Nazir, Muhammad Ashraf

    2017-01-01

    Periodontal diseases are prevalent both in developed and developing countries and affect about 20-50% of global population. High prevalence of periodontal disease in adolescents, adults, and older individuals makes it a public health concern. Several risk factors such as smoking, poor oral hygiene, diabetes, medication, age, hereditary, and stress are related to periodontal diseases. Robust evidence shows the association of periodontal diseases with systemic diseases such as cardiovascular disease, diabetes, and adverse pregnancy outcomes. Periodontal disease is likely to cause 19% increase in the risk of cardiovascular disease, and this increase in relative risk reaches to 44% among individuals aged 65 years and over. Type 2 diabetic individuals with severe form of periodontal disease have 3.2 times greater mortality risk compared with individuals with no or mild periodontitis. Periodontal therapy has been shown to improve glycemic control in type 2 diabetic subjects. Periodontitis is related to maternal infection, preterm birth, low birth weight, and preeclampsia. Oral disease prevention strategies should be incorporated in chronic systemic disease preventive initiatives to curtail the burden of disease in populations. The reduction in the incidence and prevalence of periodontal disease can reduce its associated systemic diseases and can also minimize their financial impact on the health-care systems. It is hoped that medical, dental practitioners, and other health-care professionals will get familiar with perio-systemic link and risk factors, and need to refer to the specialized dental or periodontal care.

  1. Prevalence of periodontal disease, its association with systemic diseases and prevention

    Science.gov (United States)

    Nazir, Muhammad Ashraf

    2017-01-01

    Periodontal diseases are prevalent both in developed and developing countries and affect about 20-50% of global population. High prevalence of periodontal disease in adolescents, adults, and older individuals makes it a public health concern. Several risk factors such as smoking, poor oral hygiene, diabetes, medication, age, hereditary, and stress are related to periodontal diseases. Robust evidence shows the association of periodontal diseases with systemic diseases such as cardiovascular disease, diabetes, and adverse pregnancy outcomes. Periodontal disease is likely to cause 19% increase in the risk of cardiovascular disease, and this increase in relative risk reaches to 44% among individuals aged 65 years and over. Type 2 diabetic individuals with severe form of periodontal disease have 3.2 times greater mortality risk compared with individuals with no or mild periodontitis. Periodontal therapy has been shown to improve glycemic control in type 2 diabetic subjects. Periodontitis is related to maternal infection, preterm birth, low birth weight, and preeclampsia. Oral disease prevention strategies should be incorporated in chronic systemic disease preventive initiatives to curtail the burden of disease in populations. The reduction in the incidence and prevalence of periodontal disease can reduce its associated systemic diseases and can also minimize their financial impact on the health-care systems. It is hoped that medical, dental practitioners, and other health-care professionals will get familiar with perio-systemic link and risk factors, and need to refer to the specialized dental or periodontal care. PMID:28539867

  2. The Olig family affects central nervous system development and disease

    Institute of Scientific and Technical Information of China (English)

    Botao Tan; Jing Yu; Ying Yin; Gongwei Jia; Wei Jiang; Lehua Yu

    2014-01-01

    Neural cell differentiation and maturation is a critical step during central nervous system devel-opment. The oligodendrocyte transcription family (Olig family) is known to be an important factor in regulating neural cell differentiation. Because of this, the Olig family also affects acute and chronic central nervous system diseases, including brain injury, multiple sclerosis, and even gliomas. Improved understanding about the functions of the Olig family in central nervous system development and disease will greatly aid novel breakthroughs in central nervous system diseases. This review investigates the role of the Olig family in central nervous system develop-ment and related diseases.

  3. Applications of Next-generation Sequencing in Systemic Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Yiyangzi Ma

    2015-08-01

    Full Text Available Systemic autoimmune diseases are a group of heterogeneous disorders caused by both genetic and environmental factors. Although numerous causal genes have been identified by genome-wide association studies (GWAS, these susceptibility genes are correlated to a relatively low disease risk, indicating that environmental factors also play an important role in the pathogenesis of disease. The intestinal microbiome, as the main symbiotic ecosystem between the host and host-associated microorganisms, has been demonstrated to regulate the development of the body’s immune system and is likely related to genetic mutations in systemic autoimmune diseases. Next-generation sequencing (NGS technology, with high-throughput capacity and accuracy, provides a powerful tool to discover genomic mutations, abnormal transcription and intestinal microbiome identification for autoimmune diseases. In this review, we briefly outlined the applications of NGS in systemic autoimmune diseases. This review may provide a reference for future studies in the pathogenesis of systemic autoimmune diseases.

  4. Applications of Next-generation Sequencing in Systemic Autoimmune Diseases

    Institute of Scientific and Technical Information of China (English)

    Yiyangzi Ma; Na Shi; Mengtao Li; Fei Chen; Haitao Niu

    2015-01-01

    Systemic autoimmune diseases are a group of heterogeneous disorders caused by both genetic and environmental factors. Although numerous causal genes have been identified by genome-wide association studies (GWAS), these susceptibility genes are correlated to a relatively low disease risk, indicating that environmental factors also play an important role in the pathogen-esis of disease. The intestinal microbiome, as the main symbiotic ecosystem between the host and host-associated microorganisms, has been demonstrated to regulate the development of the body’s immune system and is likely related to genetic mutations in systemic autoimmune diseases. Next-generation sequencing (NGS) technology, with high-throughput capacity and accuracy, provides a powerful tool to discover genomic mutations, abnormal transcription and intestinal microbiome identification for autoimmune diseases. In this review, we briefly outlined the applications of NGS in systemic autoimmune diseases. This review may provide a reference for future studies in the pathogenesis of systemic autoimmune diseases.

  5. A computer-aided diagnostic system for kidney disease

    Science.gov (United States)

    Jahantigh, Farzad Firouzi; Malmir, Behnam; Avilaq, Behzad Aslani

    2017-01-01

    Background Disease diagnosis is complicated since patients may demonstrate similar symptoms but physician may diagnose different diseases. There are a few number of investigations aimed to create a fuzzy expert system, as a computer aided system for disease diagnosis. Methods In this research, a cross-sectional descriptive study conducted in a kidney clinic in Tehran, Iran in 2012. Medical diagnosis fuzzy rules applied, and a set of symptoms related to the set of considered diseases defined. The input case to be diagnosed defined by assigning a fuzzy value to each symptom and then three physicians asked about each suspected diseases. Then comments of those three physicians summarized for each disease. The fuzzy inference applied to obtain a decision fuzzy set for each disease, and crisp decision values attained to determine the certainty of existence for each disease. Results Results indicated that, in the diagnosis of seven cases of kidney disease by examining 21 indicators using fuzzy expert system, kidney stone disease with 63% certainty was the most probable, renal tubular was at the lowest level with 15%, and other kidney diseases were at the other levels. The most remarkable finding of this study was that results of kidney disease diagnosis (e.g., kidney stone) via fuzzy expert system were fully compatible with those of kidney physicians. Conclusion The proposed fuzzy expert system is a valid, reliable, and flexible instrument to diagnose several typical input cases. The developed system decreases the effort of initial physical checking and manual feeding of input symptoms.

  6. The application of PET/CT in immunoglobulin G4-related disease%免疫球蛋白G4相关性疾病的PET/CT应用

    Institute of Scientific and Technical Information of China (English)

    张慧玮; 赵军

    2013-01-01

    Immunoglobulin G4 (IgG4)-related disease is a kind of new systemic entity characterized by mass-forming lesions in various organs that consist of lymphoplasmacytic infiltrates and fibrosclerosis with numerous IgG4-positive plasma cells.PET/CT has been widely used in clinical as a whole body examination that earned plenty of successful experience,especially in multiple locations and organs cancer such as lymphoma.PET/CT also has a potential application in IgG4-relatcd diseases as its feature of systemic.%免疫球蛋白G4(IgG4)相关性疾病是一种以器官肿胀、IgG4水平升高和IgG4阳性淋巴浆细胞的组织浸润为特征的纤维炎症性疾病,该疾病可累及多器官.PET/CT作为一种全身检查现已广泛应用于临床,特别是在以淋巴瘤为代表的累及多部位、多器官疾病的应用中已经取得了成功的经验.又因IgG4相关性疾病具有系统性疾病的特点,PET/CT在该疾病中可能具有广阔的应用前景.

  7. A case of immunoglobulin G4-related respiratory disease with multiple lung cysts: A case report

    Directory of Open Access Journals (Sweden)

    Hironori Mikumo

    2017-01-01

    Full Text Available A 48-year-old man was admitted for evaluation of abnormal shadows on chest radiograph. Chest computed tomography (CT showed cysts, nodules, and cervical and axillary lymphadenopathies. Elevated serum levels of IgG4 and interleukin (IL-6 suggested IgG4-related disease (IgG4-RD or multicentric Castleman's disease (MCD. Histologic findings of the cervical lymph node and right lung S6 biopsies revealed numerous IgG4-positive plasma cells. Although CT findings of the lungs were atypical for IgG4-RD, consistent histologic findings, clinical symptoms, and laboratory data made us conclude IgG4-RD. Because histologic findings of IgG4-RD and MCD have similarities, differentiating between the two diseases should consider the clinical presentation.

  8. Research Progression of Cellular Autophagy in Liver System Diseases

    Directory of Open Access Journals (Sweden)

    Chunyun Liu

    2013-09-01

    Full Text Available Autophagy is a basic biological phenomenon widely existed in eukaryotic cells and an important mechanism for cells to adjust to the surrounding environment, prevent invasion of pathogenic micro-organisms and maintain homeostasis, whose activity changes evidently in multiple liver system diseases, suggesting that there is close association between autophagy and the generation and development of liver system diseases. It is also reported that autophagy develops and exerts an important function in many liver-related diseases, such as hepatic carcinoma, non-alcoholic fatty liver disease, alcoholic liver disease, viral liver disease and acute liver injury. Therefore, this study aimed to summarize the relationship between autophagy and multiple liver diseases, hoping to explore the effect of autophagy in liver system diseases and further study the regulative effect of autophagy so as to provide new thoughts for their treatment.

  9. Research Progression of Cellular Autophagy in Liver System Diseases

    Institute of Scientific and Technical Information of China (English)

    Liu Chunyun; Gong Xiangwen; Xiao Xinfa; Yuan Xiangying

    2013-01-01

    Autophagy is a basic biological phenomenon widely existed in eukaryotic cells and an important mechanism for cells to adjust to the surrounding environment, prevent invasion of pathogenic micro-organisms and maintain homeostasis, whose activity changes evidently in multiple liver system diseases, suggesting that there is close association between autophagy and the generation and development of liver system diseases. It is also reported that autophagy develops and exerts an important function in many liver-related diseases, such as hepatic carcinoma, non-alcoholic fatty liver disease, alcoholic liver disease, viral liver disease and acute liver injury. Therefore, this study aimed to summarize the relationship between autophagy and multiple liver diseases, hoping to explore the effect of autophagy in liver system diseases and further study the regulative effect of autophagy so as to provide new thoughts for their treatment.

  10. Evaluation of an automated connective tissue disease screening assay in Korean patients with systemic rheumatic diseases.

    Science.gov (United States)

    Jeong, Seri; Yang, Heeyoung; Hwang, Hyunyong

    2017-01-01

    This study aimed to evaluate the diagnostic utilities of the automated connective tissues disease screening assay, CTD screen, in patients with systemic rheumatic diseases. A total of 1093 serum samples were assayed using CTD screen and indirect immunofluorescent (IIF) methods. Among them, 162 were diagnosed with systemic rheumatic disease, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCT). The remaining 931 with non-systemic rheumatic disease were assigned to the control group. The median ratios of CTD screen tests were significantly higher in the systemic rheumatic disease group than in the control group. The positive likelihood ratios of the CTD screen were higher than those of IIF in patients with total rheumatic diseases (4.1 vs. 1.6), including SLE (24.3 vs. 10.7). The areas under the receiver operating characteristic curves (ROC-AUCs) of the CTD screen for discriminating total rheumatic diseases, RA, SLE, and MCT from controls were 0.68, 0.56, 0.92 and 0.80, respectively. The ROC-AUCs of the combinations with IIF were significantly higher in patients with total rheumatic diseases (0.72) and MCT (0.85) than in those of the CTD screen alone. Multivariate analysis indicated that both the CTD screen and IIF were independent variables for predicting systemic rheumatic disease. CTD screen alone and in combination with IIF were a valuable diagnostic tool for predicting systemic rheumatic diseases, particularly for SLE.

  11. Disease Compass- a navigation system for disease knowledge based on ontology and linked data techniques.

    Science.gov (United States)

    Kozaki, Kouji; Yamagata, Yuki; Mizoguchi, Riichiro; Imai, Takeshi; Ohe, Kazuhiko

    2017-06-19

    Medical ontologies are expected to contribute to the effective use of medical information resources that store considerable amount of data. In this study, we focused on disease ontology because the complicated mechanisms of diseases are related to concepts across various medical domains. The authors developed a River Flow Model (RFM) of diseases, which captures diseases as the causal chains of abnormal states. It represents causes of diseases, disease progression, and downstream consequences of diseases, which is compliant with the intuition of medical experts. In this paper, we discuss a fact repository for causal chains of disease based on the disease ontology. It could be a valuable knowledge base for advanced medical information systems. We developed the fact repository for causal chains of diseases based on our disease ontology and abnormality ontology. This section summarizes these two ontologies. It is developed as linked data so that information scientists can access it using SPARQL queries through an Resource Description Framework (RDF) model for causal chain of diseases. We designed the RDF model as an implementation of the RFM for the fact repository based on the ontological definitions of the RFM. 1554 diseases and 7080 abnormal states in six major clinical areas, which are extracted from the disease ontology, are published as linked data (RDF) with SPARQL endpoint (accessible API). Furthermore, the authors developed Disease Compass, a navigation system for disease knowledge. Disease Compass can browse the causal chains of a disease and obtain related information, including abnormal states, through two web services that provide general information from linked data, such as DBpedia, and 3D anatomical images. Disease Compass can provide a complete picture of disease-associated processes in such a way that fits with a clinician's understanding of diseases. Therefore, it supports user exploration of disease knowledge with access to pertinent information

  12. Interstitial Lung disease in Systemic Sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Ooi, G.C.; Mok, M.Y.; Tsang, K.W.T.; Khong, P.L.; Fung, P.C.W.; Chan, S.; Tse, H.F.; Wong, R.W.S.; Lam, W.K.; Lau, C.S. [Queen Mary Hospital, Hong Kong (China). Dept. of Diagnostic Radiology and Medicine; Wong, Y. [Queen Mary Hospital, Hong Kong (China). Dept. of Radiology

    2003-05-01

    Purpose: To evaluate high-resolution CT (HRCT) parameters of inflammation and fibrosis in systemic sclerosis (SSc), for correlation with lung function, skin scores and exercise tolerance. Material and Methods: : 45 SSc patients (40 women, 48.5{+-}13.4 years), underwent thoracic HRCT, lung function assessment, and modified Rodnan skin scores. Exercise tolerance was also graded. HRCT were scored for extent of 4 HRCT patterns of interstitial lung disease (ILD): ground glass opacification (GGO), reticular, mixed and honeycomb pattern in each lobe. Total HRCT score, inflammation index (GGO and mixed score) and fibrosis index (reticular and honeycomb scores) were correlated with lung function and clinical parameters. Results: ILD was present in 39/45 (86.7%) patients. Abnormal (<80% predicted) forced vital capacity (FVC), total lung capacity (TLC) and carbon monoxide diffusion factor (DLco) were detected in 30%, 22% and 46% of patients. Total HRCT score correlated with FVC (r=0.43, p=0.008), FEV1 (forced expiratory volume) (r=-0.37, p=0.03), TLC (r=-0.47, p=0.003), and DLCO (r=-0.43, p=0.008); inflammatory index with DLCO (r=-0.43, p=0.008) and exercise tolerance (r=-0.39, p < 0.05); and fibrosis index with FVC (r=-0.31, p=0.05) and TLC (r=-0.38, p=0.02). Higher total HRCT score, and inflammation and fibrosis indices were found in patients with abnormal lung function. Conclusion: Qualitative HRCT is able to evaluate inflammation and fibrosis, showing important relationships with diffusion capacity and lung volume, respectively.

  13. IgG4 Inflammatory Pseudotumor of the Kidney

    Directory of Open Access Journals (Sweden)

    Ahmed N. Alkhasawneh

    2012-01-01

    Full Text Available Hyper-IgG4 disease is a rare systemic disorder that usually affects middle age males. It is characterized by elevated serum IgG4 levels and infiltration of organs by IgG4 positive plasma cells associated with fibrosis. Patients usually present with mass or masses in the involved organ that mimic neoplasia. While initially described in the pancreas, IgG4-related inflammatory tumors have been now described in many organs. We describe an unusual case of an IgG4-related pseudotumor of the kidney.

  14. Corneal manifestations of selected systemic diseases: A review

    Directory of Open Access Journals (Sweden)

    Wayne D.H. Gillan

    2015-03-01

    Full Text Available The corneal manifestations of several selected systemic diseases are reviewed. Metabolic, immunologic and inflammatory and infectious diseases are included. A brief overview of each disease and how it manifests in the cornea is discussed. The importance of conducting a slit-lamp examination on every patient is emphasised.

  15. Chapter 15. Plant pathology and managing wildland plant disease systems

    Science.gov (United States)

    David L. Nelson

    2004-01-01

    Obtaining specific, reliable knowledge on plant diseases is essential in wildland shrub resource management. However, plant disease is one of the most neglected areas of wildland resources experimental research. This section is a discussion of plant pathology and how to use it in managing plant disease systems.

  16. Peripheral Vestibular System Disease in Vestibular Schwannomas

    DEFF Research Database (Denmark)

    Møller, Martin Nue; Hansen, Søren; Caye-Thomasen, Per

    2015-01-01

    density of the peripheral vestibular nerve branches, and atrophy of the neuroepithelium of the vestibular end organs. In cases with small tumors, peripheral disease occurred only in the tissue structures innervated by the specific nerve from which the tumor originated. CONCLUSION: Vestibular schwannomas...... are associated with distinctive disease of the peripheral vestibular tissue structures, suggesting anterograde degeneration and that dizziness in these patients may be caused by deficient peripheral vestibular nerve fibers, neurons, and end organs. In smaller tumors, a highly localized disease occurs, which...

  17. The effect of liver disease on the cardiovascular system

    DEFF Research Database (Denmark)

    Henriksen, Jens Henrik; Møller, Søren

    2007-01-01

    and clinical practice discussed by the best authors.It includes unique sections on: Symptoms and signs in liver diseaseIndustrial diseases affecting the liverThe effects of diseases of other systems on the liverThe effects of liver diseases on other systemsIt 's bigger and more extensive than other books...... into the practical applications to benefit people).Edited by ten leading experts in the liver and biliary tract and their diseases, along with outstanding contributions from over 200 international clinicians, this text has global references, evidence and extensive subject matter - giving you the best science...

  18. Diagnosis of Skin Diseases using Online Expert System

    Directory of Open Access Journals (Sweden)

    Muhammad Zubair Asghar

    2011-06-01

    Full Text Available This paper describes Expert System (ES for diagnosis and management of skin diseases. More than 13 types of skin diseases can be diagnosed and treated by our system. It is rule based web-supported expert system, assisting skin specialists, medical students doing specialization in dermatology, researchers as well as skin patients having computer know-how. System was developed with Java Technology. The expert rules were developed on the symptoms of each type of skin disease, and they were presented using tree-graph and inferred using forward-chaining with depth-first search method. User interaction with system is enhanced with efficient user interfaces. The web based expert system described in this paper can detect and give early diagnosis of thirteen plus skin diseases. This ES can be extended to diagnose all types of skin-diseases.

  19. The Diagnostic Value of Skin Disease Diagnosis Expert System.

    Science.gov (United States)

    Jeddi, Fatemeh Rangraz; Arabfard, Masoud; Arabkermany, Zahra; Gilasi, Hamidreza

    2016-02-01

    Evaluation is a necessary measure to ensure the effectiveness and efficiency of all systems, including expert systems. The aim of this study was to determine the diagnostic value of expert system for diagnosis of complex skin diseases. A case-control study was conducted in 2015 to determine the diagnostic value of an expert system. The study population included patients who were referred to Razi Specialized Hospital, affiliated to Tehran University of Medical Sciences. The control group was selected from patients without the selected skin diseases. Data collection tool was a checklist of clinical signs of diseases including pemphigus vulgaris, lichen planus, basal cell carcinoma, melanoma, and scabies. The sample size formula estimated 400 patients with skin diseases selected by experts and 200 patients without the selected skin diseases. Patient selection was undertaken with randomized stratified sampling and their sign and symptoms were logged into the system. Physician's diagnosis was determined as the gold standard and was compared with the diagnosis of expert system by SPSS software version 16 and STATA. Kappa statistics, indicators of sensitivity, specificity, accuracy and confidence intervals were calculated for each disease. An accuracy of 90% was considered appropriate. Comparing the results of expert system and physician's diagnosis at the evaluation stage showed an accuracy of 97.1%, sensitivity of 97.5% and specificity of 96.5% The Kappa test indicated a high agreement of 93.6%. The expert system can diagnose complex skin diseases. Development of such systems is recommended to identify all skin diseases.

  20. ACUTE RESPIRATORY DISEASE AS THE DEBUT OF SYSTEMIC LUPUS ERYTHEMATOSUS

    Directory of Open Access Journals (Sweden)

    A. Yu. Ischenko

    2015-01-01

    Full Text Available Systemic lupus erythematosus — a chronic autoimmune disease that is often associated with infectious processes. The paper presents two clinical cases of systemic lupus erythematosus , debuted with acute respiratory infection.

  1. Hansen's disease mimicking a systemic vasculitis.

    Science.gov (United States)

    Sampaio, L; Silva, L; Terroso, G; Pimenta, S; Brandão, F; Pinto, J; Prisca, A; Brito, J; Ventura, F

    2011-01-01

    Hansen's disease, caused by Mycobacterium leprae, classically presents with cutaneous and neurological manifestations. Rheumatologic manifestations present in 1 to 5% of the patients, and include arthritis, arthralgias, Charcot arthropathy, erythema nodosum and vasculitis. We report a case of a 86 year old woman with polyarthritis, subcutaneous nodules and leg ulcers whose differential diagnosis included primary vasculitis and diffuse connective tissue diseases and ended to be leprosy in a non endemic country.

  2. Early Stage Disease Diagnosis System Using Human Nail Image Processing

    Directory of Open Access Journals (Sweden)

    Trupti S. Indi

    2016-07-01

    Full Text Available Human’s hand nail is analyzed to identify many diseases at early stage of diagnosis. Study of person hand nail color helps in identification of particular disease in healthcare domain. The proposed system guides in such scenario to take decision in disease diagnosis. The input to the proposed system is person nail image. The system will process an image of nail and extract features of nail which is used for disease diagnosis. Human nail consist of various features, out of which proposed system uses nail color changes for disease diagnosis. Here, first training set data is prepared using Weka tool from nail images of patients of specific diseases. A feature extracted from input nail image is compared with the training data set to get result. In this experiment we found that using color feature of nail image average 65% results are correctly matched with training set data during three tests conducted.

  3. [Pregnancy in systemic autoimmune diseases: Myths, certainties and doubts].

    Science.gov (United States)

    Danza, Álvaro; Ruiz-Irastorza, Guillermo; Khamashta, Munther

    2016-10-07

    Systemic autoimmune diseases especially affect young women during childbearing age. The aim of this review is to update systemic lupus erythematosus, antiphospholipid syndrome and systemic sclerosis management during pregnancy. These diseases present variable maternal and fetal risks. Studies show that an appropriate disease control and a reasonable remission period prior to pregnancy are associated with satisfactory obstetric outcomes. Antiphospholipid autoantibodies profile, anti-Ro/anti-La antibodies, pulmonary pressure and activity evaluation are crucial to assess the pregnancy risk. Monitoring requires a multidisciplinary team, serial analytic controls and Doppler ultrasound of maternal and fetal circulation. Evaluation of the activity of the disease is essential.

  4. [Development of expert diagnostic system for common respiratory diseases].

    Science.gov (United States)

    Xu, Wei-hua; Chen, You-ling; Yan, Zheng

    2014-03-01

    To develop an internet-based expert diagnostic system for common respiratory diseases. SaaS system was used to build architecture; pattern of forward reasoning was applied for inference engine design; ASP.NET with C# from the tool pack of Microsoft Visual Studio 2005 was used for website-interview medical expert system.The database of the system was constructed with Microsoft SQL Server 2005. The developed expert system contained large data memory and high efficient function of data interview and data analysis for diagnosis of various diseases.The users were able to perform this system to obtain diagnosis for common respiratory diseases via internet. The developed expert system may be used for internet-based diagnosis of various respiratory diseases,particularly in telemedicine setting.

  5. Australia's notifiable disease status, 2014: Annual report of the National Notifiable Diseases Surveillance System.

    Science.gov (United States)

    2016-03-31

    In 2014, 69 diseases and conditions were nationally notifiable in Australia. States and territories reported a total of 275,581 notifications of communicable diseases to the National Notifiable Diseases Surveillance System, an increase of 22% on the number of notifications in 2013. In 2014, the most frequently notified diseases were sexually transmissible infections (105,719 notifications, 38% of total notifications), vaccine preventable diseases (101,400 notifications, 37% of total notifications), and gastrointestinal diseases (40,367 notifications, 15% of total notifications). There were 17,411 notifications of bloodborne diseases; 8,125 notifications of vectorborne diseases; 1,942 notifications of other bacterial infections; 615 notifications of zoonoses and 2 notifications of quarantinable diseases.

  6. Disease management in soilless culture systems

    NARCIS (Netherlands)

    Os, van E.A.

    2010-01-01

    EU legislation, laid down in the Water Framework Directive, demands to minimize emissions of nitrogen, phosphate and crop protection products to achieve an excellent chemical and ecological quality in 2015. The aim is to force growers to a better water and disease management. Supply water of excelle

  7. Disease management in soilless culture systems

    NARCIS (Netherlands)

    Os, van E.A.

    2010-01-01

    EU legislation, laid down in the Water Framework Directive, demands to minimize emissions of nitrogen, phosphate and crop protection products to achieve an excellent chemical and ecological quality in 2015. The aim is to force growers to a better water and disease management. Supply water of

  8. Disease management in soilless culture systems

    NARCIS (Netherlands)

    Os, van E.A.

    2010-01-01

    EU legislation, laid down in the Water Framework Directive, demands to minimize emissions of nitrogen, phosphate and crop protection products to achieve an excellent chemical and ecological quality in 2015. The aim is to force growers to a better water and disease management. Supply water of excelle

  9. Immunomodulation by the autonomic nervous system: therapeutic approach for cancer, collagen diseases, and inflammatory bowel diseases.

    Science.gov (United States)

    Abo, Toru; Kawamura, Toshihiko

    2002-10-01

    The distribution of leukocytes is regulated by the autonomic nervous system in humans and animals. The number and function of granulocytes are stimulated by sympathetic nerves whereas those of lymphocytes are stimulated by parasympathetic nerves. This is because granulocytes bear adrenergic receptors, but lymphocytes bear cholinergic receptors on the surface. These regulations may be beneficial to protect the body of living beings. However, when the autonomic nervous system deviates too much to one direction, we fall victim to certain diseases. For example, severe physical or mental stress --> sympathetic nerve activation --> granulocytosis --> tissue damage, including collagen diseases, inflammatory bowel diseases, and cancer. If we introduce the concept of immunomodulation by the autonomic nervous system, a new approach for collagen diseases, inflammatory bowel diseases, and even cancer is raised. With this approach, we believe that these diseases are no longer incurable.

  10. PERIODONTAL INFECTION AS A POTENTIAL RISK FACTOR FOR SYSTEMIC DISEASE

    Directory of Open Access Journals (Sweden)

    Sumintarti Sumintarti

    2015-06-01

    Full Text Available Oral infection can have an adverse effect on other organs of the body. Oral infections, especially periodontitis, may affect the course and pathogenesis of a number of systemic diseases, such as diabetes mellitus, cardiovascular disease, pre-term low birth weight infant and respiratory disease. The purpose of this article is to evaluate the current status of oral infection especially periodontitis as a potential risk factor of systemic diseases. Three main pathways linking oral infection to secondary systemic effects have been proposed: metastatic infection, metastatic injury and metastatic inflammation. Periodontitis can cause bacteria to enter the blood stream and activate immune cells. These activated cells produce inflammatory cytokines that have a destructive effect throughout the entire body. Therefore, periodontitis as a major oral infection may affect the host’s susceptibility to systemic disease.

  11. POSSIBILITYIES OF PHYTOTHERAPY AT DIGESTIVE SYSTEM DISEASES

    Directory of Open Access Journals (Sweden)

    A. V. Kurkina

    2016-01-01

    Full Text Available In the present paper the modern approaches to the reasonable using of phytopharmaceuticals for diseases of gastrointestinal tract were discussed. Substantiates using groups of medicinal plants for the treatment of diseases of gastrointestinal tract based on important relationship «biologically active substance-pharmacological effect». According modern data of chemical composition and pharmacological activity in the attitude to this pathology the most important species of medicinal plants are considered. The importance of principle of phytotherapy’s safety as a main approach of evidential medicine was highlighted. This scientific paper will help experts to make an evidence-based decision of medicinal plants and phytopreparations based on them in clinical practice.

  12. Automated system for periodontal disease diagnosis

    Science.gov (United States)

    Albalat, Salvador E.; Alcaniz-Raya, Mariano L.; Juan, M. Carmen; Grau Colomer, Vincente; Monserrat, Carlos

    1997-04-01

    Evolution of periodontal disease is one of the most important data for the clinicians in order to achieve correct planning and treatment. Clinical measure of the periodontal sulcus depth is the most important datum to know the exact state of periodontal disease. These measures must be done periodically study bone resorption evolution around teeth. Time factor of resorption indicates aggressiveness of periodontitis. Manual probes are commonly used with direct reading. Mechanical probes give automatic signal but this method uses complicated and heavy probes that are only limited for University researchers. Probe position must be the same to have right diagnosis. Digital image analysis of periodontal probing provides practical, accurate and easy tool. Gum and plaque index could also be digitally measured with this method.

  13. Microbial Nucleic Acid Sensing in Oral and Systemic Diseases.

    Science.gov (United States)

    Crump, K E; Sahingur, S E

    2016-01-01

    One challenge in studying chronic infectious and inflammatory disorders is understanding how host pattern recognition receptors (PRRs), specifically toll-like receptors (TLRs), sense and respond to pathogen- or damage-associated molecular patterns, their communication with each other and different components of the immune system, and their role in propagating inflammatory stages of disease. The discovery of innate immune activation through nucleic acid recognition by intracellular PRRs such as endosomal TLRs (TLR3, TLR7, TLR8, and TLR9) and cytoplasmic proteins (absent in melanoma 2 and DNA-dependent activator of interferon regulatory factor) opened a new paradigm: Nucleic acid sensing is now implicated in multiple immune and inflammatory conditions (e.g., atherosclerosis, cancer), viral (e.g., human papillomavirus, herpes virus) and bacterial (e.g., Helicobacter pylori, pneumonia) diseases, and autoimmune disorders (e.g., systemic lupus erythematosus, rheumatoid arthritis). Clinical investigations reveal the overexpression of specific nucleic acid sensors in diseased tissues. In vivo animal models show enhanced disease progression associated with receptor activation. The involvement of nucleic acid sensors in various systemic conditions is further supported by studies reporting receptor knockout mice being either protected from or prone to disease. TLR9-mediated inflammation is also implicated in periodontal diseases. Considering that persistent inflammation in the oral cavity is associated with systemic diseases and that oral microbial DNA is isolated at distal sites, nucleic acid sensing may potentially be a link between oral and systemic diseases. In this review, we discuss recent advances in how intracellular PRRs respond to microbial nucleic acids and emerging views on the role of nucleic acid sensors in various systemic diseases. We also highlight new information on the role of intracellular PRRs in the pathogenesis of oral diseases including periodontitis

  14. The microbiome-systemic diseases connection

    NARCIS (Netherlands)

    van der Meulen, T. A.; Harmsen, H. J. M.; Bootsma, H.; Spijkervet, F. K. L.; Kroese, F. G. M.; Vissink, A.

    2016-01-01

    The human microbiome consists of all microorganisms occupying the skin, mucous membranes and intestinal tract of the human body. The contact of the mucosal immune system with the human microbiome is a balanced interplay between defence mechanisms of the immune system and symbiotic or pathogenic micr

  15. Evaluation of malnutrition in patients with nervous system disease.

    Science.gov (United States)

    Li, Feng; Liu, Yao-wen; Wang, Xue-feng; Liu, Guang-wei

    2014-10-01

    Nutritional deficiencies are independent risk factors for adverse outcomes in patients with nervous system disease. Patients with nervous system disease can often become malnourished due to swallowing difficulties or unconsciousness. This malnourishment increases hospitalization duration; average total hospital cost; occurrence of infection, pressure ulcers, and other complications. These problems need to be addressed in the clinic. In this paper, we review the relevant literature, including studies on influencing factors, evaluations, indexes, and methods: Our aim is to understand the current status of malnutrition in patients with nervous system disease and reasons associated with nutritional deficiencies by using malnutrition evaluation methods to assess the risk of nutritional deficiencies in the early stages.

  16. Multi-disease data management system platform for vector-borne diseases.

    Directory of Open Access Journals (Sweden)

    Lars Eisen

    Full Text Available BACKGROUND: Emerging information technologies present new opportunities to reduce the burden of malaria, dengue and other infectious diseases. For example, use of a data management system software package can help disease control programs to better manage and analyze their data, and thus enhances their ability to carry out continuous surveillance, monitor interventions and evaluate control program performance. METHODS AND FINDINGS: We describe a novel multi-disease data management system platform (hereinafter referred to as the system with current capacity for dengue and malaria that supports data entry, storage and query. It also allows for production of maps and both standardized and customized reports. The system is comprised exclusively of software components that can be distributed without the user incurring licensing costs. It was designed to maximize the ability of the user to adapt the system to local conditions without involvement of software developers. Key points of system adaptability include 1 customizable functionality content by disease, 2 configurable roles and permissions, 3 customizable user interfaces and display labels and 4 configurable information trees including a geographical entity tree and a term tree. The system includes significant portions of functionality that is entirely or in large part re-used across diseases, which provides an economy of scope as new diseases downstream are added to the system at decreased cost. CONCLUSIONS: We have developed a system with great potential for aiding disease control programs in their task to reduce the burden of dengue and malaria, including the implementation of integrated vector management programs. Next steps include evaluations of operational implementations of the current system with capacity for dengue and malaria, and the inclusion in the system platform of other important vector-borne diseases.

  17. Dynamic Recommendation: Disease Prediction and Prevention Using Recommender System

    Directory of Open Access Journals (Sweden)

    Mahdi Nasiri

    2016-06-01

    Full Text Available Background: In today’s world, chronic diseases are predominant health problems and cause heavy burden on society; therefore early diagnosis and even prediction of the disease is a way to reduce this burden. In this project, we tried to use recommender system to predict which other diseases a chronic patient is susceptible for. Methods: In this study, through a dynamic recommender system, we evaluated patients’ treatment destiny during the time. Results: It was shown that our method increased accuracy and reduced error compared with other recommendation methods in disease prediction. Conclusion: Compared to current usual methods, in our method we used previous patients’ characteristics as one of the factorization variables to predict destiny of future patients. Furthermore, using this method, we can predict which complication or disease the patient would suffer from first in future. Therefore, we can manage policies toward disease burden reduction by implementing prevention programs.

  18. [Kidney allograft: a target for systemic disease].

    Science.gov (United States)

    Canaud, Guillaume; Legendre, Christophe

    2012-03-01

    Recurrence of disease after transplantation is frequent and represents the third cause of allograft loss. Recurrence of lupus nephritis after transplantation is rare. Kidney transplantation in patients with antiphospholipid syndrome or lupus anticoagulant is challenging due to the high risk of immediate post-transplant thrombosis and bleeding risk associated to the subsequent anticoagulation. Moreover, vascular changes associated to the presence of antiphospholipid antibodies negatively impact allograft rate survival. Recurrence of pauci immune glomerulonephritis or Goodpasture syndrome is exceptional. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  19. [Mental disorders in digestive system diseases - internist's and psychiatrist's insight].

    Science.gov (United States)

    Kukla, Urszula; Łabuzek, Krzysztof; Chronowska, Justyna; Krzystanek, Marek; Okopień, BogusŁaw

    2015-05-01

    Mental disorders accompanying digestive system diseases constitute interdisciplinary yet scarcely acknowledged both diagnostic and therapeutic problem. One of the mostly recognized examples is coeliac disease where patients endure the large spectrum of psychopathological symptoms, starting with attention deficit all the way down to the intellectual disability in extreme cases. It has not been fully explained how the pathomechanism of digestive system diseases affects patient's mental health, however one of the hypothesis suggests that it is due to serotonergic or opioid neurotransmission imbalance caused by gluten and gluten metabolites effect on central nervous system. Behavioral changes can also be invoked by liver or pancreatic diseases, which causes life-threatening abnormalities within a brain. It occurs that these abnormalities reflexively exacerbate the symptoms of primary somatic disease and aggravate its course, which worsens prognosis. The dominant mental disease mentioned in this article is depression which because of its effect on a hypothalamuspituitary- adrenal axis and on an autonomic nervous system, not only aggravates the symptoms of inflammatory bowel diseases but may accelerate their onset in genetically predisposed patients. Depression is known to negatively affects patients' ability to function in a society and a quality of their lives. Moreover, as far as children are concerned, the occurrence of digestive system diseases accompanied by mental disorders, may adversely affect their further physical and psychological development, which merely results in worse school performance. All those aspects of mental disorders indicate the desirability of the psychological care for patients with recognized digestive system disease. The psychological assistance should be provided immediately after diagnosis of a primary disease and be continued throughout the whole course of treatment.

  20. Relationship between periodontal disease and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Carlos Marcelo da Silva Figueiredo

    2008-01-01

    Full Text Available There has been a constant search for a definition of criteria of susceptibility to periodontal diseases in modern Periodontology, as it is of the greatest importance, since it enables improvements in prevention, early diagnosis, treatment and prognosis of this disease. It is in this context that this literature review was conducted, with the object of verifying whether there is a greater propensity to periodontal disease in individuals with systemic lúpus erythematosus, a chronic inflammatory disease, whose pathogenesis presents some characteristics similar to those of the pathogenesis of periodontal disease. The majority of existent studies about the subject have pointed towards the possible existence of such susceptibility. Nevertheless, the relationship between this autoimmune disease and periodontal disease has not yet been well established, and the mechanisms by which the systemic disease influences the periodontal health of patients affected with it are not yet clear. Further studies are required in order to affirm that individuals with systemic lúpus erythematosus are a group of risk for the development of periodontal disease, and in order to understand the mechanisms involved in this possible relationship of susceptibility.

  1. Forest Insect and Disease Tally System (FINDIT) user manual

    Science.gov (United States)

    Barbara J. Bentz

    2000-01-01

    FINDIT, the Forest Insect and Disease Tally System, is an easy-to-use tool for analyzing insect and disease population information taken during stand surveys. Incidence of insects, pathogens, and other biotic and abiotic influences on forest ecosystems are summarized using traditional mensurational measurements. Information is summarized by diameter class, tree species...

  2. Information Supply Chain System for Managing Rare Infectious Diseases

    Science.gov (United States)

    Gopalakrishna-Remani, Venugopal

    2012-01-01

    Timely identification and reporting of rare infectious diseases has important economic, social and health implications. In this study, we investigate how different stakeholders in the existing reporting system influence the timeliness in identification and reporting of rare infectious diseases. Building on the vision of the information supply…

  3. Information Supply Chain System for Managing Rare Infectious Diseases

    Science.gov (United States)

    Gopalakrishna-Remani, Venugopal

    2012-01-01

    Timely identification and reporting of rare infectious diseases has important economic, social and health implications. In this study, we investigate how different stakeholders in the existing reporting system influence the timeliness in identification and reporting of rare infectious diseases. Building on the vision of the information supply…

  4. Conceptual Foundations of Systems Biology Explaining Complex Cardiac Diseases.

    Science.gov (United States)

    Louridas, George E; Lourida, Katerina G

    2017-02-21

    Systems biology is an important concept that connects molecular biology and genomics with computing science, mathematics and engineering. An endeavor is made in this paper to associate basic conceptual ideas of systems biology with clinical medicine. Complex cardiac diseases are clinical phenotypes generated by integration of genetic, molecular and environmental factors. Basic concepts of systems biology like network construction, modular thinking, biological constraints (downward biological direction) and emergence (upward biological direction) could be applied to clinical medicine. Especially, in the field of cardiology, these concepts can be used to explain complex clinical cardiac phenotypes like chronic heart failure and coronary artery disease. Cardiac diseases are biological complex entities which like other biological phenomena can be explained by a systems biology approach. The above powerful biological tools of systems biology can explain robustness growth and stability during disease process from modulation to phenotype. The purpose of the present review paper is to implement systems biology strategy and incorporate some conceptual issues raised by this approach into the clinical field of complex cardiac diseases. Cardiac disease process and progression can be addressed by the holistic realistic approach of systems biology in order to define in better terms earlier diagnosis and more effective therapy.

  5. Conceptual Foundations of Systems Biology Explaining Complex Cardiac Diseases

    Directory of Open Access Journals (Sweden)

    George E. Louridas

    2017-02-01

    Full Text Available Systems biology is an important concept that connects molecular biology and genomics with computing science, mathematics and engineering. An endeavor is made in this paper to associate basic conceptual ideas of systems biology with clinical medicine. Complex cardiac diseases are clinical phenotypes generated by integration of genetic, molecular and environmental factors. Basic concepts of systems biology like network construction, modular thinking, biological constraints (downward biological direction and emergence (upward biological direction could be applied to clinical medicine. Especially, in the field of cardiology, these concepts can be used to explain complex clinical cardiac phenotypes like chronic heart failure and coronary artery disease. Cardiac diseases are biological complex entities which like other biological phenomena can be explained by a systems biology approach. The above powerful biological tools of systems biology can explain robustness growth and stability during disease process from modulation to phenotype. The purpose of the present review paper is to implement systems biology strategy and incorporate some conceptual issues raised by this approach into the clinical field of complex cardiac diseases. Cardiac disease process and progression can be addressed by the holistic realistic approach of systems biology in order to define in better terms earlier diagnosis and more effective therapy.

  6. Conceptual Foundations of Systems Biology Explaining Complex Cardiac Diseases

    Science.gov (United States)

    Louridas, George E.; Lourida, Katerina G.

    2017-01-01

    Systems biology is an important concept that connects molecular biology and genomics with computing science, mathematics and engineering. An endeavor is made in this paper to associate basic conceptual ideas of systems biology with clinical medicine. Complex cardiac diseases are clinical phenotypes generated by integration of genetic, molecular and environmental factors. Basic concepts of systems biology like network construction, modular thinking, biological constraints (downward biological direction) and emergence (upward biological direction) could be applied to clinical medicine. Especially, in the field of cardiology, these concepts can be used to explain complex clinical cardiac phenotypes like chronic heart failure and coronary artery disease. Cardiac diseases are biological complex entities which like other biological phenomena can be explained by a systems biology approach. The above powerful biological tools of systems biology can explain robustness growth and stability during disease process from modulation to phenotype. The purpose of the present review paper is to implement systems biology strategy and incorporate some conceptual issues raised by this approach into the clinical field of complex cardiac diseases. Cardiac disease process and progression can be addressed by the holistic realistic approach of systems biology in order to define in better terms earlier diagnosis and more effective therapy. PMID:28230815

  7. End-Stage Renal Disease Prospective Payment System

    Data.gov (United States)

    U.S. Department of Health & Human Services — This final rule implements a case-mix adjusted bundled prospective payment system (PPS) for Medicare outpatient end-stage renal disease (ESRD) dialysis facilities...

  8. The use and role of predictive systems in disease management.

    Science.gov (United States)

    Gent, David H; Mahaffee, Walter F; McRoberts, Neil; Pfender, William F

    2013-01-01

    Disease predictive systems are intended to be management aids. With a few exceptions, these systems typically do not have direct sustained use by growers. Rather, their impact is mostly pedagogic and indirect, improving recommendations from farm advisers and shaping management concepts. The degree to which a system is consulted depends on the amount of perceived new, actionable information that is consistent with the objectives of the user. Often this involves avoiding risks associated with costly disease outbreaks. Adoption is sensitive to the correspondence between the information a system delivers and the information needed to manage a particular pathosystem at an acceptable financial risk; details of the approach used to predict disease risk are less important. The continuing challenge for researchers is to construct tools relevant to farmers and their advisers that improve upon their current management skill. This goal requires an appreciation of growers' decision calculus in managing disease problems and, more broadly, their overall farm enterprise management.

  9. COPD is a systemic disease – the ex trapulmonary manifestations

    African Journals Online (AJOL)

    COPD is defined as a preventable and treatable respiratory disease ... inhalation of noxious particles, particularly cigarette smoke. Although ... Depression and anxiety .... Cruetzberg E. Leptin in relation to systemic inflammation and regulation.

  10. Advances in diagnosis and treatment of cerebral venous system diseases

    Directory of Open Access Journals (Sweden)

    Xiao-yun LIU

    2016-11-01

    Full Text Available Cerebral venous system diseases include cerebral venous thrombosis (CVT, venous sinus stenosis, carotid cavernous fistula (CCF, intracranial arteriovenous malformation (AVM and so on. In recent years, due to the rapid development of neuroimaging and interventional technology, more and more cerebral venous system diseases have been timely diagnosed and treated, such as magnetic resonance black-blood thrombus imaging (MRBTI in the diagnosis of CVT, stenting in the treatment of venous sinus stenosis, micro coil plus Onyx glue or covered stents in the treatment of CCF, which allow us to make a deeper recognition of cerebral venous system diseases. Therefore, this paper will introduce the latest diagnosis and treatment of cerebral venous system diseases. DOI: 10.3969/j.issn.1672-6731.2016.11.006

  11. The eye in systemic disease | Lenake | South African Family Practice

    African Journals Online (AJOL)

    Abstract. The eye is a unique organ which is often involved in systemic disease. ... virus/acquired immune deficiency syndrome, syphilis, some dermatological conditions and the ocular side-effects of certain drugs, are discussed in this article.

  12. Human Disease Diagnosis Using a Fuzzy Expert System

    CERN Document Server

    Hasan, Mir Anamul; Chowdhury, Ahsan Raja

    2010-01-01

    Human disease diagnosis is a complicated process and requires high level of expertise. Any attempt of developing a web-based expert system dealing with human disease diagnosis has to overcome various difficulties. This paper describes a project work aiming to develop a web-based fuzzy expert system for diagnosing human diseases. Now a days fuzzy systems are being used successfully in an increasing number of application areas; they use linguistic rules to describe systems. This research project focuses on the research and development of a web-based clinical tool designed to improve the quality of the exchange of health information between health care professionals and patients. Practitioners can also use this web-based tool to corroborate diagnosis. The proposed system is experimented on various scenarios in order to evaluate it's performance. In all the cases, proposed system exhibits satisfactory results.

  13. Psoriasis and systemic inflammatory diseases: potential mechanistic links between skin disease and co-morbid conditions.

    NARCIS (Netherlands)

    Davidovici, B.B.; Sattar, N.; Prinz, J.C.; Puig, L.; Emery, P.; Barker, J.N.; Kerkhof, P.C.M. van de; Stahle, M.; Nestle, F.O.; Girolomoni, G.; Krueger, J.G.

    2010-01-01

    Psoriasis is now classified as an immune-mediated inflammatory disease (IMID) of the skin. It is being recognized that patients with various IMIDs, including psoriasis, are at higher risk of developing "systemic" co-morbidities, e.g., cardiovascular disease (CVD), metabolic syndrome, and overt diabe

  14. Predictive modeling of coral disease distribution within a reef system.

    Directory of Open Access Journals (Sweden)

    Gareth J Williams

    Full Text Available Diseases often display complex and distinct associations with their environment due to differences in etiology, modes of transmission between hosts, and the shifting balance between pathogen virulence and host resistance. Statistical modeling has been underutilized in coral disease research to explore the spatial patterns that result from this triad of interactions. We tested the hypotheses that: 1 coral diseases show distinct associations with multiple environmental factors, 2 incorporating interactions (synergistic collinearities among environmental variables is important when predicting coral disease spatial patterns, and 3 modeling overall coral disease prevalence (the prevalence of multiple diseases as a single proportion value will increase predictive error relative to modeling the same diseases independently. Four coral diseases: Porites growth anomalies (PorGA, Porites tissue loss (PorTL, Porites trematodiasis (PorTrem, and Montipora white syndrome (MWS, and their interactions with 17 predictor variables were modeled using boosted regression trees (BRT within a reef system in Hawaii. Each disease showed distinct associations with the predictors. Environmental predictors showing the strongest overall associations with the coral diseases were both biotic and abiotic. PorGA was optimally predicted by a negative association with turbidity, PorTL and MWS by declines in butterflyfish and juvenile parrotfish abundance respectively, and PorTrem by a modal relationship with Porites host cover. Incorporating interactions among predictor variables contributed to the predictive power of our models, particularly for PorTrem. Combining diseases (using overall disease prevalence as the model response, led to an average six-fold increase in cross-validation predictive deviance over modeling the diseases individually. We therefore recommend coral diseases to be modeled separately, unless known to have etiologies that respond in a similar manner to

  15. Interneurons in the human olfactory system in Alzheimer's disease.

    Science.gov (United States)

    Saiz-Sanchez, Daniel; Flores-Cuadrado, Alicia; Ubeda-Bañon, Isabel; de la Rosa-Prieto, Carlos; Martinez-Marcos, Alino

    2016-02-01

    The principal olfactory structures display Alzheimer's disease (AD) related pathology at early stages of the disease. Consequently, olfactory deficits are among the earliest symptoms. Reliable olfactory tests for accurate clinical diagnosis are rarely made. In addition, neuropathological analysis postmortem of olfactory structures is often not made. Therefore, the relationship between the clinical features and the underlying pathology is poorly defined. Traditionally, research into Alzheimer's disease has focused on the degeneration of cortical temporal projection neurons and cholinergic neurons. Recent evidence has demonstrated the neurodegeneration of interneuron populations in AD. This review provides an updated overview of the pathological involvement of interneuron populations in the human olfactory system in Alzheimer's disease.

  16. Venous thromboembolic disease in systemic autoimmune diseases: an association to keep in mind.

    Science.gov (United States)

    Silvariño, Ricardo; Danza, Álvaro; Mérola, Valentina; Bérez, Adriana; Méndez, Enrique; Espinosa, Gerard; Cervera, Ricard

    2012-12-01

    Systemic autoimmune diseases are conditions of unknown etiology, characterized by the simultaneous or successive involvement of most organs and systems, as well as the presence of autoantibodies as biological markers. Venous thromboembolic disease has a higher incidence in this population when compared to healthy individuals. This responds to the increase in congenital and acquired risk factors in this group. One of the main risk factors is linked to the presence of antiphospholipid antibodies, whose prevalence is increased among patients with such conditions.

  17. Biologic Therapy in Inflammatory Immunomediated Systemic Diseases: Safety Profile.

    Science.gov (United States)

    Moroncini, Gianluca; Albani, Lisa; Nobili, Lorenzo; Gabrielli, Armando

    2016-01-01

    The discovery of some key molecular mechanisms underlying the dysregulation of the immune system responsible for inflammatory systemic diseases as severe as Systemic Lupus Erythematosus (SLE), Systemic Sclerosis (SSc), and Systemic Vasculitides, led to the development and subsequent introduction into clinical practice of biological drugs which are significantly improving the management of such complex disorders. This novel molecular targeted therapeutics represents in fact a valid alternative or complementary treatment to conventional immunosuppressive strategies, characterized by broad, unspecific actions and severe adverse effects. Main advantages of the use of biologic drugs reside in their steroid-sparing effect and in the ability of inducing remission of refractory disease states or curing specific organ involvements. Aim of this article is to review and briefly discuss the scientific evidence supporting the use of biologics in these diseases, with a particular emphasis on their efficacy and safety profile compared to the canonical drugs.

  18. Bile acid nuclear receptor FXR and digestive system diseases

    Directory of Open Access Journals (Sweden)

    Lili Ding

    2015-03-01

    Full Text Available Bile acids (BAs are not only digestive surfactants but also important cell signaling molecules, which stimulate several signaling pathways to regulate some important biological processes. The bile-acid-activated nuclear receptor, farnesoid X receptor (FXR, plays a pivotal role in regulating bile acid, lipid and glucose homeostasis as well as in regulating the inflammatory responses, barrier function and prevention of bacterial translocation in the intestinal tract. As expected, FXR is involved in the pathophysiology of a wide range of diseases of gastrointestinal tract, including inflammatory bowel disease, colorectal cancer and type 2 diabetes. In this review, we discuss current knowledge of the roles of FXR in physiology of the digestive system and the related diseases. Better understanding of the roles of FXR in digestive system will accelerate the development of FXR ligands/modulators for the treatment of digestive system diseases.

  19. Complement system in dermatological diseases – fire under the skin

    Directory of Open Access Journals (Sweden)

    Jaana Helena Panelius

    2015-01-01

    Full Text Available The complement system plays a key role in several dermatological diseases. Overactivation, deficiency or abnormality of the control proteins are often related to a skin disease. Autoimmune mechanisms with autoantibodies and a cytotoxic effect of the complement membrane attack complex (MAC on epidermal or vascular cells can cause direct tissue damage and inflammation e.g. in SLE, phospholipid antibody syndrome and bullous skin diseases like pemphigoid. By evading complement attack, some microbes like borrelia spirochetes and staphylococci can persist in the skin and cause prolonged symptoms. In this review we present the most important skin diseases connected to abnormalities in the function of the complement system. Drugs having an effect on the complement system are also briefly described. On one hand, drugs with free hydroxyl on amino groups (e.g. hydralazine, procainamide could interact with C4A, C4B or C3 and cause an SLE-like disease. On the other hand, progress in studies on complement has led to novel anti-complement drugs (recombinant C1 inhibitor and anti-C5 antibody, eculizumab that could alleviate symptoms in diseases associated with excessive complement activation.The main theme of the manuscript is to show how relevant the complement system is as an immune effector system in contributing to tissue injury and inflammation in a broad range of skin disorders.

  20. Expert System Diagnosis Dental Disease Using Certainty Factor Method

    Directory of Open Access Journals (Sweden)

    Whisnu Ulinnuha Setiabudi

    2017-05-01

    Full Text Available Technological development is growing rapidly among with the increasing of human needs especially in mobile technology where the technology that often be used is android. The existence of this android facilitates the user in access of information. This android can be used for healthy needs, for example is detecting dental disease. One of the branches of computer science that can help society in detecting dental disease is expert system. In this research, making expert system to diagnosis dental disease by using certainty factor method. Dental disease diagnosis application can diagnose the patient based on griping of the patient about dental disease so it can be obtained diseases possibility of the patient itself. This application is an expert system application that operates on android platform. Furthermore, in the measurement accuracy of the system test performed by 20 patients, there were 19 cases of corresponding and 1 cases that do not fit. So, from system testing performed by 20 patients resulted in a 95% accuracy rate. 

  1. An architecture model for multiple disease management information systems.

    Science.gov (United States)

    Chen, Lichin; Yu, Hui-Chu; Li, Hao-Chun; Wang, Yi-Van; Chen, Huang-Jen; Wang, I-Ching; Wang, Chiou-Shiang; Peng, Hui-Yu; Hsu, Yu-Ling; Chen, Chi-Huang; Chuang, Lee-Ming; Lee, Hung-Chang; Chung, Yufang; Lai, Feipei

    2013-04-01

    Disease management is a program which attempts to overcome the fragmentation of healthcare system and improve the quality of care. Many studies have proven the effectiveness of disease management. However, the case managers were spending the majority of time in documentation, coordinating the members of the care team. They need a tool to support them with daily practice and optimizing the inefficient workflow. Several discussions have indicated that information technology plays an important role in the era of disease management. Whereas applications have been developed, it is inefficient to develop information system for each disease management program individually. The aim of this research is to support the work of disease management, reform the inefficient workflow, and propose an architecture model that enhance on the reusability and time saving of information system development. The proposed architecture model had been successfully implemented into two disease management information system, and the result was evaluated through reusability analysis, time consumed analysis, pre- and post-implement workflow analysis, and user questionnaire survey. The reusability of the proposed model was high, less than half of the time was consumed, and the workflow had been improved. The overall user aspect is positive. The supportiveness during daily workflow is high. The system empowers the case managers with better information and leads to better decision making.

  2. Serum levels of IgG and IgG4 in Hashimoto thyroiditis.

    Science.gov (United States)

    Kawashima, Sachiko-Tsukamoto; Tagami, Tetsuya; Nakao, Kanako; Nanba, Kazutaka; Tamanaha, Tamiko; Usui, Takeshi; Naruse, Mitsuhide; Minamiguchi, Sachiko; Mori, Yusuke; Tsuji, Jun; Tanaka, Issei; Shimatsu, Akira

    2014-03-01

    Although IgG4-related disease is characterized by extensive infiltration of IgG4-positive plasma cells and lymphocytes of various organs, the details of this systemic disease are still unclear. We screened serum total IgG levels in the patients with Hashimoto thyroiditis (HT) to illustrate the prevalence of IgG4-related thyroiditis in HT. Twenty-four of 94 patients with HT (25.5%) had elevated serum IgG levels and their serum IgG4 was measured. Five of the 24 cases had more than 135 mg/dL of IgG4, which is the serum criterion of IgG4-related disease. One was a female patient who was initially treated as Graves' disease and rapidly developed a firm goiter and hypothyroidism. The biopsy of her thyroid gland revealed that follicular cells were atrophic with squamous metaplasia, replaced with fibrosis, which was compatible with the fibrous variant of HT. Immunohistochemical examination revealed diffuse infiltration of IgG4-positive plasma cells, and the serum IgG4 level was 179 mg/dL. The levels of IgG and IgG4 were positively correlated with the titers of anti-thyroglobulin antibody or anti-thyroid peroxidase antibody. In conclusion, at least a small portion of patients with HT with high titers of anti-thyroid antibodies may overlap the IgG4-related thyroiditis.

  3. Communicable Disease Reporting Systems in the World: A System­atic Review Article

    Directory of Open Access Journals (Sweden)

    Ali JANATI

    2015-11-01

    Full Text Available Background: Communicable disease reporting and surveillance system has poor infrastructure and supporters in most of countries. Its quality improvement is a challenge and requires an accurate and efficient care and reporting systems at all levels to achieve new and simple models. This study evaluates reporting systems of communicable diseases using systematic review.Methods: This was a systematic review study. For data collection, we used the following database and search engines: Proquest, Science direct, Pub MED, Scopes, Springer, and EBESCO. For Persian databases, we used SID, Iranmedex and Magiran. Our key words were "Communicable Diseases", "Notifiable Disease", "Disease Notification", "Reporting System"," Surveillance Systems" and "evaluation". Two independent researchers reviewed the resources and the results were classified in different domains.Results: From 1889 cases, only 66 resources were studied. The results were classified in several domains, including those who were reporting, reporting methods and procedures, responsibilities and reporting system characteristics, problems and solutions of the report, the reporting process, and receptor level.Conclusion: Disease-reporting system has similar problems in all parts of the world. Change, improve, update and continuous monitoring of the reporting system are very important. Although the reporting process can vary in different regions, but being perfect and timely are important principles in system design. Detailed explanations of tasks and providing appropriate instructions are the most important points to integrate an efficient reporting system. Keywords: Reporting, Diseases surveillance system, Disease control

  4. Diseases of the nervous system associated with calcium channelopathies

    NARCIS (Netherlands)

    Todorov, Boyan Bogdanov

    2010-01-01

    The aim of the studies described in this thesis was to investigate how abnormal CaV2.1 channel function can cause disease, in particular motor coordination dysfunction. The chapters illustrate how various neuronal cell types in the periphery (peripheral nervous system) and the central nervous system

  5. Diseases of the nervous system associated with calcium channelopathies

    NARCIS (Netherlands)

    Todorov, Boyan Bogdanov

    2010-01-01

    The aim of the studies described in this thesis was to investigate how abnormal CaV2.1 channel function can cause disease, in particular motor coordination dysfunction. The chapters illustrate how various neuronal cell types in the periphery (peripheral nervous system) and the central nervous system

  6. Commentary on Special Issue : CNS Diseases and the Immune System

    NARCIS (Netherlands)

    't Hart, Bert A.; den Dunnen, Wilfred F.

    2013-01-01

    In an increasing number of central nervous system (CNS) diseases a pathogenic contribution of the immune system is proposed. However, the exact underlying mechanisms are often poorly understood. The collection of articles in this special issue presents a state-of-the-art review of adaptive and innat

  7. [The diseases of circulatory system in employees of railway transport].

    Science.gov (United States)

    Molodtsov, R N; Shemetova, G N

    2013-01-01

    The article presents the epidemiologic and medical social aspects of diseases of circulatory system in employees of railway transport in 2000-2010 exemplified by Privolzhskiy railroad. The established tendencies in prevalence of pathology of cardio-vascular system in railroad workers makes the issues of practical implementation of priority of prevention in the organization of medical care to this group of patients to come to foreground. The main directions for complex prevention of diseases of circulatory system in employees of railway transport are presented.

  8. Determination of autoantibodies to annexin XI in systemic autoimmune diseases

    DEFF Research Database (Denmark)

    Jorgensen, C S; Levantino, G; Houen, Gunnar

    2000-01-01

    Annexin XI, a calcyclin-associated protein, has been shown to be identical to a 56,000 Da antigen recognized by antibodies found in sera from patients suffering from systemic autoimmune diseases. In this work hexahistidine-tagged recombinant annexin XI (His6- rAnn XI) was used as antigen in ELISA...... experiments for determination of autoantibodies to annexin XI in sera of patients with systemic rheumatic autoimmune diseases. Immunoblotting with HeLa cell extract and with His6-rAnn XI as antigen was used for confirmation of positive ELISA results. We found eleven anti-annexin XI positive sera (3.9%) out...... of 282 sera from patients with systemic rheumatic diseases. The highest number of annexin XI positive sera were found in primary antiphospholipid syndrome (3/17), and in subacute lupus erythematosus (1/6), while lower frequencies of positive sera were found in patients with systemic sclerosis (5...

  9. Endoscopic scoring systems for inflammatory bowel disease: pros and cons.

    Science.gov (United States)

    Tontini, Gian Eugenio; Bisschops, Raf; Neumann, Helmut

    2014-07-01

    Endoscopy plays a pivotal role for diagnosis and assessment of disease activity and extent in patients with inflammatory bowel diseases. International guidelines recommend the use of endoscopic scoring systems for evaluation of the prognosis and efficacy of medical treatments. Ideal scoring systems are easy to use, reproducible, reliable, responsive to changes, and validated in different clinical settings in order to guide therapeutic strategies. However, currently available endoscopic scoring systems often appear as complex for routine endoscopy and suffer from insufficient interobserver agreement and lack of formal validation which often limit their use in clinical trials. Here, we describe the role of endoscopic scoring systems in inflammatory bowel diseases focusing on pros and cons in the era of advanced endoscopic imaging and mucosal healing.

  10. Development of CAD prototype system for Crohn's disease

    Science.gov (United States)

    Oda, Masahiro; Kitasaka, Takayuki; Furukawa, Kazuhiro; Watanabe, Osamu; Ando, Takafumi; Goto, Hidemi; Mori, Kensaku

    2010-03-01

    The purpose of this paper is to present a CAD prototype system for Crohn's disease. Crohn's disease causes inflammation or ulcers of the gastrointestinal tract. The number of patients of Crohn's disease is increasing in Japan. Symptoms of Crohn's disease include intestinal stenosis, longitudinal ulcers, and fistulae. Optical endoscope cannot pass through intestinal stenosis in some cases. We propose a new CAD system using abdominal fecal tagging CT images for efficient diagnosis of Crohn's disease. The system displays virtual unfolded (VU), virtual endoscopic, curved planar reconstruction, multi planar reconstruction, and outside views of both small and large intestines. To generate the VU views, we employ a small and large intestines extraction method followed by a simple electronic cleansing method. The intestine extraction is based on the region growing process, which uses a characteristic that tagged fluid neighbor air in the intestine. The electronic cleansing enables observation of intestinal wall under tagged fluid. We change the height of the VU views according to the perimeter of the intestine. In addition, we developed a method to enhance the longitudinal ulcer on views of the system. We enhance concave parts on the intestinal wall, which are caused by the longitudinal ulcer, based on local intensity structure analysis. We examined the small and the large intestines of eleven CT images by the proposed system. The VU views enabled efficient observation of the intestinal wall. The height change of the VU views helps finding intestinal stenosis on the VU views. The concave region enhancement made longitudinal ulcers clear on the views.

  11. Adult neurogenesis in the olfactory system and neurodegenerative disease.

    Science.gov (United States)

    Gallarda, B W; Lledo, P-M

    2012-12-01

    The olfactory system is unique in many respects-two of which include the process of adult neurogenesis which continually supplies it with newborn neurons, and the fact that neurodegenerative diseases are often accompanied by a loss of smell. A link between these two phenomena has been hypothesized, but recent evidence for the lack of robust adult neurogenesis in the human olfactory system calls into question this hypothesis. Nevertheless, model organisms continue to play a critical role in the exploration of neurodegenerative disease. In part one of this review we discuss the most promising recent technological advancements for studying adult neurogenesis in the murine olfactory system. Part two continues by looking at emerging evidence related to adult neurogenesis in neurodegenerative disease studied in model organisms and the differences between animal and human olfactory system adult neurogenesis. Hopefully, the careful application of advanced research methods to the study of neurodegenerative disease in model organisms, while taking into account the recently reported differences between the human and model organism olfactory system, will lead to a better understanding of the reasons for the susceptibility of olfaction to disease.

  12. Treatment of systemic hypertension associated with kidney disease.

    Science.gov (United States)

    Buoncompagni, Simona; Bowles, Mary H

    2013-05-01

    Systemic hypertension is an increasingly diagnosed disorder in dogs and cats and frequently occurs secondary to chronic kidney disease. Prevention of damage to organs such as the kidneys, brain, heart, and eyes is one of the primary concerns in the management of veterinary patients with hypertension. This article reviews the guidelines for antihypertensive therapy in patients with, or at risk for, kidney disease, including the initiation of treatment and currently recommended medications.

  13. A Systems Overview of Immunology, Disease and Related Data Processing

    OpenAIRE

    Asachenkov, A.L.; Mohler, R.R.

    1989-01-01

    This paper gives a brief but systematic overview of topics in "mathematical immunology" and may be considered as an introduction to a new IIASA activity on these topics. Theoretical and experimental research in this area is aimed at an understanding of the precise manner by which the immune system controls (or attempts to control) infectious diseases and diseases such as AIDS and cancer. Available mathematical methods, the difficulties arising in the respective problems, and some possibilitie...

  14. [Modern threats and burden of respiratory system diseases in Poland].

    Science.gov (United States)

    Płusa, Tadeusz

    2013-11-01

    Polish population according to the National Census of Population and Housing, which was conducted in 2011, was 38 511.8 thousand. The average life expectancy in Poland is 71.0 years for men and 79.7 years for women. The reason for hospitalization in Poland are primarily cardiovascular disease (18%), tumors (11.4%), digestive diseases (10.6%), respiratory (9.3%), trauma (9.1%), infectious diseases (2.3%) and others (39%). Mortality rates determined on the basis of the analyzes and simulations in different disease groups indicates that the predominant causes of death of Polish citizens are strongly cardiovascular disease and cancer. Respiratory diseases occupy fourth place. World analyses clearly show that the number of deaths in 2030 due to lung diseases will be the fourth (COPD), fifth (pneumonia) and sixth (lung cancer) cause of death. As it turns out, the existence of various pathologies affecting the country's economic status. Respiratory allergies are observed more often, including in approximately 20% of Europeans are symptoms of allergic rhinitis (15-20% severe) and in 5-11% are diagnosed with asthma. Malignant tumors are the second most common causes of death in the group with the highest risk of life for the residents of Polish, particularly for men, is lung cancer, because of which in 2001, 20 570 people died. Incurred costs of the social security system are mainly caused by inflammatory diseases of the respiratory system, which corresponds to the number of days of sick leave, especially in the age group 19-28 years, with a decrease in the age group above 59 years of age. Numbers hospitalized for respiratory diseases according to data from the National Health Fund also clearly indicate the cause of inflammation and cancer, and in the population aged 41-60 years, the need for hospital treatment is multiplied. The data indicate the constant threat of respiratory diseases.

  15. Effect of biodiversity changes in disease risk: exploring disease emergence in a plant-virus system.

    Directory of Open Access Journals (Sweden)

    Israel Pagán

    Full Text Available The effect of biodiversity on the ability of parasites to infect their host and cause disease (i.e. disease risk is a major question in pathology, which is central to understand the emergence of infectious diseases, and to develop strategies for their management. Two hypotheses, which can be considered as extremes of a continuum, relate biodiversity to disease risk: One states that biodiversity is positively correlated with disease risk (Amplification Effect, and the second predicts a negative correlation between biodiversity and disease risk (Dilution Effect. Which of them applies better to different host-parasite systems is still a source of debate, due to limited experimental or empirical data. This is especially the case for viral diseases of plants. To address this subject, we have monitored for three years the prevalence of several viruses, and virus-associated symptoms, in populations of wild pepper (chiltepin under different levels of human management. For each population, we also measured the habitat species diversity, host plant genetic diversity and host plant density. Results indicate that disease and infection risk increased with the level of human management, which was associated with decreased species diversity and host genetic diversity, and with increased host plant density. Importantly, species diversity of the habitat was the primary predictor of disease risk for wild chiltepin populations. This changed in managed populations where host genetic diversity was the primary predictor. Host density was generally a poorer predictor of disease and infection risk. These results support the dilution effect hypothesis, and underline the relevance of different ecological factors in determining disease/infection risk in host plant populations under different levels of anthropic influence. These results are relevant for managing plant diseases and for establishing conservation policies for endangered plant species.

  16. Effect of biodiversity changes in disease risk: exploring disease emergence in a plant-virus system.

    Science.gov (United States)

    Pagán, Israel; González-Jara, Pablo; Moreno-Letelier, Alejandra; Rodelo-Urrego, Manuel; Fraile, Aurora; Piñero, Daniel; García-Arenal, Fernando

    2012-01-01

    The effect of biodiversity on the ability of parasites to infect their host and cause disease (i.e. disease risk) is a major question in pathology, which is central to understand the emergence of infectious diseases, and to develop strategies for their management. Two hypotheses, which can be considered as extremes of a continuum, relate biodiversity to disease risk: One states that biodiversity is positively correlated with disease risk (Amplification Effect), and the second predicts a negative correlation between biodiversity and disease risk (Dilution Effect). Which of them applies better to different host-parasite systems is still a source of debate, due to limited experimental or empirical data. This is especially the case for viral diseases of plants. To address this subject, we have monitored for three years the prevalence of several viruses, and virus-associated symptoms, in populations of wild pepper (chiltepin) under different levels of human management. For each population, we also measured the habitat species diversity, host plant genetic diversity and host plant density. Results indicate that disease and infection risk increased with the level of human management, which was associated with decreased species diversity and host genetic diversity, and with increased host plant density. Importantly, species diversity of the habitat was the primary predictor of disease risk for wild chiltepin populations. This changed in managed populations where host genetic diversity was the primary predictor. Host density was generally a poorer predictor of disease and infection risk. These results support the dilution effect hypothesis, and underline the relevance of different ecological factors in determining disease/infection risk in host plant populations under different levels of anthropic influence. These results are relevant for managing plant diseases and for establishing conservation policies for endangered plant species.

  17. Possibilities of Strawberry Integrated Disease Management in Different Cultivation System

    Directory of Open Access Journals (Sweden)

    Tihomir Miličević

    2006-12-01

    Full Text Available In the last few years strawberry production in Croatia is constantly increasing. One of the main problems in production are diseases. During two-year trials in strawberry plantations in northern Croatia, the occurrence of diseases was monitored in order to establish the most effective methods of integrated disease management. Trials were performed in three cultivation system: open field, greenhouse and hydroponics. The most frequent disease in all three production systems was gray mould (Botrytis cinerea. In open field production, the occurrence of common leaf spot (Mycosphaerella fragariae and leaf scorch (Diplocarpon earliana were also frequently observed, while leaf blotch (Gnomonia comari, leaf blight (Phomopsis obscurans and fruit anthracnose (Colletotrichum spp. were only sporadically present. For the control of the most important disease, gray mould, forecast model BOTMAN was implemented. As relatively simple model based on meteorological data, BOTMAN allowed effective, ecologically and economically more acceptable control, based on integrated chemical and biological measures. Meteorological data were obtained from the State Hydrometeorological Department (DHMZ. Results showed no significant difference in intensity of gray mould infection between usual chemical control and BOTMAN-based control. Two-years research on strawberry disease management in Croatia revealed perspective possibilities of integrated strawberry disease management.

  18. Localized Knowledge based System for Human Disease Diagnosis

    Directory of Open Access Journals (Sweden)

    Adane Nega Tarekegn

    2016-03-01

    Full Text Available —Knowledge based system can be designed to solve complex medical problems. It incorporates the expert‟s knowledge that has been coded into facts, rules, heuristics and procedures. Incorporation of local languages with the knowledge based system allows endusers communicate with the system in a simpler and easier way. In this study a localized knowledge based system is developed for TB disease diagnosis using Ethiopian national language. To develop the localized knowledge based system, tacit knowledge is acquired from domain experts using interviewing techniques and explicit knowledge is captured from documented sources using relevant documents analysis method. Then the acquired knowledge is modeled using decision tree structure that represents concepts and procedures involved in diagnosis of disease. Production rules are used to represent domain knowledge. The localized knowledge based system is developed using SWI Prolog version 6.4.1 programming language. Prolog supports natural language processing feature to localize the system. As a result, the system is implemented using Amharic language (the national language of Ethiopia user interface. With Localization, users at remote areas and users who are not good in foreign languages are benefited enormously. The system is tested and evaluated to ensure that whether the performance of the system is accurate and the system is usable by physicians and patients. The average performance of the localized knowledge based system has registered 81.5%.

  19. Oxidative Stress in Oral Diseases: Understanding Its Relation with Other Systemic Diseases.

    Science.gov (United States)

    Kumar, Jaya; Teoh, Seong Lin; Das, Srijit; Mahakknaukrauh, Pasuk

    2017-01-01

    Oxidative stress occurs in diabetes, various cancers, liver diseases, stroke, rheumatoid arthritis, chronic inflammation, and other degenerative diseases related to the nervous system. The free radicals have deleterious effect on various organs of the body. This is due to lipid peroxidation and irreversible protein modification that leads to cellular apoptosis or programmed cell death. During recent years, there is a rise in the oral diseases related to oxidative stress. Oxidative stress in oral disease is related to other systemic diseases in the body such as periodontitis, cardiovascular, pancreatic, gastric, and liver diseases. In the present review, we discuss the various pathways that mediate oxidative cellular damage. Numerous pathways mediate oxidative cellular damage and these include caspase pathway, PERK/NRF2 pathway, NADPH oxidase 4 pathways and JNK/mitogen-activated protein (MAP) kinase pathway. We also discuss the role of inflammatory markers, lipid peroxidation, and role of oxygen species linked to oxidative stress. Knowledge of different pathways, role of inflammatory markers, and importance of low-density lipoprotein, fibrinogen, creatinine, nitric oxide, nitrates, and highly sensitive C-reactive proteins may be helpful in understanding the pathogenesis and plan better treatment for oral diseases which involve oxidative stress.

  20. Homozygosity for moyamoya disease risk allele leads to moyamoya disease with extracranial systemic and pulmonary vasculopathy.

    Science.gov (United States)

    Fukushima, Hiroyuki; Takenouchi, Toshiki; Kosaki, Kenjiro

    2016-09-01

    Moyamoya disease is characterized by diffuse distal intracranial stenosis. Recently, RNF213 has been identified as a susceptibility gene in the development of this condition. Pulmonary hypertension is a rare progressive vasculopathy with an unknown etiology. The co-occurrence of pulmonary hypertension and Moyamoya disease has been described in four patients; however, whether this co-occurrence represents a chance association or a common vascular pathology has remained unknown. Here, we report two unrelated male patients who presented during their childhood with dyspnea on exertion. Systemic vascular imaging studies revealed the presence of pulmonary hypertension and Moyamoya disease in both patients. Medical exome sequencing revealed that both patients had a homozygous mutation for p.Arg4810Lys in RNF213. We suggest that homozygosity in RNF213 may lead to a novel entity involving the brain and lung. Interestingly, when present in a heterozygous state, this mutation causes a classic cerebral vascular disease, Moyamoya disease. In the homozygous state, the exact same mutation led to Moyamoya disease with extracranial systemic vasculopathy in at least two patients. From a clinical standpoint, cerebrovascular or pulmonary vascular investigations may be warranted in patients with pulmonary hypertension or Moyamoya disease, respectively. © 2016 Wiley Periodicals, Inc.

  1. Event communication in a regional disease surveillance system.

    Science.gov (United States)

    Loschen, Wayne; Coberly, Jacqueline; Sniegoski, Carol; Holtry, Rekha; Sikes, Marvin; Happel Lewis, Sheryl

    2007-10-11

    When real-time disease surveillance is practiced in neighboring states within a region, public health users may benefit from easily sharing their concerns and findings regarding potential health threats. To better understand the need for this capability, an event communications component (ECC) was added to the National Capital Region Disease Surveillance System, an operational biosurveillance system employed in the District of Columbia and in surrounding Maryland and Virginia counties. Through usage analysis and user survey methods, we assessed the value of the enhanced system in daily operational use and during two simulated exercises. Results suggest that the system has utility for regular users of the system as well as suggesting several refinements for future implementations.

  2. Vitamin D in systemic and organ-specific autoimmune diseases.

    Science.gov (United States)

    Agmon-Levin, Nancy; Theodor, Emanuel; Segal, Ramit Maoz; Shoenfeld, Yehuda

    2013-10-01

    Lately, vitamin D has been linked with metabolic and immunological processes, which established its role as an essential component of human health preservation. Vitamin D has been defined as natural immune modulators, and upon activation of its receptors (VDRs), it regulates calcium metabolism, cellular growth, proliferation and apoptosis, and other immunological functions. Epidemiological data underline a strong correlation between poor vitamin D status and higher risk for chronic inflammatory illnesses of various etiologies, including autoimmune diseases. Epidemiological, genetic, and basic studies indicated a potential role of vitamin D in the pathogenesis of certain systemic and organ-specific autoimmune diseases. These studies demonstrate correlation between low vitamin D and prevalence of diseases. In addition, VDRs' polymorphisms observed in some of these autoimmune diseases may further support a plausible pathogenic link. Notably, for some autoimmune disease, no correlation with vitamin D levels could be confirmed. Thus, in the current review we present the body of evidence regarding the plausible roles of vitamin D and VDR's polymorphism in the pathogenesis of autoimmunity. We summarize the data regarding systemic (i.e., systemic lupus erythematosus, rheumatoid arthritis, etc.) and organ-specific (i.e., multiple sclerosis, diabetes mellitus, primary biliary cirrhosis, etc.) autoimmune diseases, in which low level of vitamin D was found comparing to healthy subjects. In addition, we discuss the correlations between vitamin D levels and clinical manifestations and/or activity of diseases. In this context, we address the rational for vitamin D supplementation in patients suffering from autoimmune diseases. Further studies addressing the mechanisms by which vitamin D affects autoimmunity and the proper supplementation required are needed.

  3. Healthcare decision support system for administration of chronic diseases.

    Science.gov (United States)

    Woo, Ji-In; Yang, Jung-Gi; Lee, Young-Ho; Kang, Un-Gu

    2014-07-01

    A healthcare decision-making support model and rule management system is proposed based on a personalized rule-based intelligent concept, to effectively manage chronic diseases. A Web service was built using a standard message transfer protocol for interoperability of personal health records among healthcare institutions. An intelligent decision service is provided that analyzes data using a service-oriented healthcare rule inference function and machine-learning platform; the rules are extensively compiled by physicians through a developmental user interface that enables knowledge base construction, modification, and integration. Further, screening results are visualized for the self-intuitive understanding of personal health status by patients. A recommendation message is output through the Web service by receiving patient information from the hospital information recording system and object attribute values as input factors. The proposed system can verify patient behavior by acting as an intellectualized backbone of chronic diseases management; further, it supports self-management and scheduling of screening. Chronic patients can continuously receive active recommendations related to their healthcare through the rule management system, and they can model the system by acting as decision makers in diseases management; secondary diseases can be prevented and health management can be performed by reference to patient-specific lifestyle guidelines.

  4. The automated system for prevention of industrial-caused diseases

    Science.gov (United States)

    Varnavsky, A. N.

    2017-01-01

    The paper presents the automated system intended to prevent industrial-caused diseases of workers, the basis of which is represented by algorithms of preventing several negative functional conditions (stress, monotony). The emergence of such state shall be determined based on an analysis of bioelectric signals, in particular, skin-galvanic reactions. Proceeding from the dynamics of the functional state, the automated system offers to perform an optimized set of measures to restore the health of the worker. Implementation of an automated system is presented in Visual Programming system LabVIEW.

  5. Monitoring diseases across borders: African regional integrative information systems.

    Science.gov (United States)

    Simbini, Tungamirirai; Foster, Rosemary; Nesara, Paul; Hullin Lucay Cossio, Carola

    2010-01-01

    In African countries, communicable diseases remain the chief cause of a heavy disease burden. Regional economic, political and social integration bring new challenges in the management of these diseases, many of which are treatable. Information Communication Technology (ICT) applied through electronic health systems has the potential to strengthen healthcare service delivery and disease surveillance within these countries. This paper discusses the importance of well-defined e-Health strategies within countries and, in addition, proposes that countries within regions collaborate in planning for health information exchange across borders. It is suggested that particular attention be paid to technical and data standards enabling interoperability, and also to issues of security, patient privacy and governance.

  6. Parkinson's disease Assessment using Fuzzy Expert System and Nonlinear Dynamics

    Directory of Open Access Journals (Sweden)

    GEMAN, O.

    2013-02-01

    Full Text Available This paper proposes a new screening system for quantitative evaluation and analysis, designed for the early stage detection of Parkinson disease. This has been carried out in the view of improving the diagnosis currently established upon a basis of subjective scores. Parkinson?s disease (PD appears as a result of dopamine loss, a chemical mediator that is responsible for the body?s ability to control movements. The symptoms reflect the loss of nerve cells, due to an unknown. The input parameters of the system are represented by amplitude, frequency, the spectral characteristic and trembling localization. The main symptoms include trembling of hand, arms, movement difficulties, postural instability, disturbance of coordination and equilibrium, sleep disturbance, difficulties in speaking, reducing of voice volume. The medical knowledge in PD field is characterized by imprecision, uncertainty and vagueness. The proposed system (fuzzy expert systems is non-invasive and, easy to use by both physicians and patients at home.

  7. A wireless portable system with microsensors for monitoring respiratory diseases.

    Science.gov (United States)

    Cao, Zhe; Zhu, Rong; Que, Rui-Yi

    2012-11-01

    A wireless portable monitoring system for respiratory diseases using microsensors is proposed. The monitoring system consists of two sensor nodes integrating with Bluetooth transmitters that measure user's respiratory airflow, blood oxygen saturation, and body posture. The utility of micro-hot-film flow sensor makes the monitor can acquire comprehensive respiration parameters which are useful for diagnoses of obstructive sleep apnea, chronic obstructive pulmonary disease, and asthma. The system can serve as both sleep recorder and spirometer. Additionally, a mobile phone or a PC connected to the Internet serving as a monitoring and transfer terminal makes telemedicine achievable. Several experiments were conducted to verify the feasibility and effectiveness of the proposed system for monitoring and diagnosing OSA, COPD, and asthma.

  8. Chemokines and their receptors in central nervous system disease.

    Science.gov (United States)

    Biber, Knut; de Jong, Eiko K; van Weering, Hilmar R J; Boddeke, Hendrikus W G M

    2006-01-01

    Almost a decade ago, it was discovered that the human deficiency virus (HIV) makes use of chemokine receptors to infect blood cells. This appreciation of the clinical relevance of specific chemokine receptors has initiated a considerable boost in the field of chemokine research. It is clear today that chemokine signaling orchestrates the immune system and is widely involved in both physiological and pathophysiological processes. Since the chemokine system offers various targets through which pathology could be influenced, most pharmaceutical companies have chosen this system as a therapeutic target for a variety of diseases. Here recent developments concerning the role of chemokines in diseases of the central nervous system (CNS) as well as their possible therapeutic relevance are discussed.

  9. Quality Control System of the Endoplasmic Reticulum and Related Diseases

    Institute of Scientific and Technical Information of China (English)

    Jun-Chao WU; Zhong-Qin LIANG; Zheng-Hong QIN

    2006-01-01

    The quality control (QC) system of the endoplasmic reticulum (ER) is an important monitoring mechanism in the protein maturation process, which ensures export of properly folded proteins from the ER.Incorrectly or incompletely folded proteins are retained in the ER for refolding or degradation by the ER-residing proteasome. The calnexin/calreticulin cycle and ER-associated degradation are the key elements in QC. These two mechanisms work together to allow incorrectly folded proteins have additional opportunities to achieve their native conformations. The QC dysfunction is involved in many diseases caused by mutant proteins, many of which are causes of neurodegenerative disorders. A better understanding of molecular regulation in the QC system will uncover the molecular pathogenic mechanisms of many diseases caused by protein misfolding and help discover novel strategies for preventing or treating these diseases.

  10. Genome integrity and disease prevention in the nervous system.

    Science.gov (United States)

    McKinnon, Peter J

    2017-06-15

    Multiple DNA repair pathways maintain genome stability and ensure that DNA remains essentially unchanged over the life of a cell. Various human diseases occur if DNA repair is compromised, and most of these impact the nervous system, in some cases exclusively. However, it is often unclear what specific endogenous damage underpins disease pathology. Generally, the types of causative DNA damage are associated with replication, transcription, or oxidative metabolism; other direct sources of endogenous lesions may arise from aberrant topoisomerase activity or ribonucleotide incorporation into DNA. This review focuses on the etiology of DNA damage in the nervous system and the genome stability pathways that prevent human neurologic disease. © 2017 McKinnon; Published by Cold Spring Harbor Laboratory Press.

  11. [VARICELLA ZOSTER VIRUS AND DISEASES OF CENTRAL NERVOUS SYSTEM VESSELS].

    Science.gov (United States)

    Kazanova, A S; Lavrov, V F; Zverev, V V

    2015-01-01

    Systemized data on epidemiology, pathogenesis, clinical manifestation, diagnostics and therapy of VZV-vasculopathy--a disease, occurring due to damage of arteries of the central nervous system by Varicella Zoster virus, are presented in the review. A special attention in the paper is given to the effect of vaccine prophylaxis of chicken pox and herpes zoster on the frequency of development and course of VZV-vasculopathy.

  12. An Intelligent System based on Fuzzy Inference System to prophesy the brutality of Cardio Vascular Disease

    Directory of Open Access Journals (Sweden)

    Sivagowry shathesh

    2015-11-01

    Full Text Available To unravel hidden relationships and diagnose diseases efficiently, Data Mining along with Soft Computing Techniques are used in several researches.  Cardio Vascular Disease is a condition which leads to severe disability and death.  Since the diagnosis involves vague symptoms and tedious procedures, diagnosis is usually time-consuming and erroneous.  For the healthier analysis and treatment of heart disease based on brutality, an Intellectual, accurate and proficient investigative system is needed.  For diagnosing heart disease with improved effectiveness, an Intelligent Fuzzy Inference System is needed.  This paper illustrates how Fuzzy Inference System is used to envisage the severity of disease by constructing an effective Fuzzy Rule Base.  It is also proved that a precision of 95.23% is obtained when Fuzzy System is used in severity prediction

  13. Disease suppressive soilless culture systems; characterisation of its microflora

    NARCIS (Netherlands)

    Postma, J.; Willemsen-de Klein, M.J.E.I.M.; Rattink, H.; Os, van E.A.

    2001-01-01

    The trend in glasshouse horticulture has always been to start culture systems as aseptic as possible. However, several root diseases still cause problems under these conditions. The present paper shows the importance of the microflora to suppress Pythium aphanidermatum, a fungal root pathogen which

  14. Disease scoring systems for oral lichen planus; a critical appraisal

    NARCIS (Netherlands)

    Wang, J.; van der Waal, I.

    2015-01-01

    The aim of the present study has been to critically review 22 disease scoring systems (DSSs) on oral lichen planus (OLP) that have been reported in the literature during the past decades. Although the presently available DSSs may all have some merit, particularly for research purposes, the diversity

  15. Importance of the brain Angiotensin system in Parkinson's disease.

    Science.gov (United States)

    Wright, John W; Harding, Joseph W

    2012-01-01

    Parkinson's disease (PD) has become a major health problem affecting 1.5% of the world's population over 65 years of age. As life expectancy has increased so has the occurrence of PD. The primary direct consequence of this disease is the loss of dopaminergic (DA) neurons in the substantia nigra and striatum. As the intensity of motor dysfunction increases, the symptomatic triad of bradykinesia, tremors-at-rest, and rigidity occur. Progressive neurodegeneration may also impact non-DA neurotransmitter systems including cholinergic, noradrenergic, and serotonergic, often leading to the development of depression, sleep disturbances, dementia, and autonomic nervous system failure. L-DOPA is the most efficacious oral delivery treatment for controlling motor symptoms; however, this approach is ineffective regarding nonmotor symptoms. New treatment strategies are needed designed to provide neuroprotection and encourage neurogenesis and synaptogenesis to slow or reverse this disease process. The hepatocyte growth factor (HGF)/c-Met receptor system is a member of the growth factor family and has been shown to protect against degeneration of DA neurons in animal models. Recently, small angiotensin-based blood-brain barrier penetrant mimetics have been developed that activate this HGF/c-Met system. These compounds may offer a new and novel approach to the treatment of Parkinson's disease.

  16. Disease scoring systems for oral lichen planus; a critical appraisal

    NARCIS (Netherlands)

    Wang, J.; van der Waal, I.

    2015-01-01

    The aim of the present study has been to critically review 22 disease scoring systems (DSSs) on oral lichen planus (OLP) that have been reported in the literature during the past decades. Although the presently available DSSs may all have some merit, particularly for research purposes, the diversity

  17. The influence of systemic diseases on the diagnosis of oral diseases: a problem-based approach

    NARCIS (Netherlands)

    Lockhart, P.B.; Hong, C.H.L.; van Diermen, D.E.

    2011-01-01

    lthough all dentists are taught about the importance of oral health to general health and that systemic disease can manifest in the oral cavity, the 4-year dental school curriculum does not allow time to gain competency in these relationships. Nevertheless, all dentists must have skills in taking a

  18. Periodontitis and systemic diseases : a record of discussions of working group 4 of the Joint EFP/AAP Workshop on Periodontitis and Systemic Diseases

    NARCIS (Netherlands)

    Linden, Gerry J; Herzberg, Mark C; van Winkelhoff, Arie

    2013-01-01

    BACKGROUND: There has been an explosion in research into possible associations between periodontitis and various systemic diseases and conditions. AIM: To review the evidence for associations between periodontitis and various systemic diseases and conditions, including chronic obstructive pulmonary

  19. Periodontitis and systemic diseases : a record of discussions of working group 4 of the Joint EFP/AAP Workshop on Periodontitis and Systemic Diseases

    NARCIS (Netherlands)

    Linden, Gerry J; Herzberg, Mark C; van Winkelhoff, Arie

    2013-01-01

    BACKGROUND: There has been an explosion in research into possible associations between periodontitis and various systemic diseases and conditions. AIM: To review the evidence for associations between periodontitis and various systemic diseases and conditions, including chronic obstructive pulmonary

  20. Periodontitis and systemic diseases : a record of discussions of working group 4 of the Joint EFP/AAP Workshop on Periodontitis and Systemic Diseases

    NARCIS (Netherlands)

    Linden, Gerry J; Herzberg, Mark C; van Winkelhoff, Arie

    BACKGROUND: There has been an explosion in research into possible associations between periodontitis and various systemic diseases and conditions. AIM: To review the evidence for associations between periodontitis and various systemic diseases and conditions, including chronic obstructive pulmonary

  1. Involvement of the autonomic nervous system in Chagas heart disease

    Directory of Open Access Journals (Sweden)

    Edison Reis Lopes

    1983-12-01

    Full Text Available The autonomic nervous system and especially the intracardiac autonomic nervous system is involved in Chagas' disease. Ganglionitis and periganglionitis were noted in three groups ofpatients dying with Chagas'disease: 1 Those in heart failure; 2 Those dying a sudden, non violent death and; 3 Those dying as a consequence ofaccidents or homicide. Hearts in the threegroups also revealed myocarditis and scattered involvement of intramyocardial ganglion cells as well as lesions of myelinic and unmyelinic fibers ascribable to Chagas'disease. In mice with experimentally induced Chagas' disease weobserved more intensive neuronal lesions of the cardiac ganglia in the acute phase of infection. Perhaps neuronal loss has a role in the pathogenesis of Chagas cardiomyopathy. However based on our own experience and on other data from the literature we conclude that the loss of neurones is not the main factor responsible for the manifestations exhibited by chronic chagasic patients. On the other hand the neuronal lesions may have played a role in the sudden death ofone group of patients with Chagas'disease but is difficult to explain the group of patients who did not die sudderly but instead progressed to cardiac failure.

  2. Epstein-Barr virus in systemic autoimmune diseases.

    Science.gov (United States)

    Draborg, Anette Holck; Duus, Karen; Houen, Gunnar

    2013-01-01

    Systemic autoimmune diseases (SADs) are a group of connective tissue diseases with diverse, yet overlapping, symptoms and autoantibody development. The etiology behind SADs is not fully elucidated, but a number of genetic and environmental factors are known to influence the incidence of SADs. Recent findings link dysregulation of Epstein-Barr virus (EBV) with SAD development. EBV causes a persistent infection with a tight latency programme in memory B-cells, which enables evasion of the immune defence. A number of immune escape mechanisms and immune-modulating proteins have been described for EBV. These immune modulating functions make EBV a good candidate for initiation of autoimmune diseases and exacerbation of disease progression. This review focuses on systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren's syndrome (SS) and sum up the existing data linking EBV with these diseases including elevated titres of EBV antibodies, reduced T-cell defence against EBV, and elevated EBV viral load. Together, these data suggest that uncontrolled EBV infection can develop diverse autoreactivities in genetic susceptible individuals with different manifestations depending on the genetic background and the site of reactivation.

  3. Epstein-Barr Virus in Systemic Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Anette Holck Draborg

    2013-01-01

    Full Text Available Systemic autoimmune diseases (SADs are a group of connective tissue diseases with diverse, yet overlapping, symptoms and autoantibody development. The etiology behind SADs is not fully elucidated, but a number of genetic and environmental factors are known to influence the incidence of SADs. Recent findings link dysregulation of Epstein-Barr virus (EBV with SAD development. EBV causes a persistent infection with a tight latency programme in memory B-cells, which enables evasion of the immune defence. A number of immune escape mechanisms and immune-modulating proteins have been described for EBV. These immune modulating functions make EBV a good candidate for initiation of autoimmune diseases and exacerbation of disease progression. This review focuses on systemic lupus erythematosus (SLE, rheumatoid arthritis (RA, and Sjögren’s syndrome (SS and sum up the existing data linking EBV with these diseases including elevated titres of EBV antibodies, reduced T-cell defence against EBV, and elevated EBV viral load. Together, these data suggest that uncontrolled EBV infection can develop diverse autoreactivities in genetic susceptible individuals with different manifestations depending on the genetic background and the site of reactivation.

  4. "Gestaltomics": Systems Biology Schemes for the Study of Neuropsychiatric Diseases.

    Science.gov (United States)

    Gutierrez Najera, Nora A; Resendis-Antonio, Osbaldo; Nicolini, Humberto

    2017-01-01

    The integration of different sources of biological information about what defines a behavioral phenotype is difficult to unify in an entity that reflects the arithmetic sum of its individual parts. In this sense, the challenge of Systems Biology for understanding the "psychiatric phenotype" is to provide an improved vision of the shape of the phenotype as it is visualized by "Gestalt" psychology, whose fundamental axiom is that the observed phenotype (behavior or mental disorder) will be the result of the integrative composition of every part. Therefore, we propose the term "Gestaltomics" as a term from Systems Biology to integrate data coming from different sources of information (such as the genome, transcriptome, proteome, epigenome, metabolome, phenome, and microbiome). In addition to this biological complexity, the mind is integrated through multiple brain functions that receive and process complex information through channels and perception networks (i.e., sight, ear, smell, memory, and attention) that in turn are programmed by genes and influenced by environmental processes (epigenetic). Today, the approach of medical research in human diseases is to isolate one disease for study; however, the presence of an additional disease (co-morbidity) or more than one disease (multimorbidity) adds complexity to the study of these conditions. This review will present the challenge of integrating psychiatric disorders at different levels of information (Gestaltomics). The implications of increasing the level of complexity, for example, studying the co-morbidity with another disease such as cancer, will also be discussed.

  5. Empirical validation of the Horowitz Multiple Systemic Infectious Disease Syndrome Questionnaire for suspected Lyme disease

    Directory of Open Access Journals (Sweden)

    Citera M

    2017-09-01

    Full Text Available Maryalice Citera,1 Phyllis R Freeman,2 Richard I Horowitz2 1Department of Psychology, State University of New York at New Paltz, New Paltz, NY, 2Hudson Valley Healing Arts Center, Hyde Park, NY, USA Purpose: Lyme disease is spreading worldwide, with multiple Borrelia species causing a broad range of clinical symptoms that mimic other illnesses. A validated Lyme disease screening questionnaire would be clinically useful for both providers and patients. Three studies evaluated such a screening tool, namely the Horowitz Multiple Systemic Infectious Disease Syndrome (MSIDS Questionnaire. The purpose was to see if the questionnaire could accurately distinguish between Lyme patients and healthy individuals.Methods: Study 1 examined the construct validity of the scale examining its factor structure and reliability of the questionnaire among 537 individuals being treated for Lyme disease. Study 2 involved an online sample of 999 participants, who self-identified as either healthy (N=217 or suffering from Lyme now (N=782 who completed the Horowitz MSIDS Questionnaire (HMQ along with an outdoor activity survey. We examined convergent validity among components of the scale and evaluated discriminant validity with the Big Five personality characteristics. The third study compared a sample of 236 patients with confirmed Lyme disease with an online sample of 568 healthy individuals.Results: Factor analysis results identified six underlying latent dimensions; four of these overlapped with critical symptoms identified by Horowitz – neuropathy, cognitive dysfunction, musculoskeletal pain, and fatigue. The HMQ showed acceptable levels of internal reliability using Cronbach’s coefficient alpha and exhibited evidence of convergent and divergent validity. Components of the HMQ correlated more highly with each other than with unrelated traits.Discussion: The results consistently demonstrated that the HMQ accurately differentiated those with Lyme disease from

  6. Chronotherapeutic drug delivery systems: an approach to circadian rhythms diseases.

    Science.gov (United States)

    Sunil, S A; Srikanth, M V; Rao, N Sreenivasa; Uhumwangho, M U; Latha, K; Murthy, K V Ramana

    2011-11-01

    The purpose of writing this review on chronotherapeutic drug delivery systems (ChrDDs) is to review the literatures with special focus on ChrDDs and the various dosage forms, techniques that are used to target the circadian rhythms (CR) of various diseases. Many functions of the human body vary considerably in a day. ChrDDs refers to a treatment method in which in vivo drug availability is timed to match circadian rhythms of disease in order to optimize therapeutic outcomes and minimize side effects. Several techniques have been developed but not many dosage forms for all the diseases are available in the market. ChrDDs are gaining importance in the field of pharmaceutical technology as these systems reduce dosing frequency, toxicity and deliver the drug that matches the CR of that particular disease when the symptoms are maximum to worse. Finally, the ultimate benefit goes to the patient due the compliance and convenience of the dosage form. Some diseases that follow circadian rhythms include cardiovascular diseases, asthma, arthritis, ulcers, diabetes etc. ChrDDs in the market were also discussed and the current technologies used to formulate were also stated. These technologies include Contin® , Chronotopic®, Pulsincaps®, Ceform®, Timerx®, Oros®, Codas®, Diffucaps®, Egalet®, Tablet in capsule device, Core-in-cup tablet technology. A coated drug-core tablet matrix, A bi-layered tablet, Multiparticulate-based chronotherapeutic drug delivery systems, Chronoset and Controlled release microchips.

  7. Influence of Endocrine Diseases on Reproductive System Functioning

    Directory of Open Access Journals (Sweden)

    О.О. Коrytko

    2015-12-01

    Full Text Available In the lecture the questions of endocrine diseases influence on the reproductive system functioning are being considered. The reproductive system of woman is analyzed as a complex of interdependent structural elements: hypothalamus, pituitary organ, ovaries, organs-targets and other endocrine organs that provide realization of genesis function. During pregnancy, hormone metabolism changes that is an important factor for the diagnosis and treatment of endocrine diseases in pregnant women and women planning to become pregnant. It is shown that subclinical endocrine disorder is a factor reducing the normal hormonal functional response necessary for the adequate development of induced pregnancy. Therefore, in any violations of reproductive function (infertility, miscarriage, it is necessary to evaluate functioning of endocrine system, and pregnancy in women with endocrine pathology requires a significant correction of the conducted therapy.

  8. [Immune system and rheumatic diseases in the elderly].

    Science.gov (United States)

    Schirmer, Michael

    2016-06-01

    Impairments of the immune system play an important role in all immun-mediated rheumatic diseases. Recently, the following news were reported: · Early aging of the immune system with thymus insufficiency has now been reported for both patients with rheumatoid arthritis and axial spondyloarthritis, without prethymic lack of progenitors at least in rheumatoid arthritis.. · For giant cell arteritis, the most frequent vasculitis in the elderly, an increased expression of IL-17A in temporal artery biopsies coincides with good prognosis and reponse to glucocorticoids.. · Concerning immunosenescence in systemic lupus erythematosus, BAFF appears to have an important role for relapses after B-cell depletion.. For the future it can be anticipated that the use of unified classification criteria for rheumatic diseases (as with the new 2012 EULAR / ACR classification criteria for polymyalgia rheumatica) will ensure better comparability of immunological studies also in the elderly.

  9. Pulmonary disease management system with distributed wearable sensors.

    Science.gov (United States)

    Fu, Yongji; Ayyagari, Deepak; Colquitt, Nhedti

    2009-01-01

    A pulmonary disease management system with on-body and near-body sensors is introduced in this presentation. The system is wearable for continuous ambulatory monitoring. Distributed sensor data is transferred through a wireless body area network (BAN) to a central controller for real time analysis. Physiological and environmental parameters are monitored and analyzed using prevailing clinical guidelines for self-management of environmentally-linked pulmonary ailments. The system provides patients with reminders, warnings, and instructions to reduce emergency room and physician visits, and improve clinical outcomes.

  10. A prediction model for progressive disease in systemic sclerosis

    Science.gov (United States)

    Meijs, Jessica; Schouffoer, Anne A; Ajmone Marsan, Nina; Stijnen, Theo; Putter, Hein; Ninaber, Maarten K; Huizinga, Tom W J; de Vries-Bouwstra, Jeska K

    2015-01-01

    Objective To develop a model that assesses the risk for progressive disease in patients with systemic sclerosis (SSc) over the short term, in order to guide clinical management. Methods Baseline characteristics and 1 year follow-up results of 163 patients with SSc referred to a multidisciplinary healthcare programme were evaluated. Progressive disease was defined as: death, ≥10% decrease in forced vital capacity, ≥15% decrease in diffusing capacity for carbon monoxide, ≥10% decrease in body weight, ≥30% decrease in estimated-glomerular filtration rate, ≥30% increase in modified Rodnan Skin Score (with Δ≥5) or ≥0.25 increase in Scleroderma Health Assessment Questionnaire. The number of patients with progressive disease was determined. Univariable and multivariable logistic regression analyses were used to assess the probability of progressive disease for each individual patient. Performance of the prediction model was evaluated using a calibration plot and area under the receiver operating characteristic curve. Results 63 patients had progressive disease, including 8 patients who died ≤18 months after first evaluation. Multivariable analysis showed that friction rubs, proximal muscular weakness and decreased maximum oxygen uptake as % predicted, adjusted for age, gender and use of immunosuppressive therapy at baseline, were significantly associated with progressive disease. Using the prediction model, the predicted chance for progressive disease increased from a pretest chance of 37% to 67–89%. Conclusions Using the prediction model, the chance for progressive disease for individual patients could be doubled. Friction rubs, proximal muscular weakness and maximum oxygen uptake as % predicted were identified as relevant parameters. PMID:26688749

  11. Orchid Classification Disease Identification And Healthiness Prediction System

    Directory of Open Access Journals (Sweden)

    K. W. V Sanjaya

    2015-03-01

    Full Text Available Abstract Floriculture has become one of Sri Lankas major foreign exchange ventures and it has grown substantially during the last few years. Currently we can find three major types of growers in floriculture. They are Large Commercial Ventures Middle Level growers and Village Level growers. Both Middle Level and Village level growers usually go for low cost cultivation with minimum advanced techniques sticking to conventional methods. Orchid cultivation is more pleasurable and profitable than any other floriculture ventures. As the orchid cultivation is so pleasurable we can introduce another group of growers who cultivate orchid in their home gardens for making their home gardens beautiful. But the problem is that most of these growers may not have the knowledge to identify the specie of the plants as there are a number of similar looking plants which are in different species. And also they may not have the knowledge about the orchid diseases. Because of that they may not be able to get the maximum outcome from their cultivations. So the aim of our project is to address the above mentioned issues by introducing a system which can identify orchid species amp diseases and predict the healthiness of the orchid plants. The only input to this system is an image of an orchid leaf and the system will provide the orchid specie name diseases if there any healthiness of the orchid plant and suggestions to overcome the issues associated with the orchid plant as the output. We identify the orchid species and diseases by extracting the features of orchid plant leaf in the input image using image processing technics and with the use of data mining technics we predict the healthiness of the orchid plant. So this system will be a great help for the people who love to grow orchids but dont have knowledge about the orchid species and diseases. And also they will be able to find the healthiness of their orchid plants.

  12. Empirical validation of the Horowitz Multiple Systemic Infectious Disease Syndrome Questionnaire for suspected Lyme disease.

    Science.gov (United States)

    Citera, Maryalice; Freeman, Phyllis R; Horowitz, Richard I

    2017-01-01

    Lyme disease is spreading worldwide, with multiple Borrelia species causing a broad range of clinical symptoms that mimic other illnesses. A validated Lyme disease screening questionnaire would be clinically useful for both providers and patients. Three studies evaluated such a screening tool, namely the Horowitz Multiple Systemic Infectious Disease Syndrome (MSIDS) Questionnaire. The purpose was to see if the questionnaire could accurately distinguish between Lyme patients and healthy individuals. Study 1 examined the construct validity of the scale examining its factor structure and reliability of the questionnaire among 537 individuals being treated for Lyme disease. Study 2 involved an online sample of 999 participants, who self-identified as either healthy (N=217) or suffering from Lyme now (N=782) who completed the Horowitz MSIDS Questionnaire (HMQ) along with an outdoor activity survey. We examined convergent validity among components of the scale and evaluated discriminant validity with the Big Five personality characteristics. The third study compared a sample of 236 patients with confirmed Lyme disease with an online sample of 568 healthy individuals. Factor analysis results identified six underlying latent dimensions; four of these overlapped with critical symptoms identified by Horowitz - neuropathy, cognitive dysfunction, musculoskeletal pain, and fatigue. The HMQ showed acceptable levels of internal reliability using Cronbach's coefficient alpha and exhibited evidence of convergent and divergent validity. Components of the HMQ correlated more highly with each other than with unrelated traits. The results consistently demonstrated that the HMQ accurately differentiated those with Lyme disease from healthy individuals. Three migratory pain survey items (persistent muscular pain, arthritic pain, and nerve pain/paresthesias) robustly identified individuals with verified Lyme disease. The results support the use of the HMQ as a valid, efficient, and low

  13. Nutritional status in chronic obstructive pulmonary disease and systemic sclerosis: two systemic diseases involving the respiratory system.

    Science.gov (United States)

    Mękal, D; Doboszyńska, A; Kądalska, E; Świetlik, E; Rudnicka, L

    2015-01-01

    This study aimed to assess and compare the nutritional status and life quality of patients with chronic obstructive pulmonary disease (COPD) and systemic sclerosis (SSc). Thirty patients with stable COPD and 32 patients with SSc were examined. In all patients, the following parameters were measured: fat mass, fat-free mass, total body water, FEV1, and blood gases. COPD patients' life quality was assessed with St. George's Respiratory Questionnaire, and in SSc patients with a Quality of Life Questionnaire. The results show that among COPD patients 13% had normal body weight, 60% were obese, and 27% were overweight. In SSc patients, 59% had normal body weight, 31% were overweight, 1 patient was obese, and 2 were underweight. The mean life quality score in COPD patients was 57.3±16.5, while that in SSc patients was 35.8±18.2. COPD patients had a statistically significant lower life quality than SSc patients. The mean value of FEV1 was 45.5±12.2% pred. in COPD patients, and 86.8±21.2% pred. in the SSc group. We conclude that nutritional disorders are more frequent in COPD patients compared to those with SSc.

  14. Decision Support in Heart Disease Prediction System using Naive Bayes

    Directory of Open Access Journals (Sweden)

    G.Subbalakshmi,

    2011-04-01

    Full Text Available Data Mining refers to using a variety of techniques to identify suggest of information or decision making knowledge in thedatabase and extracting these in a way that they can put to use in areas such as decision support, predictions, forecasting and estimation. The healthcare industry collects huge amounts of healthcare data which, unfortunately, are not “mined” to discover hidden information for effective decision making. Discovering relations that connect variables in a database is the subject of data mining. This research has developed a Decision Support in Heart Disease Prediction System (DSHDPS using data mining modeling technique, namely, Naïve Bayes. Using medical profiles such as age, sex, blood pressure and blood sugar it can predict the likelihood of patients getting a heart disease. It is implemented as web based questionnaire application. It can serve a training tool to train nurses and medical students to diagnose patients with heart disease.

  15. Design of Knowledge Management System for Diabetic Complication Diseases

    Science.gov (United States)

    Fiarni, Cut

    2017-01-01

    This paper examines how to develop a Model for Knowledge Management System (KMS) for diabetes complication diseases. People with diabetes have a higher risk of developing a series of serious health problems. Each patient has different condition that could lead to different disease and health problem. But, with the right information, patient could have early detection so the health risk could be minimized and avoided. Hence, the objective of this research is to propose a conceptual framework that integrates social network model, Knowledge Management activities, and content based reasoning (CBR) for designing such a diabetes health and complication disease KMS. The framework indicates that the critical knowledge management activities are in the process to find similar case and the index table for algorithm to fit the framework for the social media. With this framework, KMS developers can work with healthcare provider to easily identify the suitable IT associated with the CBR process when developing a diabetes KMS.

  16. Cardiovascular disease due to accelerated atherosclerosis in systemic vasculitides.

    Science.gov (United States)

    Cohen Tervaert, Jan Willem

    2013-02-01

    Patients with different forms of systemic vasculitis experience long-term morbidity and mortality caused by cardiovascular disease due to premature atherosclerosis. Epidemiologic reports of patients with GCA suggest that long-term mortality in this disease is not increased compared with the general population of the same age. The risk of a stroke, however, in particular in the vertebrobasilar territory, is increased. In addition, the occurrence of aortic aneurysmal disease and aortic dissection is also clearly increased in GCA. Mortality due to ischaemic heart disease, however, is not increased. In Takayasu arteritis accelerated atherosclerosis has been clearly documented both clinically and in autopsy reports. Atherosclerotic plaques in the carotid artery may be present in the carotid arteries especially in patients with a documented history of arteritis involving the carotid artery. It is controversial whether Kawasaki disease is associated with accelerated atherosclerosis. Young adults with a history of Kawasaki disease may have abnormal brachial artery reactivity, increased carotid IMT values and increased arterial stiffness. At autopsy examinations of KD patients, however, no significant atherosclerotic lesions are detected and carotid IMT measurements were found to be clearly different from those in young adults with familiar hypercholesterolaemia, suggesting that the remodeling process in KD is different from atherosclerosis. In ANCA-associated vasculitis (AAV), an increased mortality as a consequence of cardiovascular disease is well-documented. In these patients the relative risk for coronary heart disease is two- to fourfold that in control subjects. In addition, a similar relative risk has been found for stroke. Diabetes, hypertension, dyslipidemia, abdominal obesity (metabolic syndrome), impaired renal function, persistent proteinuria and increased production of C-reactive protein are common risk factors for premature atherosclerosis in patients with

  17. IgG4相关疾病误诊为多中心型Castleman病3例并文献回顾%IgG4-related Disease Misdiagnosed as Multicentric Castleman's Disease: A Three-cases Report and Literature Review

    Institute of Scientific and Technical Information of China (English)

    焦洋; 张文; 黄晓明; 曾学军

    2013-01-01

    目的 通过病例资料分析IgG4相关疾病特点,总结IgG4相关疾病被误诊为多中心型Castleman病的原因,提高对此类疾病的诊断准确性.方法 回顾性分析2008年1月至2012年12月北京协和医院诊治的3例IgG4相关疾病患者误诊为多中心型Castleman病的临床资料.结果 3例患者均为男性,年龄为53~57岁,均有全身广泛的淋巴结肿大及多系统受累,均曾行1次以上淋巴结活检.经病理检查,2例确诊、1例疑诊为多中心型Castleman病.筛查3例患者血清IgG4水平均明显升高,均大于1350 mg/L;正电子发射计算机断层显像仪-CT(positron-emission tomography/computed tomography,PET-CT)显示全身广泛淋巴结及多脏器摄取增高;2例患者病理免疫组化证实IgG4阳性淋巴细胞占淋巴细胞50%以上.3例患者最终被确诊为IgG4相关疾病.经足量糖皮质激素及环磷酰胺治疗后3例患者的临床、血清学及影像学异常均迅速显著缓解.结论 IgG4相关疾病易被误诊为多中心型Castleman病,对临床表现、血清IgG4水平、PET-CT检查及病理免疫组化的综合分析有助于IgG4相关疾病的诊断.%Objective To analyze the clinical features of IgG4-related disease and determine the reasons why The patients with IgG4-related disease are misdiagnosed with multicentric Castleman's disease. Methods We retrospectively reviewed the medical records of the three patients with IgG4-related disease, who were admitted to Peking Union Medical College Hospital ( PUMCH) and had been misdiagnosed as multicentric Castleman' s disease. Results All three patients were males aged 53 ~ 57. They presented with generalized lymphadenopathy and multiple organ involvement. Biopsy results of their lymph nodes were consistent with multicentric Castleman' s disease. However, all their serum IgG4 levels were significantly elevated. Fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) scan detected abnormal FDG

  18. Nervous system Lyme disease, chronic Lyme disease, and none of the above.

    Science.gov (United States)

    Halperin, John J

    2016-03-01

    Lyme borreliosis, infection with the tick-borne spirochete Borrelia burgdorferi sensu lato, causes nervous system involvement in 10-15 % of identified infected individuals. Not unlike the other well-known spirochetosis, syphilis, infection can be protracted, but is microbiologically curable in virtually all patients, regardless of disease duration. Diagnosis relies on 2-tier serologic testing, which after the first 4-6 weeks of infection is both highly sensitive and specific. After this early, acute phase, serologic testing should rely only on IgG reactivity. Nervous system involvement most commonly presents with meningitis, cranial neuritis and radiculoneuritis, but can also present with a broader array of peripheral nervous system manifestations. Central nervous system infection typically elicits a cerebrospinal fluid pleocytosis and, often, intrathecal production of specific antibody, findings that should not be expected in disease not affecting the CNS. Treatment with recommended courses of oral or, when necessary, parenteral antibiotics is highly effective. The attribution of chronic, non-specific symptoms to "chronic Lyme disease", in the absence of specific evidence of ongoing B. burgdorferi infection, is inappropriate and unfortunate, leading not only to unneeded treatment and its associated complications, but also to missed opportunities for more appropriate management of patients' often disabling symptoms.

  19. [Involvement of Syk in pathology of systemic autoimmune disease].

    Science.gov (United States)

    Iwata, Shigeru; Yamaoka, Kunihiro; Niiro, Hiroaki; Nakano, Kazuhisa; Wang, Sheau-Pey; Saito, Kazuyoshi; Akashi, Koichi; Tanaka, Yoshiya

    2012-01-01

    Biological products have proven its high efficacy on autoimmune disease such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Meanwhile, small molecular drugs have attracted attention over the years because of its availability of oral administration and cost effectiveness. Spleen tyrosine kinase (Syk) is a 72 kDa protein tyrosine kinase widely expressed on cells that are involved in the immune system and inflammation such as B cells, T cells, macrophages and synovial fibroblast. Syk is involved in intracellular signaling of the multi-chain immune receptors, including B cell receptor (BCR), ζchain of T-cell receptor (TCR), FcR and integrins, which contains the immune-receptor tyrosine-based activation motif (ITAM). Recently, Syk inhibitor fostamatinib has exerted potent therapeutic efficacy against autoimmune and allergic diseases such as rheumatoid arthritis (RA), bronchial asthma and thrombocytopenic purpura (ITP). Moreover, Syk blockade prevented the development of skin and kidney lesions in lupus-prone mice, however the mechanism of action is unclear. We have revealed that Syk-mediated BCR-signaling is prerequisite for optimal induction of toll-like receptor (TLR)-9, thereby allowing efficient propagation of CD40- and TLR9- signaling in human B cells. These results indicate that inhibition of Syk have a potential to regulate B-cell mediated inflammatory diseases such as SLE. We here document the in vitro and in vivo effects of a Syk inhibitor for the treatment of autoimmune diseases, mainly in RA and SLE.

  20. Enhancement on infectious diseases nursing plan information system.

    Science.gov (United States)

    Yeh, Mei-Lin; Hao, Te-Hui; Hsu, Chien-Yeh

    2009-01-01

    Based on researches, the most time-consuming nursing activities, in teaching hospital, are: room patrols, the blood pressure survey, the body temperature pulse breath survey, the nursing record maintenance. The nursing record is one way to communicate data. It can allow the medical service team to understand what measures the nursing staff once did for sickness, as well as responses from sickness. Nevertheless, it is the key component to utilize the record with a clinical nursing plan, so as to provide a proficient health management. Since the maintenance of nursing plan is costly and time-consuming, therefore, it is essential to establish the nursing plan information system, which can effectively promote the nursing quality. This research main body comes from one infectious disease division nursing plan information system, which was developed in 1992, and its data base covers entire courtyard compatibility and various faculties characteristic nursing plan. The nursing staff often complained that this system is not user-friendly, its contents are not comprehensive, and sometimes it does not let staff choose the right diagnosis. Therefore this research is based on history analysis and the questionnaire survey procedure first, the infectious disease nursing plan use number of times, the frequency and the project content, then by the literature scientific theory and result of the improvement group discussion together. The original 38 infectious disease division nursing plan will be expanded to 45 nursing plans. Moreover, the common 38 infectious disease code (ICD-9), and its corresponding diagnosis items, shall automatically appear in the disease diagnose code field, so it would be better off for the nursing staff to set up the nursing plan efficiently. Infectious disease division nursing plan information system utilization ratio is promoted 9.6-folds, according to research outcome. Each task consumes 3.68 minutes beforehand-including computer program operation, the

  1. Systemic disease manifestations associated with epilepsy in tuberous sclerosis complex.

    Science.gov (United States)

    Jeong, Anna; Wong, Michael

    2016-09-01

    Epilepsy is one of the most disabling symptoms of tuberous sclerosis complex (TSC) and is a leading cause of morbidity and mortality in affected individuals. The relationship between systemic disease manifestations and the presence of epilepsy has not been thoroughly investigated. This study utilizes a multicenter TSC Natural History Database including 1,816 individuals to test the hypothesis that systemic disease manifestations of TSC are associated with epilepsy. Univariate analysis was used to identify patient characteristics (e.g., age, gender, race, and TSC mutation status) associated with the presence of epilepsy. Individual logistic regression models were built to examine the association between epilepsy and each candidate systemic or neurologic disease variable, controlling for the patient characteristics found to be significant on univariate analysis. Finally, a multivariable logistic regression model was constructed, using the variables found to be significant on the individual analyses as well as the patient characteristics that were significant on univariate analysis. Nearly 88% of our cohort had a history of epilepsy. After adjusting for age, gender, and TSC mutation status, multiple systemic disease manifestations including cardiac rhabdomyomas (odds ratio [OR] 2.3, 95% confidence interval [CI] 1.3-3.9, p = 0.002), retinal hamartomas (OR 2.1, CI 1.0-4.3, p = 0.04), renal cysts (OR 2.1, CI 1.3-3.4, p = 0.002), renal angiomyolipomas (OR 3.0, CI 1.8-5.1, p < 0.001), shagreen patches (OR 1.7, CI 1.0-2.7, p = 0.04), and facial angiofibromas (OR 1.7, CI 1.1-2.9, p = 0.03) were associated with a higher likelihood of epilepsy. In the multivariable logistic regression model, cardiac rhabdomyomas (OR 1.9, CI 1.0-3.5, p = 0.04) remained significantly associated with the presence of epilepsy. The identification of systemic disease manifestations such as cardiac rhabdomyomas that confer a higher risk of epilepsy development in TSC could contribute to disease

  2. [Eales' disease involving central nervous system white matter].

    Science.gov (United States)

    Antigüedad, A; Zarranz, J J

    1994-01-01

    Eales' disease (ED) is a rare condition characterized by repeated retinal and vitreous hemorrhages. The only extraocular involvement described occasionally in the literature is neurological. Histologically, vasculitis in ED is usually restricted to the eye, but occasionally involves the central nervous system, where demyelinizing lesions may also occur. We present a 34-year-old male with ED and subclinical central nervous system involvement. Craneal magnetic resonance images (MR) suggested demyelinization; brainstem auditory and somatosensory evoked potentials were abnormal. There was moderate pleocytosis in CSF and intratecal production of immunoglobulins with oligoclonal bands. Follow-up over a period of 2.5 years showed no clinical, MR or CSF changes in spite of continued opthamological impairment. Little is known about factors that affect the development or not of demyelinizing lesions in ED patients with neurological involvement demonstrated by intratecal production of immunoglobulins. Identification of such factors may contribute to our understanding of other diseases, such as multiple sclerosis.

  3. Statin Induced Myopathy a Patient with Multiple Systemic Diseases

    Directory of Open Access Journals (Sweden)

    Özgül Uçar

    2011-04-01

    Full Text Available Hydroxymethylglutaryl-coenzyme A reductase inhibitors (statins are the most successful class of drugs for the treatment of hypercholesterolaemia and dyslipidaemia. However, the popular profile of statins in terms of efficacy has been maligned by theiradverse effects. Statin induced myopathy, which can be seen at any time during the course of therapy, is a clinically important cause of statin intolerance and discontinuation. When a patient with multiple systemic diseases who use numerous medications represent with myalgia and muscle cramps, statin induced myopathy may not be remembered at first. We present a patient with multiple systemic diseases, alcohol and morphine abuse in whom myopathy developed. After exclusion of other etiologies, we concluded that myopathy was related to statin therapy.

  4. Organ culture system as a means to detect celiac disease.

    Science.gov (United States)

    Picarelli, Antonio; Libanori, Valerio; De Nitto, Daniela; Saponara, Annarita; Di Tola, Marco; Donato, Giuseppe

    2010-01-01

    Anti-endomysial and anti-transglutaminase antibodies can be produced in vitro by the intestinal mucosa of celiac disease (CD) patients in clinical remission, when the culture is performed in the presence of gliadin peptides. Our aim was to use this organ culture system as a means to detect the pathognomonic antibodies of celiac disease (CD) in the culture supernatants. Organ culture was performed in the presence of three different activators to evaluate which one induced the strongest antibody response in intestinal mucosa from patients in clinical remission of CD. Our data confirm the high efficiency of synthetic peptide 31-43 as a specific immunological activator in CD and demonstrate its capability to stimulate production/secretion of CD-specific antibodies. We envision that this organ culture system may prove to be useful as a new technique for CD diagnosis.

  5. Cellular systems biology profiling applied to cellular models of disease.

    Science.gov (United States)

    Giuliano, Kenneth A; Premkumar, Daniel R; Strock, Christopher J; Johnston, Patricia; Taylor, Lansing

    2009-11-01

    Building cellular models of disease based on the approach of Cellular Systems Biology (CSB) has the potential to improve the process of creating drugs as part of the continuum from early drug discovery through drug development and clinical trials and diagnostics. This paper focuses on the application of CSB to early drug discovery. We discuss the integration of protein-protein interaction biosensors with other multiplexed, functional biomarkers as an example in using CSB to optimize the identification of quality lead series compounds.

  6. Therapies in the Pipeline for Systemic Autoimmune Diseases

    OpenAIRE

    Juan Felipe Betancur; Jose Fernando Molina; Gabriel J. Tobón

    2016-01-01

    The current goals in the development of novel therapeutics of systemic autoimmune diseases are to develop agents more effective than conventional therapies as well as to reduce the risk of organ damage. To achieve this goal, large multicentre randomised controlled trials are needed to confirm the efficacy and safety of novel agents. Whether these novel modalities are synergistic to conventional drugs, the optimal dosages, and duration of treatment, need to be explored. As expected, the dev...

  7. Statin Induced Myopathy a Patient with Multiple Systemic Diseases

    OpenAIRE

    Özgül Uçar; İbrahim Kocaoğlu; Ahmet Karagöz; Serkan Gökaslan

    2011-01-01

    Hydroxymethylglutaryl-coenzyme A reductase inhibitors (statins) are the most successful class of drugs for the treatment of hypercholesterolaemia and dyslipidaemia. However, the popular profile of statins in terms of efficacy has been maligned by theiradverse effects. Statin induced myopathy, which can be seen at any time during the course of therapy, is a clinically important cause of statin intolerance and discontinuation. When a patient with multiple systemic diseases who use numerous medi...

  8. Prevalence of Systemic Diseases in Patients with Dental Infections

    OpenAIRE

    Seyed Mohsen Khoshniat Nikoo; Mohammad Bayat; Fatemeh Afshar Hezarkhani

    2013-01-01

    Background and Aims: The aim of this study was to investigate the prevalence of diabetes and other risk factors in patients with dental infections.Materials and Methods: A cross-sectional study was conducted among 50 patients who preferred in maxillofacial word of shariaty hospital with acute dental infections in 9 months. A self-administered questionnaire was administered during a dental appointment in order to gather demographic information and recorded past history of systemic disease, OPG...

  9. Relationship between systemic diseases and endodontics: an online study guide.

    Science.gov (United States)

    2008-05-01

    The Editorial Board of the Journal of Endodontics has developed a literature-based study guide of topical areas related to endodontics. This study guide is intended to give the reader a focused review of the essential endodontic literature and does not cite all possible articles related to each topic. Although citing all articles would be comprehensive, it would defeat the idea of a study guide. This section will cover the relationship between systemic diseases and endodontics.

  10. Role of the ubiquitin proteasome system in Parkinson's disease

    OpenAIRE

    2007-01-01

    Abstract Parkinson's disease (PD) is the most common neurodegenerative movement disorder. Although a subject of intense research, the etiology of PD remains poorly understood. Recently, several lines of evidence have implicated an intimate link between aberrations in the ubiquitin proteasome system (UPS) and PD pathogenesis. Derangements of the UPS, which normally functions as a type of protein degradation machinery, lead to alterations in protein homeostasis that could conceivably promote th...

  11. Pervasive mobile healthcare systems for chronic disease monitoring.

    Science.gov (United States)

    Huzooree, Geshwaree; Kumar Khedo, Kavi; Joonas, Noorjehan

    2017-05-01

    Pervasive mobile healthcare system has the potential to improve healthcare and the quality of life of chronic disease patients through continuous monitoring. Recently, many articles related to pervasive mobile healthcare system focusing on health monitoring using wireless technologies have been published. The main aim of this review is to evaluate the state-of-the-art pervasive mobile healthcare systems to identify major technical requirements and design challenges associated with the realization of a pervasive mobile healthcare system. A systematic literature review was conducted over IEEE Xplore Digital Library to evaluate 20 pervasive mobile healthcare systems out of 683 articles from 2011 to 2016. The classification of the pervasive mobile healthcare systems and other important factors are discussed. Potential opportunities and challenges are pointed out for the further deployment of effective pervasive mobile healthcare systems. This article helps researchers in health informatics to have a holistic view toward understanding pervasive mobile healthcare systems and points out new technological trends and design challenges that researchers have to consider when designing such systems for better adoption, usability, and seamless integration.

  12. Clinical and biochemical landmarks in systemic autoinflammatory diseases.

    Science.gov (United States)

    Cantarini, Luca; Rigante, Donato; Brizi, Maria Giuseppina; Lucherini, Orso Maria; Sebastiani, Gian Domenico; Vitale, Antonio; Gianneramo, Valentina; Galeazzi, Mauro

    2012-11-01

    Systemic autoinflammatory diseases are a group of inherited disorders of the innate immune system characterized by seemingly unprovoked inflammation recurring at variable intervals and involving skin, serosal membranes, joints, and gastrointestinal apparatus, with reactive amyloidosis as a possible severe long-term complication. Recent advances in genetics and molecular biology have improved our understanding of the pathogenesis of these diseases, including familial Mediterranean fever, mevalonate kinase deficiency syndrome, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes, and hereditary pyogenic and granulomatous disorders: the vast majority of these conditions are related to the activation of the interleukin-1 pathway, which results in (or from?) a common unifying pathogenetic mechanism. Their diagnostic identification derives from the combination of clinical data, evaluation of acute phase reactants, clinical efficacy in response to specific drugs, and recognition of specific mutations in the relevant genes, although genetic tests may be unconstructive in some cases. This review will discuss clinical and laboratory clues useful for a diagnostic approach to systemic autoinflammatory diseases.

  13. Elevated Adiponectin Serum Levels in Women with Systemic Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Éric Toussirot

    2010-01-01

    Full Text Available Adipose tissue produces a wide range of proteins that may influence the immune system. In this study, we assessed the serum levels of leptin, adiponectin, and ghrelin, in association with the measurements of body composition, in 15 female patients with various autoimmune diseases (systemic lupus erythematosus, primary Sjögren's syndrome, sarcoidosis, mixed connective tissue disease, vasculitis, CREST syndrome, and polymyositis and in 15 healthy female controls. There were no statistically significant differences between the patients and controls with regard to serum leptin, serum ghrelin, global fat mass, adiposity, and fat mass in the android or gynoid regions, whereas serum adiponectin levels were higher in patients than controls (16.3±1.6 μg/mL versus 9.7±0.6 μg/mL; =.01. As adiponectin is known to exhibit potent anti-inflammatory properties, a high adiponectinemia in patients with systemic autoimmune disease may mitigate the inflammatory response. However, the precise consequences of these elevated serum adiponectin levels on the metabolic syndrome development and atherosclerotic cardiovascular risk in this patient population still needs to be determined.

  14. Plasmin system of Alzheimer's disease patients: CSF analysis.

    Science.gov (United States)

    Martorana, Alessandro; Sancesario, Giulia M; Esposito, Zaira; Nuccetelli, Marzia; Sorge, Roberto; Formosa, Amanda; Dinallo, Vincenzo; Bernardi, Giorgio; Bernardini, Sergio; Sancesario, Giuseppe

    2012-07-01

    Alzheimer's disease (AD) is a multifactorial neurodegenerative disorder characterized by the extracellular deposit of Amyloid beta (Aβ), mainly of the Amyloid beta(1-42) (Aβ(1-42)) peptide in the hippocampus and neocortex leading to progressive cognitive decline and dementia. The possible imbalance between the Aβ production/degradation process was suggested to contribute to the pathogenesis of AD. Among others, the serine protease plasmin has shown to be involved in Aβ(1-42) clearance, a hypothesis strengthened by neuropathological studies on AD brains. To explore whether there is a change in plasmin system in CSF of AD patients, we analyzed CSF samples from AD and age-matched controls, looking at plasminogen, tissue plasminogen activator (t-PA) and plasminogen activator inhibitor (PAI-1) protein levels and t-PA and urokinase plasminogen activator (u-PA) enzymatic activities. We also measured Aβ(1-42), total-tau and phospho-tau (181) CSF levels and sought for a possible relationship between them and plasmin system values. Our findings showed that t-PA, plasminogen and PAI-1 levels, as t-PA enzymatic activity, remained unchanged in AD with respect to controls; u-PA activity was not detected. We conclude that CSF analysis of plasminogen system does not reflect changes observed post-mortem. Unfortunately, the CSF detection of plasmin system could not be a useful biomarker for either AD diagnosis or disease progression. However, these findings do not exclude the possible involvement of the plasmin system in AD.

  15. Chronic obstructive pulmonary disease and obstructive sleep apnea: overlaps in pathophysiology, systemic inflammation, and cardiovascular disease.

    LENUS (Irish Health Repository)

    McNicholas, Walter T

    2012-02-01

    Chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea syndrome represent two of the most prevalent chronic respiratory disorders in clinical practice, and cardiovascular diseases represent a major comorbidity in each disorder. The two disorders coexist (overlap syndrome) in approximately 1% of adults but asymptomatic lower airway obstruction together with sleep-disordered breathing is more prevalent. Although obstructive sleep apnea syndrome has similar prevalence in COPD as the general population, and vice versa, factors such as body mass index and smoking influence relationships. Nocturnal oxygen desaturation develops in COPD, independent of apnea\\/hypopnea, and is more severe in the overlap syndrome, thus predisposing to pulmonary hypertension. Furthermore, upper airway flow limitation contributes to nocturnal desaturation in COPD without apnea\\/hypopnea. Evidence of systemic inflammation in COPD and sleep apnea, involving C-reactive protein and IL-6, in addition to nuclear factor-kappaB-dependent pathways involving tumor necrosis factor-alpha and IL-8, provides insight into potential basic interactions between both disorders. Furthermore, oxidative stress develops in each disorder, in addition to activation and\\/or dysfunction of circulating leukocytes. These findings are clinically relevant because systemic inflammation may contribute to the pathogenesis of cardiovascular diseases and the cell\\/molecular pathways involved are similar to those identified in COPD and sleep apnea. However, the pathophysiological and clinical significance of systemic inflammation in COPD and sleep apnea is not proven, and thus, studies of patients with the overlap syndrome should provide insight into the mechanisms of systemic inflammation in COPD and sleep apnea, in addition to potential relationships with cardiovascular disease.

  16. Ubiquitin-proteasome system involvement in Huntington’s disease

    Directory of Open Access Journals (Sweden)

    Zaira eOrtega

    2014-09-01

    Full Text Available Huntington’s disease (HD is a genetic autosomal dominant neurodegenerative disease caused by the expansion of a CAG repeat in the huntingtin (htt gene. This triplet expansion encodes a polyglutamine stretch (polyQ in the N-terminus of the high molecular weight (348-kDa and ubiquitously expressed protein huntingtin (htt. Normal individuals have between 6 and 35 CAG triplets, while expansions longer than 40 repeats lead to HD. The onset and severity of the disease depend on the length of the polyQ tract: the longer the polyQ is, the earlier the disease begins and the more severe the symptoms are. One of the main histopathological hallmarks of HD is the presence of intraneuronal proteinaceous inclusion bodies (IBs, whose prominent and invariant feature is the presence of Ubiquitin (Ub; therefore, they can be detected with anti-ubiquitin and anti-proteasome antibodies. This, together with the observation that mutations in components of the Ubiquitin Proteasome system (UPS give rise to some neurodegenerative diseases, suggests that UPS impairment may be causative of HD. Even though the link between disrupted Ub homeostasis and protein aggregation to HD is undisputed, the functional significance of these correlations and their mechanistic implications remains unresolved. Moreover, there is no consistent evidence documenting an accompanying decrease in levels of free Ub or disruption of Ub pool dynamics in neurodegenerative disease or models thus suggesting that the Ub-conjugate accumulation may be benign and just underlie lesion in 26S function. In this chapter we will elaborate on the different studies that have been performed using different experimental approaches, in order to shed light to this matter.

  17. Cerebrospinal fluid interleukin-6 in central nervous system inflammatory diseases.

    Directory of Open Access Journals (Sweden)

    Alexandre Wullschleger

    Full Text Available BACKGROUND: Interleukin (IL-6 is recognised as an important cytokine involved in inflammatory diseases of the central nervous system (CNS. OBJECTIVE: To perform a large retrospective study designed to test cerebrospinal fluid (CSF IL-6 levels in the context of neurological diseases, and evaluate its usefulness as a biomarker to help discriminate multiple sclerosis (MS from other inflammatory neurological diseases (OIND. PATIENTS AND METHODS: We analyzed 374 CSF samples for IL-6 using a quantitative enzyme-linked immunosorbent assay. Groups tested were composed of demyelinating diseases of the CNS (DD, n = 117, including relapsing-remitting MS (RRMS, n = 65, primary progressive MS (PPMS, n = 11, clinically isolated syndrome (CIS, n = 11, optic neuritis (ON, n = 30; idiopathic transverse myelitis (ITM, n = 10; other inflammatory neurological diseases (OIND, n = 35; and non-inflammatory neurological diseases (NIND, n = 212. Differences between groups were analysed using Kruskal-Wallis test and Mann-Whitney U-test. RESULTS: CSF IL-6 levels exceeded the positivity cut-off of 10 pg/ml in 18 (51.4% of the 35 OIND samples, but in only three (3.9% of the 76 MS samples collected. CSF IL-6 was negative for all NIND samples tested (0/212. IL-6 cut-off of 10 pg/ml offers 96% sensitivity to exclude MS. CONCLUSION: CSF IL-6 may help to differentiate MS from its major differential diagnosis group, OIND.

  18. Familial occurrence of systemic mast cell activation disease.

    Directory of Open Access Journals (Sweden)

    Gerhard J Molderings

    Full Text Available Systemic mast cell activation disease (MCAD comprises disorders characterized by an enhanced release of mast cell mediators accompanied by accumulation of dysfunctional mast cells. Demonstration of familial clustering would be an important step towards defining the genetic contribution to the risk of systemic MCAD. The present study aimed to quantify familial aggregation for MCAD and to investigate the variability of clinical and molecular findings (e.g. somatic mutations in KIT among affected family members in three selected pedigrees. Our data suggest that systemic MCAD pedigrees include more systemic MCAD cases than would be expected by chance, i.e., compared with the prevalence of MCAD in the general population. The prevalence of MCAD suspected by symptom self-report in first-degree relatives of patients with MCAD amounted to approximately 46%, compared to prevalence in the general German population of about 17% (p<0.0001. In three families with a high familial loading of MCAD, the subtype of MCAD and the severity of mediator-related symptoms varied between family members. In addition, genetic alterations detected in KIT were variable, and included mutations at position 816 of the amino acid sequence. In conclusion, our data provide evidence for common familial occurrence of MCAD. Our findings observed in the three pedigrees together with recent reports in the literature suggest that, in familial cases (i.e., in the majority of MCAD, mutated disease-related operator and/or regulator genes could be responsible for the development of somatic mutations in KIT and other proteins important for the regulation of mast cell activity. Accordingly, the immunohistochemically different subtypes of MCAD (i.e. mast cell activation syndrome and systemic mastocytosis should be more accurately regarded as varying presentations of a common generic root process of mast cell dysfunction, than as distinct diseases.

  19. DISEASES

    DEFF Research Database (Denmark)

    Pletscher-Frankild, Sune; Pallejà, Albert; Tsafou, Kalliopi;

    2015-01-01

    Text mining is a flexible technology that can be applied to numerous different tasks in biology and medicine. We present a system for extracting disease-gene associations from biomedical abstracts. The system consists of a highly efficient dictionary-based tagger for named entity recognition...... of human genes and diseases, which we combine with a scoring scheme that takes into account co-occurrences both within and between sentences. We show that this approach is able to extract half of all manually curated associations with a false positive rate of only 0.16%. Nonetheless, text mining should...... not stand alone, but be combined with other types of evidence. For this reason, we have developed the DISEASES resource, which integrates the results from text mining with manually curated disease-gene associations, cancer mutation data, and genome-wide association studies from existing databases...

  20. Periodontal disease in Chinese patients with systemic lupus erythematosus.

    Science.gov (United States)

    Zhang, Qiuxiang; Zhang, Xiaoli; Feng, Guijaun; Fu, Ting; Yin, Rulan; Zhang, Lijuan; Feng, Xingmei; Li, Liren; Gu, Zhifeng

    2017-08-01

    Disease of systemic lupus erythematosus (SLE) and periodontal disease (PD) shares the common multiple characteristics. The aims of the present study were to evaluate the prevalence and severity of periodontal disease in Chinese SLE patients and to determine the association between SLE features and periodontal parameters. A cross-sectional study of 108 SLE patients together with 108 age- and sex-matched healthy controls was made. Periodontal status was conducted by two dentists independently. Sociodemographic characteristics, lifestyle factors, medication use, and clinical parameters were also assessed. The periodontal status was significantly worse in SLE patients compared to controls. In univariate logistic regression, SLE had a significant 2.78-fold [95% confidence interval (CI) 1.60-4.82] increase in odds of periodontitis compared to healthy controls. Adjusted for potential risk factors, patients with SLE had 13.98-fold (95% CI 5.10-38.33) increased odds against controls. In multiple linear regression model, the independent variable negatively and significantly associated with gingival index was education (P = 0.005); conversely, disease activity (P periodontitis of SLE in multivariate logistic regression (OR 1.348; 95% CI: 1.183-1.536, P < 0.001). Chinese SLE patients were likely to suffer from higher odds of PD. These findings confirmed the importance of early interventions in combination with medical therapy. It is necessary for a close collaboration between dentists and clinicians when treating those patients.

  1. Systems biology strategies to study lipidomes in health and disease.

    Science.gov (United States)

    Hyötyläinen, Tuulia; Orešič, Matej

    2014-07-01

    Lipids are a diverse group of metabolites that have many key biological functions, acting as structural components of cell membranes, energy storage sources and intermediates in signaling pathways. Due to their importance lipids are under tight homeostatic control and exhibit spatial and dynamic complexity at multiple levels. It is thus not surprising that altered lipid metabolism plays important roles in the pathogenesis of most of the common diseases. Lipidomics emerged as a discipline which is dedicated to global study of lipidomes, including pathways and networks of lipids in biological systems. When studying the lipidomes at a systems level, one of the key challenges is how to address the lipid functionality at many physiological levels, from metabolic and signaling pathways to spatial systems such as cellular membranes and lipoprotein particles. Besides the better analytical techniques to study lipids, computational techniques have started to emerge which enable modeling of lipidomes in their spatial and dynamic context. Together, the recent methodological advances in lipidomics have a potential to open novel avenues for predictive and preventive medicine. This review focuses on progress in systems approaches to study lipids in health and disease, with specific emphasis on clinical applications.

  2. Clearance systems in the brain-implications for Alzheimer disease.

    Science.gov (United States)

    Tarasoff-Conway, Jenna M; Carare, Roxana O; Osorio, Ricardo S; Glodzik, Lidia; Butler, Tracy; Fieremans, Els; Axel, Leon; Rusinek, Henry; Nicholson, Charles; Zlokovic, Berislav V; Frangione, Blas; Blennow, Kaj; Ménard, Joël; Zetterberg, Henrik; Wisniewski, Thomas; de Leon, Mony J

    2015-08-01

    Accumulation of toxic protein aggregates-amyloid-β (Aβ) plaques and hyperphosphorylated tau tangles-is the pathological hallmark of Alzheimer disease (AD). Aβ accumulation has been hypothesized to result from an imbalance between Aβ production and clearance; indeed, Aβ clearance seems to be impaired in both early and late forms of AD. To develop efficient strategies to slow down or halt AD, it is critical to understand how Aβ is cleared from the brain. Extracellular Aβ deposits can be removed from the brain by various clearance systems, most importantly, transport across the blood-brain barrier. Findings from the past few years suggest that astroglial-mediated interstitial fluid (ISF) bulk flow, known as the glymphatic system, might contribute to a larger portion of extracellular Aβ (eAβ) clearance than previously thought. The meningeal lymphatic vessels, discovered in 2015, might provide another clearance route. Because these clearance systems act together to drive eAβ from the brain, any alteration to their function could contribute to AD. An understanding of Aβ clearance might provide strategies to reduce excess Aβ deposits and delay, or even prevent, disease onset. In this Review, we describe the clearance systems of the brain as they relate to proteins implicated in AD pathology, with the main focus on Aβ.

  3. P-Glycoprotein and Drug Resistance in Systemic Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Andrea Picchianti-Diamanti

    2014-03-01

    Full Text Available Autoimmune diseases such as systemic lupus erythematosus (SLE, rheumatoid arthritis (RA and psoriatic arthritis (PsA are chronic inflammatory disorders of unknown etiology characterized by a wide range of abnormalities of the immune system that may compromise the function of several organs, such as kidney, heart, joints, brain and skin. Corticosteroids (CCS, synthetic and biologic immunosuppressive agents have demonstrated the capacity to improve the course of autoimmune diseases. However, a significant number of patients do not respond or develop resistance to these therapies over time. P-glycoprotein (P-gp is a transmembrane protein that pumps several drugs out of the cell, including CCS and immunosuppressants; thus, its over-expression or hyper-function has been proposed as a possible mechanism of drug resistance in patients with autoimmune disorders. Recently, different authors have demonstrated that P-gp inhibitors, such as cyclosporine A (CsA and its analogue Tacrolimus, are able to reduce P-gp expression and or function in SLE, RA and PsA patients. These observations suggest that P-gp antagonists could be adopted to revert drug resistance and improve disease outcome. The complex inter-relationship among drug resistance, P-gp expression and autoimmunity still remains elusive.

  4. Zoonotic disease surveillance--inventory of systems integrating human and animal disease information.

    Science.gov (United States)

    Wendt, A; Kreienbrock, L; Campe, A

    2015-02-01

    Although 65% of recent major disease outbreaks throughout the world have a zoonotic origin, there is still a sharp division among the disciplines into the human and animal health sectors. In the last few decades, a global integrative concept, often referred to as 'One Health', has been strongly endorsed. Surveillance and monitoring efforts are major components for effective disease prevention and control. As human health and animal health are inextricably linked, it is assumed that a cross-sectoral data interpretation of zoonotic disease information will improve their prevention, prediction and control. To provide an overview of existing systems throughout the world which integrate information from humans and animals on zoonotic diseases, a literature review was conducted. Twenty projects were identified and described regarding their concepts and realization. They all vary widely depending on their surveillance purpose, their structure and the source of information they use. What they have in common is that they quite often use data which have already been collected for another purpose. Therefore, the challenges of how to make use of such secondary data are of great interest.

  5. PTPN22 gene polymorphisms in autoimmune diseases with special reference to systemic lupus erythematosus disease susceptibility

    Directory of Open Access Journals (Sweden)

    Pradhan V

    2010-01-01

    Full Text Available Systemic lupus erythematosus (SLE is a prototype autoimmune disease. SLE is a result of one or more immune mechanisms, like autoantibody production, complement activation, multiple inflammation and immune complex deposition leading to organ tissue damage. SLE affected patients are susceptible to common and opportunistic infections. There are several reports suggesting that Mycobacterium tuberculosis infection precipitates SLE in patients from endemic areas. Genetic factors and environmental factors also play an important role in the overall susceptibility to SLE pathophysiology. Recently, protein tyrosine phosphatase, non-receptor type 22 (PTPN22 gene, has been found to be associated with several autoimmune diseases like SLE, Grave′s disease and Hashimoto thyroiditis. The missense R620W polymorphism, rs 2476601, in PTPN22 gene at the nucleotide 1858 in codon 620 (620Arg > Trp has been associated with autoimmune diseases. The PTPN22 locus is also found to be responsible for development of pulmonary tuberculosis in certain populations. The PTPN22 1858C/T gene locus will be ideal to look for SLE susceptibility to tuberculosis in the Indian population. In this review, we focus on human PTPN22 gene structure and function as well as the association of PTPN22 gene polymorphisms with SLE susceptibility

  6. Systems genetics of complex diseases using RNA-sequencing methods

    DEFF Research Database (Denmark)

    Mazzoni, Gianluca; Kogelman, Lisette; Suravajhala, Prashanth

    2015-01-01

    Next generation sequencing technologies have enabled the generation of huge quantities of biological data, and nowadays extensive datasets at different ‘omics levels have been generated. Systems genetics is a powerful approach that allows to integrate different ‘omics level and understand...... non-coding RNAs (ncRNAs). The integration of transcriptomics data with genomic data in a systems genetics context represents a valuable possibility to go deep into the causal and regulatory mechanisms that generate complex traits and diseases. However RNA-Seq data have to be treated carefully...... principally on merits and demerits of tools for post mapping quality control, normalization, differential expression analysis, gene network analysis, and integration of different omics data in order to generate a comprehensive guideline to systems genetics analysis using RNA-Seq data....

  7. Genetic Factors in Systemic Lupus Erythematosus: Contribution to Disease Phenotype

    Science.gov (United States)

    Ceccarelli, Fulvia; Perricone, Carlo; Borgiani, Paola; Ciccacci, Cinzia; Rufini, Sara; Cipriano, Enrica; Alessandri, Cristiano; Spinelli, Francesca Romana; Sili Scavalli, Antonio; Novelli, Giuseppe; Valesini, Guido; Conti, Fabrizio

    2015-01-01

    Genetic factors exert an important role in determining Systemic Lupus Erythematosus (SLE) susceptibility, interplaying with environmental factors. Several genetic studies in various SLE populations have identified numerous susceptibility loci. From a clinical point of view, SLE is characterized by a great heterogeneity in terms of clinical and laboratory manifestations. As widely demonstrated, specific laboratory features are associated with clinical disease subset, with different severity degree. Similarly, in the last years, an association between specific phenotypes and genetic variants has been identified, allowing the possibility to elucidate different mechanisms and pathways accountable for disease manifestations. However, except for Lupus Nephritis (LN), no studies have been designed to identify the genetic variants associated with the development of different phenotypes. In this review, we will report data currently known about this specific association. PMID:26798662

  8. Therapies in the Pipeline for Systemic Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Juan Felipe Betancur

    2016-07-01

    Full Text Available The current goals in the development of novel therapeutics of systemic autoimmune diseases are to develop agents more effective than conventional therapies as well as to reduce the risk of organ damage. To achieve this goal, large multicentre randomised controlled trials are needed to confirm the efficacy and safety of novel agents. Whether these novel modalities are synergistic to conventional drugs, the optimal dosages, and duration of treatment, need to be explored. As expected, the development of new molecules for the treatment of autoimmune diseases is constant, and there are different ongoing clinical trials. We review the different molecules in the pipeline, summarised in Tables 1, 2, and 3. We also show the successes, failures, and molecules that require more evidence.

  9. Linking Microbiota to Human Diseases: A Systems Biology Perspective.

    Science.gov (United States)

    Wu, Hao; Tremaroli, Valentina; Bäckhed, Fredrik

    2015-12-01

    The human gut microbiota encompasses a densely populated ecosystem that provides essential functions for host development, immune maturation, and metabolism. Alterations to the gut microbiota have been observed in numerous diseases, including human metabolic diseases such as obesity, type 2 diabetes (T2D), and irritable bowel syndrome, and some animal experiments have suggested causality. However, few studies have validated causality in humans and the underlying mechanisms remain largely to be elucidated. We discuss how systems biology approaches combined with new experimental technologies may disentangle some of the mechanistic details in the complex interactions of diet, microbiota, and host metabolism and may provide testable hypotheses for advancing our current understanding of human-microbiota interaction.

  10. The utility of information collected by occupational disease surveillance systems.

    Science.gov (United States)

    Money, A; Carder, M; Hussey, L; Agius, R M

    2015-11-01

    The Health and Occupation Research (THOR) network in the UK and the Republic of Ireland (ROI) is an integrated system of surveillance schemes collecting work-related ill-health (WRIH) data since 1989. In addition to providing information about disease incidence, trends in incidence and the identification of new hazards, THOR also operates an ad hoc data enquiry service enabling interested parties to request information about cases of WRIH reported to THOR. To examine requests for information made to a network of surveillance schemes for WRIH in the UK. Analysis via SPSS of data requests received by THOR between 2002 and 2014. A total of 631 requests were received by THOR between 2002 and 2014. Requests were predominantly submitted by participating THOR physicians (34%) and the main THOR funder-the UK Health & Safety Executive (HSE) (31%). The majority (67%) of requests were for information about work-related respiratory or skin disease with relatively few requests for other diagnoses, such as musculoskeletal or mental ill-health. Requests frequently related to a specific industry and/or occupation (42%) and/or a specific causal agent (58%). Data collected by occupational disease surveillance systems such as THOR are an extremely useful source of information, the use of which extends beyond informing government on disease incidence and trends in incidence. The data collected provide a framework that can assist a wide range of enquirers with clinical diagnoses, identification of suspected causative agents/exposures and to highlight growing risks in particular industrial and occupational sectors. © The Author 2015. Published by Oxford University Press on behalf of the Society of Occupational Medicine. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  11. Variants associated with Gaucher disease in multiple system atrophy

    Science.gov (United States)

    Mitsui, Jun; Matsukawa, Takashi; Sasaki, Hidenao; Yabe, Ichiro; Matsushima, Masaaki; Dürr, Alexandra; Brice, Alexis; Takashima, Hiroshi; Kikuchi, Akio; Aoki, Masashi; Ishiura, Hiroyuki; Yasuda, Tsutomu; Date, Hidetoshi; Ahsan, Budrul; Iwata, Atsushi; Goto, Jun; Ichikawa, Yaeko; Nakahara, Yasuo; Momose, Yoshio; Takahashi, Yuji; Hara, Kenju; Kakita, Akiyoshi; Yamada, Mitsunori; Takahashi, Hitoshi; Onodera, Osamu; Nishizawa, Masatoyo; Watanabe, Hirohisa; Ito, Mizuki; Sobue, Gen; Ishikawa, Kinya; Mizusawa, Hidehiro; Kanai, Kazuaki; Hattori, Takamichi; Kuwabara, Satoshi; Arai, Kimihito; Koyano, Shigeru; Kuroiwa, Yoshiyuki; Hasegawa, Kazuko; Yuasa, Tatsuhiko; Yasui, Kenichi; Nakashima, Kenji; Ito, Hijiri; Izumi, Yuishin; Kaji, Ryuji; Kato, Takeo; Kusunoki, Susumu; Osaki, Yasushi; Horiuchi, Masahiro; Kondo, Tomoyoshi; Murayama, Shigeo; Hattori, Nobutaka; Yamamoto, Mitsutoshi; Murata, Miho; Satake, Wataru; Toda, Tatsushi; Filla, Alessandro; Klockgether, Thomas; Wüllner, Ullrich; Nicholson, Garth; Gilman, Sid; Tanner, Caroline M; Kukull, Walter A; Stern, Mathew B; Lee, Virginia M-Y; Trojanowski, John Q; Masliah, Eliezer; Low, Phillip A; Sandroni, Paola; Ozelius, Laurie J; Foroud, Tatiana; Tsuji, Shoji

    2015-01-01

    Objective Glucocerebrosidase gene (GBA) variants that cause Gaucher disease are associated with Parkinson disease (PD) and dementia with Lewy bodies (DLB). To investigate the role of GBA variants in multiple system atrophy (MSA), we analyzed GBA variants in a large case–control series. Methods We sequenced coding regions and flanking splice sites of GBA in 969 MSA patients (574 Japanese, 223 European, and 172 North American) and 1509 control subjects (900 Japanese, 315 European, and 294 North American). We focused solely on Gaucher-disease-causing GBA variants. Results In the Japanese series, we found nine carriers among the MSA patients (1.65%) and eight carriers among the control subjects (0.89%). In the European series, we found three carriers among the MSA patients (1.35%) and two carriers among the control subjects (0.63%). In the North American series, we found five carriers among the MSA patients (2.91%) and one carrier among the control subjects (0.34%). Subjecting each series to a Mantel–Haenszel analysis yielded a pooled odds ratio (OR) of 2.44 (95% confidence interval [CI], 1.14–5.21) and a P-value of 0.029 without evidence of significant heterogeneity. Logistic regression analysis yielded similar results, with an adjusted OR of 2.43 (95% CI 1.15–5.37) and a P-value of 0.022. Subtype analysis showed that Gaucher-disease-causing GBA variants are significantly associated with MSA cerebellar subtype (MSA-C) patients (P = 7.3 × 10−3). Interpretation The findings indicate that, as in PD and DLB, Gaucher-disease-causing GBA variants are associated with MSA. PMID:25909086

  12. Immunoglobulin G4-Related Inflammatory Abdominal Aortic Aneurysm Associated With Myasthenia Gravis, With Contained Rupture.

    Science.gov (United States)

    Jun, Heungman; Jung, Cheol Woong

    2016-11-01

    Immunoglobulin (Ig) G4-related disease is reportedly among the various causes of inflammatory abdominal aortic aneurysm (IAAA). Many IgG4-related diseases are closely related to allergic constitution and autoimmune disease. We report a case of a 72-year-old man with IgG4-related IAAA associated with myasthenia gravis, with contained rupture.

  13. Computer-aided diagnosis expert system for cerebrovascular diseases.

    Science.gov (United States)

    Chen, Xu; Wang, Zhijun; Sy, Chrisopher; Liu, Xiaokun; Qian, Jinwu; Zheng, Jia; Dong, Zhiqiang; Cao, Limei; Geng, Xiang; Xu, Shuye; Liu, Xueyuan

    2014-05-01

    To establish an expert diagnosis system for cerebrovascular diseases (CVDs) and assess accuracy of the diagnosis system. An expert diagnosis system for CVDs was established and evaluated using actual clinical cases. An expert diagnosis system for CVDs was established and tested in 319 clinical patients. Diagnosis accordance was obtained in 307 patients (the diagnosis accordance rate was 96.2%). Involved were 223, 7, 23, 54 and 12 patients with cerebral thrombosis, cerebral embolism, transient ischemic attack, cerebral hemorrhage and subarachnoid hemorrhage, respectively; and diagnosis accordance was obtained in 219 (98.2%), 6 (85.7%), 23 (100%), 48 (88.9%) and 11 (91.7%), respectively. Overall, the case analysis results support and demonstrate the diagnostic reasoning accuracy of the expert diagnosis system for CVDs. With the expert diagnosis system, medical experts' diagnosis of CVDs can be effectively mimicked and auxiliary diagnosis of CVDs has been preliminarily realized, laying a foundation for increasing the diagnostic accuracy of clinical diagnoses as it pertains to CVDs.

  14. Systems Biology of Meridians, Acupoints, and Chinese Herbs in Disease

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    Li-Ling Lin

    2012-01-01

    Full Text Available Meridians, acupoints, and Chinese herbs are important components of traditional Chinese medicine (TCM. They have been used for disease treatment and prevention and as alternative and complementary therapies. Systems biology integrates omics data, such as transcriptional, proteomic, and metabolomics data, in order to obtain a more global and complete picture of biological activity. To further understand the existence and functions of the three components above, we reviewed relevant research in the systems biology literature and found many recent studies that indicate the value of acupuncture and Chinese herbs. Acupuncture is useful in pain moderation and relieves various symptoms arising from acute spinal cord injury and acute ischemic stroke. Moreover, Chinese herbal extracts have been linked to wound repair, the alleviation of postmenopausal osteoporosis severity, and anti-tumor effects, among others. Different acupoints, variations in treatment duration, and herbal extracts can be used to alleviate various symptoms and conditions and to regulate biological pathways by altering gene and protein expression. Our paper demonstrates how systems biology has helped to establish a platform for investigating the efficacy of TCM in treating different diseases and improving treatment strategies.

  15. [Systemic diseases--the significance of early diagnosis exemplified by systemic lupus erythematosus and Wegener's granulomatosis].

    Science.gov (United States)

    Türk, H

    1993-12-01

    New therapeutic modalities have shown remarkable advances in the fields of systemic lupus erythematosus and Wegener's granulomatosis. For an optimal clinical outcome therapy has to be started early and must be adapted to disease activity. Concerning these two points early diagnosis is essential. This aim can be reached by a detailed evaluation of the patient's history by focusing on early symptoms and on typical clinical constellations, taking into account that both diseases show a great variability and sometimes even an insidious course. Immunological tests, capillary microscopy, echocardiography and computed tomography can be very helpful in the diagnosis of these diseases.

  16. Systemic and Pulmonary Vascular Remodelling in Chronic Obstructive Pulmonary Disease.

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    Mariana Muñoz-Esquerre

    Full Text Available Chronic Obstructive Pulmonary Disease (COPD is associated with subclinical systemic atherosclerosis and pulmonary vascular remodelling characterized by intimal hyperplasia and luminal narrowing. We aimed to determine differences in the intimal thickening of systemic and pulmonary arteries in COPD subjects and smokers. Secondary aims include comparisons with a non-smokers group; determining the clinical variables associated with systemic and pulmonary intimal thickening, and the correlations between systemic and pulmonary remodelling changes.All consecutive subjects undergoing lung resection were included and divided into 3 groups: 1 COPD, 2 smokers, and 3 non-smokers. Sections of the 5th intercostal artery and muscular pulmonary arteries were measured by histo-morphometry. Four parameters of intimal thickening were evaluated: 1 percentage of intimal area (%IA, 2 percentage of luminal narrowing, 3 intimal thickness index, and 4 intima-to-media ratio.In the adjusted analysis, the systemic arteries of COPD subjects showed greater intimal thickening (%IA than those of smokers (15.6±1.5% vs. 14.2±1.6%, p = 0.038. In the pulmonary arteries, significant differences were observed for %IA between the 2 groups (37.3±2.2% vs. 29.3±2.3%, p = 0.016. Among clinical factors, metabolic syndrome, gender and COPD status were associated with the systemic intimal thickening, while only COPD status was associated with pulmonary intimal thickening. A correlation between the %IA of the systemic and pulmonary arteries was observed (Spearman's rho = 0.46, p = 0.008.Greater intimal thickening in systemic and pulmonary arteries is observed in COPD patients than in smokers. There is a correlation between systemic and pulmonary vascular remodelling in the overall population.

  17. Metabolite monitoring to guide thiopurine therapy in systemic autoimmune diseases.

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    Chapdelaine, Aurélie; Mansour, Anne-Marie; Troyanov, Yves; Williamson, David R; Doré, Maxime

    2017-01-27

    6-Thioguanine nucleotide (6-TGN) is the active metabolite of thiopurine drugs azathioprine and 6-mercaptopurine. 6-Methylmercaptopurine (6-MMP) is an inactive and potentially hepatotoxic metabolite. A subgroup of patients (shunters) preferentially produce 6-MMP instead of 6-TGN, therefore displaying thiopurine resistance and risk for hepatotoxicity. Outside inflammatory bowel disease literature, few data exist regarding individualized thiopurine therapy based on metabolite monitoring. This study sought to describe metabolite monitoring in patients receiving weight-based thiopurine for systemic autoimmune diseases. Patients were enrolled using a laboratory database, and data were retrospectively collected. The correlation between the highest thiopurine dose (mg/kg) and the 6-TGN concentration (pmol/8 × 10(8) erythrocytes) was estimated with Pearson's correlation coefficient. Seventy-one patients with various systemic autoimmune conditions were enrolled. The correlation between the thiopurine dose and the 6-TGN level was weak for the overall patient sample (r = 0.201, p = 0.092) and for the subgroup of non-shunters (r = 0.278, p = 0.053). Subjects with 6-MMP levels >5700 pmol/8 × 10(8) erythrocytes had more hepatic cytolysis compared to subjects with 6-MMP 5700. Eleven non-shunters had hepatotoxicity, one of which had 6-MMP >5700. Thiopurine metabolite monitoring shows wide variability in 6-TGN levels among patients treated with weight-based thiopurine for systemic autoimmune diseases. Thirty-one percent of the patients in our series fulfilled the shunter definition. Thiopurine metabolite monitoring and dose adjustment to improve maintenance of remission and avoid hepatotoxicity should be studied prospectively.

  18. Systemic diseases in patients with HTLV-1-associated uveitis.

    Science.gov (United States)

    Nakao, Kumiko; Abematsu, Noriko; Sakamoto, Taiji

    2017-07-08

    Human T-lymphotropic virus type 1 (HTLV-1) carriers may develop severe systemic diseases, such as adult T cell leukaemia (ATL) or HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). This study aims to investigate systemic diseases of HTLV-1 carriers who had developed HTLV-1-associated uveitis (HAU). We investigated the occurrence of systemic diseases in 200 patients with HAU by performing a retrospective investigation of their medical records and examining the results of a postal survey. The mean age of HAU onset was 49 years, and the total person-years from HAU onset was 1627. There were two cases of ATL. Of these, one was diagnosed with smouldering ATL at the time of HAU onset and the other developed acute-type ATL 4 years after HAU onset. There were 26 cases of HAM/TSP; of these, HAM/TSP occurred first in 13 cases and HAU occurred first in 11 cases. The interval between the onset of HAM/TSP and HAU ranged from 6 months to 6 years, with no significant difference observed based on whether HAM/TSP or HAU occurred first. Hyperthyroidism was noted in 45 cases and preceded onset in all cases. HAU onset occurred after starting thiamazole treatment, and in two cases HAU recurred each time thiamazole treatment was restarted. HTLV-1 carriers with HAU may develop HAM/TSP more frequently than general carriers. HTLV-1 carriers undergoing treatment for hyperthyroidism may be prone to developing HAU. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  19. C. elegans as a model system for Parkinson disease

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Parkinson disease( PD) is characterized by the selective loss of dopaminergic neurons in the substantia nigra.Although investigation in mammalian animal models of PD has enhanced our understanding of PD, the complexity of the mammalian nervous system and our inability to visualize DA neurons in vivo restricts the advances in elucidating the molecular mechanisms of PD. Conservation between C. elegans and mammals in genomic, biosynthetic and metabolic pathways as well as the advantages of observing DA neurons morphology in vivo and the ease of transgenic and genetic manipulation make C. elegans an excellent model organism for PD.

  20. Current approach for urinary system stone disease in pregnant women

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    Orcun Celik

    2016-01-01

    Full Text Available Urinary system stones can be classified according to size, location, X-ray characteristics, aetiology of formation, composition, and risk of recurrence. Especially urolithiasis during pregnancy is a diagnostic and therapeutic challenge. In most cases, it becomes symptomatic in the second or third trimester. Diagnostic options in pregnant women are limited due to the possible teratogenic, carcinogenic, and mutagenic risk of foetal radiation exposure. Clinical management of a pregnant urolithiasis patient is complex and demands close collaboration between patient, obstetrician and urologist. We would like to review current diagnosis and treatment modalities of stone disease of pregnant woman.