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Sample records for scleroderma localized

  1. Localized Scleroderma

    Science.gov (United States)

    ... How Is Localized Scleroderma Diagnosed? Doctors who are familiar with scleroderma, or who are experts at examining ... systemic treatment with a medication or other treatment interventions (for example, ultraviolet light), are reserved for more ...

  2. Localized scleroderma: imaging features

    International Nuclear Information System (INIS)

    Liu, P.; Uziel, Y.; Chuang, S.; Silverman, E.; Krafchik, B.; Laxer, R.

    1994-01-01

    Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with ''en coup de sabre'' facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients. (orig.)

  3. Localized scleroderma: imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Liu, P. (Dept. of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)); Uziel, Y. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada)); Chuang, S. (Dept. of Diagnostic Imaging, Hospital for Sick Children, Toronto, ON (Canada)); Silverman, E. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada)); Krafchik, B. (Div. of Dermatology, Dept. of Pediatrics, Hospital for Sick Children, Toronto, ON (Canada)); Laxer, R. (Div. of Rheumatology, Hospital for Sick Children, Toronto, ON (Canada))

    1994-06-01

    Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with ''en coup de sabre'' facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients. (orig.)

  4. Surgical Management of Localized Scleroderma.

    Science.gov (United States)

    Lee, Jae Hyun; Lim, Soo Yeon; Lee, Jang Hyun; Ahn, Hee Chang

    2017-09-01

    Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients.

  5. [Localized scleroderma (morphea)].

    Science.gov (United States)

    Bono, Waafa; Dupin, Nicolas

    2006-12-01

    DEFINITION AND FREQUENCY: Localized scleroderma, also known as morphea, is a sclerotic condition limited to the skin. The specific clinical entity depends on the extent, linear disposition and depth of the lesions. Morphea is ten times more prevalent than systemic sclerosis, and its prognosis is generally good: superficial forms resolve within 3 years. In the absence of symptoms, examinations to detect systemic involvement are purposeless. Plaque morphea is the most frequent clinical presentation. Serious manifestations include extensive morphea that may involve the entire skin or linear forms, especially in children, where they may be severe, especially on the face. There are no immunological markers clearly associated with morphea and no causative agents have been implicated in its pathogenesis, although sclerodermiform dermatitis is reported to be associated with some drugs and toxic agents. There is no consensual treatment for morphea. Treatment should be decided according to severity and extent of lesions. Limited lesions may be treated with local steroids such as class IV corticosteroids. Systemic treatment (methotrexate) should be discussed in extensive and linear forms when there is a risk of functional or esthetic complications.

  6. BILATERAL CHOROIDAL EXCAVATION IN JUVENILE LOCALIZED SCLERODERMA.

    Science.gov (United States)

    Franklin, Mackenzie L; Day, Shelley

    2018-01-01

    To describe a case of bilateral choroidal excavation in a patient with juvenile localized scleroderma. Case report. An asymptomatic 12-year-old boy with localized scleroderma presented for examination and was found to have bilateral areas of choroidal excavation temporal to the fovea. Previous reports of ocular complications of localized scleroderma have primarily described adnexal and anterior segment changes. This is the second report of choroidal changes in a patient with localized scleroderma, and the first in a pediatric patient.

  7. [Localized scleroderma (morphea) in childhood].

    Science.gov (United States)

    Weibel, L

    2012-02-01

    Localized scleroderma or morphea is a sclerosing connective tissue disease of the skin, which may affect underlying tissues such as subcutis, muscle and bone. Many patients show extracutaneous symptoms and antinuclear antibodies, however, secondary transformation into systemic sclerosis does not occur. Localized scleroderma usually begins in childhood with a wide variation in its clinical spectrum. The linear variant is the most common subtype in children, associated with a progressive course and increased risk of complications. The disease may progress over years and result in severe functional and cosmetic disability. The etiology of localized scleroderma remains unknown. A genetic background is suspected, while triggers such as trauma, vaccinations and infections may lead to secondary immunologic phenomena. Localized scleroderma often remains unrecognized for a long time, resulting in substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as first-line therapy for progressive (usually linear) disease, whereas phototherapy (UVA-1 or UVB-narrow band) is suitable for adolescents with superficial circumscribed subtypes.

  8. [Nodules on localized scleroderma or morphea].

    Science.gov (United States)

    Bayle, P; Bazex, J; Marguery, M-C; Lamant, L

    2005-02-01

    Localized scleroderma or morphea usually appears as flat or depressed lesions. We report 3 cases of morphea with atypical appearance, alternating pigmented and depigmented patches with nodules or sclerous bands, occurring in adult men. The occurrence of nodular elements on generalized or localized scleroderma, although rare, was first reported in the literature by Addisson in 1884. These nodules usually appear during evolution. These scleroderma are then described as being keloidal or nodular. We report 3 cases of nodules on localized scleroderma which appeared at the beginning of the dermatosis and where the scleroderma had a similar unusual irregularly pigmented appearance.

  9. Localized Scleroderma: A Clinical Review.

    Science.gov (United States)

    Tratenberg, Mark; Gutwein, Farrah; Rao, Varuni; Sperber, Kirk; Wasserrman, Amy; Ash, Julia

    2017-01-01

    Localized scleroderma (LS) is characterized by excessive collagen deposition leading to thickening of the dermis, subcutaneous tissue or both. The outcome for most patients with localized scleroderma is directly related to the type and stage of the affected tissue. The major challenge for untreated patients is not increased mortality risk, rather deformity and growth defects from skin, muscle and bone abnormalities. Treatment is individualized to type and stage of the lesion and may include pharmacologic and non-pharmacologic therapies. Among the pharmacologic modalities, methotrexate with systemic glucocorticoids is currently the mainstay of treatment. More controlled trials are needed to determine the length of treatment and the maintenance dose of this combination therapy. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  10. Localized scleroderma: clinical spectrum and therapeutic update.

    Science.gov (United States)

    Careta, Mariana Figueiroa; Romiti, Ricardo

    2015-01-01

    Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

  11. Localized scleroderma: clinical spectrum and therapeutic update*

    Science.gov (United States)

    Careta, Mariana Figueiroa; Romiti, Ricardo

    2015-01-01

    Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma. PMID:25672301

  12. Localized scleroderma and regional inflammatory myopathy.

    Science.gov (United States)

    Zivković, Saša A; Freiberg, William; Lacomis, David; Domsic, Robyn T; Medsger, Thomas A

    2014-05-01

    Inflammatory myopathy is rare in localized scleroderma. We report 2 new cases of regional inflammatory myopathy associated with localized scleroderma and review 10 reported cases of localized scleroderma associated with an inflammatory myopathy with regional muscle involvement, more often in the upper extremities. Serum creatine kinase was mildly elevated or normal. Histopathology often showed perimysial inflammation and plasma cell infiltration. These cases demonstrate that inflammatory myopathy should be considered in patients with localized scleroderma and regional muscle weakness, pain or atrophy. Muscle biopsy can confirm the diagnosis of myositis, which if identified, will require anti-inflammatory and/or immunosuppressive therapy. Published by Elsevier B.V.

  13. Localized scleroderma: a series of 52 patients.

    Science.gov (United States)

    Toledano, C; Rabhi, S; Kettaneh, A; Fabre, B; Fardet, L; Tiev, K P; Cabane, J

    2009-05-01

    Localized scleroderma also called morphea is a skin disorder of undetermined cause. The widely recognized Mayo Clinic Classification identifies 5 main morphea types: plaque, generalized, bullous, linear and deep. Whether each of these distinct types has a particular clinical course or is associated with some patient-related features is still unclear. We report here a retrospective series of patients with localized scleroderma with an attempt to identify features related to the type of lesion involved. The medical records of all patients with a diagnosis of localized scleroderma were reviewed by skilled practitioners. Lesions were classified according to the Mayo Clinic Classification. The relationship between each lesion type and various clinical features was tested by non-parametrical methods. The sample of 52 patients included 43 females and 9 males. Median age at onset was 30 y (range 1-76). Frequencies of patients according to morphea types were: plaque morphea 41 (78.8%) (including morphea en plaque 30 (57.7%) and atrophoderma of Pasini-Pierini 11 (21.1%)), linear scleroderma 14 (26.9%). Nine patients (17.3%) had both types of localized scleroderma. Median age at onset was lower in patients with linear scleroderma (8 y (range 3-44)) than in others (36 y (range 1-77)) (p=0.0003). Head involvement was more common in patients with linear scleroderma (37.5%) than in other subtypes (11.1%) (p=0.05). Atrophoderma of Pasini-Pierini was never located at the head. Systemic symptoms, antinuclear antibodies and the rheumatic factor were not associated with localized scleroderma types or subtypes. These results suggest that morphea types, in adults are not associated with distinct patient features except for age at disease onset (lower) and the localization on the head (more frequent), in patients with lesions of the linear type.

  14. Periostin in Mature Stage Localized Scleroderma.

    Science.gov (United States)

    Kim, Min-Woo; Park, Jung Tae; Kim, Jung Ho; Koh, Seong-Joon; Yoon, Hyun-Sun; Cho, Soyun; Park, Hyun-Sun

    2017-06-01

    Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare. To investigate the expression of periostin and other molecules in localized scleroderma. A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken. Fourteen age-matched and biopsy site-matched subjects with normal skin were included as controls. Collagen fiber deposition, periostin, procollagen, transforming growth factor-β, and matrix metalloproteinase (MMP)-1 expression were assessed and compared between the two groups. Co-localization of α-smooth muscle actin and periostin was evaluated using confocal microscopy. Periostin was predominantly expressed along the dermo-epidermal junction in the controls. Conversely, patients with localized scleroderma demonstrated increased collagen fiber deposition and periostin expression that was more widely distributed along the entire dermis. MMP-1 staining showed increased expression in the epidermis and dermis of patients compared to scanty expression in the controls. A semi-quantitative evaluation showed a higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, p =0.013), diffuse periostin positivity (42.9% vs. 0%, p =0.016), and moderate MMP-1 positivity (71.4% vs. 7.1%, p =0.001) in patients than in the controls. Compared to the controls, patients with localized scleroderma had enhanced periostin expression corresponding to increased collagen fiber deposition and unexpected overexpression of MMP-1. The results of this human in vivo study may implicate the pathogenesis of localized scleroderma.

  15. Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk

    DEFF Research Database (Denmark)

    Hesselvig, Jeanette Halskou; Kofoed, Kristian; Wu, Jashin J

    2018-01-01

    Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18...... and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint. A total of 697 patients with localized scleroderma and 1......,962 patients with systemic sclerosis were identified and compared with 5,428,380 people in the reference population. In systemic sclerosis, the adjusted HR was 2.22 (95% confidence interval 1.99-2.48). No association was seen between patients with localized scleroderma and cardiovascular disease. In conclusion...

  16. Localized scleroderma of the breast

    International Nuclear Information System (INIS)

    Opere, Elisa; Oleaga, Laura; Ibanez, Teresa; Grande, Domingo

    2002-01-01

    We report a 44-year-old patient with right-breast morphea. Mammography, MRI and needle biopsy were used for assessment of the case. Mammography demonstrated thickening of the skin and the subcutaneous tissue. The MRI showed replacement of the subcutaneous and breast fat by a low signal intensity, non-enhancing tissue. Skin biopsy confirmed the histological features of scleroderma. (orig.)

  17. Localized scleroderma of the breast

    Energy Technology Data Exchange (ETDEWEB)

    Opere, Elisa; Oleaga, Laura; Ibanez, Teresa; Grande, Domingo [Department of Radiology, Hospital de Basurto, Bilbao (Spain)

    2002-06-01

    We report a 44-year-old patient with right-breast morphea. Mammography, MRI and needle biopsy were used for assessment of the case. Mammography demonstrated thickening of the skin and the subcutaneous tissue. The MRI showed replacement of the subcutaneous and breast fat by a low signal intensity, non-enhancing tissue. Skin biopsy confirmed the histological features of scleroderma. (orig.)

  18. Musculoskeletal MRI findings of juvenile localized scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Eutsler, Eric P. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Wilmington, DE (United States); Washington University School of Medicine, Mallinckrodt Institute of Radiology, St. Louis, MO (United States); Horton, Daniel B. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Division of Rheumatology, Department of Pediatrics, Wilmington, DE (United States); Rutgers Robert Wood Johnson Medical School, Department of Pediatrics, New Brunswick, NJ (United States); Epelman, Monica [Nemours Children' s Health System/Nemours Children' s Hospital, Department of Medical Imaging, Orlando, FL (United States); Finkel, Terri [Nemours Children' s Health System/Nemours Children' s Hospital, Department of Pediatrics, Orlando, FL (United States); Averill, Lauren W. [Nemours Children' s Health System/Alfred I. duPont Hospital for Children, Wilmington, DE (United States)

    2017-04-15

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol. (orig.)

  19. Musculoskeletal MRI findings of juvenile localized scleroderma

    International Nuclear Information System (INIS)

    Eutsler, Eric P.; Horton, Daniel B.; Epelman, Monica; Finkel, Terri; Averill, Lauren W.

    2017-01-01

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol. (orig.)

  20. Musculoskeletal MRI findings of juvenile localized scleroderma.

    Science.gov (United States)

    Eutsler, Eric P; Horton, Daniel B; Epelman, Monica; Finkel, Terri; Averill, Lauren W

    2017-04-01

    Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings. Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings). Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful. Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol.

  1. Pediatric scleroderma: systemic or localized forms.

    Science.gov (United States)

    Torok, Kathryn S

    2012-04-01

    Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of patients showing extracutaneous disease manifestations such as arthritis and uveitis. Vascular, cutaneous, gastrointestinal, pulmonary, and musculoskeletal involvement are most commonly seen in children with SSc. Treatment of both forms targets the active inflammatory stage and halts disease progression; however, progress needs to be made toward the development of more effective antifibrotic therapy to help reverse disease damage. Copyright © 2012 Elsevier Inc. All rights reserved.

  2. Pathogenesis and treatment modalities of localized scleroderma.

    Science.gov (United States)

    Valančienė, Greta; Jasaitienė, Daiva; Valiukevičienė, Skaidra

    2010-01-01

    Localized scleroderma is a chronic inflammatory disease primarily of the dermis and subcutaneous fat that ultimately leads to a scar-like sclerosis of connective tissue. The disorder manifests as various plaques of different shape and size with signs of skin inflammation, sclerosis, and atrophy. This is a relatively rare inflammatory disease characterized by a chronic course, unknown etiology, and insufficiently clear pathogenesis. Many factors may influence its appearance: trauma, genetic factors, disorders of the immune system or hormone metabolism, viral infections, toxic substances or pharmaceutical agents, neurogenic factors, and Borrelia burgdorferi infection. Various therapeutic modalities are being used for the treatment of localized scleroderma. There is no precise treatment scheme for this disease. A majority of patients can be successfully treated with topical pharmaceutical agents and phototherapy, but some of them with progressive, disseminated, and causing disability localized scleroderma are in need of systemic treatment. The aim of this article is not only to dispute about the clinical and morphological characteristics of localized scleroderma, but also to present the newest generalized data about the possible origin, pathogenesis, and treatment modalities of this disease.

  3. Morphea and other localized forms of scleroderma.

    Science.gov (United States)

    Vasquez, Rebecca; Sendejo, Chelsea; Jacobe, Heidi

    2012-11-01

    Morphea, also known as localized scleroderma, is a disorder of excessive collagen deposition leading to thickening of the dermis and/or subcutaneous tissues and may cause significant morbidity. This review will describe new developments in the evaluation and management of morphea as well as its pathophysiology. The reader will be able to apply these findings to patient management. The recent development of validated outcome measures (i.e. the localized scleroderma cutaneous assessment tool) as well as consensus treatment recommendations provide a platform for collaboration among specialties to develop both standardized assessment tools and therapeutic trials. New studies have also begun to investigate the immunological underpinnings of morphea. The promise of evidence-based treatments for morphea in the near future will provide better care for patients with morphea and understanding its pathophysiology will lay groundwork for the development of new treatments.

  4. Case for diagnosis. Localized scleroderma or morphea.

    Science.gov (United States)

    Tomiyoshi, Carolina; Wojcik, Adma Silva de Lima; Vencato, Elisa Milani Oba; Taques, Guilherme Ribas; Fillus Neto, José; Brenner, Fabiane Andrade Mulinari

    2010-01-01

    Localized scleroderma or morphea is a chronic disease of the connective tissue. Its etiology may be autoimmune and the condition results from a disturbance in collagen synthesis and deposition, clinically represented by sclerotic skin lesions. Some plaques may be yellowish, which can be misleading at diagnosis. This article reports the case of an adolescent girl who concomitantly presented erythematous lesions and yellowish lesions, both of which constitute clinical manifestations of the disease.

  5. Systemic involvement in localized scleroderma/morphea.

    Science.gov (United States)

    Gorkiewicz-Petkow, Anna; Kalinska-Bienias, Agnieszka

    2015-01-01

    Localized scleroderma (LoSc), also known as morphea, is a rare fibrosing disorder of the skin and underlying tissues. Sclerosis is mainly limited to the skin, but subcutaneous tissue, fascia, and underlying muscles and bone may also be involved. In some cases, systemic manifestation with visceral abnormalities may occur. Several publications have focused on significant aspects of LoSc: genetics, immunity, epidemiology, scoring systems, and unification of classifications. Clinical studies featuring large cohorts with the disease published by various international study groups have been of great value in furthering the diagnostic and therapeutic management of LoSc. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Scleroderma

    Science.gov (United States)

    Scleroderma means hard skin. It is a group of diseases that cause abnormal growth of connective tissue. ... their shape and helps keep them strong. In scleroderma, the tissue gets hard or thick. It can ...

  7. Oxidative stress parameters in localized scleroderma patients.

    Science.gov (United States)

    Kilinc, F; Sener, S; Akbaş, A; Metin, A; Kirbaş, S; Neselioglu, S; Erel, O

    2016-11-01

    Localized scleroderma (LS) (morphea) is a chronic, inflammatory skin disease with unknown cause that progresses with sclerosis in the skin and/or subcutaneous tissues. Its pathogenesis is not completely understood. Oxidative stress is suggested to have a role in the pathogenesis of localized scleroderma. We have aimed to determine the relationship of morphea lesions with oxidative stress. The total oxidant capacity (TOC), total antioxidant capacity (TAC), paroxonase (PON) and arylesterase (ARES) activity parameters of PON 1 enzyme levels in the serum were investigated in 13 LS patients (generalized and plaque type) and 13 healthy controls. TOC values of the patient group were found higher than the TOC values of the control group (p < 0.01). ARES values of the patient group was found to be higher than the control group (p < 0.0001). OSI was significantly higher in the patient group when compared to the control (p < 0.005). Oxidative stress seems to be effective in the pathogenesis. ARES levels have increased in morphea patients regarding to the oxidative stress and its reduction. Further controlled studies are required in wider series.

  8. [Localized scleroderma: a retrospective study about 92 cases].

    Science.gov (United States)

    El Fékih, Nadia; Réjaibi, Iménc; Kamoun, Hajer; Zéglaoui, Faten; Fazaa, Bécima; Kharfi, Monia; Kamoun, Mohamed Ridha

    2009-09-01

    Sclerodermas are rare affections which can be located or generalized. Localized form is the most frequent. The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature. We have performed a retrospective study on all patients followed in the department of dermatology of the Hospital Charles Nicole during 14 years period. Our study was about 92 cases of localized scleroderma (73 were females and 19 males). The mean age was 35 years (between 2 and 72 years). The majority of localised sclerodermas (66.2% of the cases) appeared before 40 years with a maximum of frequency between 10 and 30 years (41.6%). Only 11.9% of the cases were observed before 10 years. They were 51 cases (55%) of morphea, 35 cases (38%) of scleroderma in bands including 32 linear scleroderma and 3 scleroderma en coup de sabre, 5 cases (5.5%) of generalized morphea and 1 case (0.15%) of deep morphea. Average therapeutic was specified among 63 patients (87%), and the evolution could be appreciated among 45 patients. The epidemiologic data observed in our series are comparable with those reported in the literature. Therapeutic difficulties and risks of functional after-effects, particular in scleroderma in bands, remain the principal concern for all the authors.

  9. Presentations and treatment of childhood scleroderma: localized scleroderma, eosinophilic fasciitis, systemic sclerosis, and graft-versus-host disease.

    Science.gov (United States)

    Hedrich, Christian Michael; Fiebig, Barbara; Hahn, Gabriele; Suttorp, Meinolf; Gahr, Manfred

    2011-07-01

    Juvenile scleroderma is a rare connective tissue disease that involves the skin and subcutaneous tissue. Among all presentations of juvenile scleroderma, localized scleroderma (JLSc) is the most frequent, followed by systemic disease (JSSc) and eosinophilic fasciitis (EF). In posttransplantation chronic graft-versus-host disease (GvHD), scleroderma-like skin involvement can occur. Systemic forms of juvenile scleroderma and GvHD can affect the internal organs, such as the lungs, the gastrointestinal tract, the heart, and kidneys and cause disability and severe, sometimes lethal, complications. Here, the authors give an overview of different presentations of juvenile scleroderma. They report their experience with the different forms and presentations of scleroderma, diagnostic workups, treatment, and outcome of all forms of childhood scleroderma in the context of the existing literature.

  10. [Clinical characteristics of patients with juvenile localized scleroderma].

    Science.gov (United States)

    Sun, Qiu-Ning; Du, Wei; Hu, Bin; Liu, Pai; Yuan, Xie

    2009-02-01

    To investigate the clinical characteristics of juvenile localized scleroderma (JLS). The clinical data of 100 outpatients with JLS who were admitted to PUMC Hospital from 2000 to 2008 were retrospectively analyzed. Of a total of 100 cases, 51 (51%) were confirmed as linear scleroderma, 26 (26%) as plaque morphea, 26 (26%) as deep morphea, 12 (12%) as generalized morphea, and 15 (15%) as a mixed subtype. Nine patients (9%) had family histories of rheumatic or autoimmune diseases, while 16 (16%) might be triggered by unknown factors. Totally 84 patients underwent antinuclear antibody tests and 38 patients (45.2%) had positive results. Linear scleroderma are the most frequent subtype of JLS. Localized scleroderma may be associated with some autoimmune-related causes.

  11. Predictors of Longitudinal Quality of Life in Juvenile Localized Scleroderma.

    Science.gov (United States)

    Ardalan, Kaveh; Zigler, Christina K; Torok, Kathryn S

    2017-07-01

    Localized scleroderma can negatively affect children's quality of life (QoL), but predictors of impact have not been well described. We sought to identify predictors of QoL impact in juvenile localized scleroderma patients. We analyzed longitudinal data from a single-center cohort of juvenile localized scleroderma patients, using hierarchical generalized linear modeling (HGLM) to identify predictors of QoL impact. HGLM is useful for nested data and allows for evaluation of both time-variant and time-invariant predictors. The number of extracutaneous manifestations (ECMs; e.g., joint contracture and hemifacial atrophy) and female sex predicted negative QoL impact, defined as a Children's Dermatology Life Quality Index score >1 (P = 0.019 for ECMs and P = 0.002 for female sex). As the time since the initial visit increased, the odds of reporting a negative QoL impact decreased (P scleroderma than cutaneous features. Further study is required to determine which ECMs have the most impact on QoL, which factors underlie sex differences in QoL in localized scleroderma, and why increasing the time since the initial visit appears to be protective. An improved understanding of predictors of QoL impact may allow for the identification of patients at risk of poorer outcomes and for the tailoring of treatment and psychosocial support. © 2016, American College of Rheumatology.

  12. German guidelines for the diagnosis and therapy of localized scleroderma.

    Science.gov (United States)

    Kreuter, Alexander; Krieg, Thomas; Worm, Margitta; Wenzel, Jörg; Moinzadeh, Pia; Kuhn, Annegret; Aberer, Elisabeth; Scharffetter-Kochanek, Karin; Horneff, Gerd; Reil, Emma; Weberschock, Tobias; Hunzelmann, Nicolas

    2016-02-01

    Localized scleroderma designates a heterogeneous group of sclerotic skin disorders. Depending on the subtype, severity, and site affected, adjacent structures such as adipose tissue, muscles, joints, and bones may be involved. This is an update of the existing German AWMF (Association of the Scientific Medical Societies in Germany) guidelines (classification: S2k). These guidelines provide an overview of the definition, epidemiology, classification, pathogenesis, laboratory workup, histopathology, clinical scoring systems, as well as imaging and device-based workup of localized scleroderma. Moreover, consensus-based recommendations are given on the management of localized scleroderma depending on its clinical subtype. Treatment recommendations are presented in a therapeutic algorithm. No financial support was given by any pharmaceutical company. The guidelines are valid until July 2019. © 2016 The Authors | Journal compilation © Blackwell Verlag GmbH, Berlin.

  13. Evaluation of mean platelet volume in localized scleroderma.

    Science.gov (United States)

    Bahali, Anil Gulsel; Su, Ozlem; Emiroglu, Nazan; Cengiz, Fatma Pelin; Kaya, Mehmet Onur; Onsun, Nahide

    2017-01-01

    Localized scleroderma is a chronic inflammatory skin disease characterized by sclerosis of the dermis and subcutaneous tissue. Platelets play an important role in inflammation. Following activation, platelets rapidly release numerous mediators and cytokines, which contribute to inflammation. To evaluate whether there was any relation between localized scleroderma and platelet parameters. Forty-one patients with localized scleroderma were enrolled in the study. The control group consisted of 30 healthy subjects. The mean platelet volume level in the patient group was 9.9 ± 1.3 fl and in the control group was 7.6 ± 1.1 fl. This difference was statistically significant (pscleroderma. Platelet parameters may be used as markers for evaluating disease severity and inflammatory processes. Thus, there is a need for more detailed and prospective studies.

  14. Parry-Romberg Syndrome Associated with Localized Scleroderma

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    Jelena Maletic

    2010-06-01

    Full Text Available Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea.

  15. Parry-Romberg Syndrome Associated with Localized Scleroderma

    Science.gov (United States)

    Maletic, Jelena; Tsirka, Vassiliki; Ioannides, Panos; Karacostas, Dimitrios; Taskos, Nikolaos

    2010-01-01

    Parry-Romberg syndrome is a rare neurocutaneous disorder of unknown origin. It is characterized by progressive facial hemiatrophy and frequently overlaps with a condition known as linear scleroderma ‘en coup de sabre’. Neurological involvement is frequently described in these patients, including migraine, facial pain and epilepsy, which represent the commonest neurological conditions, sometimes associated with brain abnormalities ipsilaterally to the skin lesions. We present a case of Parry-Romberg syndrome with neurological involvement in a patient with diagnosed localized scleroderma (morphea). PMID:20671858

  16. A Patient with Localized Scleroderma Successfully Treated with Etretinate

    Science.gov (United States)

    Shima, Tomoko; Yamamoto, Yuki; Ikeda, Takaharu; Furukawa, Fukumi

    2014-01-01

    There are several treatment methods for localized scleroderma, but treatment is difficult when the lesion is widely distributed. We encountered a case who was treated successfully with etretinate, a vitamin A derivative. The usefulness of this agent is discussed. PMID:25408646

  17. A Patient with Localized Scleroderma Successfully Treated with Etretinate

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    Tomoko Shima

    2014-09-01

    Full Text Available There are several treatment methods for localized scleroderma, but treatment is difficult when the lesion is widely distributed. We encountered a case who was treated successfully with etretinate, a vitamin A derivative. The usefulness of this agent is discussed.

  18. Localized severe scleroderma: a retrospective study of 26 pediatric patients.

    Science.gov (United States)

    Beltramelli, Matilde; Vercellesi, Paolo; Frasin, Adina; Gelmetti, Carlo; Corona, Fabrizia

    2010-01-01

    Juvenile localized scleroderma includes different conditions characterized by skin hardening with increased collagen deposition. Although juvenile localized scleroderma is considered a relatively benign disease, lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Furthermore, extracutaneous manifestations are described. We retrospectively analyzed a cohort of 26 patients with severe Juvenile localized scleroderma with particular attention to clinical features, therapy, and long-term outcome. A subgroup of three patients has been further evaluated with infrared thermography. Our findings were consistent with the current literature for demographic, laboratory, and clinical characteristics at disease onset, but, with our patients, the prevalence of extracutaneous manifestations was higher, thus confirming the potential for severe juvenile localized scleroderma to affect organs other than the skin, without increased risk of development toward systemic sclerosis. Correlation between various treatments and clinical endpoint showed that systemic therapy lead to a better outcome: in particular, methotrexate appeared the most effective drug, capable in halting the progression of the disease and sometimes inducing its regression. © 2010 Wiley Periodicals, Inc.

  19. Clinicopathological study of 81 cases of localized and systemic scleroderma.

    Science.gov (United States)

    Succaria, F; Kurban, M; Kibbi, A-G; Abbas, O

    2013-02-01

    Scleroderma is a connective tissue disease that includes localized and systemic forms. Our recent encounter with a morphea case exhibiting prominent perineural inflammation microscopically prompted us to assess the features of all patients diagnosed with morphea/scleroderma at our institution. To describe the clinicopathological features of all patients diagnosed with morphea/scleroderma at American University of Beirut Medical Center (AUB-MC) between 1999 and 2010, and compare our findings with those published in the literature. A total of 81 cases (63 women and 18 men) were identified, of which 73 were localized (morphea) and eight were systemic scleroderma. Clinically, plaque type morphea was the most common variant both in adults and children, and seven (9%) cases of morphea were associated with lichen sclerosis et atrophicus (LSA). Histopathologically, perineural inflammation was observed in 49% of cases, and may serve, in addition to other features including lichen sclerosis-like changes (observed in exclusively nine cases of morphea), more diffuse dermal and less subcutaneous sclerosis, and intense inflammation, as clues favouring diagnosis of morphea over systemic sclerosis. The features of morphea/scleroderma patients in this study are generally comparable to those published in the literature, with few differences. Clinically, plaque type morphea was the most common variant both in adults and children and LSA was a frequent association. Histopathologically, perineural inflammation was commonly observed and may serve in addition to lichen sclerosis-like changes and intense inflammation as clues favouring diagnosis of morphea over systemic sclerosis. © 2012 The Authors. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology.

  20. Update on the classification and treatment of localized scleroderma.

    Science.gov (United States)

    Bielsa Marsol, I

    2013-10-01

    Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach. Copyright © 2011 Elsevier España, S.L. and AEDV. All rights reserved.

  1. A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide.

    Science.gov (United States)

    Ferguson, Ian D; Weiser, Peter; Torok, Kathryn S

    2015-01-01

    Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments. Infliximab is a potential treatment option for pediatric localized scleroderma patients who have progression of disease or who are unable to tolerate the side effect profile of more standard systemic therapy. Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

  2. Pediatric Scleroderma –Systemic and Localized Forms

    Science.gov (United States)

    Torok, Kathryn S.

    2012-01-01

    Synopsis statement Pediatric scleroderma includes two major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, with an initial inflammatory phase associated with endothelial activation, and a later fibrotic phase evidenced by collagenization of tissue and appreciable skin thickness, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, and though not fatal like SSc, up to a quarter of the patients may have extracutaneous disease manifestations, such as arthritis and uveitis. While any organ may be affected in SSc, vascular (Raynaud’s phenomenon), cutaneous (skin thickening), GI, pulmonary and musculoskeletal involvement are most commonly seen in children. Auto-antibody profiles in childhood onset SSc can assist in predicting internal organ involvement. Treatment for both forms of scleroderma targets the active inflammatory stage and halts disease progression; however, progress still needs to be made towards the development of a more effective anti-fibrotic therapy to help reverse disease damage. PMID:22560576

  3. Cytokine profiles in localized scleroderma and relationship to clinical features.

    Science.gov (United States)

    Kurzinski, Katherine; Torok, Kathryn S

    2011-08-01

    Localized scleroderma (LS) is a disfiguring autoimmune disease of the skin and underlying tissue that mainly affects the pediatric population. Inflammation of the tissue leads to fibrosis and atrophy, causing physical and psychological disability that can continue throughout childhood into adulthood. Available therapies for LS have had variable effects and are associated with morbidity themselves. A better understanding of the pathophysiology of LS, especially during the active inflammatory phase, would lead to more directed and efficacious therapies. As in systemic sclerosis (SSc), the other form of scleroderma, T-helper (Th) cells and their associated cytokines have been suggested to contribute significantly to the pathophysiology of LS supported by the presence of cytokines from these lineages in the sera and tissue of LS patients. It is postulated that the imbalance between Th1/Th2/Th17 cell subsets drives inflammation in the early stages of disease (Th1 and Th17 predominant) and fibrosis in the later stages of scleroderma (Th2 predominant). We review the available experimental data regarding cytokines in LS and compare them to available clinical disease severity and activity features. This provides the platform to launch further investigations into the role of select cytokines in the pathogenesis of LS and to provide directed therapeutic options in the future. Published by Elsevier Ltd.

  4. Treatment of morphea or localized scleroderma: review of the literature.

    Science.gov (United States)

    Vilela, Fernanda Aguiar Santos; Carneiro, Sueli; Ramos-e-Silva, Marcia

    2010-10-01

    Scleroderma is a disease that affects the microvasculature and the connective tissue. These alterations produce fibrosis and blood vessel occlusion. Its cause is still unknown, although the exaggerated synthesis of collagens I and IV, detected in skin and vessels, may be related to genetic, immunologic and, less frequently, exogenous factors as inhalation of silica and polyvinyl chloride. There is a localized and a systemic form, which affects both adults and children. The treatment of the localized form, also called morphea, is still controversial, and, in this article, the authors will discuss the main agents that were found to improve the lesions and symptoms.

  5. Localized scleroderma and its implications on quality of life

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    Nathalia Mebius

    2014-12-01

    Full Text Available Scleroderma is a disease of unknown etiology, which affects the connective tissue, is characterized histologically by a change in collagen synthesis with increased deposition, and dermal infiltration and thickening of the skin, asymmetric distribution without involvement of internal organs in this way (localized of the disease, but can lead, in some cases, important aesthetic and functional impairment of the patient. In our case, it is believed that the disease has already developed completely and now there is an overlap disease of old age, which makes their daily activities and independence.

  6. New developments in juvenile systemic and localized scleroderma.

    Science.gov (United States)

    Foeldvari, Ivan

    2013-11-01

    Juvenile localized scleroderma (jLS) and juvenile systemic sclerosis (jSS) are both orphan diseases, with jLS around 10 times more frequent than jSS. In recent years the time gap between the appearance of symptoms and diagnosis has become significantly shorter. This review focuses on the new classifications of jSS and jLS, and on the developments and adaptations of the outcome measures for certain organ involvements whereby progress has been made regarding pediatric patients. Copyright © 2013 Elsevier Inc. All rights reserved.

  7. Localized scleroderma en coup de sabre in the Neurology Clinic.

    Science.gov (United States)

    Pinho, João; Rocha, João; Sousa, Filipa; Macedo, Cristiana; Soares-Fernandes, João; Cerqueira, João; Maré, Ricardo; Lourenço, Esmeralda; Pereira, João

    2016-07-01

    Localized scleroderma en coup de sabre (LScs) is a form of localized scleroderma thought to be an autoimmune disorder. Central nervous system involvement is not rare and neurological manifestations include seizures, focal neurological deficits, headache and neuropsychiatric changes. Patients attending the Neurology Clinic with the final diagnosis of LScs with neurological manifestations were identified and clinical and imagiological records reviewed. Five patients (0.024%) had LScs with neurological involvement, presenting with transient focal neurologic deficits, seizures, headache or migraine with aura. Neuroimaging studies confirmed localized skin depression and showed bone thinning, white matter lesions, brain calcifications, sulcal effacement and meningeal enhancement. Three patients experienced clinical improvement after immunosuppressive therapy, and in two of these patients neuroimaging findings also improved. Recognizing typical dermatologic changes is keystone for the diagnosis of LScs with neurological involvement. It is a diagnosis of exclusion and extensive etiological diagnostic evaluation should be performed. Treatment options, including conservative follow-up or immunosuppressive therapy, should be carefully considered. Copyright © 2016 Elsevier B.V. All rights reserved.

  8. Evaluation of heart rhythm variability and arrhythmia in children with systemic and localized scleroderma.

    Science.gov (United States)

    Wozniak, Jacek; Dabrowski, Rafal; Luczak, Dariusz; Kwiatkowska, Malgorzata; Musiej-Nowakowska, Elzbieta; Kowalik, Ilona; Szwed, Hanna

    2009-01-01

    To evaluate possible disturbances in autonomic regulation and cardiac arrhythmias in children with localized and systemic scleroderma. There were 40 children included in the study: 20 with systemic and 20 with localized scleroderma. The control group comprised 20 healthy children. In 24-hour Holter recording, the average rate of sinus rhythm was significantly higher in the groups with systemic and localized scleroderma than in the control group, but there was no significant difference between them. The variability of heart rhythm in both groups was significantly decreased. In the group with systemic scleroderma, single supraventricular ectopic beats were observed in 20% and runs were seen in 40% of patients. In the group with localized scleroderma, supraventricular single ectopic beats occurred in 35% of patients and runs in 45% of those studied. Ventricular arrhythmia occurred in 2 children with systemic scleroderma, but in 1 child, it was complex. The most frequent cardiac arrhythmias in both types of scleroderma in children were of supraventricular origin, whereas ventricular arrhythmias did not occur very often. There were no significant differences in autonomic disturbances manifesting as a higher heart rate and decreased heart rate variability between localized and systemic scleroderma.

  9. Measurement of transepidermal water loss in localized scleroderma.

    Science.gov (United States)

    Ďurčanská, Veronika; Jedličková, Hana; Vašků, Vladimír

    2016-05-01

    Localized scleroderma (LS) is a disease characterized by fibrotic changes in the dermis. Connective tissue growth factor and transforming growth factor β2 are the main mediators of fibrogenesis; this, along with excessive connective tissue production, affects epidermal keratinocytes, and thereby contributes to the changed quality of skin barrier. The objective of this article was to study the objective measurement of the skin barrier quality in LS with transepidermal water loss (TEWL) meter. The measurements of TEWL were performed on LS plaques in all three stages of various body locations. Control measurements were made on the contralateral side of healthy skin. The difference between TEWL in LS area and the contralateral side of the healthy skin was evaluated. A higher average TEWL 7.86 g/m(2) /h (SD 5.29) was observed on LS plaques compared with the control measurements on healthy skin 6.39 g/m(2) /h (SD 2.77). TEWL average values decreased from the inflammatory stage, through the sclerotic and to the atrophic stage. The mean difference 1.301 g/m(2) /h (SD 5.16) was found between TEWL on LS plaques and on the contralateral healthy skin in 82 measurements, i.e., a higher TEWL was observed in LS. The difference was statistically significant with p = 0.0250. Although fibrogenesis in scleroderma is localized in dermis, the skin barrier changes can be detected. © 2016 Wiley Periodicals, Inc.

  10. Prevalence of localized scleroderma in the Krasnodar territory

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    M. M. Tlish

    2015-01-01

    Full Text Available The authors conducted a study of particular features of the course of localized scleroderma in the south of Russia (Krasnodar territory. 65 case histories were analyzed. Women suffered from the disease 2.8 times more often than men; one half of the sample (55.4% included patients aged 35-55; the disease duration of over a year prevailed (69.8%; the disease occurred against the background of an endocrine pathology in one third of all cases; one third of all women developed the disease during the postmenopausal period. Plaque scleroderma (56.9%, white spot disease (von Zumbusch psoriasis (16.9% and idiopathic atrophoderma of Pasini and Pierini (12.3% belonged to the key clinical forms. The average number of lesions was 2.4 ± 1.2 while almost a half of the patients (47.7% had three or more lesions. The clinical manifestations localized mostly on the back (46.5% and stomach (34.9%.

  11. A Case of Localized Scleroderma in a Sculptor and His Wife

    Science.gov (United States)

    Bakst, Richard; Kovarik, Carrie; Werth, Victoria P.

    2011-01-01

    SUMMARY The etiology of localized scleroderma is unknown, and its pathogenetic relationship to its systemic counterpart is unclear. Environmental exposures, notably to silica dust, have long been suspected in the pathogenesis of the disorder. However, its’ relationship to the localized variant has not been well described. Here we present two cases of localized scleroderma in a sculptor and his wife who have extensive exposure to silica dust. PMID:19955998

  12. From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution.

    Science.gov (United States)

    Dilia, Giuggioli; Michele, Colaci; Emanuele, Cocchiara; Amelia, Spinella; Federica, Lumetti; Clodoveo, Ferri

    2018-01-01

    Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients. We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Eight (2.4%) female patients presented both the two diagnoses in their clinical histories. Six developed LoS prior to SSc; in 4/6 cases, the presence of autoantibodies was observed before SSc diagnosis. Overall, the median time interval between LoS and SSc diagnosis was 18 (range 0-156) months. LoS and SSc are two distinct clinical entities that may coexist. Moreover, as anecdotally reported in pediatric populations, we suggested the possible development of SSc in adult patients with LoS, particularly in presence of Raynaud's phenomenon or antinuclear antibodies before the SSc onset.

  13. Choroidal sclerosis in localized scleroderma (morphea en plaque).

    Science.gov (United States)

    Milenkovic, S; Petrovic, L; Risimic, D; Kosanovic-Jakovic, N; Jaksic, V; Djakovic, Z; Stojkovic, M; Risovic, D; Ivankovic, Lj; Ivancevic-Milenkovic, M

    2008-01-01

    Plaque morphea is a superficial type of morphea (localized scleroderma) which is characterized by various fibrotic areas of the dermis without systemic features. We present a 63-year-old man with morphea en plaque. The skin on his forearms and feet was taut, thickened and hidebound with scattered telangiectatic changes. Autoantibody profile was obtained and only ANA were positive (1:80). The patient had a decreased vision in the only functional, left eye. Our case is specific because the patient negated any kind of health problem, meaning the morphea and visual deterioration were of outstanding importance for him. Choroidal sclerosis and fundus appearance was extremely impressive and, to our knowledge, this is the first report of such unique case of ocular involvement in the literature. (c) 2008 S. Karger AG, Basel

  14. Alternatively activated macrophages (M2 macrophages) in the skin of patient with localized scleroderma.

    Science.gov (United States)

    Higashi-Kuwata, Nobuyo; Makino, Takamitsu; Inoue, Yuji; Takeya, Motohiro; Ihn, Hironobu

    2009-08-01

    Localized scleroderma is a connective tissue disorder that is limited to the skin and subcutaneous tissue. Macrophages have been reported to be particularly activated in patients with skin disease including systemic sclerosis and are potentially important sources for fibrosis-inducing cytokines, such as transforming growth factor beta. To clarify the features of immunohistochemical characterization of the immune cell infiltrates in localized scleroderma focusing on macrophages, skin biopsy specimens were analysed by immunohistochemistry. The number of cells stained with monoclonal antibodies, CD68, CD163 and CD204, was calculated. An evident macrophage infiltrate and increased number of alternatively activated macrophages (M2 macrophages) in their fibrotic areas were observed along with their severity of inflammation. This study revealed that alternatively activated macrophages (M2 macrophages) may be a potential source of fibrosis-inducing cytokines in localized scleroderma, and may play a crucial role in the pathogenesis of localized scleroderma.

  15. Juvenile localized scleroderma with port wine stain: Coincidental or possible common pathogenetic association

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    Seval Dogruk Kacar

    2015-01-01

    Full Text Available Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain. Coexistence of both diseases is not found yet. We herein present a case of juvenile localized scleroderma on the left side of trunk, with congenital port wine stain located on the ipsilateral face at V1-V2 distribution.

  16. Juvenile localized scleroderma with port wine stain: coincidental or possible common pathogenetic association.

    Science.gov (United States)

    Kacar, Seval Dogruk; Ozuguz, Pinar; Polat, Serap; Kacar, Emre; Polat, Onur; Tokyol, Cigdem

    2015-01-01

    Port wine stain and juvenile localized scleroderma are two different dermatoses usually encountered in pediatric age group. Up to now, there are reports of morphea patients initially diagnosed and treated as port wine stain. Coexistence of both diseases is not found yet. We herein present a case of juvenile localized scleroderma on the left side of trunk, with congenital port wine stain located on the ipsilateral face at V1-V2 distribution.

  17. Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma

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    Jolanta Budzyńska-Włodarczyk

    2016-02-01

    Full Text Available Introduction : Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components. Aim: To determine concentrations of interleukin (IL-27, transforming growth factor (TGF-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers. Material and methods: The study encompassed 17 females with localized scleroderma (aged 25–67. The control group consisted of 30 age-matched healthy women. The blood was sampled from the basilic vein. Serum levels of cytokines were determined using ELISA. Results : The TGF-β2 levels were found to be significantly lower in patients with localized scleroderma compared to controls. Concentrations of TGF-β1 were decreased in scleroderma patients when compared to controls but without statistical significance. There were no significant differences in serum IL-6, sIL-6R and IL-27 levels between patients and the control group; however, we found a significant positive correlation between the level of sIL-6 and ESR among subjects with localized scleroderma. Conclusions : The findings of decreased serum levels of TGF-β1 and TGF-β2 in patients with localized scleroderma demonstrate a possible association of these cytokines with pathogenesis of the disease. The results suggest also that sIL-6R is likely to be involved in inflammation in patients with localized scleroderma.

  18. Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma.

    Science.gov (United States)

    Budzyńska-Włodarczyk, Jolanta; Michalska-Jakubus, Małgorzata M; Kowal, Małgorzata; Krasowska, Dorota

    2016-02-01

    Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components. To determine concentrations of interleukin (IL)-27, transforming growth factor (TGF)-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers. The study encompassed 17 females with localized scleroderma (aged 25-67). The control group consisted of 30 age-matched healthy women. The blood was sampled from the basilic vein. Serum levels of cytokines were determined using ELISA. The TGF-β2 levels were found to be significantly lower in patients with localized scleroderma compared to controls. Concentrations of TGF-β1 were decreased in scleroderma patients when compared to controls but without statistical significance. There were no significant differences in serum IL-6, sIL-6R and IL-27 levels between patients and the control group; however, we found a significant positive correlation between the level of sIL-6 and ESR among subjects with localized scleroderma. The findings of decreased serum levels of TGF-β1 and TGF-β2 in patients with localized scleroderma demonstrate a possible association of these cytokines with pathogenesis of the disease. The results suggest also that sIL-6R is likely to be involved in inflammation in patients with localized scleroderma.

  19. Localized scleroderma: a clinical study at a single center in Korea.

    Science.gov (United States)

    Noh, Jung Won; Kim, Jinseok; Kim, Jae-Wang

    2013-08-01

    Localized scleroderma (morphea) is a rare autoimmune disease limited to the skin, characterized by cutaneous fibrosing and obstructive vasculopathy. Localized scleroderma may invade into the subcutaneous fat layer and cause permanent functional disability. Because of its rarity, there have been few clinical surveys of patients with localized scleroderma in Korea. The aim of this study was to elucidate the clinical presentation, serological data, and clinical outcomes of localized scleroderma. This was a retrospective survey conducted by reviewing available medical records during a 7 year-period from 2004 to 2010 in a single medical center in Jeju Island, South Korea. In total 43 patients with localized scleroderma were included. Localized scleroderma occurred primarily in females (female to male ratio 2.6 : 1.0). Most patients were between 10 and 29 years of age and the mean age at diagnosis was 26.2 years. Plaque (51.2%) and linear morphea (37.2%) were most common. No case was associated with systemic scleroderma (systemic sclerosis). The most common site of plaque morphea was the trunk (47.8%). In the linear type, the most common site was head-neck (52.9%). Fluorescent antinuclear antibody was positive in 23.3% of all cases. Treatment included systemic corticosteroids, colchicine, anti-malarial agents, D-penicillamine or intralesional triamcinolone injection. Clinical improvement, including significant and partial response, was seen in only 62.8% of treated patients. Localized scleroderma is a chronic inflammatory condition confined to the skin. In order to exclude other conditions, thorough history taking, physical examination, serologic studies and histopathologic examinations should be conducted. © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  20. Clinicohistopathological correlations in juvenile localized scleroderma: studies on a subset of children with hypopigmented juvenile localized scleroderma due to loss of epidermal melanocytes.

    Science.gov (United States)

    Sung, Joanne J; Chen, Tina S; Gilliam, Anita C; McCalmont, Timothy H; Gilliam, Amy E

    2011-08-01

    Localized scleroderma or morphea is a connective tissue disorder characterized by fibrosis of the skin and subcutaneous tissue. Excessive accumulation of collagen underlies the fibrosis, yet the pathogenesis is unknown. A subset of localized scleroderma/morphea, juvenile localized scleroderma (JLS), affects children and adolescents. The clinical and microscopic features of JLS have not been fully characterized. The goal is to better characterize the microscopic features of JLS. We collected a distinctive data set of 35 children with JLS, 19 (54%) of whom presented with hypopigmented lesions, and performed a retrospective chart and pathology review. We had adequate tissue for immunostaining studies on 8 of these individuals. We found that: (1) CD34 and factor XIIIa immunostaining, reported previously in adult morphea and scleroderma, when used with clinical information, is valuable for confirming a diagnosis of JLS; and (2) presence of hypopigmented lesions in JLS correlates with immunostaining studies. Decreased numbers of MelanA(+) melanocytes were present at the dermoepidermal junction in lesional skin in two of 3 children with hypopigmented JLS and in two of 4 children with nonhypopigmented JLS. The number of cases is small, a function of the small number of children who have biopsy specimens with material sufficient for multiple immunostaining procedures. These results provide a useful immunostaining method for confirmation of the diagnosis of JLS. They suggest a complex autoimmune phenotype in some children with JLS. Copyright © 2010 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  1. Peripheral blood cytokine and chemokine profiles in juvenile localized scleroderma

    Science.gov (United States)

    Torok, Kathryn S.; Kurzinski, Katherine; Kelsey, Christina; Yabes, Jonathan; Magee, Kelsey; Vallejo, Abbe N.; Medsger, Thomas; Feghali-Bostwick, Carol A.

    2015-01-01

    Objective To evaluate peripheral blood T-helper (TH) cell associated cytokine and chemokine profiles in localized scleroderma (LS), and correlate them with clinical disease features, including disease activity parameters. Methods A 29-plex Luminex platform was used to analyze the humoral profile of plasma samples from 69 pediatric LS patients and 71 healthy pediatric controls. Cytokine/chemokine levels were compared between these two groups and within LS patients, focusing on validated clinical outcome measures of disease activity and damage in LS. Results Plasma levels of IP-10, MCP-1, IL-17a, IL-12p70, GM-CSF, PDGF-bb, IFN-α2, and IFN-γ were significantly higher in LS compared to healthy controls. Analysis within the LS group demonstrated IP-10, TNF-α and GM-CSF correlated with clinical measures of disease activity. Several cytokines/chemokines correlated with anti-histone antibody, while only a few correlated with positive ANA and single-stranded DNA antibody. Conclusion This is the first time that multiple cytokines and chemokines have been examined simultaneously LS. In general, a TH-1 (IFN-γ) and TH-17 (IL-17a) predominance was demonstrated in LS compared to healthy controls. There is also an IFN–γ signature with elevated IP-10, MCP-1 and IFN-γ, which has been previously demonstrated in systemic sclerosis, suggesting a shared pathophysiology. Within the LS patients, those with active disease demonstrated IP-10, TNF-α and GM-CSF, which may potentially serve as biomarkers of disease activity in the clinical setting. PMID:26254121

  2. Development of Consensus Treatment Plans for Juvenile Localized Scleroderma

    Science.gov (United States)

    Li, Suzanne C.; Torok, Kathryn S.; Pope, Elena; Dedeoglu, Fatma; Hong, Sandy; Jacobe, Heidi T.; Rabinovich, C. Egla; Laxer, Ronald M.; Higgins, Gloria C.; Ferguson, Polly J.; Lasky, Andrew; Baszis, Kevin; Becker, Mara; Campillo, Sarah; Cartwright, Victoria; Cidon, Michael; Inman, Christi J; Jerath, Rita; O'Neil, Kathleen M.; Vora, Sheetal; Zeft, Andrew; Wallace, Carol A.; Ilowite, Norman T.; Fuhlbrigge, Robert C

    2013-01-01

    Objective To develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile localized scleroderma (jLS). Background jLS is a chronic inflammatory skin disorder associated with substantial morbidity and disability. Although a wide range of therapeutic strategies have been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment of have made it difficult to compare approaches and identify optimal therapy. Methods A core group of pediatric rheumatologists, dermatologists and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for jLS using consensus methods/nominal group techniques. Recommendations were validated in two face-to-face conferences with a larger group of practitioners with expertise in jLS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the U.S and Canada. Results Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members. Standardized clinical assessment methods and provisional treatment response criteria were also developed. Greater than 90% of pediatric rheumatologists responding to a survey (67% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with jLS. Conclusions Using consensus methodology, we have developed standardized treatment plans and assessment methods for jLS. The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of jLS. PMID:22505322

  3. Detection of autoimmune antibodies in localized scleroderma by synthetic oligonucleotide antigens

    DEFF Research Database (Denmark)

    Samuelsen, Simone; Jørgensen, Christian Damsgaard; Mellins, Elizabeth D

    2018-01-01

    In this study, we developed a series of synthetic oligonucleotides that allowed us to investigate the details on the antigen recognition by autoimmune antibodies in localized scleroderma subjects. Besides dramatically improved analytical specificity of the assay, our data suggests a potential...... linking for antibodies to DNA to the biological status of disease state in localized scleroderma. Moreover, introducing chemical modifications into short synthetic deoxyribonucleic acid (DNA) and ribonucleic acid (RNA) molecules completely changed the binding titers of corresponding antibodies...... and their clinical relevance. The strongest observed effect was registered for the localized scleroderma skin damage index (LoSDI) on the IgG antibodies to TC dinucleotide-rich double-stranded antigen (p

  4. Health-related quality of life, optimism, and coping strategies in persons suffering from localized scleroderma.

    Science.gov (United States)

    Szramka-Pawlak, B; Dańczak-Pazdrowska, A; Rzepa, T; Szewczyk, A; Sadowska-Przytocka, A; Żaba, R

    2013-01-01

    The clinical course of localized scleroderma may consist of bodily deformations, and bodily functions may also be affected. Additionally, the secondary lesions, such as discoloration, contractures, and atrophy, are unlikely to regress. The aforementioned symptoms and functional disturbances may decrease one's quality of life (QoL). Although much has been mentioned in the medical literature regarding QoL in persons suffering from dermatologic diseases, no data specifically describing patients with localized scleroderma exist. The aim of the study was to explore QoL in localized scleroderma patients and to examine their coping strategies in regard to optimism and QoL. The study included 41 patients with localized scleroderma. QoL was evaluated using the SKINDEX questionnaire, and levels of dispositional optimism were assessed using the Life Orientation Test-Revised. In addition, individual coping strategy was determined using the Mini-MAC scale and physical condition was assessed using the Localized Scleroderma Severity Index. The mean QoL score amounted to 51.10 points, with mean scores for individual components as follows: symptoms = 13.49 points, emotions = 21.29 points, and functioning = 16.32 points. A relationship was detected between QoL and the level of dispositional optimism as well as with coping strategies known as anxious preoccupation and helplessness-hopelessness. Higher levels of optimism predicted a higher general QoL. In turn, greater intensity of anxious preoccupied and helpless-hopeless behaviors predicted a lower QoL. Based on these results, it may be stated that localized scleroderma patients have a relatively high QoL, which is accompanied by optimism as well as a lower frequency of behaviors typical of emotion-focused coping strategies.

  5. Thrombocytopenia Associated with Localized Scleroderma: Report of Four Pediatric Cases and Review of the Literature.

    Science.gov (United States)

    Jindal, Ankur Kumar; Gupta, Anju; Dogra, Sunil; Rawat, Amit; Suri, Deepti; Ahluwalia, Jasmina; Singh, Surjit

    2017-07-01

    We report on four children with localized scleroderma (morphea) and thrombocytopenia. All four had the en coup de sabre subtype of morphea and had varying degrees of thrombocytopenia (8 × 10 9 /L to 120 × 10 9 /L). None of them had major bleeding manifestations, and thrombocytopenia resolved with treatment of morphea. (One patient was also given an injection of anti-D immunoglobulin.) We propose that thrombocytopenia associated with localized scleroderma is usually benign and requires no specific therapy. © 2017 Wiley Periodicals, Inc.

  6. Successful autologous hematopoietic stem cell transplantation for a patient with rapidly progressive localized scleroderma.

    Science.gov (United States)

    Nair, Velu; Sharma, Ajay; Sharma, Sanjeevan; Das, Satyaranjan; Bhakuni, Darshan S; Narayanan, Krishnan; Nair, Vivek; Shankar, Subramanian

    2015-03-01

    Autologous hematopoietic stem cell transplant (HSCT) for rapidly progressive disease has not been reported in localized scleroderma. Our patient, a 16-year-old girl had an aggressive variant of localized scleroderma, mixed subtype (linear-generalized) with Parry Romberg syndrome, with no internal organ involvement, that was unresponsive to immunosuppressive therapy and was causing rapid disfigurement. She was administered autologous HSCT in June 2011 and has maintained drug-free remission with excellent functional status at almost 3.5 years of follow-up. © 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd.

  7. Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma.

    Science.gov (United States)

    Li, Suzanne C; Torok, Kathryn S; Pope, Elena; Dedeoglu, Fatma; Hong, Sandy; Jacobe, Heidi T; Rabinovich, C Egla; Laxer, Ronald M; Higgins, Gloria C; Ferguson, Polly J; Lasky, Andrew; Baszis, Kevin; Becker, Mara; Campillo, Sarah; Cartwright, Victoria; Cidon, Michael; Inman, Christi J; Jerath, Rita; O'Neil, Kathleen M; Vora, Sheetal; Zeft, Andrew; Wallace, Carol A; Ilowite, Norman T; Fuhlbrigge, Robert C

    2012-08-01

    Juvenile localized scleroderma (LS) is a chronic inflammatory skin disorder associated with substantial morbidity and disability. Although a wide range of therapeutic strategies has been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment has made it difficult to compare approaches and identify optimal therapy. Our objective was to develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile LS. A core group of pediatric rheumatologists, dermatologists, and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for juvenile LS using consensus methods/nominal group techniques. Recommendations were validated in 2 face-to-face conferences with a larger group of practitioners with expertise in juvenile LS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the US and Canada. Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members. Standardized clinical assessment methods and provisional treatment response criteria were also developed. Greater than 90% of pediatric rheumatologists responding to a survey (66% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with juvenile LS. Using consensus methodology, we have developed standardized treatment plans and assessment methods for juvenile LS. The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of juvenile LS. Copyright © 2012 by the American College of Rheumatology.

  8. Localized Scleroderma, Systemic Sclerosis and Cardiovascular Risk: A Danish Nationwide Cohort Study.

    Science.gov (United States)

    Hesselvig, Jeanette Halskou; Kofoed, Kristian; Wu, Jashin J; Dreyer, Lene; Gislason, Gunnar; Ahlehoff, Ole

    2018-03-13

    Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint. A total of 697 patients with localized scleroderma and 1,962 patients with systemic sclerosis were identified and compared with 5,428,380 people in the reference population. In systemic sclerosis, the adjusted HR was 2.22 (95% confidence interval 1.99-2.48). No association was seen between patients with localized scleroderma and cardiovascular disease. In conclusion, systemic sclerosis is a significant cardiovascular disease risk factor, while patients with localized scleroderma are not at increased risk of cardiovascular disease.

  9. Use of mycophenolate mofetil in patients with severe localized scleroderma resistant or intolerant to methotrexate

    NARCIS (Netherlands)

    Mertens, Jorre S.; Marsman, Diane; van de Kerkhof, Peter C M; Hoppenreijs, Esther P A H; Knaapen, Hanneke K A; Radstake, Timothy R D; de Jong, Elke M G J; Seyger, Marieke M B

    2016-01-01

    To assess the efficacy and safety of mycophenolate mofetil (MMF) in patients with localized scleroderma (LoS) resistant or intolerant to previous treatment with methotrexate (MTX). A case series of patients with LoS treated with MMF. Outcome was assessed through clinical examination. Adverse events

  10. Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases.

    Science.gov (United States)

    Matsushita, Takashi; Hasegawa, Minoru; Matsushita, Yukiyo; Echigo, Takeshi; Wayaku, Takamasa; Horikawa, Mayuka; Ogawa, Fumihide; Takehara, Kazuhiko; Sato, Shinichi

    2007-02-01

    Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.

  11. Pirfenidone gel in patients with localized scleroderma: a phase II study.

    Science.gov (United States)

    Rodríguez-Castellanos, Marco; Tlacuilo-Parra, Alberto; Sánchez-Enríquez, Sergio; Vélez-Gómez, Ezequiel; Guevara-Gutiérrez, Elizabeth

    2015-01-28

    Localized scleroderma is an inflammatory disease in its first stages and a fibrotic process in later stages, principally mediated by the transforming growth factor β. To date, there is no standard treatment. The objective of this study was to determine the effectiveness and safety of 8% pirfenidone gel in patients with localized scleroderma. This was an open phase II clinical trial that included 12 patients. Treatment with pirfenidone was indicated, three times daily for 6 months. Patients were evaluated clinically with the modified Localized Scleroderma Skin Severity Index (mLoSSI), as well with a durometer and histologically using hematoxylin and eosin stain and Masson's trichrome stain. The baseline mLoSSI average scores were 5.83 ± 4.80 vs. 0.83 ± 1.75 (P = 0.002) at 6 months. The initial durometer induration of the scleroderma plaques was 35.79 ± 9.10 vs. 32.47 ± 8.97 at 6 months (P = 0.05). We observed histopathological improvement with respect to epidermal atrophy, inflammation, dermal or adipose tissue fibrosis and annex atrophy from 12.25 ± 3.25 to 9.75 ± 4.35 (P = 0.032). The 8% pirfenidone gel application was well tolerated, and no side effects were detected. This is the first study on the therapeutic use of pirfenidone gel in localized scleroderma. It acts on both the inflammatory and the fibrotic phases. Considering its effectiveness, good safety profile and the advantage of topical application, pirfenidone is a treatment option in this condition.

  12. [Localized Scleroderma of Lower Extremities:Clinical and Magnetic Resonance Imaging Features].

    Science.gov (United States)

    Wang, Feng-dan; Wang, Hong-wei; Wu, Zhi-hong; Hou, Bo; Jiang, Bo; Zhang, Yan; Feng, Feng; Jin, Zheng-yu; Yuan, Xie

    2015-08-01

    To evaluate the clinical and musculoskeletal characteristics of localized scleroderma with lower extremities affected. All the localized scleroderma patients,who received magnetic resonance (MR ) examinations of affected lower extremities at Peking Union Medical College Hospital from April 2013 to June 2014,were retrospectively reviewed. Their clinical data and laboratory results of antinuclear antibody,anti-double stranded-DNA antibody, and anti-extractable nuclear antigen antibody were collected and analyzed. All the MR examinations were non-contrast imaging using Siemens Skyra 3.0T MR scanner. There were 16 localized scleroderma patients with lower extremities affected, 11 of whom were linear scleroderma, 4 generalized morphea, and 1 deep morphea. Female to male ratio was 1:2.2. The mean age was 22.5 years. The mean time span was 7.4 years. Four of the 14 patients (28.6%) who received antinuclear antibody test were positive. All the 10 patients who received anti-double stranded-DNA antibody test and the 7 patients who received anti-extractable nuclear antigen antibody test were negative. The most common musculoskeletal MR features were subcutaneous septal thickening (16/16) and fascial thickening (11/16). The thickened speta and fascia could either be hypointenstiy or hyperintensity on turbo inversion recovery magnitude/proton density weighted imaging. Other MR manifestations were intramuscular speta thickening (3/16), muscular abnormal signals (1/16), and bone marrow abnormal signals (2/16). Musculoskeletal manifestations of the lower extremities with localized scleroderma can be well revealed using MR imaging.

  13. Scanning laser Doppler imaging may predict disease progression of localized scleroderma in children and young adults.

    Science.gov (United States)

    Shaw, L J; Shipley, J; Newell, E L; Harris, N; Clinch, J G; Lovell, C R

    2013-07-01

    Localized scleroderma is a rare but potentially disfiguring and disabling condition. Systemic treatment should be started early in those with active disease in key functional and cosmetic sites, but disease activity is difficult to determine clinically. Superficial blood flow has been shown to correlate with disease activity in localized scleroderma. To examine whether superficial blood flow measured by laser Doppler imaging (LDI) has the potential to predict disease progression and therefore select patients for early systemic treatment. A group of 20 individuals had clinical assessment and scanning LDI blood-flow measurements of 32 affected body sites. After a mean follow-up of 8.7 months their clinical outcome was compared with the results of the initial LDI assessment. Eleven out of 15 patients with an assessment of active LDI had progressed clinically, and 16 out of the 17 scans with inactive LDI assessment had not progressed, giving a positive predictive value of 73% and a negative predictive value of 94%. We believe that LDI can be a useful tool in predicting disease progression in localized scleroderma, and it may help clinicians to decide which patients to treat early. © 2013 The Authors BJD © 2013 British Association of Dermatologists.

  14. Scleroderma with Nodular Scleroderma

    Directory of Open Access Journals (Sweden)

    Chutika Srisuttiyakorn

    2016-11-01

    Full Text Available Background: Nodular scleroderma is a rare variant of scleroderma which can occur in connection with systemic sclerosis or morphea. A biopsy from the lesion can demonstrate the scleroderma pattern, i.e., keloid pattern or mixed type. Treatment is challenging, and several treatments modalities have been reported with unsatisfactory results. Main Observations: We present a case of systemic sclerosis in a 50-year-old female who developed nodular scleroderma in the absence of deterioration of the scleroderma condition. Although no additional treatment was given, the lesions remained stable without progression. Conclusions: Although this condition is rare, it has been reported sporadically, and clinicians should be able to recognize this variant in cases of scleroderma presenting with firm nodules or plaques.

  15. [Using the polymerase chain reaction to Borrelia burgdorferi infection in localized scleroderma injure (morphea), in Venezuelan patients].

    Science.gov (United States)

    Espinoza-León, Fabiola; Arocha, Francisco; Hassanhi, Manzur; Arévalo, Julio

    2010-09-01

    Borrelia burgdorferi is the causative agent of Lyme Borreliosis, an infectious multisystemic disease transmitted to humans by the Ixodes ticks bite. A possible association of Borrelia burgdorferi with localized scleroderma has been postulated. However, published data do not provide unequivocal results. Previous serologic analysis of patients with localized scleroderma in South American countries (including Venezuela), have been reported as yielding some reactivity. The present study looked for evidence of Borrelia burgdorferi infection in venezuelan patients with localized scleroderma, using the polymerase chain reaction to analyze 21 skin samples of patients with this skin condition. The results were negative in all the samples studied. Our data do not support an association of Borrelia burgdorferi infection and the sclerotic lesions of localized scleroderma; but do not rule out the possibility of a relationship between localized scleroderma and an unknown geno-specie of Borrelia burgdorferi sensu lato complex, a different Borrelia specie or a different spirochetal organism, as the etiological agents of the skin lesions in this area.

  16. Imaging features in calcinosis circumscripta, a rare type of subcutaneous calcification in localized scleroderma

    Directory of Open Access Journals (Sweden)

    Pratiksha Yadav

    2013-01-01

    Full Text Available Calcinosis cutis circumscripta is a rare condition in which abnormal deposition of calcium seen in the dermis and subcutaneous tissue, it is associated with localized scleroderma. A 30-year-old female presented with an area of extensive calcification involving the right gluteal region, lateral aspect of right thigh and a small area on left thigh detected on radiograph with atrophy of subcutaneous tissue. Magnetic resonance imaging and computed tomography were done for further evaluation and the findings were of calcification and atrophy involving the skin and subcutaneous tissue.

  17. Esclerodermia localizada: Diagnósticos diferenciales Localized scleroderma: Differential diagnosis

    Directory of Open Access Journals (Sweden)

    MB Leroux

    2011-09-01

    Full Text Available La esclerodermia localizada constituye un desorden autoinmune órgano específico, que compromete sobre todo la piel. Se caracteriza por inflamación seguida de esclerosis e incluye distintas formas clínicas. La etiología de la esclerodermia localizada no ha sido establecida. El diagnóstico diferencial incluye cuadros esclerodermiformes, desencadenados por factores intrínsecos y extrínsecos que están siendo estudiados. Entre ellos se incluyen: exposición a radiaciones o tóxicos, consumo de medicamentos, infecciones y enfermedades de origen endocrino-metabólico, genético e inflamatorio. En primer lugar, se debe descartar la esclerodermia sistémica. En segundo lugar, se clasifican las entidades con predominio de esclerosis o atrofia. Por último, se incluyen algunas enfermedades en un cuadro comparativo.Localized scleroderma is an autoimmune organ specific disorder with an important skin compromise. It is an inflammatory process with several distinct clinical characteristics. The etiology of localized scleroderma has not been established yet. Differential diagnosis includes sclerodermiform onset unchained by intrinsic and extrinsic factors that are presently studied. Among them must be taking into account: exposure to radiation or toxic agents, therapeutic drugs, infections and diseases of endocrine, metabolic, genetic or inflammatory etiology. Firstly, it must be point out that systemic scleroderma must be ruled out. Secondly, disorders predominantly with sclerosis and atrophy must be classified and lastly, some other diseases are included in a comparative table.

  18. Systemic sclerosis and localized scleroderma--current concepts and novel targets for therapy.

    Science.gov (United States)

    Distler, Oliver; Cozzio, Antonio

    2016-01-01

    Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Skin and organ fibrosis are key manifestations of SSc, for which no generally accepted therapy is available. Thus, there is a high unmet need for novel anti-fibrotic therapeutic strategies in SSc. At the same time, important progress has been made in the identification and characterization of potential molecular targets in fibrotic diseases over the recent years. In this review, we have selected four targeted therapies, which are tested in clinical trials in SSc, for in depths discussion of their preclinical characterization. Soluble guanylate cyclase (sGC) stimulators such as riociguat might target both vascular remodeling and tissue fibrosis. Blockade of interleukin-6 might be particularly promising for early inflammatory stages of SSc. Inhibition of serotonin receptor 2b signaling links platelet activation to tissue fibrosis. Targeting simultaneously multiple key molecules with the multityrosine kinase-inhibitor nintedanib might be a promising approach in complex fibrotic diseases such as SSc, in which many partially independent pathways are activated. Herein, we also give a state of the art overview of the current classification, clinical presentation, diagnostic approach, and treatment options of localized scleroderma. Finally, we discuss whether the novel targeted therapies currently tested in SSc could be used for localized scleroderma.

  19. Mild Cognitive Impairment as a single sign of brain hemiatrophy in patient with Localized Scleroderma and Parry-Romberg Syndrome.

    Science.gov (United States)

    Klimiec, Elzbieta; Klimkowicz-Mrowiec, Aleksandra

    2016-01-01

    Neurologic involvement is well recognized in Systemic Scleroderma and increasingly reported in Localized Scleroderma. MRI brain abnormalities are often associated with symptoms such as seizures or headaches. In some cases they may be clinically silent. We describe a 23 years old female with head, trunk and limbs scleroderma who developed Parry-Romberg Syndrome. Brain MRI showed ipsilateral temporal lobe atrophy without any prominent neurologic symptoms. Neuropsychological examination revealed Mild Cognitive Impairment. During the 7 years of follow up we have noticed progression of face atrophy but no progression of brain atrophy. Cognitive functions have been stable. This case highlight that major MRI brain abnormalities in LS may occur with only subtle clinical manifestation such as Mild Cognitive Impairment. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  20. Systemic sclerosis complicated with localized scleroderma-like lesions induced by Köbner phenomenon.

    Science.gov (United States)

    Saigusa, Ryosuke; Asano, Yoshihide; Yamashita, Takashi; Takahashi, Takehiro; Nakamura, Kouki; Miura, Shunsuke; Ichimura, Yohei; Toyama, Tetsuo; Taniguchi, Takashi; Sumida, Hayakazu; Tamaki, Zenshiro; Miyazaki, Miki; Yoshizaki, Ayumi; Sato, Shinichi

    2018-03-01

    Scleroderma is a chronic disease of unknown etiology characterized by skin fibrosis and is divided into two clinical entities: systemic sclerosis (SSc) and localized scleroderma (LSc). In general, LSc is rarely complicated with SSc, but a certain portion of SSc patients manifests bilateral symmetric LSc-like lesions on the trunk and extremities. We investigated SSc patients with LSc-like lesions to clarify the underlying pathophysiology. Nine SSc cases complicated with LSc-like lesions were clinically and histologically characterized. SSc patients with LSc-like lesions exhibited multiple progressive hyper- and/or hypo-pigmented plaques with mild sclerosis symmetrically distributed on the trunk and extremities, especially abdominal region. In histological assessment, epidermal IL-1α expression was elevated in both forearms and LSc-like lesions of these patients to a greater extent than in forearms of control patients (SSc patients without LSc-like lesions). Of note, the infiltration and degranulation of mast cells were evident throughout the dermis of LSc-like lesions, while detectable to a lesser extent in forearms of SSc patients with LSc-like lesions and control patients. The epidermis of SSc patients with LSc-like lesions seems to possess an inflammatory phenotype, leading to the activation of mast cells in the dermis of mechanically stressed skin. Köbner phenomenon may be involved in the induction of LSc-like lesions in a certain subset of SSc. Copyright © 2017 Japanese Society for Investigative Dermatology. Published by Elsevier B.V. All rights reserved.

  1. Evaluation of medium-dose UVA1 phototherapy in localized scleroderma with the cutometer and fast Fourier transform method

    NARCIS (Netherlands)

    de Rie, M. A.; Enomoto, D. N. H.; de Vries, H. J. C.; Bos, J. D.

    2003-01-01

    Purpose: To evaluate the efficacy of medium-dose UVA1 phototherapy in patients with localized scleroderma. Method: A controlled pilot study with medium-dose UVA1 (48 J/cm(2)) was performed. The results were evaluated by means of a skin score and two objective methods for quantifying sclerosis

  2. Successful combination treatment of a patient with progressive juvenile localized scleroderma (morphea) using imatinib, corticosteroids, and methotrexate.

    Science.gov (United States)

    Inamo, Yasuji; Ochiai, Toyoko

    2013-01-01

    We report a case of progressive juvenile localized scleroderma (JLS or morphea) treated with a combination of imatinib, corticosteroids, and methotrexate. This therapy halted the progressive skin thickening and the hand and finger joint deformity in the early stages of the disease. We conclude that imatinib used in addition to standard treatment with systemic corticosteroids and methotrexate may be of therapeutic benefit for individuals with JLS. © 2012 Wiley Periodicals, Inc.

  3. The Localized Scleroderma Skin Severity Index and Physician Global Assessment of Disease Activity: A Work in Progress Toward Development of Localized Scleroderma Outcome Measures

    Science.gov (United States)

    ARKACHAISRI, THASCHAWEE; VILAIYUK, SOAMARAT; LI, SUZANNE; O’NEIL, KATHLEEN M.; POPE, ELENA; HIGGINS, GLORIA C.; PUNARO, MARILYNN; RABINOVICH, EGLA C.; ROSENKRANZ, MARGALIT; KIETZ, DANIEL A.; ROSEN, PAUL; SPALDING, STEVEN J.; HENNON, TERESA R.; TOROK, KATHRYN S.; CASSIDY, ELAINE; MEDSGER, THOMAS A.

    2013-01-01

    Objective To develop and evaluate a Localized Scleroderma (LS) Skin Severity Index (LoSSI) and global assessments’ clinimetric property and effect on quality of life (QOL). Methods A 3-phase study was conducted. The first phase involved 15 patients with LS and 14 examiners who assessed LoSSI [surface area (SA), erythema (ER), skin thickness (ST), and new lesion/extension (N/E)] twice for inter/intrarater reliability. Patient global assessment of disease severity (PtGA-S) and Children’s Dermatology Life Quality Index (CDLQI) were collected for intrarater reliability evaluation. The second phase was aimed to develop clinical determinants for physician global assessment of disease activity (PhysGA-A) and to assess its content validity. The third phase involved 2 examiners assessing LoSSI and PhysGA-A on 27 patients. Effect of training on improving reliability/validity and sensitivity to change of the LoSSI and PhysGA-A was determined. Results Interrater reliability was excellent for ER [intraclass correlation coefficient (ICC) 0.71], ST (ICC 0.70), LoSSI (ICC 0.80), and PhysGA-A (ICC 0.90) but poor for SA (ICC 0.35); thus, LoSSI was modified to mLoSSI. Examiners’ experience did not affect the scores, but training/practice improved reliability. Intrarater reliability was excellent for ER, ST, and LoSSI (Spearman’s rho = 0.71–0.89) and moderate for SA. PtGA-S and CDLQI showed good intrarater agreement (ICC 0.63 and 0.80). mLoSSI correlated moderately with PhysGA-A and PtGA-S. Both mLoSSI and PhysGA-A were sensitive to change following therapy. Conclusion mLoSSI and PhysGA-A are reliable and valid tools for assessing LS disease severity and show high sensitivity to detect change over time. These tools are feasible for use in routine clinical practice. They should be considered for inclusion in a core set of LS outcome measures for clinical trials. PMID:19833758

  4. Clinical and laboratory features of systemic sclerosis complicated with localized scleroderma.

    Science.gov (United States)

    Toki, Sayaka; Motegi, Sei-ichiro; Yamada, Kazuya; Uchiyama, Akihiko; Kanai, Sahori; Yamanaka, Masayoshi; Ishikawa, Osamu

    2015-03-01

    Localized scleroderma (LSc) primarily affects skin, whereas systemic sclerosis (SSc) affects skin and various internal organs. LSc and SSc are considered to be basically different diseases, and there is no transition between them. However, LSc and SSc have several common characteristics, including endothelial cell dysfunction, immune activation, and excess fibrosis of the skin, and there exist several SSc cases complicated with LSc during the course of SSc. Clinical and laboratory characteristics of SSc patients with LSc remain unclear. We investigated the clinical and laboratory features of 8 SSc patients with LSc among 220 SSc patients (3.6%). The types of LSc included plaque (5/8), guttate (2/8), and linear type (1/8). All cases were diagnosed as having SSc within 5 years before or after the appearance of LSc. In three cases of SSc with LSc (37.5%), LSc skin lesions preceded clinical symptoms of SSc. Young age, negative antinuclear antibody, and positive anti-RNA polymerase III antibody were significantly prevalent in SSc patients with LSc. The positivity of anticentromere antibody tended to be prevalent in SSc patients without LSc. No significant difference in the frequency of complications, such as interstitial lung disease, reflux esophagitis, and pulmonary artery hypertension, was observed. The awareness of these characteristic of SSc with LSc are essential to establish an early diagnosis and treatment. © 2015 Japanese Dermatological Association.

  5. Pathogenesis and therapeutic approaches for improved topical treatment in localized scleroderma and systemic sclerosis.

    Science.gov (United States)

    Badea, I; Taylor, M; Rosenberg, A; Foldvari, M

    2009-03-01

    SSc is a chronic progressive disorder of unknown aetiology characterized by excess synthesis and deposition of collagen and other extracellular matrix components in a variety of tissues and organs. Localized scleroderma (LS) differs from SSc in that with LS only skin and occasionally subcutaneous tissues are involved. Although rarely life threatening, LS can be disfiguring and disabling and, consequently, can adversely affect quality of life. There is no known effective treatment for LS, and various options, including, as examples, corticosteroids and other immunomodulatory agents, ultraviolet radiation and vitamin D analogues, are of unproven efficacy. Clinical trials evaluating combination therapy such as corticosteroids with MTX or UVA1 exposure with psoralens have not been established as consistently effective. New immunomodulators such as tacrolimus and thalidomide are also being evaluated. A better understanding of the molecular and cellular mechanisms of LS has led to evaluation of new treatments that modulate profibrotic cytokines such as TGF-beta and IL-4, regulate assembly and deposition of extracellular matrix components, and restore Th1/Th2 immune balance by administering IL-12 or IFN-gamma. IFN-gamma acts by directly inhibiting collagen synthesis and by restoring immune balance. In this review, we evaluate current and future treatment options for LS and cutaneous involvement in SSc. Recent advances in therapy focus mainly on anti-fibrotic agents. Delivery of these drugs into the skin as the target tissue might be a key factor in developing more effective and safer therapy.

  6. Impact of a new simplified disability scoring system for adult patients with localized scleroderma.

    Science.gov (United States)

    Okiyama, Naoko; Asano, Yoshihide; Hamaguchi, Yasuhito; Jinnin, Masatoshi; Motegi, Sei-Ichiro; Koizumi, Haruka; Hasegawa, Minoru; Ishikawa, Osamu; Sato, Shinichi; Takehara, Kazuhiko; Yamamoto, Toshiyuki; Fujimoto, Manabu; Ihn, Hironobu

    2018-04-01

    Localized scleroderma (LoS) involves dermal but not internal inflammation and fibrosis. Cosmetic changes often impact quality of life (QOL), however, impairment of activities of daily living (ADL) in LoS patients has not been investigated. To determine what factor(s) are associated with ADL in adult patients with LoS, we performed a retrospective observational study in 177 Japanese adult LoS patients using a novel LoS disability score based on Barthel's indices of ADL: feeding, bathing, grooming, dressing, bowels, bladder, toilet use, transfers, mobility and stairs. LoS disability scores increased in proportion to the number of affected body parts but were not correlated to age and duration of illness. The presence of leg lesions significantly impaired ADL of LoS patients compared with lesions on other body parts. Patients treated with systemic medications, who tended to have multiple lesions, presented higher LoS disability scores than those without systemic treatments. Our study proposes that physicians evaluate ADL, not only QOL, in LoS patients. Our findings using LoS disability scoring indicate that multiple affected body parts and leg lesions are risk factors for ADL impairment. © 2018 Japanese Dermatological Association.

  7. Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation.

    Science.gov (United States)

    Borowiec, A; Dabrowski, R; Wozniak, J; Jasek, S; Chwyczko, T; Kowalik, I; Musiej-Nowakowska, E; Szwed, H

    2012-02-01

    The aim of the present study was non-invasive evaluation of the cardiovascular system in asymptomatic young adult patients with juvenile localized scleroderma (JLS) and juvenile systemic sclerosis (JSS). A group of 34 consecutive children with scleroderma were prospectively observed in the study. The control group (CG) consisted of 20 healthy subjects. In each subject 12-lead electrocardiographic, echocardiographic, ECG Holter, and ambulatory blood pressure monitoring examinations were performed at the baseline visit and after 10 years. Additionally, B-type natriuretic peptide (BNP) concentrations were measured after 10 years. Examinations were performed in 13 patients with JLS and 15 with JSS at the final visit. Two children had died (one from each group). Four patients were alive but refused the final visit. After 10 years, a higher prevalence of ventricular extrasystoles (p = 0.01) and an elevated pulmonary arterial pressure (JLS: p = 0.04, JSS: p = 0.03) were observed in both groups, but in comparison with the controls there was no significant difference at the final visit. In JLS patients more cases of left ventricle diastolic dysfunction, hypertension, and sinus tachycardia were diagnosed at the final visit (p ≤ 0.05). More atrioventricular block episodes in both groups of scleroderma patients were observed. Over the 10 years, arterial hypertension was diagnosed in three patients from the JLS group and in two with JSS. There were no significant differences in BNP concentrations at the final visit. The results of the present study show that juvenile scleroderma seems to be more benign than adult-onset disease. This observational study shows subclinical, not severe, cardiac abnormalities in adult patients with juvenile-onset disease.

  8. microRNA-7 down-regulation mediates excessive collagen expression in localized scleroderma.

    Science.gov (United States)

    Etoh, Mitsuhiko; Jinnin, Masatoshi; Makino, Katsunari; Yamane, Keitaro; Nakayama, Wakana; Aoi, Jun; Honda, Noritoshi; Kajihara, Ikko; Makino, Takamitsu; Fukushima, Satoshi; Ihn, Hironobu

    2013-01-01

    Localized scleroderma (LSc), a connective tissue disorder restricted to the skin and subcutaneous tissue, is characterized by skin fibrosis due to an excessive deposition of types I collagen. The mechanism of such fibrosis is still unknown, but epigenetics may play some roles in the excessive collagen expression. In the present study, we investigated the mechanism of fibrosis seen in LSc, focusing on microRNA (miRNA). miRNA expression was determined by PCR array, real-time PCR, and in situ hybridization. The function of miRNA was evaluated using specific inhibitor. Immunoblotting was performed to detect α2(I) collagen protein. PCR array analysis using tissue miRNA demonstrated miR-7 level was significantly decreased in LSc skin as well as keloid tissue compared to normal skin in vivo. In situ hybridization also showed miR-7 expression in dermal fibroblasts was decreased in LSc dermis. The transfection of specific inhibitor for miR-7 into cultured normal dermal fibroblasts resulted in the up-regulation of α2(I) collagen protein in vitro. Also, the serum levels of miR-7 were significantly decreased in LSc patients compared with healthy controls, but serum miR-29a levels not. Systemic or local down-regulation of miR-7 may contribute to the pathogenesis of LSc via the overexpression of α2(I) collagen, and serum miR-7 may be useful as a disease marker. Investigation of the regulatory mechanisms of LSc by miRNA may lead to new treatments by the transfection into the lesional skin of this disease.

  9. Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach.

    Science.gov (United States)

    Marrani, Edoardo; Foeldvari, Ivan; Lopez, Jordi Anton; Cimaz, Rolando; Simonini, Gabriele

    2018-03-14

    Localized scleroderma is a skin fibrosing disorder that, if untreated, may result in severe disability. The purpose of this systematic review is to compare the present evidence concerning the effectiveness of Methotrexate versus phototherapy, alone or associated with Psoralen, in childhood localized scleroderma. A systematic search between January 1996 and May 2017 was performed to identify studies investigating the efficacy of Methotrexate (MTX) or phototherapy (UVA) for treating localized scleroderma with onset ≤18 years. Due to a lack of validated clinical criteria, four clinical response criteria were used to assess the treatment efficacy as primary outcome. We determined a combined estimate of the proportion of children responding to MTX and UVA. A total of 19 studies was included (8 MTX; 11 UVA). In the methotrexate group, 193 children were included in the analysis; in the phototherapy group, a total of 48 treated children. For both groups age, disease subtype, glucocorticoids (GCs) use, and side effects of treatment were also analyzed. The meta-analysis suggested that UVA and MTX protocols have both a favorable effect in active lesions of childhood localized scleroderma. However, MTX resulted significantly superior to UVA, with or without Psoralen. Our study supports the combination of MTX and GCs in patients with a high risk of complication. Phototherapy with UVA1 could represent a therapeutic option in patients with limited scleroderma, where lesions do not cross joints and they do not lead to potential cosmetic changes. Copyright © 2018 Elsevier Inc. All rights reserved.

  10. Esophageal abnormalities in juvenile localized scleroderma: is it associated with other extracutaneous manifestations?

    Science.gov (United States)

    Valões, Clarissa C M; Novak, Glaucia V; Brunelli, Juliana B; Kozu, Katia T; Toma, Ricardo K; Silva, Clovis A

    To assess esophageal involvement (EI) in juvenile localized scleroderma (JLS) population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. For a period of 30 years, 5881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia) and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring. JLS was observed in 56/5881 patients (0.9%), mainly linear morphea subtype. EI was observed in 23/56(41%) of JLS patients. Eight(35%) of 23 EI patients with JLS were symptomatic and presented heartburn(5/8), solid and liquid dysphagia(3/8), nausea and epigastralgia(1/8). The frequency of any cumulative extracutaneous manifestations (calcinosis, arthritis/arthralgia, central nervous system, interstitial pneumonitis, mesangial nephritis and/or arrhythmia) was significantly higher in JLS patients with EI compared to those without this complication (56% vs. 24%, p=0.024). No differences were evidenced in demographic data, JLS subtypes and in each extracutaneous manifestation in both groups (p>0.05). The frequency of methotrexate use was significantly higher in JLS patients with EI compared to those without (52% vs. 12%, p=0.002). Autoantibody profile (antinuclear antibodies, anti-SCL-70, rheumatoid factor, anticentromere, anti-cardiolipin, anti-Ro/SSA and anti-La/SSB) was similar in both groups (p>0.05). Our study demonstrated that EI was frequently observed in JLS patients, mainly in asymptomatic patients with linear subtype. EI occurred in JLS patients with other extracutaneous manifestations and required methotrexate therapy. Copyright © 2016. Published by Elsevier Editora Ltda.

  11. Esophageal abnormalities in juvenile localized scleroderma: is it associated with other extracutaneous manifestations?

    Directory of Open Access Journals (Sweden)

    Clarissa C.M. Valões

    Full Text Available Abstract Objective: To assess esophageal involvement (EI in juvenile localized scleroderma (JLS population and the possible association between this gastrointestinal manifestation and demographic data, clinical features, laboratory exams, treatments and outcomes. Methods: For a period of 30 years, 5881 patients with rheumatic diseases were followed in our Pediatric Rheumatology Division. EI was defined by the presence of symptoms (solid/liquid dysphagia, heartburn, esophageal regurgitation, nausea/vomiting and epigastralgia and confirmed by at least one EI exam abnormality: barium contrast radiography, upper gastrointestinal endoscopy and 24-hour esophageal pH-monitoring. Results: JLS was observed in 56/5881 patients (0.9%, mainly linear morphea subtype. EI was observed in 23/56(41% of JLS patients. Eight(35% of 23 EI patients with JLS were symptomatic and presented heartburn(5/8, solid and liquid dysphagia(3/8, nausea and epigastralgia(1/8. The frequency of any cumulative extracutaneous manifestations (calcinosis, arthritis/arthralgia, central nervous system, interstitial pneumonitis, mesangial nephritis and/or arrhythmia was significantly higher in JLS patients with EI compared to those without this complication (56% vs. 24%, p = 0.024. No differences were evidenced in demographic data, JLS subtypes and in each extracutaneous manifestation in both groups (p > 0.05. The frequency of methotrexate use was significantly higher in JLS patients with EI compared to those without (52% vs. 12%, p = 0.002. Autoantibody profile (antinuclear antibodies, anti-SCL-70, rheumatoid factor, anticentromere, anti-cardiolipin, anti-Ro/SSA and anti-La/SSB was similar in both groups (p > 0.05. Conclusions: Our study demonstrated that EI was frequently observed in JLS patients, mainly in asymptomatic patients with linear subtype. EI occurred in JLS patients with other extracutaneous manifestations and required methotrexate therapy.

  12. Interferon-gamma inducible protein-10 as a potential biomarker in localized scleroderma

    Science.gov (United States)

    2013-01-01

    Introduction The purpose of this study was to evaluate the presence and levels of interferon-gamma inducible protein-10 (IP-10) in the plasma and skin of pediatric localized scleroderma (LS) patients compared to those of healthy pediatric controls and to determine if IP-10 levels correlate to clinical disease activity measures. Methods The presence of IP-10 in the plasma was analyzed using a Luminex panel in 69 pediatric patients with LS and compared to 71 healthy pediatric controls. Of these patients, five had available skin biopsy specimens with concurrent clinical and serological data during the active disease phase, which were used to analyze the presence and location of IP-10 in the skin by immunohistochemistry (IHC). Results IP-10 levels were significantly elevated in the plasma of LS patients compared to that of healthy controls and correlated to clinical disease activity measures in LS. Immunohistochemistry staining of IP-10 was present in the dermal infiltrate of LS patients and was similar to that found in psoriasis skin specimens, the positive disease control. Conclusions Elevation of IP-10 levels in the plasma compared to those of healthy controls and the presence of IP-10 staining in the affected skin of LS patients indicates that IP-10 is a potential biomarker in LS. Furthermore, significant elevation of IP-10 in LS patients with active versus inactive disease and correlations between IP-10 levels and standardized disease outcome measures of activity in LS strongly suggest that IP-10 may be a biomarker for disease activity in LS. PMID:24499523

  13. The Localized Scleroderma Cutaneous Assessment Tool: responsiveness to change in a pediatric clinical population.

    Science.gov (United States)

    Kelsey, Christina E; Torok, Kathryn S

    2013-08-01

    Lack of agreement on how to accurately capture disease outcomes in localized scleroderma (LS) has hindered the development of efficacious treatment protocols. The LS Cutaneous Assessment Tool (LoSCAT), consisting of the modified LS Skin Severity Index (mLoSSI) and the LS Damage Index, has potential for use in clinical trials. The goal of this article is to further evaluate the clinical responsiveness of the LoSCAT. Based on the modifiable nature of disease activity versus damage, we expected the mLoSSI to be responsive to change. At 2 study visits, a physician completed the LoSCAT and Physician Global Assessment (PGA) of Disease Activity and of Disease Damage for 29 patients with LS. Spearman correlations were used to examine the relationships between the change in the LoSCAT and the PGA scores. To evaluate contrasted group validity, patients were grouped according to disease activity classification and change scores of groups were compared. Minimal clinically important differences were calculated and compared with the standard error of measurement. Change in the mLoSSI score correlated strongly with change in the PGA of Disease Activity score, whereas change in the LS Damage Index score correlated weakly with change in the PGA of Disease Damage score. The mLoSSI and PGA of Disease Activity exhibited contrasted group validity. Minimal clinically important differences for the activity measures were greater than the respective standard errors of measurement. Only 2 study visits were included in analysis. This study gives further evidence that the LoSCAT, specifically the mLoSSI, is a responsive, valid measure of activity in LS and should be used in future treatment studies. Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  14. A long-term follow-up study of methotrexate in juvenile localized scleroderma (morphea).

    Science.gov (United States)

    Zulian, Francesco; Vallongo, Cristina; Patrizi, Annalisa; Belloni-Fortina, Anna; Cutrone, Mario; Alessio, Maria; Martino, Silvana; Gerloni, Valeria; Vittadello, Fabio; Martini, Giorgia

    2012-12-01

    Recent studies report that methotrexate (MTX) is beneficial in the treatment of juvenile localized scleroderma (JLS) but little is known about its long-term effectiveness. We assessed the therapeutic role of MTX in children with JLS who were followed up for a prolonged period. A cohort of patients with JLS, previously enrolled in a double-blind, randomized controlled trial and treated with oral MTX (15 mg/m(2)/wk) and prednisone (1 mg/kg/d, maximum 50 mg) for the first 3 months, were prospectively followed up. Lesions were evaluated clinically, with infrared thermography, and by a computerized skin score. Response to treatment was defined as: (1) no new lesions; (2) skin score rate less than 1; and (3) decrease in lesion temperature by at least 10% compared with baseline. Clinical remission (CR) on medication was defined when response was maintained, on treatment, for at least 6 months, and complete CR when response was maintained, without treatment, for at least 6 months. Of 65 patients treated with MTX, 48 (73.8%) were responders, 10 (15.4%) relapsed by 24 months since MTX start, and 7 (10.8%) were lost to follow-up. Among the responders, 35 (72.9%) maintained CR for a mean of 25 months and 13 (27.1%) were in CR on medication. Adverse effects seen in 28 patients (48.3%) were generally mild and never required treatment discontinuation. The use of objective measures not widely available, such as infrared thermography and computerized skin score, makes it difficult to compare data from previous studies. Long-term MTX therapy is beneficial and well tolerated for JLS. Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  15. Initial evaluation of an ultrasound measure for assessing the activity of skin lesions in juvenile localized scleroderma.

    Science.gov (United States)

    Li, S C; Liebling, M S; Haines, K A; Weiss, J E; Prann, A

    2011-05-01

    To evaluate the construct validity of 2 proposed measures (the Ultrasound Disease Activity [U-DA] and the Tissue Thickness Score [TTS]) for evaluating sonographic differences in juvenile localized scleroderma skin lesions. We conducted a retrospective review of juvenile localized scleroderma patients who had ultrasound scans of their skin lesions between October 2005 and February 2009. Imaged lesions were classified as active or inactive based upon clinical assessment. Lesions had to have been imaged within 1 month of a clinic visit or have the same clinical assessment during both the visit before and the visit after the scan. Two physicians scored the scans using the U-DA, which scores for differences in lesion echogenicity and vascularity compared with normal tissue. Tissue thickness differences were evaluated by percent differences and by using the TTS. Wilcoxon's rank sum test was performed to assess differences. We studied 52 scans from 21 patients, 32 scans of active skin lesions and 20 scans of inactive skin lesions. Features reported by clinicians as indicative of active disease included erythema, warmth, violaceous color, new lesion, expansion of lesion, and induration. The U-DA was significantly different between active and inactive skin lesions (P = 0.0010) with significant differences found for the parameters of total echogenicity, hypodermis echogenicity, and deep tissue layer vascularity (P = 0.0014, P = 0.0023, and P = 0.0374, respectively). No significant differences were found for tissue layer thickness or TTS. The U-DA may be a useful tool in the identification of localized scleroderma activity. Further study is needed to prospectively evaluate the validity, reliability, and sensitivity of this potential monitoring tool. Copyright © 2011 by the American College of Rheumatology.

  16. Risk Factors for Scleroderma

    Science.gov (United States)

    ... You are here: Home For Patients Risk Factors Risk Factors for Scleroderma The cause of scleroderma is ... what biological factors contribute to scleroderma pathogenesis. Genetic Risk Scleroderma does not tend to run in families ...

  17. Lupus erythematosus and localized scleroderma coexistent at the same sites: a rare presentation of overlap syndrome of connective-tissue diseases.

    Science.gov (United States)

    Pascucci, Anabella; Lynch, Peter J; Fazel, Nasim

    2016-05-01

    Overlap syndromes are known to occur with connective-tissue diseases (CTDs). Rarely, the overlap occurs at the same tissue site. We report the case of a patient with clinical and histopathologic findings consistent with the presence of discoid lupus erythematosus (DLE) and localized scleroderma within the same lesions. Based on our case and other reported cases in the literature, the following features are common in patients with an overlap of lupus erythematosus (LE) and localized scleroderma: predilection for young women, photodistributed lesions, DLE, linear morphology clinically, and positivity along the dermoepidermal junction on direct immunofluorescence. Most patients showed good response to antimalarials, topical steroids, or systemic steroids.

  18. Quantitative Assessment of Skin Stiffness in Localized Scleroderma Using Ultrasound Shear-Wave Elastography.

    Science.gov (United States)

    Wang, Liyun; Yan, Feng; Yang, Yujia; Xiang, Xi; Qiu, Li

    2017-07-01

    The purpose of this study was to evaluate the usefulness of ultrasound shear-wave elastography (US-SWE) in characterization of localized scleroderma (LS), as well as in the disease staging. A total of 21 patients with 37 LS lesions were enrolled in this study. The pathologic stage (edema, sclerosis or atrophy) of the lesions was characterized by pathologic examination. The skin elastic modulus (E-values including E mean , E min , E max and E sd ) and thickness (h) was evaluated both in LS lesions and site-matched unaffected skin (normal controls) using US-SWE. The relative difference of E-values (E RD ) was calculated between each pair of lesions and its normal control for comparison among different pathologic stages. Of the 37 LS lesions, 2 were in edema, 22 were in sclerosis and 13 were in atrophy. US-SWE results showed a significant increase of skin elastic modulus and thickness in all lesions (p < 0.001 in sclerosis and p < 0.05 in atrophy) compared with the normal controls. The measured skin elastic modulus and thickness were greater in sclerosis than in atrophy. However, once normalized by skin thickness, the atrophic lesions, which were on average thinner, appeared significantly stiffer than those of the sclerosis (normalized E RD : an increase of 316.3% in atrophy vs. 50.6% in sclerosis compared with the controls, p = 0.007). These findings suggest that US-SWE allows for quantitative evaluation of the skin stiffness of LS lesions in different stages; however, the E-values directly provided by the US-SWE system alone do not distinguish between the stages, and the normalization by skin thickness is necessary. This non-invasive, real-time imaging technique is an ideal tool for assessing and monitoring LS disease severity and progression. Copyright © 2017 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

  19. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

    OpenAIRE

    Bonilla Abadía, Fabio; Muñoz Buitrón, Evelyn; Ochoa, Carlos D.; Carrascal, Edwin; Cañas Dávila, Carlos Alberto

    2012-01-01

    The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our kno...

  20. Scleroderma Mimickers

    Science.gov (United States)

    Morgan, Nadia D.; Hummers, Laura K.

    2017-01-01

    Opinion statement Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated. PMID:28473954

  1. [When thinking to scleroderma?].

    Science.gov (United States)

    Cogan, E

    2007-09-01

    Scleroderma encompasses an heterogeneous group of autoimmune disorders characterized by an hidebound thickened skin involvement. When the changes are limited to the skin, localized scleroderma is suspected. Although the latter is most often a benign disease, it may be exceptionally associated with involvement of multiple organs, mainly the neurological system. At the opposite, systemic sclerosis is a serious disorder associated with high morbidity and even mortality and defined by an extended skin sclerosis, multiple organ involvement and general symptoms. Raynaud phenomena is nearly always present at the beginning of the disease. Identifying initial manifestations of the disease (Raynaud phenomena, diffuse non pitting edema, symmetrical polyarthritis with tendon friction rubs, dysphagia associated with mucosal telangiectasia) may allow the clinician to rapidly transfer the patient to a specialized reference center in order to organize a multidisciplinary approach and to prompt optimum therapy.

  2. Down-regulation of microRNA-196a in the sera and involved skin of localized scleroderma patients.

    Science.gov (United States)

    Makino, Takamitsu; Jinnin, Masatoshi; Etoh, Mitsuhiko; Yamane, Keitaro; Kajihara, Ikko; Makino, Katsunari; Ichihara, Asako; Igata, Toshikatsu; Sakai, Keisuke; Fukushima, Satoshi; Ihn, Hironobu

    2014-01-01

    Localized scleroderma (LSc) exhibits fibrosis of the skin and subcutaneous tissue. LSc shows an excessive deposition of type 1 collagen. To elucidate the mechanism of type 1 collagen overexpression in LSc, we investigated the epigenetics, focusing on microRNA (miRNA). miRNA expression profile was determined by PCR array analysis. The expression of microRNA-196a (miR-196a) in the skin tissue was examined by in situ hybridization or real-time PCR. The serum levels of miR-196a were measured by real-time PCR. PCR array analysis demonstrated that the miR-196a level was markedly decreased in LSc skin tissue in vivo. The transfection of specific inhibitor for miR-196a into normal cultured human dermal fibroblasts led to the up-regulation of type 1 collagen protein in vitro. Furthermore, the serum levels of miR-196a were significantly decreased in LSc patients. Down-regulation of miR-196a and subsequent overexpression of type 1 collagen in dermal fibroblasts may play a key role in the pathogenesis of LSc. The serum levels of miR-196a may be useful as a diagnostic marker of LSc.

  3. Corticosteroids in Myositis and Scleroderma

    Science.gov (United States)

    Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

    2017-01-01

    Synopsis Idiopathic inflammatory myopathies (IIM) involve inflammation of the muscles and are classified based on the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into four categories: dermatomyositis, polymyositis, inclusion body myositis, and immune mediated necrotizing myopathy. The term “scleroderma” refers to fibrosis of the skin. Localized scleroderma (morphea) is skin-limited, while systemic sclerosis (SSc) is associated with vascular and internal organ involvement. Although there is a paucity of randomized clinical trials, treatment with systemic corticosteroids (CS) is the standard of care for IIM with muscle and organ involvement. The extra-cutaneous features of systemic sclerosis are frequently treated with CS, however high doses have been associated with scleroderma renal crisis in high-risk patients. CS monotherapy is neither recommended for the cutaneous manifestations of dermatomyositis nor scleroderma. While CS can be effective first line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. PMID:26611554

  4. Access to care for children and young people diagnosed with localized scleroderma or juvenile SSc in the UK.

    Science.gov (United States)

    Hawley, Daniel P; Baildam, Eileen M; Amin, Tania S; Cruikshank, Mary K; Davidson, Joyce E; Dixon, Jennifer; Martin, Neil S; Ohlsson, Victoria; Pilkington, Clarissa; Rangaraj, Satyapal; Riley, Philip; Sundaramoorthy, Chitra; Walsh, Jo; Foster, Helen E

    2012-07-01

    To describe pathways of care and referral to paediatric rheumatology from onset of first symptom (noticed by the patient or their family) to diagnosis for children and young people diagnosed with localized scleroderma (LS) or juvenile SSc (jSSc). Retrospective case note audit of patients under paediatric rheumatology care who presented during January 2005-January 2010. Data included disease subtype, sex, age at key points in the referral pathway and health care professional (HCP) contact. All patient and HCP data were pseudo-anonymized in accordance with good clinical practice. Data were from eight UK centres that saw 89 cases: 62 females, 26 males; 73 LS, 16 jSSc. Median time from first symptom to first HCP review was 4 (range 0-72) months (LS) and 1 (range 0-50) month (jSSc). Median time from first symptom to paediatric rheumatology review was 15 (range 1-103) months (LS) and 7 (range 0-50) months (jSSc). Median time from first HCP review to first paediatric rheumatology review was 11 (range 0-103) months (LS) and 2 (range 0-10) months. First HCP seen (74%) was usually a general practitioner. The referring HCP to paediatric rheumatology was usually a dermatologist (56%) for LS. Median time from first symptom to diagnosis was 13 (range 1-102) months (LS) and 8 (range 1-50) months (jSSc). A prolonged interval occurs from first symptom to definitive diagnosis, which may adversely affect outcome. There is a need to raise awareness of this rare diagnosis and facilitate earlier recognition.

  5. Localized morphea. A rare but significant secondary complication following breast cancer radiotherapy. Case report and review of the literature on radiation reaction among patients with scleroderma/morphea

    International Nuclear Information System (INIS)

    Herrmann, Thomas; Csere, Peter; Guenther, Claudia

    2009-01-01

    Purpose and approach: to report a case of morphea (localized scleroderma) in a patient following breast cancer therapy and to summarize the current literature. Results and conclusion: the occurrence of morphea is an unexpected late effect (approximately 1 year after the end of radiation therapy) which occurs frequently in the irradiated breast in women with breast-conserving therapy. The pathogenesis is unclear. The main differential diagnoses are recurrence of carcinoma and a radiogenic subcutaneous fibrosis (in most cases, the final diagnosis can only be made by means of a biopsy). Diagnosis and therapy must be performed in cooperation between dermatologist and radiooncologist. (orig.)

  6. Localized morphea. A rare but significant secondary complication following breast cancer radiotherapy. Case report and review of the literature on radiation reaction among patients with scleroderma/morphea

    Energy Technology Data Exchange (ETDEWEB)

    Herrmann, Thomas; Csere, Peter [Dept. of Radiotherapy and Radiooncology, Medical Faculty Carl Gustav Carus, Univ. of Technology, Dresden (Germany); Guenther, Claudia [Dept. of Dermatology, Medical Faculty Carl Gustav Carus, Univ. of Technology, Dresden (Germany)

    2009-09-15

    Purpose and approach: to report a case of morphea (localized scleroderma) in a patient following breast cancer therapy and to summarize the current literature. Results and conclusion: the occurrence of morphea is an unexpected late effect (approximately 1 year after the end of radiation therapy) which occurs frequently in the irradiated breast in women with breast-conserving therapy. The pathogenesis is unclear. The main differential diagnoses are recurrence of carcinoma and a radiogenic subcutaneous fibrosis (in most cases, the final diagnosis can only be made by means of a biopsy). Diagnosis and therapy must be performed in cooperation between dermatologist and radiooncologist. (orig.)

  7. Dental Care in Scleroderma

    Science.gov (United States)

    Dental Care in Scleroderma People living with scleroderma face unique challenges while trying to maintain their oral ... They are more likely to be affected by dental conditions such as small mouth, dry mouth, jaw ...

  8. Scleroderma and Sexuality

    Science.gov (United States)

    Scleroderma and Sexuality INTRODUCTION If you or your partner have been diagnosed with scleroderma, you may be wondering how this will ... will continue to find satisfaction and enjoyment through sexuality. If you are single, you may wonder how ...

  9. Pulmonary Hypertension in Scleroderma

    Science.gov (United States)

    PULMONARY HYPERTENSION IN SCLERODERMA PULMONARY HYPERTENSION Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. If the high ... the right side of the heart. Patients with scleroderma are at increased risk for developing PH from ...

  10. Localized scleroderma: assessment of the therapeutic response to phototherapy Esclerodermia cutânea: avaliação da resposta terapêutica à fototerapia

    Directory of Open Access Journals (Sweden)

    Roberta Buense

    2012-02-01

    Full Text Available BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES: This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS: Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS: Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS: The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound.FUNDAMENTOS: A esclerodermia é uma doença autoimune caracterizada pela esclerose progressiva do tecido conjuntivo e alterações da microcirculação. A forma cutânea é considerada uma doença autolimitada. No entanto, em alguns casos, ocorrem lesões atróficas, deformantes, que dificultam o desenvolvimento normal. Relatos da literatura apontam a fototerapia como uma modalidade terapêutica com resposta favorável nas formas cutâneas da esclerodermia. OBJETIVOS

  11. Pulsed dye laser in the treatment of localized scleroderma and its effects on CD34+ and factor XIIIa+ cells: an immunohistochemical study.

    Science.gov (United States)

    Tawfik, Abeer Attia; Shokir, Hisham; Soliman, Mona; Salah, Lila; Fathy, Sahar

    2013-06-01

    Localized scleroderma (morphea) is characterized by hardening and thickening of the dermis due to excessive collagen deposition. A decreased number of CD34+ cells and an increased number of Factor XIIIa+ cells are seen in the affected skin. The flashlamp pulsed dye laser (FLPDL) has been used in the treatment of localized morphea with promising results. The purpose of this study was to evaluate the therapeutic effectiveness of the pulsed dye laser in localized scleroderma and to assess its effect on CD34+ cells, Factor XIIIa+ cells, and blood vessels. Thirty patients with plaque morphea were treated with a FLPDL (585 nm wavelength, 450 μs pulse duration). Fluence ranged from 7.5 to 8.5 J/cm(2). Sessions were performed biweekly for a maximum of 6 months. Clinical, histopathologic, and immunohistochemical assessments were performed. Patients showed varying degrees of improvement of indurated skin. There was no worsening or further improvement at the treated sites during the follow-up assessments at 3, 6, and 12 months. An increased number of CD34+ cells were found in both the upper and the lower dermis, and a decreased number of Factor XIIIa+ cells were found in the lower dermis. The FLPDL is effective in the treatment of morphea, as confirmed by the changes in the pathologic tissue and levels of CD34+ and Factor XIIIa+ cells.

  12. Challenges and strategies for coping with scleroderma: implications for a scleroderma-specific self-management program.

    Science.gov (United States)

    Milette, Katherine; Thombs, Brett D; Maiorino, Kristina; Nielson, Warren R; Körner, Annett; Peláez, Sandra

    2018-05-09

    The purpose of this study was to explore challenges faced by patients with systemic sclerosis, also called scleroderma, in coping with their disease and the strategies they used to face those challenges. Five focus groups were held with scleroderma patients (4 groups, n = 34) and health care professionals who have experience treating scleroderma (1 group, n = 8). Participants' discussions were recorded, transcribed and analyzed using thematic analysis. Participants reported challenges accessing information (e.g., knowledgeable specialists), dealing with negative emotions (e.g., stress due to misunderstandings with loved ones), and accessing resources (e.g., helpful products or devices). Strategies for overcoming challenges were also discussed (e.g., advocating for own needs). When faced with significant challenges while coping with scleroderma, patients develop strategies to manage better and improve their quality of life. To help them cope, patients would benefit from easier access to supportive interventions, including tailored scleroderma self-management programs. Although the challenges experienced by patients with scleroderma are unique, findings from this study might help better understand patients' perspectives regarding coping and disease management for other chronic diseases as well. Implications for Rehabilitation People living with rare diseases, including the rare autoimmune disease scleroderma, face unique challenges and often do not have access to disease-specific educational or other support resources. People with scleroderma report that they face challenges in accessing information, including knowledgeable healthcare providers; managing difficult social interactions and negative emotions; and accessing resources. Strategies employed by scleroderma patients to overcome these challenges include seeking connections to other people with scleroderma or scleroderma patient organizations, actively seeking out local resources, and learning to

  13. Tips for Living with Scleroderma

    Science.gov (United States)

    ... Patients Tips for Living Tips for Living with Scleroderma Ways to help manage your symptoms The Scleroderma ... help find improved therapies and a cure for scleroderma! Your gift today will be matched to have ...

  14. Genetics Home Reference: systemic scleroderma

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Systemic scleroderma Systemic scleroderma Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Systemic scleroderma is an autoimmune disorder that affects the skin ...

  15. Unilateral Enophthalmos in Linear Scleroderma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Cho, So hyun; Hwang, Hee Young; Choi, Hye Young; Kim, Hyung Sik [Gachon University, Gil Hospital, Incheon (Korea, Republic of)

    2010-04-15

    Linear scleroderma is an uncommon subtype of localized scleroderma, which is characterized by a linear streak that crosses dermatomes and is associated with the tracking of fibrosis from the skin into deeper tissues, including muscle and fascia. A severe form of this condition sometimes causes growth atrophy of bone and supporting tissue in the affected area. Enophthalmos as a manifestation of linear scleroderma is very rare and occurs due to the replacement of orbital fat and muscle with collagen, which finally leads to atrophy of the affected orbit. This report introduces imaging findings of two cases of enophthalmos caused by linear scleroderma

  16. Unilateral Enophthalmos in Linear Scleroderma: A Case Report

    International Nuclear Information System (INIS)

    Cho, So hyun; Hwang, Hee Young; Choi, Hye Young; Kim, Hyung Sik

    2010-01-01

    Linear scleroderma is an uncommon subtype of localized scleroderma, which is characterized by a linear streak that crosses dermatomes and is associated with the tracking of fibrosis from the skin into deeper tissues, including muscle and fascia. A severe form of this condition sometimes causes growth atrophy of bone and supporting tissue in the affected area. Enophthalmos as a manifestation of linear scleroderma is very rare and occurs due to the replacement of orbital fat and muscle with collagen, which finally leads to atrophy of the affected orbit. This report introduces imaging findings of two cases of enophthalmos caused by linear scleroderma

  17. Paraneoplastic Scleroderma: Are There Any Clues?

    Science.gov (United States)

    Jedlickova, Hana; Durčanská, Veronika; Vašků, Vladimír

    2016-04-01

    localized scleroderma was seen in 3 patients and generalized localized scleroderma in one case. All patients had a histological picture consistent with scleroderma, negative ANA and ENA antibodies (Table 1, Figure 1). A 66-year-old woman presented with a 10 months history of sclerodermatous plaques on her neck, trunk, and upper and lower extremities. The skin on her breasts and cheeks was diffusely indurated. Examination showed thrombocytopenia, elevated transaminases, Cancer antigen 19-9 (Ca 19-9), thyroid stimulating hormone (TSH), and anti-thyroid peroxidase antibodies, dysmotility of the lower part of esophagus, hepatosplenomegaly, cholecystolithiasis, and benign polyps of colon. She was given prednisone 40 mg/day but did not return for follow up. After 6 months she was diagnosed with cholangiogenic carcinoma with metastatic disease and died shortly afterwards. A 74-year-old woman had localized scleroderma on the trunk for three years. She was treated with procaine penicillin for positive borrelia Immunoglobulin M (IgM) antibodies. Her condition worsened suddenly with confluent scleroderma plaques on her trunk, extremities, and genital region, and vasoneurosis on her lower extremities; she was started on prednisone 35 mg/day. Examination revealed endometrial cancer. The patient underwent a hysterectomy, adnexectomy, and radiotherapy with curative effect. Scleroderma patches softened with residual hyperpigmentation, and prednisone was stopped two years later. A 80-year-old man had a month-long history of diffuse thickening and toughening of the skin on the forearms and lower legs and scleroderma patches on the thighs and shins. Examination revealed prostate adenocarcinoma, and therapy with antiandrogen bicalutamide and prednisone 15 mg/day was started. Two years after the diagnosis he continues with bicalutamide treatment, prednisone 5 mg q.a.d. and has residual toughening of the skin on his lower legs. A 62-year-old woman with seronegative rheumatoid arthritis presented

  18. Assessment of esophageal involvement in systemic sclerosis and morphea (localized scleroderma) by clinical, endoscopic, manometric and pH metric features: a prospective comparative hospital based study.

    Science.gov (United States)

    Arif, Tasleem; Masood, Qazi; Singh, Jaswinder; Hassan, Iffat

    2015-02-15

    Systemic sclerosis (SSc) is a generalized disorder of unknown etiology affecting the connective tissue of the body. It affects the skin and various internal organs. Gastrointestinal tract involvement is seen in almost 90% of the patients. Esophagus is the most frequently affected part of the gastrointestinal tract. Esophageal motility disturbance classically manifests as a reduced lower esophageal sphincter pressure (LESP) and loss of distal esophageal body peristalsis. Consequently, SSc patients may be complicated by erosive esophagitis and eventually by Barrett's esophagus and esophageal adenocarcinoma. Morphea, also known as localized scleroderma, is characterized by predominant skin involvement, with occasional involvement of subjacent muscles and usually sparing the internal organs. The involvement of esophagus in morphea has been studied very scarcely. The proposed study will investigate the esophageal involvement in the two forms of scleroderma (systemic and localized), compare the same and address any need of upper gastrointestinal evaluation in morphea (localized scleroderma) patients. 56 and 31 newly and already diagnosed cases of SSc and morphea respectively were taken up for the study. All the patients were inquired about the dyspeptic symptoms (heartburn and/or acid regurgitation and/or dysphagia). Upper gastrointestinal endoscopy, esophageal manometry and 24-hour pH monitoring were done in 52, 47 and 41 patients of SSc; and 28, 25 and 20 patients of morphea respectively. Esophageal symptoms were present in 39 cases (69.6%) of SSc which were mild in 22 (39.3%), moderate in 14 (25%), severe in three (5.3%); while only four cases (7.1%) of morphea had esophageal symptoms all of which were mild in severity. Reflux esophagitis was seen in 17 cases (32.7%) of SSc and only two cases (7.14%) of morphea. Manometric abnormalities were seen in 32 cases (68.1%) of SSc and none in morphea. Ambulatory 24-hour esophageal pH monitoring documented abnormal reflux in

  19. Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review.

    Science.gov (United States)

    Martini, Giorgia; Fadanelli, Gloria; Agazzi, Anna; Vittadello, Fabio; Meneghel, Alessandra; Zulian, Francesco

    2018-05-03

    Juvenile Localized Scleroderma (JLS) is a rare disorder that may cause severe aesthetic sequelae and functional disability. To date, data on natural history and long-term outcome are discordant and difficult to compare due to the heterogeneity of clinical subtypes, treatments and methods to evaluate activity and outcome in previous studies. A retrospective and cross-sectional study including 133 patients followed between January 1991 and December 2016 was conducted at our Pediatric Rheumatology Centre. Disease course was drawn by retrospective analysis of patients' clinical features, treatment, disease course and outcome at the last evaluation. Disease activity and severity of tissue damage were assessed by using parameters derived from the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and thermography. Most patients achieved complete remission, as only 12.5%, all with the linear subtype, had still active disease after over 10 years of follow-up. At least one disease relapse occurred in 22.2% of patients and first flare was observed 20 months after first treatment discontinuation. Mild tissue damage was observed in more than half of patients, in 25.4% was moderate and in 23.0% severe; 19.8% presented a functional limitation. The entity of skin and subcutaneous fat loss established at the early stages of the disease as 27.8% of patients with shorter disease duration had severe damage and the rates remained constant in patients with longer follow-up. The delay in start of systemic treatment was associated with longer disease activity and higher relapse rate. Patients with linear scleroderma (LS), pansclerotic morphea (PM) and mixed subtype (MS) presented more severe aesthetic and functional damage but did not differ from other subtypes as for rate of complete remission. JLS in some patients can be a very aggressive disease with persistent activity after >10 years and/or several disease relapses. As tissue damage establishes early in disease course a

  20. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

    Science.gov (United States)

    Knobler, R; Moinzadeh, P; Hunzelmann, N; Kreuter, A; Cozzio, A; Mouthon, L; Cutolo, M; Rongioletti, F; Denton, C P; Rudnicka, L; Frasin, L A; Smith, V; Gabrielli, A; Aberer, E; Bagot, M; Bali, G; Bouaziz, J; Braae Olesen, A; Foeldvari, I; Frances, C; Jalili, A; Just, U; Kähäri, V; Kárpáti, S; Kofoed, K; Krasowska, D; Olszewska, M; Orteu, C; Panelius, J; Parodi, A; Petit, A; Quaglino, P; Ranki, A; Sanchez Schmidt, J M; Seneschal, J; Skrok, A; Sticherling, M; Sunderkötter, C; Taieb, A; Tanew, A; Wolf, P; Worm, M; Wutte, N J; Krieg, T

    2017-09-01

    The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum. © 2017 European Academy of Dermatology and Venereology.

  1. Scleroderma in children: an update.

    Science.gov (United States)

    Zulian, Francesco; Cuffaro, Giorgio; Sperotto, Francesca

    2013-09-01

    Scleroderma, in its localized and systemic presentation, represents the third most frequent rheumatic condition in childhood after juvenile idiopathic arthritis and systemic lupus erythematosus. Early diagnosis, appropriate assessment and effective treatment are crucial to improve the long-term outcome. Recent studies, concerning histopathology and clinical associations with other conditions, open new horizons on the etiopathogenesis of scleroderma. New developments have been also reached in the field of outcome measures. In juvenile localized scleroderma (JLS), new techniques such as Doppler and laser Doppler imaging have shown their usefulness for the daily monitoring of the patients. In juvenile systemic sclerosis (JSSc), a new severity score has been developed and needs to be validated in future trials. Finally, a randomized, double-blind controlled trial, a multicenter consensus statement and long-term follow-up studies have confirmed the important role of methotrexate (MTX) for the treatment of JLS. Studies over recent years highlighted the role of imaging as outcome measures for JLS and introduced a severity score for JSSc. New studies on MTX confirmed its important role for the treatment of JLS.

  2. Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.

    Science.gov (United States)

    Ferreli, Caterina; Gasparini, Giulia; Parodi, Aurora; Cozzani, Emanuele; Rongioletti, Franco; Atzori, Laura

    2017-12-01

    Scleroderma refers to an autoimmune connective tissue fibrosing disease, including three different subsets: localized scleroderma, limited cutaneous systemic sclerosis, and diffuse cutaneous systemic sclerosis with divergent patterns of organ involvement, autoantibody profiles, management, and prognostic implications. Although systemic sclerosis is considered the disease prototype that causes cutaneous sclerosis, there are many other conditions that can mimic and be confused with SSc. They can be classified into immune-mediated/inflammatory, immune-mediated/inflammatory with abnormal deposit (mucinoses), genetic, drug-induced and toxic, metabolic, panniculitis/vascular, and (para)neoplastic disorders according to clinico-pathological and pathogenetic correlations. This article reviews the clinical presentation with emphasis on cutaneous disease, etiopathogenesis, diagnosis, and treatment options available for the different forms of scleroderma firstly and for scleroderma-like disorders, including scleromyxedema, scleredema, nephrogenic systemic fibrosis, eosinophilic fasciitis, chronic graft-versus-host disease, porphyria cutanea tarda, diabetic stiff-hand syndrome (diabetic cheiroartropathy), and other minor forms. This latter group of conditions, termed also scleroderma mimics, sclerodermiform diseases, or pseudosclerodermas, shares the common thread of skin thickening but presents with distinct cutaneous manifestations, skin histology, and systemic implications or disease associations, differentiating each entity from the others and from scleroderma. The lack of Raynaud's phenomenon, capillaroscopic abnormalities, or scleroderma-specific autoantibodies is also important diagnostic clues. As cutaneous involvement is the earliest, most frequent and characteristic manifestation of scleroderma and sclerodermoid disorders, dermatologists are often the first-line doctors who must be able to promptly recognize skin symptoms to provide the affected patient a correct

  3. Scleroderma Research Foundation

    Science.gov (United States)

    ... will continue. Learn more The SRF: A Four Star Charity The SRF has achieved the highest possible ... Saget share about his connection to scleroderma and what he is doing to make a difference. Click ...

  4. Supernatants from culture of type I collagen-stimulated PBMC from patients with cutaneous systemic sclerosis versus localized scleroderma demonstrate suppression of MMP-1 by fibroblasts.

    Science.gov (United States)

    Brown, Monica; Postlethwaite, Arnold E; Myers, Linda K; Hasty, Karen A

    2012-06-01

    Systemic sclerosis (SSc) is a chronic fibrosing disease characterized by vasculopathy, autoimmunity, and an accumulation of collagen in tissues. Numerous studies have shown that compared to healthy or diseased controls, the peripheral blood mononuclear cells (PBMC) from patients with SSc produce a variety of cytokines or proliferate when cultured with solubilized type I collagen (CI) or constituent α1(II) and α2(I) polypeptide chains. The purpose of this study was to determine whether PBMC isolated from patients with SSc and cultured in vitro with soluble CI elaborated soluble mediators that inhibit the production of collagenase (i.e., matrix metalloproteinase, MMP-1) by fibroblasts. Supernatants of CI-stimulated PBMC from juvenile and adult diffuse cutaneous (dc)SSc patients significantly reduced MMP-1 production by SSc dermal fibroblasts, while supernatants of CI-stimulated PBMC from patients with localized scleroderma (LS) did not. CI-stimulated PBMC culture supernatants from patients with dcSSc in contrast to patients with LS exhibited increased levels of platelet-derived growth factor (PDGF)-AA, PDGF-BB, TNF-α, IL-13, and EGF. Prolonged culture of SSc dermal fibroblasts with recombinant PDGF-BB or IL-13 inhibited the induction of MMP-1 in response to subsequent TNF-α stimulation. These data suggest that therapies aimed at reducing these cytokines may decrease collagen accumulation in SSc, preventing the development of chronic fibrosis.

  5. Neurological abnormalities in localized scleroderma of the face and head: a case series study for evaluation of imaging findings and clinical course.

    Science.gov (United States)

    Lis-Święty, Anna; Brzezińska-Wcisło, Ligia; Arasiewicz, Hubert

    2017-09-01

    Localized scleroderma (LoS) of the face and head is often associated with neurological manifestations and/or imaging abnormalities in the central nervous system (CNS). We present an analysis of 20 cases of LoS affecting the face and head. The CNS symptoms and/or abnormalities in high-resolution computed tomography (HRCT) and/or magnetic resonance imaging (MRI) were observed in 12 patients (60%). In addition to the mild and unspecific disorders (e.g. headaches), serious neurological complications probably in the course of vasculitis were revealed: epilepsy (in two patients), epilepsy and pyramidal sings (in one patient). Neurological disorders and LoS occurred at the same time (in three patients) or at the course of the disease (nine patients) and no later than 29 years since the onset of the disease. No link between neurological disorders and the LoS clinical morphology, immunological and other laboratory parameters has been established. CNS involvement is not correlated with the clinical course of the facial and head LoS and may occur years after the disease initial symptomatology. Imaging follow-up is not required if there is not any emerging neurological symptom. In some cases, however, both HRCT and MRI are useful for monitoring disease evolution and addressing therapeutic choices.

  6. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report.

    Science.gov (United States)

    Bonilla-Abadía, Fabio; Muñoz-Buitrón, Evelyn; Ochoa, Carlos D; Carrascal, Edwin; Cañas, Carlos A

    2012-12-20

    The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  7. A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report

    Directory of Open Access Journals (Sweden)

    Bonilla-Abadía Fabio

    2012-12-01

    Full Text Available Abstract Background The localized scleroderma (LS known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53 year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

  8. JUVENILE SCLERODERMA-what has changed in the meantime?

    Science.gov (United States)

    Adrovic, Amra; Sahin, Sezgin; Barut, Kenan; Kasapcopur, Ozgur

    2018-04-22

    Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition. Skin manifestations are most prominent feature of the systemic form, followed by musculoskeletal and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in childhood. Combination of disease modifying antirheumatic drugs (methotrexate, mycophenolate-mofetil, cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a useful treatment option in most severe form of the disease. Localized scleroderma is characterized with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the local scleroderma is made according to the size and localization of the skin changes. There are few different therapeutical options but there is no specific therapy for the localized scleroderma. Many data regarding disease features and treatment options in juvenile scleroderma are based on studies among adults. There is a striking need for multicentric, prospective studies among children with juvenile scleroderma.Emerging biological agents and new treatment options are showing promising results. Anyhow, juvenile scleroderma remains a mystery with many aspects of the disease waiting to be solved. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  9. Fractional carbon dioxide laser versus low-dose UVA-1 phototherapy for treatment of localized scleroderma: a clinical and immunohistochemical randomized controlled study.

    Science.gov (United States)

    Shalaby, S M; Bosseila, M; Fawzy, M M; Abdel Halim, D M; Sayed, S S; Allam, R S H M

    2016-11-01

    Morphea is a rare fibrosing skin disorder that occurs as a result of abnormal homogenized collagen synthesis. Fractional ablative laser resurfacing has been used effectively in scar treatment via abnormal collagen degradation and induction of healthy collagen synthesis. Therefore, fractional ablative laser can provide an effective modality in treatment of morphea. The study aimed at evaluating the efficacy of fractional carbon dioxide laser as a new modality for the treatment of localized scleroderma and to compare its results with the well-established method of UVA-1 phototherapy. Seventeen patients with plaque and linear morphea were included in this parallel intra-individual comparative randomized controlled clinical trial. Each with two comparable morphea lesions that were randomly assigned to either 30 sessions of low-dose (30 J/cm 2 ) UVA-1 phototherapy (340-400 nm) or 3 sessions of fractional CO 2 laser (10,600 nm-power 25 W). The response to therapy was then evaluated clinically and histopathologically via validated scoring systems. Immunohistochemical analysis of TGF-ß1 and MMP1 was done. Patient satisfaction was also assessed. Wilcoxon signed rank test for paired (matched) samples and Spearman rank correlation equation were used as indicated. Comparing the two groups, there was an obvious improvement with fractional CO 2 laser that was superior to that of low-dose UVA-1 phototherapy. Statistically, there was a significant difference in the clinical scores (p = 0.001), collagen homogenization scores (p = 0.012), and patient satisfaction scores (p = 0.001). In conclusion, fractional carbon dioxide laser is a promising treatment modality for cases of localized morphea, with proved efficacy of this treatment on clinical and histopathological levels.

  10. Mimics of scleroderma

    Directory of Open Access Journals (Sweden)

    Kaveri K Nalianda

    2017-01-01

    Full Text Available Systemic sclerosis is a rare autoimmune connective tissue disorder characterised typically by tightening and tethering of skin. However, several other disorders are also characterised by hardening and thickening of skin. These mimics can be potentially confused with systemic sclerosis, leading to a misdiagnosis. This review describes the aetiopathogenesis, clinical features and treatment of Morphea (localised scleroderma, Scleredema, Scleromyxoedema, Eosinophilic fasciitis, Nephrogenic Systemic Fibrosis, Diabetic Cheiroarthropathy, chronic GVHD, POEMS syndrome and drug induced scleroderma like illness. A careful and thorough clinical assessment is essential in order to differentiate these mimics from each other and from systemic sclerosis, establish the diagnosis, and initiate appropriate treatment.

  11. Development and initial validation of the Localized Scleroderma Skin Damage Index and Physician Global Assessment of disease Damage: a proof-of-concept study

    Science.gov (United States)

    Vilaiyuk, Soamarat; Torok, Kathryn S.; Medsger, Thomas A.

    2010-01-01

    Objective. To develop and assess the psychometric properties of the Localized Scleroderma (LS) Skin Damage Index (LoSDI) and Physician Global Assessment of disease Damage (PGA-D). Methods. Damage was defined as irreversible/persistent changes (>6 months) due to previous active disease/complications of therapy. Eight rheumatologists assessed the importance of 17 variables in formulating the PGA-D/LoSDI. LS patients were evaluated by two rheumatologists using both tools to assess their psychometric properties. LoSDI was calculated by summing three scores for cutaneous features of damage [dermal atrophy (DAT), subcutaneous atrophy (SAT) and dyspigmentation (DP)] measured at 18 anatomic sites. Patient GA of disease severity (PtGA-S), Children's Dermatology Life Quality Index (CDLQI) and PGA-D were recorded at the time of each examination. Results. Thirty LS patients (112 lesions) and nine patient-visit pairs (18 lesions) were included for inter- and intra-rater reliability study. LoSDI and its domains DAT, SAT, DP and PGA-D demonstrated excellent inter- and intra-rater reliability (reliability coefficients 0.86–0.99 and 0.74–0.96, respectively). LoSDI correlated moderately with PGA-D and poorly with PtGA-S and CDLQI. PGA-D correlated moderately with PtGA-S, but poorly with CDLQI. Conclusions. To complete the LS Cutaneous Assessment Tool (LoSCAT), we developed and evaluated the psychometric properties of the LoSDI and PGA-D in addition to the LS Skin Severity Index (LoSSI). These instruments will facilitate evaluation of LS patients for individual patient management and clinical trials. LoSDI and PGA-D demonstrated excellent reliability and high validity. LoSCAT provides an improved understanding of LS natural history. Further study in a larger group of patients is needed to confirm these preliminary findings. PMID:20008472

  12. The downregulation of microRNA let-7a contributes to the excessive expression of type I collagen in systemic and localized scleroderma.

    Science.gov (United States)

    Makino, Katsunari; Jinnin, Masatoshi; Hirano, Ayaka; Yamane, Keitaro; Eto, Mitsuhiko; Kusano, Takamitsu; Honda, Noritoshi; Kajihara, Ikko; Makino, Takamitsu; Sakai, Keisuke; Masuguchi, Shinichi; Fukushima, Satoshi; Ihn, Hironobu

    2013-04-15

    Systemic and localized scleroderma (SSc and LSc) is characterized by excessive deposition of collagen and tissue fibrosis in the skin. Although they have fundamental common characteristics including autoimmunity, little is known about the exact mechanism that mediates the excessive collagen expression in these disorders. In the current study, we tried to evaluate the possibility that microRNAs (miRNAs) play some roles in the pathogenesis of fibrosis seen in these diseases. miRNA expression patterns were evaluated by miRNA array analysis, real-time PCR, and in situ hybridization. The function of miRNAs in dermal fibroblasts was assessed using miRNA inhibitors, precursors, or protectors. In the mouse model of bleomycin-induced dermal sclerosis, the overexpression of miRNAs was performed by i.p. miRNA injection. We demonstrated let-7a expression was downregulated in SSc and LSc skin both in vivo and in vitro, compared with normal or keloid skin. The inhibition or overexpression of let-7a in human or mouse skin fibroblasts affected the protein expression of type I collagen or luciferase activity of collagen 3'-untranslated region. Also, we found let-7a was detectable and quantitative in the serum and investigated serum let-7a levels in patients with SSc or LSc. let-7a concentration was significantly decreased in these patients, especially in LSc patients. Moreover, we revealed that the intermittent overexpression of let-7a in the skin by i.p. miRNA injection improved the skin fibrosis induced by bleomycin in mice. Investigation of more detailed mechanisms of miRNA-mediated regulation of collagen expression may lead to new therapeutic approaches against SSc and LSc.

  13. Coexistence of lichen sclerosus and morphea: a retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center.

    Science.gov (United States)

    Kreuter, Alexander; Wischnewski, Jana; Terras, Sarah; Altmeyer, Peter; Stücker, Markus; Gambichler, Thilo

    2012-12-01

    The coexistence of lichen sclerosus (LiS) and localized scleroderma (LoS) has sporadically been reported in the literature. Recently, a prospective multicenter study demonstrated a surprisingly high percentage of genital LiS in patients with morphea. The aim of this study was to determine the prevalence of LiS in a cohort of patients with LoS who presented at a tertiary referral medical center for connective tissue diseases in Germany. We retrospectively evaluated the prevalence of genital and extragenital LiS in adult and pediatric patients with different subtypes of LoS. Secondary outcome measures included demographic characteristics and prevalence of other concomitant autoimmune diseases. Of the 472 patients (381 adults, 91 children; mean age: 46 years; range, 4-88 years; female to male ratio: 3.5:1 in adults and 8:1 in children) with LoS, 27 (5.7%) also presented with LiS (19 extragenital and 8 genital lesions). LiS exclusively occurred in patients with plaque-type (morphea) and generalized LoS. Twenty-six of the 27 (96.2%) patients with concomitant LoS and LiS were adults. Compared with LiS in the general population, LiS was significantly more frequent in LoS as indicated by an odds ratio of 18.1 (95% confidence interval 2.6-134.2; P morphea, should be carefully screened for concomitant LiS, including inspection of the anogenital region. Copyright © 2012 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  14. Malignancy in scleroderma patients from south west England: a population-based cohort study.

    LENUS (Irish Health Repository)

    Siau, Keith

    2010-01-08

    The pathophysiological relationship between scleroderma and malignancy remains poorly understood. Although some previous studies have demonstrated an increased malignancy risk in patients with scleroderma, others have been inconclusive. We aimed to determine if patients with scleroderma had an increased risk of malignancy compared to an age- and sex-matched local South West England population, and if there were any important differences between scleroderma patients with and without malignancy. Methods of this study are as follows. Notes were obtained on all local scleroderma patients (n = 68) locally, and those diagnosed with malignancy verified by contacting each patient\\'s general practitioner. Expected malignancy figures were obtained from age- and sex-stratified regional prevalence data provided by the South West Cancer Intelligence Service registry. Among the patients, 22.1% with scleroderma were identified with concurrent malignancy. Affected sites were of the breast (n = 5), haematological system (n = 5), skin (n = 4), and unknown primary (n = 1). Overall, malignancy risk was found to be increased in scleroderma (RR = 3.15, 95% CI 1.77-5.20, p = 0.01). In particular, this risk was the highest for haematological malignancies (RR = 18.5, 95% CI 6-43, p = 0.03), especially for non-Hodgkin\\'s lymphoma (RR = 25.8, 95% CI 5-75, p = 0.10). The majority of patients (86.7%) developed malignancy after the onset of scleroderma (mean = 6.9 years). Age of >70 and patients with limited scleroderma were significant risk factors for a patient with scleroderma to have a concurrent malignancy; however, no increased risk was found in patients with any particular pattern of organ involvement, cytotoxic usage or serology. To conclude, in this small patient cohort, we have found that scleroderma is associated with an increased risk of malignancy. This risk is statistically significant in patients with limited scleroderma. Patients who are elderly and those with limited disease

  15. Esclerodermia localizada na criança: aspectos clínicos, diagnósticos e terapêuticos Localized scleroderma in children: clinical, diagnostic and therapeutic aspects

    Directory of Open Access Journals (Sweden)

    Pedro C. Q. Zancanaro

    2009-04-01

    Full Text Available A esclerodermia localizada, ou morféia, acomete crianças em idade escolar e, em geral, é autolimitada, apesar de localmente desfiguradora. A literatura descreve inúmeros fatores etiopatogênicos, bem como modalidades de investigação e tratamento. Este artigo reúne os estudos mais recentes e discute sua aplicação clínica.Localized scleroderma or morphea affects school-aged children, is usually self-limited and a disfiguring condition. Several etiopathogenic factors, investigations and treatment options are described. This article reviews the recent literature and discusses its clinical applications.

  16. Scleroderma: a case report

    Science.gov (United States)

    Harahap, T. A.; Marpaung, B.

    2018-03-01

    Scleroderma is a complex disease in which extensive fibrosis, vascular alterations, and autoantibodies against various cellular antigens are among the principal features.[1,2] The prevalenceranging from 50 to 300 cases per 1 million persons with women are at much higher risk. The average age of diagnosis is the fifth decades of life.[2] There is no cure for scleroderma, but many of its problems and complications can be treated.[3-7]A 54-year-old female patient with main complains limitation of motion and mouth, stiffness and painful joints in the hands and feet, thickening on the skin in the chest and trunk for eight years, purplish red spots on arms and legs intermittent for tenyears. On physical examination found sclerosis lesions, sclerodactyly on fingers and toes, telangiectasias in the antebrachii and cruris region. On laboratory, examination showed ANA test 10.7 and Anti DS DNA 123. The histopathological of the skin result is scleroderma. The patient was diagnosed with scleroderma and treated with methotrexate 7.5 mg/weeks, ciclosporin 2×100 mg/day, omeprazole 2×20 mg. After seven days of therapy, there is aclinical improvement, and the patient becomes anoutpatient treatment.

  17. Prospective study to evaluate the clinical and radiological outcome of patients with scleroderma of the face.

    Science.gov (United States)

    Careta, Mariana Figueiroa; Leite, Claudia da Costa; Cresta, Fernando; Albino, Jose; Tsunami, Mirian; Romiti, Ricardo

    2013-09-01

    Scleroderma featuring rare connective tissue disease that manifests as skin sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement and localized scleroderma or morphea which classically presents benign evolution and self-limited, confined to the skin and/or underlying tissue. Recent studies show that the localized form may possibly course with involvement of internal organs and variable morbidity. This study aimed to determine the demographic characteristics, the prevalence of systemic manifestations and laboratory findings, as well as the association with autoimmune diseases, and the evolution of neurological findings, both clinical as brain MRI in patients with scleroderma of the face and its relation with the activity skin. Patients with localized scleroderma with facial involvement were evaluated and underwent neurological examination, magnetic resonance imaging and ophthalmology evaluation. After 3years, the patients were subjected again to MRI. We studied 12 patients with localized scleroderma of the face. Of this total, headache being the most frequent complaint found in 66.7% of patients, 33.3% had neurological changes possibly associated with scleroderma. As for ophthalmologic evaluation, 25% of patients showed abnormalities. The most frequent parenchymal finding was the presence of lesions with hyperintense or hypointense signal in 75% of patients, followed by ventricular asymmetry at 16.7%. Of the patients who had neurological deficits, 75% also had a change to MRI. In all patients, imaging findings after 3years were unchanged. During this interval of 3years, 25% of patients showed signs of activity of scleroderma. Patients with localized scleroderma of the face have a high prevalence of neurological and ophthalmological changes. Based on these findings, we suggest that all cases of localized scleroderma of the face should be thoroughly

  18. Evaluation of liver function tests in scleroderma patients.

    Science.gov (United States)

    Salem, Gehan Ibrahim Abdelrazek; Abdulrahman, Awni Ali

    2012-08-01

    Systemic sclerosis is a clinically heterogeneous, systemic disorder which affects the connective tissue of the skin, internal organs, and the walls of blood vessels. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen and other matrix substances in the connective tissue. This study was done to evaluate the frequency of liver disease in patients with scleroderma and, secondarily, to study the frequency of infection of hepatitis B and C virus in these patients and determine frequency of serum auto-antibodies in this disease. We studied patients with scleroderma, localized or systemic, in the outpatient clinic of rheumatology and dermatology departments, at King Khalid University Hospital. As for a comparison, healthy persons coming to the clinic with the same mean age were considered as control group. Forty patients with the diagnosis of scleroderma included in this work, 35% had elevated gamma-glutamyl-transferase (γ-GT), 30% had elevated alkaline phosphatase (AP) and in 17.5%, the alanine-amino-transferase (ALT) was above the reference values. The ALT had changed to be more in scleroderma patients than in controls. Twenty percent (20%) of the patients tested positive for anti-smooth muscle antibodies (anti-SMA) and only one patient had anti-mitochondrial antibodies (AMA). There was no statistical difference between the two groups regarding antibody testing. Anti-HCV antibodies were observed in one patient, and HBsAg was detected in another scleroderma patient. There was no patient with clinically significant hepatic disease. In this study, although changes in liver enzymes in patients with scleroderma were not uncommon, there was no scleroderma patient with clinical manifestations of liver disease.

  19. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland.

    Science.gov (United States)

    Herrick, Ariane L; Ennis, Holly; Bhushan, Monica; Silman, Alan J; Baildam, Eileen M

    2010-02-01

    Childhood scleroderma encompasses a rare, poorly understood spectrum of conditions. Our aim was to ascertain the incidence of childhood scleroderma in its different forms in the UK and Ireland, and to describe the age, sex, and ethnicity of the cases. The members of 5 specialist medical associations including pediatricians, dermatologists, and rheumatologists were asked to report all cases of abnormal skin thickening suspected to be localized (including linear) scleroderma or systemic sclerosis (SSc) in children scleroderma and 7 (7%) with SSc. This gave an incidence rate per million children per year of 3.4 (95% confidence interval [95% CI] 2.7-4.1) for localized scleroderma, including an incidence rate of 2.5 (95% CI 1.8-3.1) for linear scleroderma, and 0.27 (95% CI 0.1-0.5) for SSc. Of the 87 localized cases, 62 (71%) had linear disease. Of localized disease cases, 55 (63%) were female, 71 (82%) were classified as white British, and the patients' mean age when first seen in secondary care was 10.4 years. Of the 7 SSc cases, all were female, 6 (86%) were white British, and the mean age when first seen was 12.1 years. The median delay between onset and being first seen was 13.1 months for localized scleroderma and 7.2 months for SSc. These data provide additional estimates of the incidence of this rare disorder and its subforms.

  20. Neurologic involvement in scleroderma: a systematic review.

    Science.gov (United States)

    Amaral, Tiago Nardi; Peres, Fernando Augusto; Lapa, Aline Tamires; Marques-Neto, João Francisco; Appenzeller, Simone

    2013-12-01

    To perform a systematic review of neurologic involvement in Systemic sclerosis (SSc) and Localized Scleroderma (LS), describing clinical features, neuroimaging, and treatment. We performed a literature search in PubMed using the following MeSH terms, scleroderma, systemic sclerosis, localized scleroderma, localized scleroderma "en coup de sabre", Parry-Romberg syndrome, cognitive impairment, memory, seizures, epilepsy, headache, depression, anxiety, mood disorders, Center for Epidemiologic Studies Depression (CES-D), SF-36, Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Patient Health Questionnaire-9 (PHQ-9), neuropsychiatric, psychosis, neurologic involvement, neuropathy, peripheral nerves, cranial nerves, carpal tunnel syndrome, ulnar entrapment, tarsal tunnel syndrome, mononeuropathy, polyneuropathy, radiculopathy, myelopathy, autonomic nervous system, nervous system, electroencephalography (EEG), electromyography (EMG), magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA). Patients with other connective tissue disease knowingly responsible for nervous system involvement were excluded from the analyses. A total of 182 case reports/studies addressing SSc and 50 referring to LS were identified. SSc patients totalized 9506, while data on 224 LS patients were available. In LS, seizures (41.58%) and headache (18.81%) predominated. Nonetheless, descriptions of varied cranial nerve involvement and hemiparesis were made. Central nervous system involvement in SSc was characterized by headache (23.73%), seizures (13.56%) and cognitive impairment (8.47%). Depression and anxiety were frequently observed (73.15% and 23.95%, respectively). Myopathy (51.8%), trigeminal neuropathy (16.52%), peripheral sensorimotor polyneuropathy (14.25%), and carpal tunnel syndrome (6.56%) were the most frequent peripheral nervous system involvement in SSc. Autonomic neuropathy involving cardiovascular and gastrointestinal systems was regularly described

  1. Scleroderma, Stress and CAM Utilization

    Directory of Open Access Journals (Sweden)

    Ka-Kit Hui

    2009-01-01

    Full Text Available Scleroderma is an autoimmune disease influenced by interplay among genetic and environmental factors, of which one is stress. Complementary and alternative medicine (CAM is frequently used to treat stress and those diseases in which stress has been implicated. Results are presented from a survey of patients with scleroderma. Respondents were a convenient sample of those attending a national conference in Las Vegas in 2002. Findings implicate stress in the onset, continuation and exacerbation of scleroderma. The implication is that CAM providers may be filling an important patient need in their provision of services that identify and treat stress and its related disorders.

  2. International Team Identifies Biomarker for Scleroderma

    Science.gov (United States)

    ... Spotlight on Research International Team Identifies Biomarker for Scleroderma By Kirstie Saltsman, Ph.D. | May 5, 2014 ... molecule correlates with a more severe form of scleroderma, a chronic autoimmune disorder that involves the abnormal ...

  3. Scleroderma in hospital settings in Lomé: 50 cases.

    Science.gov (United States)

    Akakpo, A S; Teclessou, J N; Mouhari-Touré, A; Saka, B; Matakloe, H; Kakpovi, K; Kombate, K; Pitché, P

    2017-11-01

    The aim of this study was to document the epidemiological and clinical profile, treatment used, and outcome of patients with scleroderma in hospital settings in Lomé. This descriptive study examined the records of all patients seen as outpatients or admitted for scleroderma in hospital dermatology and rheumatology departments in Lomé during the 20-year period of 1993-2012. During the study period, 50 (0.04%) of the 121,021 patients seen in these departments had scleroderma. There were 29 cases of localized scleroderma and 21 systemic cases, predominantly women (sex-ratio=0.2). The patients' mean age was 36 years. All patients with systemic scleroderma had speckled achromia (100%), and most (90.48%) had cutaneous sclerosis. After a mean follow-up period of 43.5 days, 71.43% of the patients had been lost to follow-up. All of the patients with localized scleroderma had cutaneous sclerosis, and the rate of loss to follow-up (after a mean of 17 days) was 96.55%. The results of this study confirm the extreme rarity of scleroderma in the teaching hospitals in Lomé and a clear female predominance. It points out the difficulty of management, which both influences and is aggravated by the high rate of loss to follow-up.

  4. [Microstomia as a complication of scleroderma

    NARCIS (Netherlands)

    Phoa, K.H.; Baat, P. de; Baat, C. de

    2003-01-01

    Scleroderma is a rare connective tissue disease of unknown origin. It is characterized by increased collagen deposition leading to fibrosis and degeneration of the skin and sometimes of internal organs. There are two main groups of scleroderma: circumscribed scleroderma and systemic sclerosis.

  5. [3H]thymidine labeling of dermal endothelial cells in scleroderma

    International Nuclear Information System (INIS)

    Fleischmajer, R.; Perlish, J.S.

    1977-01-01

    The purpose of this study was to estimate [ 3 H] thymidine labeling of endothelial cells of skin capillaries in localized scleroderma (LS) and systemic scleroderma (SS). Skin specimens from 14 patients with SS, 5 with LS, and 9 matched controls were studied by in vitro autoradiography. Capillaries from patients with SS showed a statistically significant increase in endothelial cell labeling when compared to vessels from controls

  6. Scleroderma: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.

    Science.gov (United States)

    Fett, Nicole

    2013-01-01

    Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The nomenclature of scleroderma has changed dramatically in recent years, with morphea (localized scleroderma), limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, and systemic sclerosis sine scleroderma encompassing the currently accepted disease subtypes. Major advances have been made in the molecular studies of morphea and systemic sclerosis; however, their etiologies and pathogenesis remain incompletely understood. Although morphea and systemic sclerosis demonstrate activation of similar inflammatory and fibrotic pathways, important differences in signaling pathways and gene signatures indicate they are likely biologically distinct processes. Morphea can cause significant morbidity but does not affect mortality, whereas systemic sclerosis has the highest disease-specific mortality of all autoimmune connective tissue diseases. Treatment recommendations for morphea and systemic sclerosis are based on limited data and largely expert opinions. Current collaborative efforts in morphea and systemic sclerosis research will hopefully lead to better understanding of the etiology and pathogenesis of these rare and varied diseases and improved treatment options. Published by Elsevier Inc.

  7. Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports

    Directory of Open Access Journals (Sweden)

    Laxer Ronald M

    2011-07-01

    Full Text Available Abstract Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic resonance imaging. Using a worldwide pediatric rheumatology electronic list-serve, another patient with the same 2 conditions was identified. These two patients are reported in this study. Consideration of neuroimaging studies to disclose abnormal findings in patients with linear scleroderma en coup de sabre is important for potentially preventing and treating neurological manifestations associated with this condition.

  8. Influence of childhood scleroderma on physical function and quality of life.

    Science.gov (United States)

    Baildam, Eileen M; Ennis, Holly; Foster, Helen E; Shaw, Lindsay; Chieng, Alice S E; Kelly, Jane; Herrick, Ariane L; Richards, Helen L

    2011-01-01

    there have been few studies of quality of life in childhood scleroderma and these focused predominantly on self-perception and the influence of skin lesions. Our cross-sectional study aimed to describe the influence of childhood scleroderma on physical function and quality of life in relation to clinical and demographic measures. children with either localized scleroderma or systemic sclerosis (SSc) attending pediatric rheumatology clinics, together with their parents or guardians, were asked to complete a set of 4 validated measures. Clinical and demographic data were provided by consultant pediatric rheumatologists. in total, 28 children and their parents/guardians participated in the study (68% female, median age 13 yrs; 86% localized scleroderma, 14% SSc). The median Child Health Assessment Questionnaire (CHAQ) score was 0.1 (range 0-3, 0 indicating no impairment), the median Child Dermatology Life Quality Index (CDLQI) score was 5 (range 0-30, 0 indicating no impairment), and the median Child Quality of Life Questionnaire (CQOL) function score was 26 (range 0-105, 0 indicating no impairment). Family activity, measured by the Child Health Questionnaire (CHQ-PF50), was also moderately impaired by scleroderma, with a median score of 83 (0-100, 100 indicating no impairment). scleroderma had only a moderate effect on quality of life and physical function as measured by the 4 validated instruments. Although a small number of children reported greater impairment, this is an encouraging finding, given its potential disfiguring and debilitating effects.

  9. Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma

    DEFF Research Database (Denmark)

    Krabbe, Simon; Engelhart, Merete; Thybo, Sören

    2017-01-01

    This case report describes a patient with scleroderma who developed Mycobacterium intracellulare infection, which for more than a year mimicked worsening of her connective tissue disorder. The patient was diagnosed with scleroderma based on puffy fingers that developed into sclerodactyly, abnormal......, unfortunately with significant scarring. Immunodeficiency testing was unremarkable. In summary, an infection with Mycobacterium intracellulare was mistaken for an unusually severe progression of scleroderma....

  10. Scleroderma mimicker – Eosinophilic fasciitis

    Directory of Open Access Journals (Sweden)

    Debanjali Sinha

    2017-01-01

    Full Text Available Eosinophilic fasciitis is an uncommon connective tissue disorder characterized by thickening of the deep fascia and overlying skin and subcutaneous tissue. It may mimic scleroderma and other scleroderma-like conditions. It may be a manifestation of paraneoplastic disorders or may be associated with hematological disorders including lymphomas. Definitive diagnosis is made on histological examination of a deep skin biopsy revealing thickened deep fascia and infiltration by lymphocytes and eosinophils. Enhancement of deep fascia on Gadolinium contrast-enhanced magnetic resonance imaging may be used as a substitute for skin biopsy. Ultrasound imaging is an evolving imaging tool for diagnosing it. Glucocorticoids with or without immunosuppressive agents remains the mainstay of therapy with good response, generally. A younger age of onset, morphea like lesions and dermal fibrosclerosis is more likely to be associated with the refractory disease. Early diagnosis and appropriate treatment may result in better outcomes in terms of morbidity and quality of life of the patients.

  11. Correlação clínica e ultra-sonográfica na esclerodermia localizada cutânea Clinical and ultrasonographic correlation in localized cutaneous scleroderma

    Directory of Open Access Journals (Sweden)

    Marcio Bouer

    2008-04-01

    Full Text Available OBJETIVO: Apresentar os aspectos ultra-sonográficos da esclerodermia localizada e relacioná-los com os aspectos clínicos. MATERIAIS E MÉTODOS: Foram analisadas 23 lesões de esclerodermia localizada em 21 pacientes. Foi utilizado equipamento Logiq 700 com transdutor linear de 6-14 MHz. Foram avaliados, pelo dermatologista, o estágio da doença (inflamatório ou atrófico, e pelo radiologista, a espessura e a ecogenicidade da derme nas regiões afetadas e sãs adjacentes. Foi feito acompanhamento de sete casos após tratamento. RESULTADOS: Todas as lesões apresentaram perda do padrão ultra-sonográfico normal da derme. Os casos de lesão clinicamente atrófica (52,2%; 12/23 corresponderam a redução da espessura e aumento da ecogenicidade da derme e os casos de lesão clinicamente inflamatória (47,8%; 11/23 corresponderam a aumento da espessura e redução da ecogenicidade da derme. Controles pós-tratamento mostraram alterações na espessura da derme. CONCLUSÃO: Os achados ultra-sonográficos nos permitem associar o aumento da espessura e a redução da ecogenicidade da derme com a fase inflamatória da doença, e a redução da espessura e o aumento da ecogenicidade da derme com a fase atrófica da doença. Notamos também que é possível quantificar a espessura da derme e usar essa informação no controle pós-tratamento associada à avaliação clínica.OBJECTIVE: To describe ultrasonographic findings of localized cutaneous scleroderma and correlating them with clinical findings. MATERIALS AND METHODS: Twenty-three lesions of localized cutaneous scleroderma in 21 patients were evaluated with a Logiq 700 equipment coupled with a 6-14 MHz linear transducer. The disease stage (athrophic or inflammatory was evaluated by a dermatologist, and the ultrasonographic findings (skin thickness and echogenicity for both the affected and adjacent healthy regions were evaluated by a radiologist. Seven of the cases underwent post

  12. Neurologic Involvement in Scleroderma en Coup de Sabre

    Science.gov (United States)

    Amaral, Tiago Nardi; Marques Neto, João Francisco; Lapa, Aline Tamires; Peres, Fernando Augusto; Guirau, Caio Rodrigues; Appenzeller, Simone

    2012-01-01

    Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs. PMID:22319646

  13. Targeting miR-155 to Treat Experimental Scleroderma.

    Science.gov (United States)

    Yan, Qingran; Chen, Jie; Li, Wei; Bao, Chunde; Fu, Qiong

    2016-02-01

    Scleroderma is a refractory autoimmune skin fibrotic disorder. Alterations of microRNAs in lesional skin could be a new approach to treating the disease. Here, we found that expression of miR-155 was up regulated in lesional skin tissue from patients with either systemic or localized scleroderma, and correlated with fibrosis area. Then we demonstrated the potential of miR-155 as a therapeutic target in pre-clinical scleroderma models. MiR-155(-/-) mice were resistant to bleomycin induced skin fibrosis. Moreover, topical antagomiR-155 could effectively treat mice primed with subcutaneous bleomycin. In primary skin fibroblast, miR-155 silencing could inhibit collagen synthesis function, as well as signaling intensity of two pro-fibrotic pathways, Wnt/β-catenin and Akt, simultaneously. We further showed that miR-155 could regulate the two pathways via directly targeting casein kinase 1α (CK1α) and Src homology 2-containing inositol phosphatase-1 (SHIP-1), as previous reports. Mice with miR-155 knockout or topical antagomir-155 treatment showed inhibited Wnt/β-catenin and Akt signaling in skin upon bleomycin challenge. Together, our data suggest the potential of miR-155 silencing as a promising treatment for dermal fibrosis, especially in topical applications.

  14. Corticosteroids in Myositis and Scleroderma.

    Science.gov (United States)

    Postolova, Anna; Chen, Jennifer K; Chung, Lorinda

    2016-02-01

    Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering. Published by Elsevier Inc.

  15. Biomarkers in scleroderma: Current status

    Directory of Open Access Journals (Sweden)

    Latika Gupta

    2017-01-01

    Full Text Available Scleroderma is an autoimmune disease characterized by indolent obliterative vasculopathy and widespread fibrosis. The two main morphological manifestations of the disease overlap and may make it difficult to separate activity from damage. Many patients, especially those with the limited subset of the disease, have an indolent course without clear-cut inflammatory manifestations. There is a felt need for validated biomarkers, which can differentiate activity from damage, and yet be sensitive to change with therapy. Multiplex arrays of biomarkers have ushered an era of targeted or personalized medicine based on phenotypic characteristics in an individual.

  16. Subcutaneous administration of polymerized type I collagen downregulates interleukin (IL)-17A, IL-22 and transforming growth factor-β1 expression, and increases Foxp3-expressing cells in localized scleroderma.

    Science.gov (United States)

    Furuzawa-Carballeda, J; Ortíz-Ávalos, M; Lima, G; Jurado-Santa Cruz, F; Llorente, L

    2012-08-01

    Localized scleroderma (LS) is a disfiguring inflammatory autoimmune disease of the skin and underlying tissue. As in systemic sclerosis, a key feature is the presence of T cells in inflammatory lesions. To evaluate the effect of polymerized type I collagen vs. methylprednisolone (MP) in LS, and to determine the influence of this polymerized collagen (PC) on CD4+ peripheral T cells expressing interleukin (IL)-4, IL-17A, interferon-γ and Forkhead box protein (Foxp)3, and on cells expressing transforming growth factor (TGF)-β1, IL-17A, IL-22 and Foxp3 in the skin. In total, 16 patients with LS were treated for 3 months with monthly subcutaneous intralesional injections of 0.1 mL MP (giving a total dose of 20 mg/mL each month) and 15 patients were treated, with weekly subcutaneous intralesional injections of PC, ranging from 0.2 mL (equivalent to 1.66 mg collagen) for a lesion of 50 mm in size, up to a maximum of 1.0 mL (8.3 mg collagen) for a lesion > 100 mm in size, and followed up for a further 6 months. Skin biopsies were obtained from lesions at baseline (before treatment) and 9 months later (6 months after treatment end). Tissue sections were evaluated by histology and immunohistochemistry (IL-17A, IL-22, TGF-β1 and Foxp3). CD4+ T-cell subsets were determined in peripheral blood by flow cytometry. Abnormal tissue architecture was seen in the biopsies taken from patients treated with MP, whereas the PC treatment restored normal skin architecture. PC downregulated pro-inflammatory/profibrotic cytokine expression in peripheral cells, and upregulated the number of regulatory T cells (Tregs) in skin. PC was safe and well tolerated. PC is not only an antifibrotic/fibrolytic agent but also an immunomodulator biodrug that restores the balance between T helper (Th)1, Th2, Th17 and Tregs, downregulates production of pro-inflammatory or profibrogenic cytokines (IL-17A, IL-22 and TGF-β1), and renews skin architecture, without adverse effects. © The Author(s). CED

  17. Esophagectomy in Scleroderma: Report of a Case

    Directory of Open Access Journals (Sweden)

    Erdal Yekeler

    2008-12-01

    Full Text Available Scleroderma is a generalized autoimmune disease with variable involvement of the skin and major organs (esophagus, lung, heart and kidney. Scleroderma is essentially a skin disease that frequently involves the digestive system. In scleroderma, the esophagus is the most frequently affected organ of the digestive system, and esophageal dysmotility, reflux and stricture may be observed in the advanced stage. Balloon dilatation and bougienage are generally sufficient in patients developing stricture, and the number of cases in whom resection is performed is very low. In a 20-year-old patient with difficulty in taking even liquid foods, tests revealed sclerodermal involvement of the distal end of the esophagus and stricture. Esophageal resection and gastric replacement were performed. Such systemic diseases as scleroderma, although rare, must be considered in the differential diagnosis of nonmalignant dysphagia, and resection must be borne in mind as a surgical option in cases of advanced stricture.

  18. UVA1 a promising approach for scleroderma

    Science.gov (United States)

    Keyal, Uma; Bhatta, Anil Kumar; Wang, Xiu Li

    2017-01-01

    Scleroderma is a complex connective tissue disease characterized by fibrosis, vasculopathy, and immune system dysfunction. The heterogeneity of disease presentation and poorly understood etiology has made the management of scleroderma difficult. The available treatment options like immunosuppressive agents are associated with potentially hazardous side effects and physiotherapy, which to a certain degree helps to minimize the loss of function in digits and limbs, has only limited success. Also, studies investigating antifibrotic therapies have failed to report any significant improvement. Hence, there is currently no effective therapy for scleroderma. Recently, phototherapy has been extensively studied and found to be effective in treating scleroderma. Initially psoralen + ultraviolet A (PUVA) significantly enriched the therapeutic panel, but more recently ultraviolet A1 (UVA1) is seen to replace PUVA therapy. This might be because of UVA1 therapy being free of side effects seen with psoralens such as nausea, vomiting or photokeratitis. In addition, UVA1 is seen to lower risk of phototoxic reactions with deeper penetration of radiation. The present review will put some light on the use of UVA1 for treating cutaneous lesion in scleroderma and we aim to find the most benefitted group of patients and most effective dose of UVA1 for different types of scleroderma. PMID:28979701

  19. Keloidal Scleroderma: Case Report and Review

    Directory of Open Access Journals (Sweden)

    Sama Kassira

    2015-01-01

    Full Text Available Objective. We report a rare case of keloidal scleroderma and provide an analysis of similar cases. Results. A 41 year-old woman presented with dark brown, indurated, exophytic nodules over the chest along with smaller hyperpigmented plaques scattered over the abdomen, with concomitant sclerodactyly. The clinical, laboratory, and pathological findings were consistent with a diagnosis of keloidal scleroderma. The patient was treated with methotrexate, resulting in reduced firmness of her plaques and no new lesions. A literature review of previously reported cases was performed using keywords including keloidal morphea, keloidal scleroderma, nodular morphea, and nodular scleroderma. In our review, the majority of patients were African American and female. 91% of cases had nodular lesions with distribution on the trunk. The majority of patients exhibited sclerodactyly and pulmonary involvement was reported in 28%1. The majority of patients were ANA positive (63% and only 10% demonstrated anti-SCL-70 positivity. Conclusion. Keloidal scleroderma is a rare presentation, which can often be clinically confused with keloid and scar formation. Due to this being a rare variant, our knowledge of treatment options and efficacy is limited. Methotrexate could be considered as an initial treatment option for patients with progressive keloidal scleroderma.

  20. Scleroderma and dentistry: Two case reports.

    Science.gov (United States)

    Dixit, Shantanu; Kalkur, Chaithra; Sattur, Atul P; Bornstein, Michael M; Melton, Fred

    2016-10-24

    Scleroderma is a chronic connective tissue disorder with unknown etiology. It is characterized by excessive deposition of extracellular matrix in the connective tissues causing vascular disturbances which can result in tissue hypoxia. These changes are manifested as atrophy of the skin and/or mucosa, subcutaneous tissue, muscles, and internal organs. Such changes can be classified into two types, namely, morphea (localized) and diffuse (systemic). Morphea can manifest itself as hemifacial atrophy (Parry-Romberg syndrome) although this remains debatable. Hence, we present a case of morphea, associated with Parry-Romberg syndrome, and a second case with the classical signs of progressive systemic sclerosis. Case one: A 20-year-old man of Dravidian origin presented to our out-patient department with a complaint of facial asymmetry, difficulty in speech, and loss of taste sensation over the last 2 years. There was no history of facial trauma. After physical and radiological investigations, we found gross asymmetry of the left side of his face, a scar on his chin, tongue atrophy, relative microdontia, thinning of the ramus/body of his mandible, and sclerotic lesions on his trunk. Serological investigations were positive for antinuclear antibody for double-stranded deoxyribonucleic acid and mitochondria. A biopsy was suggestive of morphea. Hence, our final diagnosis was mixed morphea with Parry-Romberg syndrome. Case two: A 53-year-old woman of Dravidian origin presented to our out-patient department with a complaint of gradually decreasing mouth opening over the past 7 years. Her medical history was noncontributory. On clinical examination, we found her perioral, neck, and hand skin to be sclerotic. Also, her fingers exhibited bilateral telangiectasia. An oral examination revealed completely edentulous arches as well as xerostomia and candidiasis. Her serological reports were positive for antinuclear antibodies against centromere B, Scl-70, and Ro-52. A hand and

  1. Validation of the Body Concealment Scale for Scleroderma (BCSS): Replication in the Scleroderma Patient-centered Intervention Network (SPIN) Cohort

    NARCIS (Netherlands)

    Jewett, L.R.; Kwakkenbos, C.M.C.; Carrier, M.E.; Malcarne, V.L.; Harcourt, D.; Rumsey, N.; Mayes, M.D.; Assassi, S.; Körner, A.; Fox, R.S.; Gholizadeh, S.; Mills, S.D.; Fortune, C.; Thombs, B.D.

    2017-01-01

    Body concealment is an important component of appearance distress for individuals with disfiguring conditions, including scleroderma. The objective was to replicate the validation study of the Body Concealment Scale for Scleroderma (BCSS) among 897 scleroderma patients. The factor structure of the

  2. Scleroderma

    Science.gov (United States)

    ... this research; and the dissemination of information on research progress in these diseases. Contact Us NIAMS Archive Viewers and Players Social Media Moderation Policy FOIA Privacy Statement Accessibility Disclaimer Digital Strategy ...

  3. Current and future direction in the management of scleroderma.

    Science.gov (United States)

    Brady, Sean M; Shapiro, Lee; Mousa, Shaker A

    2016-09-01

    Scleroderma is a heterogeneous disease with a complex etiology. As more information is gained about the underlying mechanisms and the improved classifications of scleroderma subtypes, treatments can be better personalized. Improving scleroderma patients' early diagnosis before end organ manifestations occur should improve clinical trial design and outcomes. Two recently FDA-approved antifibrotics for idiopathic pulmonary fibrosis may be effective treatments in patients with pulmonary fibrosis secondary to scleroderma after further investigation. The potential impact of Nanobiotechnology in improving the efficacy and safety of existing antifibrotics and immunomodulators might present an exciting new approach in the management of scleroderma.

  4. Abnormalities in the Regulators of Angiogenesis in Patients with Scleroderma

    Science.gov (United States)

    HUMMERS, LAURA K.; HALL, AMY; WIGLEY, FREDRICK M.; SIMONS, MICHAEL

    2014-01-01

    Objective To determine plasma levels of regulators of angiogenesis in patients with scleroderma and to correlate those levels with manifestations of scleroderma-related vascular disease. Methods Plasma levels of vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), fibroblast growth factor-2 (FGF-2), matrix metalloproteinase-9 (MMP-9), endostatin, pro-MMP-1, hepatocyte growth factor (HGF), placental growth factor (PlGF), and FGF-4 were examined by ELISA in a cross-sectional study of 113 patients with scleroderma and 27 healthy controls. Simple and multivariate regression models were used to look for associations between factor levels and clinical disease characteristics. Results There were marked differences in the levels of pro-angiogenic growth factors between patients with scleroderma and controls, with significant elevations of VEGF, PDGF, FGF-2, and PlGF among patients with scleroderma (p scleroderma patients compared to controls (MMP-9 and pro-MMP-1) (p scleroderma, but had a positive correlation with right ventricular systolic pressure (RVSP) as measured by echocardiogram (p scleroderma (p scleroderma. The levels of some factors correlate with measures of vascular disease among patients with scleroderma. Dysregulated angiogenesis may play a role in the development of scleroderma vascular disease. PMID:19228661

  5. Caring for the Patient With Limited Systemic Scleroderma.

    Science.gov (United States)

    Lachner, Kelly Denise

    2016-01-01

    Systemic scleroderma (systemic sclerosis) is a rare, autoimmune, collagen-vascular disease of unknown etiology that affects the connective tissues of the skin, internal organs, as well as the small blood vessels. There are 3 subclasses of systemic scleroderma: limited cutaneous, diffuse cutaneous, and sine scleroderma. Prognosis depends on the extent of organ involvement. Complications of systemic scleroderma can involve the cardiovascular, pulmonary, gastrointestinal, renal, integumentary, and the skeletal-muscular systems. Because systemic scleroderma is not common, many orthopaedic nurses may be unfamiliar with how to best provide care. This article provides information about the complexity of the different types of this disease and the basic nursing care of the patient with the most common subclass of systemic scleroderma, limited cutaneous systemic scleroderma.

  6. An unusual dental phenomenon in systemic scleroderma

    International Nuclear Information System (INIS)

    ElCridly, Mahmoud

    1989-01-01

    Systemic scleroderma is a disease of unknown etiology characterized by excessive deposition of collagen and other connective tissue components in the skin and several internal organs. The most common oral findings are rigidity and thinness of lips, circumoral fibrosis which causes microstomia and inability to open the mouth widely. Involvement of tongue may lead to its decreased mobility and diminished size. Radiographic findings that have been classically associated with systemic scleroderma are widening of the periodontal ligament spaces and destruction of lamina dura, usually in posterior teeth. Intraoral radiographs of presented cases revealed apical resorption of the roots and destruction of the lamina dura verified on extraction of the teeth. This oral finding appears not to have been recorded by previous investigators of scleroderma. (author)

  7. Linear scleroderma following Blaschko′s lines

    Directory of Open Access Journals (Sweden)

    Mukhopadhyay Amiya

    2005-01-01

    Full Text Available Blaschko′s lines form a pattern, which many diseases are found to follow, but linear scleroderma following Blaschko′s lines is a controversial entity rarely reported in the literature. A 24-year-old man presented with multiple linear, atrophic, hyperpigmented lesions punctuated by areas of depigmentations on the left half of the trunk distributed on the anterior, lateral and posterior aspects. The lesions were distributed in a typical S-shaped line. Antinuclear antibody and antihistone antibody tests were negative. Histopathological examination of the skin from the affected area showed features suggestive of scleroderma. Here, we present a case of linear scleroderma following Blaschko′s lines in a male patient - an entity reported only three times so far.

  8. Nodular Scleroderma - Successful Treatment With Extracorporeal Photochemotherapy

    Directory of Open Access Journals (Sweden)

    Wollina U

    2001-01-01

    Full Text Available Nodular seleroderma is rare variant of circumscribed scleroderma (morphea. Treatment is often unsatisfactory. This report is on the use of extracorporeal photochemotherapy. A 12 year old girl and a 49 year old woman have been treated once a month on two consecutive days. A complete remission was achieved in one patient after 10 months and an almost complete remission in the young girl after 6 months. The treatment was well-tolerated and no severe side â€" effects occurred. In contrast to previous attempts in treating nodular scleroderma with different modalities, ECP seems to be an effective therapy.

  9. Acute and regressive scleroderma concomitant to an acute CMV primary infection.

    Science.gov (United States)

    Goulabchand, Radjiv; Khellaf, Lakhdar; Forestier, Amandine; Costes, Valerie; Foulongne, Vincent; le Quellec, Alain; Guilpain, Philippe

    2014-12-01

    To describe the pathophysiological mechanisms involving cytomegalovirus (CMV) primary infection and natural killer (NK) cell expansion in the development of localized scleroderma. A 43-year-old woman presented acute erythematous discoloration and skin thickening concerning face, neck, trunk, abdomen, and the four limbs, predominantly in proximal areas. Our case did not respond to systemic sclerosis criteria diagnosis. However, skin and muscle biopsy revealed early scleroderma associated with capillary thrombi, and tissue infiltration with NK cells (CD56+/Granzyme B). Scleroderma was attributed to CMV primary infection responsible for cytolytic hepatitis (7-fold over the limit) and circulating NK cell excess. After 6 months of prednisone and a 2-year follow-up, a complete resolution of symptoms was observed. Our observation suggests a potential triggering role of CMV primary infection in the development of scleroderma. Histological features from our observation addresses the role of CMV and NK cells in the development of endothelial damage and fibrotic process. Copyright © 2014 Elsevier B.V. All rights reserved.

  10. Association of Immunological Cell Profiles with Specific Clinical Phenotypes of Scleroderma Disease

    Science.gov (United States)

    Calzada, David; Mayayo, Teodoro; González-Rodríguez, María Luisa; Rabasco, Antonio María; Lahoz, Carlos

    2014-01-01

    This study aimed to search the correlation among immunological profiles and clinical phenotypes of scleroderma in well-characterized groups of scleroderma patients, comparing forty-nine scleroderma patients stratified according to specific clinical phenotypes with forty-nine healthy controls. Five immunological cell subpopulations (B, CD4+ and CD8+ T-cells, NK, and monocytes) and their respective stages of apoptosis and activation were analyzed by flow cytometry, in samples of peripheral blood mononuclear cells (PBMCs). Analyses of results were stratified according to disease stage, time since the diagnosis, and visceral damage (pulmonary fibrosis, pulmonary hypertension, and cardiac affliction) and by time of treatment with corticosteroids. An increase in the percentages of monocytes and a decrease in the B cells were mainly related to the disease progression. A general apoptosis decrease was found in all phenotypes studied, except in localized scleroderma. An increase of B and NK cells activation was found in patients diagnosed more than 10 years ago. Specific cell populations like monocytes, NK, and B cells were associated with the type of affected organ. This study shows how, in a heterogeneous disease, proper patient's stratification according to clinical phenotypes allows finding specific cellular profiles. Our data may lead to improvements in the knowledge of prognosis factors and to aid in the analysis of future specific therapies. PMID:24818126

  11. Esophageal dysmotility in scleroderma: a prospective study of 183 cases.

    Science.gov (United States)

    Lahcene, M; Oumnia, N; Matougui, N; Boudjella, M; Tebaibia, A; Touchene, B

    2009-01-01

    The goal of the study was to evaluate the prevalence and risk factors of esophageal motor disorders in systemic sclerosis. In 183 consecutive cases of scleroderma, as diagnosed by American College of Rheumatology criteria (1980). Patients' mean age was 40.6+/-13.3 years, the gender ratio was 0.13 and the average duration of disease was 6.8+/-7.5 years. A localized, cutaneous form was observed in 148 patients (81%) and a diffuse form in 35 (19%). All patients underwent upper gastrointestinal endoscopy and standard esophageal manometry. Esophageal symptoms and reflux esophagitis were found in 108 (59%) and 68 (37%) of patients, respectively. Esophageal motor disorders were present in 148 patients (81%), and were associated with a hypotensive lower esophageal sphincter in 114 (62%). The presence of these motor abnormalities was not related to age, gender, skin extension or duration of disease. Esophageal motor disorders were present in almost all patients with esophageal symptoms or esophagitis, and were also found in 48 (64%) of the asymptomatic patients. Esophageal motor disorders are frequently seen in scleroderma, especially in cases with clinical symptoms, but are not associated with a specific form of the disease.

  12. Aberrant Recapitulation of Developmental Program: Novel Target in Scleroderma

    Science.gov (United States)

    2015-12-01

    AWARD NUMBER: W81XWH-12-1-0472 TITLE: “Aberrant Recapitulation of Developmental Program: Novel Target in Scleroderma ” PRINCIPAL INVESTIGATOR...SUBTITLE Aberrant Recapitulation of Developmental Program: Novel Target in Scleroderma 5a. CONTRACT NUMBER W81XWH-12-1-0472 5b. GRANT NUMBER 5c. PROGRAM...SPONSOR/MONITOR’S REPORT NUMBER(S) 12. DISTRIBUTION / AVAILABILITY STATEMENT 13. SUPPLEMENTARY NOTES 14. ABSTRACT Fibrosis in scleroderma is associated

  13. Gastrointestinal symptoms and motility disorders in patients with systemic scleroderma

    Directory of Open Access Journals (Sweden)

    Palasciano Giuseppe

    2008-02-01

    Full Text Available Abstract Background Studies on gastrointestinal symptoms, dysfunctions, and neurological disorders in systemic scleroderma are lacking so far. Methods Thirty-eight scleroderma patients (34 limited, 4 diffuse, 60 healthy controls and 68 dyspeptic controls were scored for upper and lower gastrointestinal symptoms (dyspepsia, bowel habits, gastric and gallbladder emptying to liquid meal (functional ultrasonography and small bowel transit (H2-breath test. Autonomic nerve function was assessed by cardiovascular tests. Results The score for dyspepsia (mainly gastric fullness was greater in scleroderma patients than healthy controls, but lower than dyspeptic controls who had multiple symptoms, instead. Scleroderma patients with dyspepsia had a longer disease duration. Fasting antral area and postprandial antral dilatation were smaller in scleroderma patients than dyspeptic and healthy controls. Gastric emptying was delayed in both scleroderma patients (particularly in those with abnormal dyspeptic score and dyspeptic controls, who also showed a larger residual area. Despite gallbladder fasting and postprandial volumes were comparable across the three groups, gallbladder refilling appeared delayed in dyspeptic controls and mainly dependent on delayed gastric emptying in scleroderma. Small intestinal transit was also delayed in 74% of scleroderma and 66% of dyspeptic controls. Bowel habits were similar among the three groups. Autonomic neuropathy was not associated with dyspepsia, gastric and gallbladder motility and small intestinal transit. Conclusion In scleroderma patients dyspepsia (mainly gastric fullness, restricted distension of the gastric antrum and diffuse gastrointestinal dysmotility are frequent features. These defects are independent from the occurrence of autonomic neuropathy.

  14. Linear scleroderma en coup de sabre including abnormal dental development

    DEFF Research Database (Denmark)

    Hørberg, M; Lauesen, S R; Daugaard-Jensen, J

    2015-01-01

    BACKGROUND: Linear scleroderma en coup de sabre (SCS) is a rare skin condition, where dense collagen is deposited in a localised groove of the head and neck area resembling the stroke of a sabre. The SCS may involve the oral cavity, but the severity and relation to this skin abnormality is unknow...... with a left-sided skin defect (SCS) and a left-sided local malformation in her dentition. It is possible that there is a developmental connection between these two left-sided defects, both with an ectodermal origin.......-UP: The patient has been regularly controlled and treated since she was first diagnosed. A surgical and orthodontic treatment was performed to ensure optimal occlusion, space and alveolar bone development. The present age of the patient is 14 years and 10 months. CONCLUSION: This case demonstrated a patient...

  15. Polymyositis and the Spectrum of Scleroderma Disorders

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    Joana Cochicho

    2015-12-01

    Full Text Available Polymyositis (PM is usually associated to other autoimmune or connective tissue diseases. The authors report the case of a 59-year-old man with pulmonary fibrosis, who presented with constitutional symptoms and gradually developed proximal muscle weakness, Raynaud phenomenon, and dysphagia. Besides creatine kinase (CK elevation, he had positive anti-Polymyositis-Scleromyositis (PM-Scl and anti-Sjögren's-syndrome A (SSA antibodies. Nailfold capillaroscopy showed a scleroderma pattern and muscle biopsy revealed necrosis, regeneration of muscle fibers, and inflammatory infiltrate. Prednisolone was started, with great improvement. Taking into account the overlap features between PM and systemic sclerosis sine scleroderma, it is important to closely monitor the patient for signs of pulmonary and cardiac decompensation.

  16. Tumor-like calcifications with scleroderma

    International Nuclear Information System (INIS)

    Meyer, E.; Kulenkampff, H.A.; Kortenhaus, H.

    1987-01-01

    In patients with progressive scleroderma, interstitial calcifications are present to a varying extent. They are mostly located in the soft tissues of the fingers, resembling points, commas or dashes. They may also appear as 'calcinosis universalis' and reach a considerable size. Thus they mimic proliferative tumors. Scintigraphy, proving the existence of further calcifications can be helpful. We report the case of a female patient who presented with such a 'pseudotumor' of unusual size, site and extent in the lumbar region. (orig.) [de

  17. Progressive systemic scleroderma (A case report)

    International Nuclear Information System (INIS)

    Han, Jung Suh; Kwon, Chung Sik

    1974-01-01

    This is a report of a rare case of progressive systemic scleroderma involving the skin, lungs, bones, esophagus, duodenum, and colon in a 27 year old Korean female whose chief complaints were generalized edema, knee joint pain, hard and indurated skin lesions on the anterior chest, neck, both upper and lower extremities with loss of pain and touch sensation for one year. A review of literature is submitted

  18. Progressive systemic scleroderma (A case report)

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jung Suh; Kwon, Chung Sik [Chonnam University College of Medicine, Kwangju (Korea, Republic of)

    1974-10-15

    This is a report of a rare case of progressive systemic scleroderma involving the skin, lungs, bones, esophagus, duodenum, and colon in a 27 year old Korean female whose chief complaints were generalized edema, knee joint pain, hard and indurated skin lesions on the anterior chest, neck, both upper and lower extremities with loss of pain and touch sensation for one year. A review of literature is submitted.

  19. Gastroesophageal reflux demonstrated by hepatobiliary imaging in scleroderma

    International Nuclear Information System (INIS)

    Sawaf, N.W.; Orzel, J.A.; Weiland, F.L.

    1987-01-01

    Radionuclide hepatobiliary imaging was performed on a patient with a longstanding history of scleroderma who presented with abdominal pain suggestive of biliary disease. Cystic duct patency was documented after 10 min with tracer accumulation in the second portion of the duodenum which failed to progress consistent with the duodenal hypomotility of scleroderma. The patient was given intravenous Kinevac resulting in gastroesophageal reflux of radionuclide

  20. Gastroesophageal reflux demonstrated by hepatobiliary imaging in scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Sawaf, N.W.; Orzel, J.A.; Weiland, F.L.

    1987-03-01

    Radionuclide hepatobiliary imaging was performed on a patient with a longstanding history of scleroderma who presented with abdominal pain suggestive of biliary disease. Cystic duct patency was documented after 10 min with tracer accumulation in the second portion of the duodenum which failed to progress consistent with the duodenal hypomotility of scleroderma. The patient was given intravenous Kinevac resulting in gastroesophageal reflux of radionuclide.

  1. Phenomenon of isomorphic provoking responses in cases of limited scleroderma

    Directory of Open Access Journals (Sweden)

    Talnikova Е.Е.

    2015-09-01

    Full Text Available The article presents the historical origin of the term "Koebner phenomenon". The literature data reflect the etiology, pathogenesis and epidemiology of isomorphic mechanisms provoking responses in lichen planus, psoriasis, scleroder-ma, syphilis. Variants of the Koebner phenomenon's classifications are given. The clinical cases of limited scleroderma after mechanical injury are described.

  2. Atypical Neuroimaging Manifestations of Linear Scleroderma “en coup de sabre”

    Science.gov (United States)

    M. ALLMENDINGER, Andrew; A. RICCI, Joseph; S. DESAI, Naman; VISWANADHAN, Narayan; RODRIGUEZ, Diana

    2015-01-01

    Linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. Patients often present with neurologic symptoms. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Infratentorial lesions and other lesions not closely associated with the overlying scalp abnormality, such as those found in the cerebellum, have been reported, but are extremely uncommon. We present a case of an 8-year-old boy with a left fronto-parietal “en coup de sabre” scalp lesion and describe the neuroimaging findings of a progressively enlarging left cerebellar lesion discovered incidentally on routine magnetic resonance imaging. Interestingly, the patient had no neurologic symptoms given the size of the mass identified. PMID:26401155

  3. Capillary regeneration in scleroderma: stem cell therapy reverses phenotype?

    Directory of Open Access Journals (Sweden)

    Jo N Fleming

    2008-01-01

    Full Text Available Scleroderma is an autoimmune disease with a characteristic vascular pathology. The vasculopathy associated with scleroderma is one of the major contributors to the clinical manifestations of the disease.We used immunohistochemical and mRNA in situ hybridization techniques to characterize this vasculopathy and showed with morphometry that scleroderma has true capillary rarefaction. We compared skin biopsies from 23 scleroderma patients and 24 normal controls and 7 scleroderma patients who had undergone high dose immunosuppressive therapy followed by autologous hematopoietic cell transplant. Along with the loss of capillaries there was a dramatic change in endothelial phenotype in the residual vessels. The molecules defining this phenotype are: vascular endothelial cadherin, a supposedly universal endothelial marker required for tube formation (lost in the scleroderma tissue, antiangiogenic interferon alpha (overexpressed in the scleroderma dermis and RGS5, a signaling molecule whose expression coincides with the end of branching morphogenesis during development and tumor angiogenesis (also overexpressed in scleroderma skin. Following high dose immunosuppressive therapy, patients experienced clinical improvement and 5 of the 7 patients with scleroderma had increased capillary counts. It was also observed in the same 5 patients, that the interferon alpha and vascular endothelial cadherin had returned to normal as other clinical signs in the skin regressed, and in all 7 patients, RGS5 had returned to normal.These data provide the first objective evidence for loss of vessels in scleroderma and show that this phenomenon is reversible. Coordinate changes in expression of three molecules already implicated in angiogenesis or anti-angiogenesis suggest that control of expression of these three molecules may be the underlying mechanism for at least the vascular component of this disease. Since rarefaction has been little studied, these data may have

  4. Scleroderma renal crisis and ovarian hyperstimulation syndrome related to the use of clomiphene in a patient with scleroderma.

    Science.gov (United States)

    Kobak, Senol; Hacivelioglu, Servet; Gungor, Selen

    2014-01-01

    This paper presented a 28-year-old female with systemic sclerosis who developed scleroderma renal crisis and ovarian hyperstimulation syndrome following clomiphene administration. Urgent therapy including angiotensin-converting enzyme (ACE) inhibitors and supportive care resulted in regression and eventually resolution of all the clinical and laboratory symptoms. Although scleroderma renal crisis is a fatal complication of high-dose corticosteroids, rarely is this seen with the use of ACE inhibitors. This case report aimed to investigate the potential capacity of the selective oestrogen receptor modulator clomiphene to induce scleroderma renal crisis as well as corticosteroids. Copyright © 2012 Elsevier España, S.L. All rights reserved.

  5. [Morphea or juvenile localised scleroderma: Case report].

    Science.gov (United States)

    Strickler, Alexis; Gallo, Silvanna; Jaramillo, Pedro; de Toro, Gonzalo

    2016-01-01

    Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy. We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Recurrent ovary cancer presenting with scleroderma - A rare case report

    OpenAIRE

    Sargin, Betul; Gurer, Gulcan; Bozbas, Gulnur; Noyan, Fatih; Barut, Kayra; Tataroglu, Canten

    2017-01-01

    Scleroderma is a chronic autoimmune multisystem disorder which is characterizedby progressive fibrosis of the skin and internal organs. Ovary cancers with sclerodermahave been reported in the literature. But recurrent ovary cancer with sclerodermahas not been reported before. Here, we report a 65 -year old female patient presentingwith recurrent ovary cancer and subsequently diagnosed with scleroderma. Due toliterature sources, this is the first case of presenting with recurrent ovary cancera...

  7. Echocardiographic study of the cardiac state in systemic scleroderma

    International Nuclear Information System (INIS)

    Kotel'nikova, G.P.; Guseva, N.G.

    1986-01-01

    Echocardiographic investigation in 65 patients with systemic scleroderma showed that the left ventricular syes and indices of central hemodynamics were frequently lowered in them; an increase in sizes was observed mainly in the patients with stage 2-3 of disease. Echocardiography made it possible to specify the nature of cardiac pathology and brought to light some additional features of the involvement of different heart membranes in systemic scleroderma

  8. Validation of the Social Interaction Anxiety Scale in scleroderma: A Scleroderma Patient-centered Intervention Network cohort study

    NARCIS (Netherlands)

    Gholizadeh, S.; Kwakkenbos, C.M.C.; Carrier, M.E.; Mills, S.D.; Fox, R.S.; Jewett, L.R.; Gottesman, K.; Roesch, S.C.; Thombs, B.D.; Malcarne, V.L.

    2018-01-01

    Introduction: Individuals with visible differences due to medical conditions, such as systemic sclerosis (SSc; scleroderma), have reported difficulty navigating social situations because of issues such as staring, invasive questions, and rude comments. Fears or anxiety linked to situations in which

  9. Treatment of GI dysmotility in scleroderma with the new enterokinetic agent prucalopride

    NARCIS (Netherlands)

    Boeckxstaens, G. E.; Bartelsman, J. F. W. M.; Lauwers, L.; Tytgat, G. N. J.

    2002-01-01

    Scleroderma is a multisystem disorder frequently resulting in disturbed GI motility. Although, especially early in the disease, symptomatic improvement is achieved with prokinetic agents, more severe GI manifestations of scleroderma may be difficult to treat, leading to parenteral feeding and

  10. Novel Insights Into Causes of Scleroderma Offer Potential New Treatment Strategies

    Science.gov (United States)

    ... Spotlight on Research Novel Insights Into Causes of Scleroderma Offer Potential New Treatment Strategies By Kirstie Saltsman, Ph.D. | December 15, 2013 In scleroderma, immune cells invade the skin, but the role ...

  11. Lung diffusion of soluble radioaerosols in scleroderma

    International Nuclear Information System (INIS)

    Chopra, S.K.; Taplin, G.V.; Tashkin, D.P.; Elam, D.

    1978-01-01

    Diffusion rates of soluble radioaerosols of sodium pertechnetate (/sup 99m/TcO 4 ; mol. wt. 163) and diethylentriaminepentaacetate (/sup 99m/Tc-DTPA; mol. wt. 492) were determined in ten normal subjects and ten patients with scleroderma having lung involvement. Twenty millicuries (mCi) each of /sup 99m/TcO 4 and /sup 99m/Tc-DTPA in 5 ml saline were aerosolized and inhaled on two different days. Initial lung retention after three minutes of administration was approximately 2 mCi. Two regions of interest over each posterior lung field were monitored with a scintillation camera and data were stored on magnetic tape. Decreasing levels of radioactivity were plotted semilogarithmically and half time (T 1 / 2 ) removal rates were calculated

  12. Biomarkers in Scleroderma: Progressing from Association to Clinical Utility.

    Science.gov (United States)

    Ligon, Colin; Hummers, Laura K

    2016-03-01

    Scleroderma is a heterogenous disease characterized by autoimmunity, a characteristic vasculopathy, and often widely varying extents of deep organ fibrosis. Recent advances in the understanding of scleroderma's evolution have improved the ability to identify subgroups of patients with similar prognosis in order to improve risk stratification, enrich clinical trials for patients likely to benefit from specific therapies, and identify promising therapeutic targets for intervention. High-throughput technologies have recently identified fibrotic and inflammatory effectors in scleroderma that exhibit strong prognostic ability and may be tied to disease evolution. Increasingly, the use of collections of assayed circulating proteins and patterns of gene expression in tissue has replaced single-marker investigations in understanding the evolution of scleroderma and in objectively characterizing disease extent. Lastly, identification of shared patterns of disease evolution has allowed classification of patients into latent disease subtypes, which may allow rapid clinical prognostication and targeted management in both clinical and research settings. The concept of biomarkers in scleroderma is expanding to include nontraditional measures of aggregate protein signatures and disease evolution. This review examines the recent advances in biomarkers with a focus on those approaches poised to guide prospective management or themselves serve as quantitative surrogate disease outcomes.

  13. Race and Association With Disease Manifestations and Mortality in Scleroderma

    Science.gov (United States)

    Manno, Rebecca L.; Shah, Ami A.; Woods, Adrianne; Le, Elizabeth N.; Boin, Francesco; Hummers, Laura K.; Wigley, Fredrick M.

    2013-01-01

    Abstract Experience suggests that African Americans may express autoimmune disease differently than other racial groups. In the context of systemic sclerosis (scleroderma), we sought to determine whether race was related to a more adverse expression of disease. Between January 1, 1990, and December 31, 2009, a total of 409 African American and 1808 white patients with scleroderma were evaluated at a single university medical center. While the distribution by sex was virtually identical in both groups, at 82% female, African American patients presented to the center at a younger mean age than white patients (47 vs. 53 yr; p scleroderma-specific autoantibody status, and for the socioeconomic measures of educational attainment and health insurance status, diminished these risk estimates (RR, 1.3; 95% CI, 1.0–1.6). The heightened risk of mortality persisted in strata defined by age at disease onset, diffuse cutaneous disease, anticentromere seropositivity, decade of care at the center, and among women. These findings support the notion that race is related to a distinct phenotypic profile in scleroderma, and a more unfavorable prognosis among African Americans, warranting heightened diagnostic evaluation and vigilant care of these patients. Further, we provide a chronologic review of the literature regarding race, organ system involvement, and mortality in scleroderma; we furnish synopses of relevant reports, and summarize findings. PMID:23793108

  14. Cancer and Scleroderma: A Paraneoplastic Disease with Implications for Malignancy Screening

    Science.gov (United States)

    Shah, Ami A.; Rosen, Livia Casciola

    2015-01-01

    Purpose of review Recent data suggest a paraneoplastic mechanism of scleroderma pathogenesis in unique subsets of scleroderma patients. In this article, we review these data, explore potential links between cancer and scleroderma, and propose an approach to malignancy screening in scleroderma. Recent findings Emerging data have demonstrated that patients with scleroderma and RNA polymerase III autoantibodies have a significantly increased risk of cancer within a few years of scleroderma onset. Genetic alterations in the gene encoding RNA polymerase III (POLR3A) have been identified, and patients with somatic mutations in POLR3A have evidence of mutation specific T cell immune responses with generation of cross-reactive RNA polymerase III autoantibodies. These data strongly suggest that scleroderma is a by-product of anti-tumor immune responses in some patients. Additional epidemiologic data demonstrate that patients developing scleroderma at older ages may also have a short cancer-scleroderma interval, suggestive of paraneoplastic disease. Summary Scleroderma may be a paraneoplastic disease in unique patient subsets. Aggressive malignancy screening in these patients may aid in early cancer detection. Further study is required to determine whether cancer therapy could improve scleroderma outcomes in this patient population. PMID:26352736

  15. Scleroderma of geriatric age and scleroderma-like paraneoplastic syndrome – description of two cases

    Directory of Open Access Journals (Sweden)

    Magdalena Marek

    2016-06-01

    Full Text Available Systemic sclerosis (Ssc is an autoimmune connective tissue disease of unknown origin, characterized by progressive fibrosis of the skin and internal organs. Immune reactions taking part in Ssc pathogenesis may contribute to cancer development; therefore patients with risk factors for this disease require observation for a neoplastic process. On the other hand, symptoms of Ssc may be a mask of various cancers. Differentiating between the idiopathic form of Ssc and scleroderma-like paraneoplastic syndrome often causes a lot of difficulties. The article presents two cases of Ssc at the beginning of the disease after 60 years of age. The first case was diagnosed as Ssc, whereas in the second case the defined diagnosis was scleroderma-like syndrome in the course of colorectal cancer. This paper presents an analysis of differential diagnostic procedures which were performed and led to the final diagnosis, mentions types of cancers co-occurring with Ssc and suggests a screening scheme for cancer development in patients with a diagnosis of Ssc.

  16. Molecular subsets in the gene expression signatures of scleroderma skin.

    Directory of Open Access Journals (Sweden)

    Ausra Milano

    2008-07-01

    Full Text Available Scleroderma is a clinically heterogeneous disease with a complex phenotype. The disease is characterized by vascular dysfunction, tissue fibrosis, internal organ dysfunction, and immune dysfunction resulting in autoantibody production.We analyzed the genome-wide patterns of gene expression with DNA microarrays in skin biopsies from distinct scleroderma subsets including 17 patients with systemic sclerosis (SSc with diffuse scleroderma (dSSc, 7 patients with SSc with limited scleroderma (lSSc, 3 patients with morphea, and 6 healthy controls. 61 skin biopsies were analyzed in a total of 75 microarray hybridizations. Analysis by hierarchical clustering demonstrates nearly identical patterns of gene expression in 17 out of 22 of the forearm and back skin pairs of SSc patients. Using this property of the gene expression, we selected a set of 'intrinsic' genes and analyzed the inherent data-driven groupings. Distinct patterns of gene expression separate patients with dSSc from those with lSSc and both are easily distinguished from normal controls. Our data show three distinct patient groups among the patients with dSSc and two groups among patients with lSSc. Each group can be distinguished by unique gene expression signatures indicative of proliferating cells, immune infiltrates and a fibrotic program. The intrinsic groups are statistically significant (p<0.001 and each has been mapped to clinical covariates of modified Rodnan skin score, interstitial lung disease, gastrointestinal involvement, digital ulcers, Raynaud's phenomenon and disease duration. We report a 177-gene signature that is associated with severity of skin disease in dSSc.Genome-wide gene expression profiling of skin biopsies demonstrates that the heterogeneity in scleroderma can be measured quantitatively with DNA microarrays. The diversity in gene expression demonstrates multiple distinct gene expression programs in the skin of patients with scleroderma.

  17. Limited scleroderma and early detection of visceral changes

    International Nuclear Information System (INIS)

    Kalyuzhnaya, L.D.; Potsibina, V.V.; Stychinskaya, L.P.; Turik, N.V.

    1989-01-01

    The state of liver, kidneys, osteoarticular apparatus at the early stages of development of limited scleroderma and with the exclusion of visceral changes on the basis of clinical-laboratory studies is investigated. 11 patients with scleroderma in the age of 7-18 years were examined. Osteoscintigraphy with 99m TC-phosphone and dynamic scintigraphy of kidneys without additional introduction of RF, and hepatocholecyctoscintigraphy with 99m tc-HIPA of the patients were realized. The conclusion is made that radionuclide investigation methods permit to reveal various visceral changes, which are not recognizable by clinical methods

  18. Exploring Sources of Emotional Distress among People Living with Scleroderma: A Focus Group Study.

    Directory of Open Access Journals (Sweden)

    Stephanie T Gumuchian

    Full Text Available Systemic sclerosis, or scleroderma, is a chronic and rare connective tissue disease with negative physical and psychological implications. Sources of emotional distress and the impact they have on the lives of people with scleroderma are not well understood.To gain an in-depth understanding of the emotional experiences and sources of emotional distress for women and men living with scleroderma through focus group discussions.Three semi-structured focus group discussions were conducted (two in English, one in French with a total of 22 people with scleroderma recruited through the Scleroderma Society of Ontario in Hamilton, Ontario and a scleroderma clinic in Montreal, Canada. Interviews were recorded, transcribed, and then coded for emerging themes using thematic inductive analysis.Core themes representing sources of emotional distress were identified, including: (a facing a new reality; (b the daily struggle of living with scleroderma; (c handling work, employment and general financial burden; (d changing family roles; (e social interactions; and (f navigating the health care system. Collectively, these themes refer to the stressful journey of living with scleroderma including the obstacles faced and the emotional experiences beginning prior to receiving a diagnosis and continuing throughout the participants' lives.Scleroderma was portrayed as being an unpredictable and overwhelming disease, resulting in many individuals experiencing multiple sources of emotional distress. Interventions and supportive resources need to be developed to help individuals with scleroderma and people close to them manage and cope with the emotional aspects of the disease.

  19. Primary biliary cirrhosis and scleroderma complicated by Barrett's ...

    African Journals Online (AJOL)

    1991-04-06

    Apr 6, 1991 ... primary biliary cirrhosis, CREST syndrome, and chronic pancreatitis. Thorax. 1983; 38: 316-317. 9. Okano Y, Nisbikai M, Sato A. Scleroderma, primary biliary cirrhosis, and. Sjogren's syndrome after cosmetic breast augmentation with silicone injec- tion: a case reporfof possible human adjuvant disease.

  20. Pediatric morphea (localized scleroderma): review of 136 patients.

    Science.gov (United States)

    Christen-Zaech, Stéphanie; Hakim, Miriam D; Afsar, F Sule; Paller, Amy S

    2008-09-01

    Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement. We sought to determine the clinical features of morphea in a large pediatric cohort. We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006. Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients. Two patients with rapidly progressing generalized or extensive linear morphea and arthralgias developed restrictive pulmonary disease. Initial oral corticosteroid treatment and long-term methotrexate administration stabilized and/or led to disease improvement in most patients with aggressive disease. Retrospective analysis, relatively small sample size, and risk of a selected referral population to the single site are limitations. These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. Visceral manifestations rarely occur; the presence of progressive problematic cutaneous disease and arthralgias should trigger closer patient monitoring.

  1. MRI findings in deep and generalized morphea (localized scleroderma).

    Science.gov (United States)

    Horger, Marius; Fierlbeck, Gerhard; Kuemmerle-Deschner, Jasmin; Tzaribachev, Nikolay; Wehrmann, Manfred; Claussen, Claus D; Fritz, Jan

    2008-01-01

    Our objective was to describe the spectrum of MRI features in patients with deep and generalized morphea. Imaging features of morphea are not specific and usually overlap with those of other disorders involving the skin, fascia, and musculature, such as some types of fasciitis, myositis, and so forth. Nevertheless, the imaging features of morphea reflect pathomorphologic changes of this rare disorder and enable a complete assessment of the disease extent, including depth of infiltration and disease activity.

  2. Clinical and Autoimmune Profile of Scleroderma Patients from Western India

    Directory of Open Access Journals (Sweden)

    Vandana Pradhan

    2014-01-01

    Full Text Available Background. Systemic sclerosis (SSc, scleroderma is a disorder characterized by fibrosis of skin and visceral organs. Pathogenesis of scleroderma is complex and is incompletely understood as yet. Autoantibodies in SSc represent a serologic hallmark which have clinical relevance, with diagnostic and prognostic potential. Objectives. To study distribution of clinical manifestations and to identify frequency of autoantibodies among subtypes of scleroderma patients from Western India. Methodology. One hundred and ten scleroderma patients were clinically classified according to the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR criteria. All these patients were in active stage of disease. Clinical manifestations were recorded at the time of presentation. Autoantibodies were tested in them by indirect immunofluorescence test and ELISA. Immunoglobulin levels were estimated by nephelometer. These parameters were further correlated with clinical presentation of the disease. Results. Scleroderma patients had M : F ratio of 1 : 10 where mean age at evaluation was 34.7±10.7 years and a mean disease duration was 43.7±35 months. Clinical subtypes showed that 45 patients (40.9% had diffused cutaneous (dcSSc lesions, 32 patients (29.1% had limited cutaneous (lcSSc lesions, and 33 patients (30% had other autoimmune overlaps. The overall frequency of ANA in SSc patients studied was 85.5%. The frequency of anti-Scl70, anti-centromere, anti-endothelial cell antibodies (AECA, and anti-keratinocyte antibodies (AKA was 62.7%, 22.7%, 30%, and 40.9%, respectively. Anti-Scl70 antibodies were significantly high (75.6% versus 46.9% among dcSSc patients (P<0.0115 whereas anti-centromere antibodies were significantly high (9% versus 38% among lcSSc patients when these two subtypes were compared (P<0.0044. Conclusion. This study supports that there are geoepidemiological variations among scleroderma patients for their clinical

  3. Predictive value of European Scleroderma Group Activity Index in an early scleroderma cohort.

    Science.gov (United States)

    Nevskaya, Tatiana; Baron, Murray; Pope, Janet E

    2017-07-01

    To estimate the effect of disease activity, as measured by the European Scleroderma Research Group Activity Index (EScSG-AI), on the risk of subsequent organ damage in a large systemic sclerosis (SSc) cohort. Of 421 SSc patients from the Canadian Scleroderma Research Group database with disease duration of ⩽ 3 years, 197 who had no evidence of end-stage organ damage initially and available 3 year follow-up were included. Disease activity was assessed by the EScSG-AI with two variability measures: the adjusted mean EScSG-AI (the area under the curve of the EScSG-AI over the observation period) and persistently active disease/flare. Outcomes were based on the Medsger severity scale and included accrual of a new severity score (Δ ⩾ 1) overall and within organ systems or reaching a significant level of deterioration in health status. After adjustment for covariates, the adjusted mean EScSG-AI was the most consistent predictor of risk across the study outcomes over 3 years in dcSSc: disease progression defined as Δ ⩾ 1 in any major internal organ, significant decline in forced vital capacity and diffusing capacity of carbon monoxide, severity of visceral disease and HAQ Disability Index worsening. In multivariate analysis, progression of lung disease was predicted solely by adjusted mean EScSG-AI, while the severity of lung disease was predicted the adjusted mean EScSG-AI, older age, modified Rodnan skin score (mRSS) and initial severity. The EScSG-AI was associated with patient- and physician-assessed measures of health status and overpowered the mRSS in predicting disease outcomes. Disease activity burden quantified with the adjusted mean EScSG-AI predicted the risk of deterioration in health status and severe organ involvement in dcSSc. The EScSG-AI is more responsive when done repeatedly and averaged. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email

  4. Lichen sclerosus et atrophicans, scleroderma en coup de sabre and Lyme borreliosis

    Directory of Open Access Journals (Sweden)

    Serena Bonin

    2011-09-01

    Full Text Available Lichen sclerosus et atrophicans (LSA is a chronic, inflammatory skin disease of unknown etiology, characterized by atrophy. We report a case of LSA with frontoparietal distribution, mimicking scleroderma en coup de sabre, causing scarring alopecia. The case was associated with Borrelia infection. The lesion improved with 2 cycles of antibiotic therapy with ceftriaxone 2 gr /day i.v for 21 days associated with UVA-1 therapy and local and systemic vitamin E supply (400 mg 2x/day per os for 3 months. This case stresses the importance of identifying clinical manifestations associated with Lyme disease and the use of tissue PCR to detect borrelial DNA in patients with these lesions, but characterized by negative serology for Borrelia.

  5. Barrett esophagus in scleroderma: Increased incidence and radiographic findings

    International Nuclear Information System (INIS)

    Recht, M.P.; Levine, M.S.; Katzka, D.A.; Reynolds, J.C.; Saul, S.H.; Ouyang, A.; Cohen, S.

    1986-01-01

    Thirteen patients with scleroderma underwent esophagography and endoscopy because of symptoms of reflux esophagitis or dysphagia, or both. Five had biopsy-proved Barrett esophagus, and two of those five had esophageal adencarcinomas. In a blinded review of the barium studies, Barrett esophagus was thought to be probable in one case, possible in seven, and unlikely in five. Five of eight patients in the first two groups had Barrett esophagus on endoscopy, but none in the third group had this disease. The author's initial experience, therefore, suggests that a definitive radiologic diagnosis of Barrett esophagus is not possible in most patients with scleroderma. However, endoscopy may be avoided when Barrett esophagus is considered unlikely on esophagography

  6. A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

    Science.gov (United States)

    McCann, Liza J; Pain, Clare E

    2016-02-01

    Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

  7. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  8. Use of the international systemic scleroderma activity index

    Directory of Open Access Journals (Sweden)

    Maya Nikolayevna Starovoitova

    2013-01-01

    Full Text Available Up to now, it is difficult to determine systemic scleroderma (SSD activity because of the lack of validated tools to estimate changes in the pathological process. Attempts have been made to develop unified activity assessing methods for many years. The indices proposed by the European SSD Group are most popular today. This paper gives the results of using this index in a cohort of Russian patients.

  9. The skeletal changes of progressive systemic scleroderma (PSS), of exclusive Raynaud phenomenon and of circumscript scleroderma in a gamma camera scintigraph

    International Nuclear Information System (INIS)

    Ulbrich, T.

    1987-01-01

    The bases of this study are the skeletal scintigraphs of altogether 34 patients with the three forms of scleroderma mentioned in the title. The PSS shows a polytropic, symmetric distribution pattern, the exclusive Raynaud's phenomenon shows a basic skeleton which is unchanged in contrast to circumscript scleroderma, with which also the larger joints are relatively more frequently attacked than the little ones. As a result of high sensitivity skeletal scintiscanning makes possible an early recognition of osseous changes and is there with superior to X-radiology and should then see an increased use for the clarification of the diagnosis of arthropathy in scleroderma. (TRV) [de

  10. The EULAR Scleroderma Trials and Research Group (EUSTAR): an international framework for accelerating scleroderma research.

    Science.gov (United States)

    Tyndall, Alan; Ladner, Ulf M; Matucci-Cerinic, Marco

    2008-11-01

    Systemic sclerosis has a complex pathogenesis and a multifaceted clinical spectrum without a specific treatment. Under the auspices of the European League Against Rheumatism, the European League Against Rheumatism Scleroderma Trials And Research group (EUSTAR) has been founded in Europe to foster the study of systemic sclerosis with the aim of achieving equality of assessment and care of systemic sclerosis patients throughout the world according to evidence-based principles. EUSTAR created the minimal essential data set, a simple two-page form with basic demographics and mostly yes/no answers to clinical and laboratory parameters, to track patients throughout Europe. Currently, over 7000 patients are registered from 150 centres in four continents, and several articles have been published with the data generated by the minimal essential data set. A commitment of EUSTAR is also to teaching and educating, and for this reason there are two teaching courses and a third is planned for early in 2009. These courses have built international networks among young investigators improving the quality of multicentre clinical trials. EUSTAR has organized several rounds of 'teach the teachers' to further standardize the skin scoring. EUSTAR activities have extended beyond European borders, and EUSTAR now includes experts from several nations. The growth of data and biomaterial might ensure many further fruitful multicentre studies, but the financial sustainability of EUSTAR remains an issue that may jeopardize the existence of this group as well as that of other organizations in the world.

  11. Esclerose sistêmica e sarcoidose Scleroderma and sarcoidosis

    Directory of Open Access Journals (Sweden)

    Fernanda Guidolin

    2005-10-01

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

  12. Scleroderma in the Caribbean: characteristics in a Dominican case series.

    Science.gov (United States)

    Gottschalk, Paola; Vásquez, Ricardo; López, Persio David; Then, Jossiell; Tineo, Carmen; Loyo, Esthela

    2014-01-01

    Scleroderma is a rare disease with limited data in Latin America. Preliminary genetic studies suggest a strong African ascendance in the Dominican Republic, which could modulate the expression of the disease. The objective of this study is to describe the clinical and demographic characteristics of scleroderma in a series of 26 Dominican patients. Patients who fulfilled the EULAR/ACR criteria for scleroderma were selected from the Rheumatology Department of a tertiary health center; systemic sclerosis subtypes were defined according to the EULAR classification. Clinical and demographic information was obtained retrospectively from clinical records. Mean age at time of onset was 32.6±15 years; 68% of patients had 40 years of age or less. 73% of patients was feminine, with a female:male ratio of 2.7:1. The most affected systems were pulmonary and gastrointestinal; renal affection was scarce. Anti-Scl-70 antibodies were positive in 64% of patients, sometimes in coexistence with anti-centromere antibodies. The prevalence of systemic sclerosis is lower in the Dominican population than the reported elsewhere. The age of onset of the disease seems to be lower in the Dominican population than that reported in literature. A different pattern of autoantibodies is observed in this population. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  13. Discovery or Extinction of New Scleroderma Species in Amazonia?

    Directory of Open Access Journals (Sweden)

    Iuri G Baseia

    Full Text Available The Amazon Forest is a hotspot of biodiversity harboring an unknown number of undescribed taxa. Inventory studies are urgent, mainly in the areas most endangered by human activities such as extensive dam construction, where species could be in risk of extinction before being described and named. In 2015, intensive studies performed in a few locations in the Brazilian Amazon rainforest revealed three new species of the genus Scleroderma: S. anomalosporum, S. camassuense and S. duckei. The two first species were located in one of the many areas flooded by construction of hydroelectric dams throughout the Amazon; and the third in the Reserva Florestal Adolpho Ducke, a protected reverse by the INPA. The species were identified through morphology and molecular analyses of barcoding sequences (Internal Transcribed Spacer nrDNA. Scleroderma anomalosporum is characterized mainly by the smooth spores under LM in mature basidiomata (under SEM with small, unevenly distributed granules, a characteristic not observed in other species of the genus, the large size of the basidiomata, up to 120 mm diameter, and the stelliform dehiscence; S. camassuense mainly by the irregular to stellate dehiscence, the subreticulated spores and the bright sulfur-yellow colour, and Scleroderma duckei mainly by the verrucose exoperidium, stelliform dehiscence, and verrucose spores. Description, illustration and affinities with other species of the genus are provided.

  14. End-Stage Kidney Disease From Scleroderma in the United States, 1996 to 2012

    Directory of Open Access Journals (Sweden)

    Donal J. Sexton

    2018-01-01

    Conclusion: The incidence of ESKD from scleroderma appears to have declined in the United States since 1996. ESKD from scleroderma is associated with an enhanced likelihood of recovery of kidney function and death, a reduced likelihood of transplantation, and similar outcomes after transplantation.

  15. Exploring sources of emotional distress among people living with scleroderma: A focus group study

    NARCIS (Netherlands)

    Gumuchian, S.T.; Peláez, S.; Delisle, V.C.; Carrier, M.E.; Jewett, L.R.; El-Baalbaki, G.; Fortune, C.; Hudson, M.; Impens, A.; Körner, A.; Persmann, J.; Kwakkenbos, C.M.C.; Bartlett, S.J.; Thombs, B.D.

    2016-01-01

    Background: Systemic sclerosis, or scleroderma, is a chronic and rare connective tissue disease with negative physical and psychological implications. Sources of emotional distress and the impact they have on the lives of people with scleroderma are not well understood. Objectives: To gain an

  16. [Clinical, endoscopic and morphological manifestations of oesophageal lesion in systemic scleroderma].

    Science.gov (United States)

    Karateev, A E; Movsiian, A E; Anan'eva, M M; Radenska-Lopovok, S G

    2014-01-01

    Oesophageal lesion is the commonest visceral manifestation of systemic scleroderma (SSD) affecting the quality of life and fraught with serious complications. The aim of this study was to evaluate clinical, endoscopic andmorphological manifestations of oesophageal lesion in systemic scleroderma and its relationships with other clinical symptoms and pharmacotherapy of the disease. 479 patients with SSD (93.7% women, 6.3% men, mean age 48.7 +/- 19.2 yr). All of them underwent EGDS in 2005-2010. 123 patients were examined for the detection of Barrett's oesophagus (BO), total screening regardless of complaints was conducted in 2010. Control group included 1018 age and sex-matched patients with RA who underwent EGDS in 2008-2009. Oesophageal lesions occurred much more frequently in SSD than in RA. Oesophageal symptoms were documented in 70.0 and 29.9% cases, non-erosive oesopahgitis in 28.8 and 1.5%, erosive esophagitis in 22.5 and 2.2% ulcers in 0.8 and 0% (p < 0.001). BO manifested as intestinal metaplasia (histological study of mucosal biopsy) was found in 30 SSD patients (4.2%). Screening revealed BO in 8.9% of the patients. The development of erosive oesophagitis was unrelated to the age of the patients, duration of the disease and its form (localized or diffusive), lung pathology or Sjogren's syndrome. Cytotoxic medicines significantly increased the frequency of erosive oesophagitis, it tended to increase under effect of NSAID and low doses of aspirin. Long-term intake of PPI did not reduce the risk of oesophagitis and BO. Half of the patients with SSD have oesophagitis. Over 20% of them suffer its complications (erosion and ulcers) and 9% have BO. All such patients need endoscopic study ofoesophagus regardless of clinical symptoms.

  17. Oligosaccharide modification by N-acetylglucosaminyltransferase-V in macrophages are involved in pathogenesis of bleomycin-induced scleroderma.

    Science.gov (United States)

    Kato, Arisa; Yutani, Mizuki; Terao, Mika; Kimura, Akihiro; Itoi, Saori; Murota, Hiroyuki; Miyoshi, Eiji; Katayama, Ichiro

    2015-08-01

    Oligosaccharide modification by N-acetylglucosaminyltransferase-V (GnT-V), which catalyses the formation of β1,6 GlcNAc (N-acetylglucosamine) branches on N-glycans, is associated with various pathologies, such as cancer metastasis, multiple sclerosis and liver fibrosis. In this study, we demonstrated the involvement of GnT-V in the pathophysiology of scleroderma. High expression of GnT-V was observed in infiltrating cells in skin section samples from systemic and localized patients with scleroderma. Most of the infiltrating cells were T cells and macrophages, most of which were CD163(+) M2 macrophages. To determine the role of GnT-V in scleroderma, we next investigated skin sclerosis in GnT-V knockout (MGAT5(-/-) ) mice. Expression of GnT-V was also elevated in bleomycin (BLM)-injected sclerotic skin, and MGAT5(-/-) mice were resistant to BLM-induced skin sclerosis with reduced collagen type 1 α1 content, suggesting the biological significance of GnT-V in skin sclerosis. Furthermore, the number of CD163(+) M2 macrophages and CD3-positive T cells in BLM-induced skin sclerosis was significantly fewer in MGAT5(-/-) mice. In bone marrow-derived macrophages (BMDMs), IL-4-induced expressions of Fizz1 and Ym1 were significantly reduced in MGAT5(-/-) mice-derived BMDMs. Taken together, these results suggest the induction of GnT-V in skin sclerosis progression is possibly dependent on increased numbers of M2 macrophages in the skin, which are important for tissue fibrosis and remodelling. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. The frequency of pulmonary hypertension in patients with juvenile scleroderma

    Directory of Open Access Journals (Sweden)

    Amra Adrovic

    2015-08-01

    Full Text Available Juvenile scleroderma (JS represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests, Forced vital capacity (FVC and Carbon monoxide diffusion capacity (DLCO in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP and risk factors for pulmonary arterial hypertension (PAH were made by the measurement of maximum tricuspid insufficiency (TI, end-diastolic pulmonary insufficiency (PI, ratio of acceleration time (AT to ejection time (ET (AT/ET, right atrial pressure (RAP and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years. The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years and 3.57 years (median 2 years, range 0.5-14.5 years, respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

  19. The frequency of pulmonary hypertension in patients with juvenile scleroderma.

    Science.gov (United States)

    Adrovic, Amra; Oztunc, Funda; Barut, Kenan; Koka, Aida; Gojak, Refet; Sahin, Sezgin; Demir, Tuncalp; Kasapcopur, Ozgur

    2015-08-22

    Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

  20. ANCA-associated vasculitis in scleroderma: a case series of fourteen patients

    Directory of Open Access Journals (Sweden)

    Kimberly P. Liang

    2011-01-01

    Full Text Available Antimyeloperoxidase (MPO, perinuclear antineutrophil cytoplasmic antibodies (pANCA, and/or clinically evident vasculitis in patients with scleroderma have been reported only rarely. The clinical significance and prognosis of ANCA-associated vasculitis in systemic sclerosis is uncertain. To report a case and identify the clinical characteristics of scleroderma patients with ANCA-associated vasculitis. Patients with both vasculitis and scleroderma occurring between 1976 to 2006 were identified using an electronic diagnostic index. These diagnoses were confirmed by retrospective review of complete medical records. Clinical features and outcomes recorded included age at vasculitis diagnosis, connective tissue disease (CTD features, type of scleroderma (limited or diffuse; ANCA serology, vasculitic organ system manifestations; and death. Fourteen cases of scleroderma patients with ANCA-associated and/or small vessel vasculitis were identified. The majority (71% were female, with mean age at vasculitis diagnosis 53 years. Seven patients (50% had overlap CTD features, and the majority (79% had limited variant of scleroderma. All of the 10 patients tested were MPO and pANCA positive. Seven patients (50% had glomerulonephritis, 11 (79% pulmonary involvement including 3 with pulmonary-renal syndrome, 6 skin purpura, and 5 mononeuritis multiplex and/or peripheral neuropathy. Six patients (43% died during followup to 2008. The presence of pANCA-associated small vessel vasculitis is a rarely reported complication of scleroderma. It occurs most commonly in women with limited scleroderma and most commonly includes pulmonary and/or renal involvement, including severe organ-threatening manifestations and death. Further studies are needed to clarify the role and clinical impact of ANCA in scleroderma patients with and without vasculitis.

  1. Scleroderma Renal Crisis: A Reversible Cause of Left Ventricular Dysfunction.

    Science.gov (United States)

    Martínez-Milla, Juan; Gaebelt, Hans Paul; Sánchez-Pernaute, Olga; Kallmeyer, Andrea; Romero, José; Farré, Jerónimo

    2018-05-02

    We report a case of acute left ventricular dysfunction due to myocarditis, in the setting of a scleroderma renal crisis. The case is particularly intriguing for the favorable outcome of both symptoms and heart function following immunosuppressive therapy. We also highlight the changes observed over time with image techniques as well as in electrocardiograms. Copyright © 2018 Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. Tumor-like calcifications with scleroderma. Thibierge-Weissenbach-Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Meyer, E.; Kulenkampff, H.A.; Kortenhaus, H.

    1987-12-01

    In patients with progressive scleroderma, interstitial calcifications are present to a varying extent. They are mostly located in the soft tissues of the fingers, resembling points, commas or dashes. They may also appear as 'calcinosis universalis' and reach a considerable size. Thus they mimic proliferative tumors. Scintigraphy, proving the existence of further calcifications can be helpful. We report the case of a female patient who presented with such a 'pseudotumor' of unusual size, site and extent in the lumbar region.

  3. Renal diagnostic nuclear medicine procedures in progressive systemic scleroderma (PSS)

    Energy Technology Data Exchange (ETDEWEB)

    Ammari, B.; Hotze, A.; Gruenwald, F.; Biersack, H.J.; Blitz, H.; Kuester, W.; Kreysel, H.W.

    1989-02-01

    The involvement of kidneys in progressive systemic scleroderma (PSS) is one of the most frequent causes of death in this disease. Using clinical criteria and laboratory tests only the frequency of kidney involvement would be clearly underestimated. Invasive diagnostic procedures such as biopsy and angiography can not be applied in those patients. Nuclear medicine techniques (hippurate clearance, DMSA-scan), however, offer non invasive and sensitive methods in the diagnosis of renal involvement in PSS patients. In our study 46 of 76 patients (60%) revealed pathologic findings. The mentioned diagnostic techniques show a high sensitivity and are in agreement with pathological findings described in PSS.

  4. Renal diagnostic nuclear medicine procedures in progressive systemic scleroderma (PSS)

    International Nuclear Information System (INIS)

    Ammari, B.; Hotze, A.; Gruenwald, F.; Biersack, H.J.; Blitz, H.; Kuester, W.; Kreysel, H.W.

    1989-01-01

    The involvement of kidneys in progressive systemic scleroderma (PSS) is one of the most frequent causes of death in this disease. Using clinical criteria and laboratory tests only the frequency of kidney involvement would be clearly underestimated. Invasive diagnostic procedures such as biopsy and angiography can not be applied in those patients. Nuclear medicine techniques (hippurate clearance, DMSA-scan), however, offer non invasive and sensitive methods in the diagnosis of renal involvement in PSS patients. In our study 46 of 76 patients (60%) revealed pathologic findings. The mentioned diagnostic techniques show a high sensitivity and are in agreement with pathological findings described in PSS. (orig.) [de

  5. A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face

    Science.gov (United States)

    Kumar, M Hari; Kumar, M Siva; Kumar, Sabitha Hari; Kumar, Kingsly Selva

    2016-01-01

    Limited cutaneous scleroderma is a subtype of scleroderma limited to the skin of the face, hands, feet and forearms. We present a case of a 45-year-old woman affected by limited cutaneous systemic scleroderma involving the orofacial region and causing restricted mouth opening. The patient showed noteworthy improvement of the skin lesion by use of a combination of intralesional corticosteroid with hyaluronidase and various multiantioxidants, resulting in amelioration of her mouth opening problem. The patient gave her full informed written consent to this report being published. PMID:27033280

  6. A neoteric multidrug combination: novel approach to limited cutaneous systemic scleroderma involving the face.

    Science.gov (United States)

    Kumar, M Hari; Kumar, M Siva; Kumar, Sabitha Hari; Kumar, Kingsly Selva

    2016-03-31

    Limited cutaneous scleroderma is a subtype of scleroderma limited to the skin of the face, hands, feet and forearms. We present a case of a 45-year-old woman affected by limited cutaneous systemic scleroderma involving the orofacial region and causing restricted mouth opening. The patient showed noteworthy improvement of the skin lesion by use of a combination of intralesional corticosteroid with hyaluronidase and various multiantioxidants, resulting in amelioration of her mouth opening problem. The patient gave her full informed written consent to this report being published. 2016 BMJ Publishing Group Ltd.

  7. X-ray findings in scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Kauffmann, G.W.; Reinbold, W.D.; Hagedorn, M.

    1983-05-01

    In 28 patients with progressive systemic sclerosis clinical symptoms are correlated with roentgen findings. The systemic disease of connective tissue shows typical roentgenological signs, such as soft tissue calcifications, generalized or localized osteoporosis, arthritis, dysfunction of oesophagus, small and large bowel, pulmonary fibrosis and cardiomegaly. A limited prognostic statement according to a clinical and radiological classification seems possible.

  8. X-ray findings in scleroderma

    International Nuclear Information System (INIS)

    Kauffmann, G.W.; Reinbold, W.D.; Hagedorn, M.; Freiburg Univ.

    1983-01-01

    In 28 patients with progressive systemic sclerosis clinical symptoms are correlated with roentgen findings. The systemic disease of connective tissue shows typical roentgenological signs, such as soft tissue calcifications, generalized or localized osteoporosis, arthritis, dysfunction of oesophagus, small and large bowel, pulmonary fibrosis and cardiomegaly. A limited prognostic statement according to a clinical and radiological classification seems possible. (orig.) [de

  9. Systemic scleroderma diagnosed after undergoing radiation therapy for breast cancer

    International Nuclear Information System (INIS)

    Sasaki, Tetsuo; Kakei, Masae

    1994-01-01

    A case of systemic scleroderma in which the symptoms became prominent after undergoing radiation therapy for breast cancer is reported. A 68-year-old woman, who had undergone a radical mastectomy for breast carcinoma at the age of 63 and thereafter received radiotherapy at 65, 66 and 67 years of age, visited our clinic complaining of skin sclerosis and Raynaud's phenomenon which she had noticed since the age of 65. The physical examination revealed not only postirradiation fibrosis and pigmentation, but also edematous sclerosis and the pigmentation of her extremities, as well as short frenulum of the tongue and digital pitting scars. She demonstrated serum anti-topoisomerase I antibodies, esophageal dysfunction and lung fibrosis. The histopathology of the forearm skin showed edema of the upper dermis as well as increased and homogenized collagen bundles in the middle and lower dermis. Since similar cases have been reported, it is considerable that radiation therapy may have thus worsened the lesions of scleroderma in this patient as well. (author)

  10. Systemic scleroderma diagnosed after undergoing radiation therapy for breast cancer

    Energy Technology Data Exchange (ETDEWEB)

    Sasaki, Tetsuo; Kakei, Masae (Yokohama City Univ. (Japan). Faculty of Medicine)

    1994-10-01

    A case of systemic scleroderma in which the symptoms became prominent after undergoing radiation therapy for breast cancer is reported. A 68-year-old woman, who had undergone a radical mastectomy for breast carcinoma at the age of 63 and thereafter received radiotherapy at 65, 66 and 67 years of age, visited our clinic complaining of skin sclerosis and Raynaud's phenomenon which she had noticed since the age of 65. The physical examination revealed not only postirradiation fibrosis and pigmentation, but also edematous sclerosis and the pigmentation of her extremities, as well as short frenulum of the tongue and digital pitting scars. She demonstrated serum anti-topoisomerase I antibodies, esophageal dysfunction and lung fibrosis. The histopathology of the forearm skin showed edema of the upper dermis as well as increased and homogenized collagen bundles in the middle and lower dermis. Since similar cases have been reported, it is considerable that radiation therapy may have thus worsened the lesions of scleroderma in this patient as well. (author).

  11. Race and association with disease manifestations and mortality in scleroderma: a 20-year experience at the Johns Hopkins Scleroderma Center and review of the literature.

    Science.gov (United States)

    Gelber, Allan C; Manno, Rebecca L; Shah, Ami A; Woods, Adrianne; Le, Elizabeth N; Boin, Francesco; Hummers, Laura K; Wigley, Fredrick M

    2013-07-01

    Experience suggests that African Americans may express autoimmune disease differently than other racial groups. In the context of systemic sclerosis (scleroderma), we sought to determine whether race was related to a more adverse expression of disease. Between January 1, 1990, and December 31, 2009, a total of 409 African American and 1808 white patients with scleroderma were evaluated at a single university medical center. While the distribution by sex was virtually identical in both groups, at 82% female, African American patients presented to the center at a younger mean age than white patients (47 vs. 53 yr; p scleroderma-specific autoantibody status, and for the socioeconomic measures of educational attainment and health insurance status, diminished these risk estimates (RR, 1.3; 95% CI, 1.0-1.6). The heightened risk of mortality persisted in strata defined by age at disease onset, diffuse cutaneous disease, anticentromere seropositivity, decade of care at the center, and among women. These findings support the notion that race is related to a distinct phenotypic profile in scleroderma, and a more unfavorable prognosis among African Americans, warranting heightened diagnostic evaluation and vigilant care of these patients. Further, we provide a chronologic review of the literature regarding race, organ system involvement, and mortality in scleroderma; we furnish synopses of relevant reports, and summarize findings.

  12. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children

    NARCIS (Netherlands)

    E.F. Elst (Elisabeth); L.W.A. van Suijlekom-Smit (Lisette); A.P. Oranje (Arnold)

    1999-01-01

    textabstractLinear scleroderma is a connective tissue disorder that characteristically involves the skin. Skin induration and pigmentary changes present in a linear distribution. Severe functional and cosmetic disability may occur, especially in growing children. No effective therapy for the

  13. Predictors of end stage lung disease in a cohort of patients with scleroderma

    OpenAIRE

    Morgan, C; Knight, C; Lunt, M; Black, C; Silman, A

    2003-01-01

    Objectives: To estimate the incidence of severe lung disease in patients with scleroderma and identify the combination(s) of features present at first assessment which would be useful to predict future risk of severe lung disease.

  14. INTERSTITIAL LUNG-DISEASE AND MYOSITIS IN A PATIENT WITH SIMULTANEOUSLY OCCURRING SARCOIDOSIS AND SCLERODERMA

    NARCIS (Netherlands)

    GROEN, H; POSTMA, DS; KALLENBERG, CGM

    1993-01-01

    A patient initially presented with sarcoidosis in combination with myositis of sarcoid origin and Raynaud's phenomenon. During the course of his disease, he additionally developed scleroderma. Bronchoalveolar lavage, performed because of increase of interstitial markings in the presence of enlarged

  15. Emergency Surgery in a Patient with Scleroderma - Anaesthetic Challenges: A Case Report

    Directory of Open Access Journals (Sweden)

    Teena Bansal

    2013-06-01

    Full Text Available Scleroderma (progressive systemic sclerosis is a multisystem disease involving the skin, airway, musculoskeletal, gastrointestinal, pulmonary, renal and cardiac systems that can pose a significant challenge for the anaesthetist. The multisystem involvement of scleroderma can impact on every aspect of anaesthetic care especially airway management. There are no specific contraindications to the use of any type of anaesthesia, although the selection must be guided by identification of organ dysfunction. The anaesthetist must be aware of the organs involved, the severity of the disease and the associated anaesthetic considerations and potential risks in order to safely & skilfully manage the patient with scleroderma. We hereby present a case report of a patient with scleroderma for emergency orbital decompression because of orbital cellulitis.

  16. First record of Scleroderma polyrhizum Pers. (Gasteromycetes from Brazil Primeiro registro de Scleroderma polyrhizum Pers. (Gasteromycetes para o Brasil

    Directory of Open Access Journals (Sweden)

    Iuri Goulart Baseia

    2000-08-01

    Full Text Available The ectomycorrhizal, gasteroid fungus, Scleroderma polyrhizum is recorded from Brazil for the first time, growing under Caryocar brasiliense Camb. (Caryocaraceae a widespread native tree of the Brazilian "cerrado" vegetation. Macro and microscopic features were described using basidiocarps from fresh and dried material treated according to the traditional methodology for Gasteromycetes. The characteristics of the material were close to those of the original description given by Persoon. All material collected was associated with roots of C brasiliense.Scleroderma polyrhizum, um gasteromiceto ectomicorrízico, é registrado pela primeira vez para o Brasil, crescendo sob Caryocar brasillense Camb. (Caryiocaraceae espécie arbórea comum e nativa da vegetação de cerrado brasileiro. Os caracteres macro e microscópicos foram descritos a partir de basidiocarpos frescos e secos, segundo a metodologia tradicional utilizada em estudos taxonómicos de Gasteromycetes. As características do material analisado foram similares à descrição original fornecida por Persoon. Todo material coletado estava associado a raízes de C. brasillense.

  17. New directions for patient-centred care in scleroderma: the Scleroderma Patient-centred Intervention Network (SPIN)

    Science.gov (United States)

    Thombs, Brett D.; Jewett, Lisa R.; Assassi, Shervin; Baron, Murray; Bartlett, Susan J.; Costa Maia, Angela; El-Baalbaki, Ghassan; Furst, Daniel E.; Gottesman, Karen; Haythornthwaite, Jennifer A.; Hudson, Marie; Ann Impens, PhD; Korner, Annett; Leite, Catarina; Mayes, Maureen D.; Malcarne, Vanessa L.; Motivala, Sarosh J.; Mouthon, Luc; Nielson, Warren R.; Plante, Diane; Poiraudeau, Serge; Poole, Janet L.; Pope, Janet; Sauve, Maureen; Steele, Russell J.; Suarez-Almazor, Maria E.; Taillefer, Suzanne; van den Ende, Cornelia H.; Erin Arthurs, BSc; Bassel, Marielle; Delisle, Vanessa; Milette, Katherine; Leavens, Allison; Razykov, Ilya; Khanna, Dinesh

    2014-01-01

    Systemic sclerosis (SSc), or scleroderma, is a chronic multisystem autoimmune disorder characterised by thickening and fibrosis of the skin and by the involvement of internal organs such as the lungs, kidneys, gastrointestinal tract, and heart. Because there is no cure, feasibly-implemented and easily accessible evidence-based interventions to improve health-related quality of life (HRQoL) are needed. Due to a lack of evidence, however, specific recommendations have not been made regarding non-pharmacological interventions (e.g. behavioural/psychological, educational, physical/occupational therapy) to improve HRQoL in SSc. The Scleroderma Patient-centred Intervention Network (SPIN) was recently organised to address this gap. SPIN is comprised of patient representatives, clinicians, and researchers from Canada, the USA, and Europe. The goal of SPIN, as described in this article, is to develop, test, and disseminate a set of accessible interventions designed to complement standard care in order to improve HRQoL outcomes in SSc. PMID:22244687

  18. Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

    DEFF Research Database (Denmark)

    Jacobsen, Søren; Halberg, P; Ullman, S

    1998-01-01

    To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement.......To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement....

  19. Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma

    Directory of Open Access Journals (Sweden)

    Fatemeh Mokhtari

    2018-01-01

    Full Text Available Subcorneal pustular dermatosis (SPD or Sneddon-Wilkinson disease is a rare, benign, chronic, sterile pustular eruption which is associated with various systemic diseases including immunoglobinopathies, neoplasms, and autoimmune disorders. This paper reports a case of SPD in a patient with diffuse scleroderma in a 37-year-old woman. The hypothesis that immune dysregulation may play a role in the pathogenesis of SPD was supposed by the coexistence of diffuse scleroderma and SPD in our patient.

  20. Determination of muscle microcirculation of the limbs of healthy persons and patients with scleroderma by means of /sup 133/Xe clearance. 2. Examination of patients with progressive scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Poenitzsch, I.; Wiemers, U.; Haustein, U.F.; Schneider, G. (Karl-Marx-Universitaet, Leipzig (German Democratic Republic). Radiologische Klinik)

    1984-01-01

    By means of /sup 133/Xe muscle clearance the blood flow of the musculus tibialis anterior and the musculus opponens pollicis was determined during nonischemic work and after 3 minutes ischemia in patients with progressive scleroderma and additionally of the ischemic musculus opponens pollicis following contrast baths. In relation to 53 patients with normal vessels the reactive hyperemia of the musculus tibialis anterior after ischemia and of the musculus opponens pollicis after heat, cold as well as arterial flow decreasing was significantly decreased in patients with scleroderma and was as a disturbance of the microcirculation realized. For scientific problems in progressive scleroderma the /sup 133/Xe muscle clearance is suitable as to the musculus opponens pollicis.

  1. Systemic Sclerosis Sine Scleroderma in Mexican Patients. Case Reports.

    Science.gov (United States)

    Vera-Lastra, Olga; Sauceda-Casas, Christian Alexis; Domínguez, María Del Pilar Cruz; Alvarez, Sergio Alberto Mendoza; Sepulceda-Delgado, Jesús

    2017-01-03

    Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  2. Porphyria Cutanea Tarda Presenting with Scleroderma, Ichthyosis, Alopecia, and Vitiligo

    Directory of Open Access Journals (Sweden)

    Megan E. MacGillivray

    2018-05-01

    Full Text Available Porphyria cutanea tarda (PCT is a cutaneous porphyria that presents later in life with cutaneous findings in sun-exposed sites. We report a complex case of PCT in a 67-year-old woman with an unusual constellation of cutaneous findings: scleroderma, acquired ichthyosis, and nonscarring alopecia. Possible triggers for her PCT include tamoxifen treatment for breast cancer and carrier status of the hemochromatosis gene. High-dose chloroquine was used to successfully achieve clinical remission and normalize her uroporphyrins. While on chloroquine she developed extensive classic vitiligo. It is not clear if this is another feature of her complex and unusual PCT, or a consequence of her antimalarial therapy.

  3. A cross-sectional electromyography assessment in linear scleroderma patients

    Science.gov (United States)

    2014-01-01

    Background Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcutaneous and muscle atrophy. The aim was investigate nerve conduction and muscle involvement by electromyography in pediatric patients with LS. Methods We conducted a retrospective review of LS pediatric patients who had regular follow up at a single pediatric center from 1997–2013. We selected participants if they had consistently good follow up and enrolled consecutive patients in the study. We examined LS photos as well as clinical, serological and imaging findings. Electromyograms (EMG) were performed with bilateral symmetric technique, using surface and needle electrodes, comparing the affected side with the contralateral side. Abnormal muscle activity was categorized as a myopathic or neurogenic pattern. Results Nine LS subjects were selected for EMG, 2 with Parry-Romberg/Hemifacial Atrophy Syndrome, 7 linear scleroderma of an extremity and 2 with mixed forms (linear and morphea). Electromyogram analysis indicated that all but one had asymmetric myopathic pattern in muscles underlying the linear streaks. Motor and sensory nerve conduction was also evaluated in upper and lower limbs and one presented a neurogenic pattern. Masticatory muscle testing showed a myopathic pattern in the atrophic face of 2 cases with head and face involvement. Conclusion In our small series of LS patients, we found a surprising amount of muscle dysfunction by EMG. The muscle involvement may be possibly related to a secondary peripheral nerve involvement due to LS inflammation and fibrosis. Further collaborative studies to confirm these findings are needed. PMID:25053924

  4. The spectrum of muscle histopathologic findings in 42 weak scleroderma patients

    Science.gov (United States)

    Paik, Julie J.; Wigley, Fredrick M.; Lloyd, Thomas E.; Corse, Andrea M.; Casciola-Rosen, Livia; Shah, Ami A.; Boin, Francesco; Hummers, Laura K.; Mammen, Andrew L.

    2015-01-01

    Objective To determine if distinct muscle pathological features exist in scleroderma subjects with weakness. Methods This retrospective study included weak scleroderma subjects with muscle biopsies available for review. Biopsies were systematically assessed for individual pathologic features including inflammation, necrosis, fibrosis, and acute neurogenic atrophy. Based on the aggregate individual features, biopsies were assigned a histopathologic category of polymyositis, dermatomyositis, necrotizing myopathy, non-specific myositis, “acute denervation”, “fibrosis only”, or “other”. Clinical data analyzed included autoantibody profiles, scleroderma subtype and disease duration, Medsger muscle severity scores, creatine kinase (CK), electromyography (EMG), and muscle magnetic resonance imaging (MRI). Results 42 subjects (79% female and 64% diffuse scleroderma) were included in this study. Necrosis (67%), inflammation (48%), acute neurogenic atrophy (48%), and fibrosis (33%) were the most prevalent pathologic features. The presence of fibrosis was strongly associated with anti-PM-Scl antibodies. Histopathologic categories included non-specific myositis (36%), necrotizing myopathy (21%), dermatomyositis (7%), “acute denervation” (7%), “fibrosis only” (7%), and polymyositis (5%). Disease duration of scleroderma at the time of muscle biopsy was shorter in polymyositis than other histopathologic categories. Patients with anti-PM-Scl and Scl-70 antibodies also had a shorter disease duration than those with other auto-antibody profiles. Conclusion Non-specific myositis and necrotizing myopathy were the most common histopathologic categories in weak scleroderma subjects. Surprisingly, nearly half of the subjects studied had histological evidence of acute motor denervation (acute neurogenic atrophy); this has not been previously reported. Taken together, these observations suggest that a variety of pathologic mechanisms may underlie the development of

  5. Autoantigens targeted in scleroderma patients with vascular disease are enriched in endothelial lineage cells

    Science.gov (United States)

    McMahan, Zsuzsanna H.; Cottrell, Tricia R.; Wigley, Fredrick M.; Antiochos, Brendan; Zambidis, Elias T.; Park, Tea Soon; Halushka, Marc K.; Gutierrez-Alamillo, Laura; Cimbro, Raffaello; Rosen, Antony; Casciola-Rosen, Livia

    2016-01-01

    Objective Scleroderma patients with autoantibodies to centromere proteins (CENPs) and/or interferon-inducible protein 16 (IFI16) are at increased risk of severe vascular complications. We set out to define whether these autoantigens are enriched in cells of the vasculature. Methods Successive stages of embryoid bodies (EBs) as well as vascular progenitors were used to evaluate the expression of scleroderma autoantigens IFI16 and CENP by immunoblotting. CD31 was included to mark early blood vessels. IFI16 and CD31 expression were defined in skin paraffin sections from scleroderma patients and from healthy controls. IFI16 expression was determined by flow cytometry in circulating endothelial cells (CECs) and circulating progenitor cells (CPCs). Results Expression of CENP-A, IFI16 and CD31 was enriched in EBs at days 10 and 12 of differentiation, and particularly in cultures enriched in vascular progenitors (IFI16, CD31, CENPs A and-B). This pattern was distinct from that of comparator autoantigens. Immunohistochemical staining of skin paraffin sections showed enrichment of IFI16 in CD31-positive vascular endothelial cells in biopsies from scleroderma patients and normal controls. Flow cytometry analysis revealed IFI16 expression in CPCs, but minimal expression in CECs. Conclusion Expression of scleroderma autoantigens IFI16 and CENPs, which are associated with severe vascular disease, is increased in vascular progenitors and mature endothelial cells. High level, lineage-enriched expression of autoantigens may explain the striking association between clinical phenotypes and the immune targeting of specific autoantigens. PMID:27159521

  6. Hippocampal atrophy and developmental regression as first sign of linear scleroderma "en coup de sabre".

    Science.gov (United States)

    Verhelst, Helene E; Beele, Hilde; Joos, Rik; Vanneuville, Benedicte; Van Coster, Rudy N

    2008-11-01

    An 8-year-old girl with linear scleroderma "en coup de sabre" is reported who, at preschool age, presented with intractable simple partial seizures more than 1 year before skin lesions were first noticed. MRI revealed hippocampal atrophy, controlaterally to the seizures and ipsilaterally to the skin lesions. In the following months, a mental and motor regression was noticed. Cerebral CT scan showed multiple foci of calcifications in the affected hemisphere. In previously reported patients the skin lesions preceded the neurological signs. To the best of our knowledge, hippocampal atrophy was not earlier reported as presenting symptom of linear scleroderma. Linear scleroderma should be included in the differential diagnosis in patients with unilateral hippocampal atrophy even when the typical skin lesions are not present.

  7. Experiencing work as a daily challenge: the case of scleroderma.

    Science.gov (United States)

    Mendelson, Cindy; Poole, Janet L; Allaire, Saralynn

    2013-01-01

    Little is known about the physical and discretionary aspects of work that people with scleroderma (SSc) find difficult. This article describes the findings from a study that explored the challenges and adaptations made by individuals with SSc to continue to work. Thirty-two employed individuals with SSc participated. Participants were predominantly women (82%), white (79%), and well educated (M = 16.9 years). The average age was 47.3 years, and 60.6% were married. Mean disease duration was 9.7 years, and 56.2% had diffuse SSc. Mean years on the job was 10.2 (SD ± 8.8), and 71.9% worked at least 35 hours per week. Participants engaged in a single structured interview about work-related challenges and adaptations. Content and thematic analysis was used to identify key themes across the interviews. Employees with SSc experienced Work as a daily challenge. This central theme described the general work experience for most participants. Three subthemes described their specific experiences: The work environment: Opportunities, challenges, and accommodations; Career planning; and Supportive others. The participants were anxious to find scenarios that allowed them to continue to work. Worksite accommodations and flexibility in scheduling can make the difference between working and disability.

  8. Scleroderma and the temporomandibular joint: reconstruction in 2 variants.

    Science.gov (United States)

    MacIntosh, Robert Bruce; Shivapuja, Prasanna-Kumar; Naqvi, Rabia

    2015-06-01

    This article reviews the pathophysiology of scleroderma (systemic sclerosis [SSc]) and its destructive effects on the mandible in general and the temporomandibular joint (TMJ) in particular. It discusses the considerations of operating on patients with devastating chronic disease and presents 2 cases of TMJ reconstruction in patients with the diagnosis. Two patients with different degrees of SSc involvement underwent TMJ reconstruction with costochondral grafts. The patients represent the surgical considerations pertinent to this disease and different outcomes as determined by the variance in severity of their afflictions. The 2 patients tolerated the surgeries well and exhibited improvement in function in the long-term. One patient thrives and continues to do well despite her SSc approximately 10 years postoperatively; the second patient died of her disease approximately 9 years after her initial surgical care. The experience with these 2 cases showed that patients with SSc can safely undergo TMJ reconstruction with anticipated good results, but that the overall severity of the disease remains paramount in determining the feasibility of corrective surgery under this diagnosis. Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  9. local

    Directory of Open Access Journals (Sweden)

    Abílio Amiguinho

    2005-01-01

    Full Text Available The process of socio-educational territorialisation in rural contexts is the topic of this text. The theme corresponds to a challenge to address it having as main axis of discussion either the problem of social exclusion or that of local development. The reasons to locate the discussion in this last field of analysis are discussed in the first part of the text. Theoretical and political reasons are there articulated because the question is about projects whose intentions and practices call for the political both in the theoretical debate and in the choices that anticipate intervention. From research conducted for several years, I use contributions that aim at discuss and enlighten how school can be a potential locus of local development. Its identification and recognition as local institution (either because of those that work and live in it or because of those that act in the surrounding context are crucial steps to progressively constitute school as a partner for development. The promotion of the local values and roots, the reconstruction of socio-personal and local identities, the production of sociabilities and the equation and solution of shared problems were the dimensions of a socio-educative intervention, markedly globalising. This scenario, as it is argued, was also, intentionally, one of transformation and of deliberate change of school and of the administration of the educative territoires.

  10. Development and preliminary validation of the Scleroderma Support Group Leader Self-efficacy Scale

    NARCIS (Netherlands)

    Pal, N.E.; Gumuchian, S.T.; Delisle, V.C.; Pé pin, M.; Malcarne, V.L.; Carrier, M.E.; Kwakkenbos, C.M.C.; Pelá ez, S.; El-Baalbaki, G.; Thombs, B.D.

    2018-01-01

    Support groups are an important resource for people living with systemic sclerosis (SSc; scleroderma). Peer support group leaders play an important role in the success and sustainability of SSc support groups, but face challenges that include a lack of formal training. An SSc support group leader

  11. Nailfold capillaroscopy in systemic sclerosis: data from the EULAR scleroderma trials and research (EUSTAR) database.

    Science.gov (United States)

    Ingegnoli, Francesca; Ardoino, Ilaria; Boracchi, Patrizia; Cutolo, Maurizio

    2013-09-01

    The aims of this study were to obtain cross-sectional data on capillaroscopy in an international multi-center cohort of Systemic Sclerosis (SSc) and to investigate the frequency of the capillaroscopic patterns and their disease-phenotype associations. Data collected between June 2004 and October 2011 in the EULAR Scleroderma Trials and Research (EUSTAR) registry were examined. Patients' profiles based on clinical and laboratory data were obtained by cluster analysis and the association between profiles and capillaroscopy was investigated by multinomial logistic regression. 62 of the 110 EUSTAR centers entered data on capillaroscopy in the EUSTAR database. 376 of the 2754 patients (13.65%) were classified as scleroderma pattern absent, but non-specific capillary abnormalities were noted in 55.48% of the cases. Four major patients' profiles were identified characterized by a progressive severity for skin involvement, as well as an increased number of systemic manifestations. The "early" and "active" scleroderma patterns were generally observed in patients with mild/moderate skin involvement and a low number of disease manifestations, while the "late" scleroderma pattern was found more frequently in the more severe forms of the disease. These data indicate the importance of capillaroscopy in SSc management and that capillaroscopic patterns are directly related to the extent of organ involvement. Copyright © 2013 Elsevier Inc. All rights reserved.

  12. Frequency of motor alterations detected through manometry in patients with esophageal symptoms and scleroderma.

    Science.gov (United States)

    Pérez Y López, N; Lugo-Zamudio, G; Barbosa-Cobos, R E; Wong-Lam, A; Torres-López, E

    Scleroderma can present with esophageal involvement causing important morbidity. To describe the manometric findings and clinical characteristics of patients with scleroderma and esophageal symptoms. Patients with scleroderma and esophageal symptoms were evaluated through esophageal manometry within the time frame of one year. Descriptive statistics were carried out and the continuous variables were expressed as means and standard deviation. Frequencies were expressed as percentages. The study included 24 female patients with a mean age of 53.5 years and mean disease progression of 7.84 years. The most frequent findings were short and hypotonic lower esophageal sphincter (mean length 1.58cm and mean tone 9.49mmHg) and ineffective esophageal motility (mean non-transmitted waves 92.91%, mean effective primary peristalsis 40.05%, and mean amplitude 13.11mmHg). The most frequent symptom was dysphagia. Scleroderma is associated with lower esophageal sphincter alterations and symptomatic ineffective esophageal motility. Copyright © 2017 Asociación Mexicana de Gastroenterología. Publicado por Masson Doyma México S.A. All rights reserved.

  13. U3 snoRNP associates with fibrillarin a component of the scleroderma clumpy nucleolar domain

    DEFF Research Database (Denmark)

    Herrera-Esparza, Rafael; Kruse, Lars; von Essen, Marina

    2002-01-01

    by ELISA was recognized by the clumpy scleroderma serum from the majority of patients. In situ hybridization assays showed that the fibrillarin tagged by the elicited antibodies was colocalized with U3 snoRNP in the nucleolus in a clumpy manner and coprecipitated the U3 snoRNP. In conclusion...

  14. Reduced coronary flow and resistance reserve in primary scleroderma myocardial disease

    International Nuclear Information System (INIS)

    Nitenberg, A.; Foult, J.M.; Kahan, A.; Perennec, J.; Devaux, J.Y.; Menkes, C.J.; Amor, B.

    1986-01-01

    The maximum coronary vasodilator capacity after intravenous dipyridamole (0.14 mg X kg-1 X min-1 X 4 minutes) was studied in seven patients with primary scleroderma myocardial disease and compared to that of seven control subjects. Hemodynamic data and left ventricular angiographic data were not different in the two groups. The coronary flow reserve was evaluated by the dipyridamole/basal coronary sinus blood flow ratio (D/B CSBF) and the coronary resistance reserve by the dipyridamole/basal coronary resistance ratio (D/B CR). Coronary reserve was greatly impaired in the group with primary scleroderma myocardial disease: D/B CSBF was lower than in the control group (2.54 +/- 1.37 vs 4.01 +/- 0.56, respectively; p less than 0.05) and D/B CR was higher than in the control group (0.47 +/- 0.25 vs 0.23 +/- 0.04, respectively; p less than 0.05). Such a decreased coronary flow and resistance reserve in patients with primary scleroderma myocardial disease was not explained by an alteration of left ventricular function. It may be an important contributing factor in the pathogenesis of primary scleroderma myocardial disease

  15. Parapiptadenia rigida MYCORRHIZATION WITH SPORES OF Scleroderma citrinum

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    Gerusa Pauli Kist Steffen

    2017-06-01

    Full Text Available Ectomycorrhizal fungal inoculation in forestry seedlings aids plant establishment and growth in the field. The objectives of this study were: to determine the mycorrhizal capacity of the ectomycorrhizal fungus Scleroderma citrinum in Parapiptadenia rigida (red angico seedlings and to evaluate the viability of a mycorrhizal inoculation technique for forest seedlings involving the use of spores. Mature spores were inoculated in the substrate (75% soil and 25% carbonized rice husk, totaling 1.5 grams of fungal spores per liter of substrate. P. rigida seeds were sown in substrates inoculated or not inoculated with fungal spores in presence or absence of Pinus echinata and Eucalyptus citriodora essential oil: not inoculated (T1, inoculated (T2, inoculated more pine essential oil (T3, inoculated more eucalyptus essential oil (T4. Seedlings of Pinus elliottii were used for a positive control of mycorrhizal inoculation (T5 and not inoculated (T6 with fungal spores. At 90 days after sowing, the base stem diameter, height, fresh and dry weight of shoots and roots, percentage of root colonization and Dickson Index were determined. The presence of fungal structures in P. rigida and P. elliottii roots inoculated with S. citrinum spores was observed, demonstrating the occurrence of an ectomycorrhizal association. The application of pine and eucalyptus essential oils in the substrate increased the percentage of ectomycorrhizal colonization in P. rigida seedlings. The addition of S. citrinum mature spores in the substrate used for seedling production is a viable practice for ectomycorrhizal inoculation and it can be used in forest nurseries in controlled mycorrhization programs.

  16. Fox-2 protein regulates the alternative splicing of scleroderma-associated lysyl hydroxylase 2 messenger RNA.

    Science.gov (United States)

    Seth, Puneet; Yeowell, Heather N

    2010-04-01

    Scleroderma (systemic sclerosis [SSc]) is a complex connective tissue disorder characterized by hardening and thickening of the skin. One hallmark of scleroderma is excessive accumulation of collagen accompanied by increased levels of pyridinoline collagen crosslinks derived from hydroxylysine residues in the collagen telopeptide domains. Lysyl hydroxylase 2 (LH2), an important alternatively spliced enzyme in collagen biosynthesis, acts as a collagen telopeptide hydroxylase. Changes in the pattern of LH2 alternative splicing, favoring increased inclusion of the alternatively spliced LH2 exon 13A, thereby increasing the levels of the long transcript of LH2 (LH2[long]), are linked to scleroderma disease. This study was undertaken to examine the role played by RNA binding protein Fox-2 in regulating exon 13A inclusion, which leads to the generation of scleroderma-associated LH2(long) messenger RNA (mRNA). Phylogenetic sequence analysis of introns flanking exon 13A was performed. A tetracycline-inducible system in T-Rex 293 cells was used to induce Fox-2 protein, and endogenous LH2(long) mRNA was determined by reverse transcriptase-polymerase chain reaction. An LH2 minigene was designed, validated, and used in Fox-2 overexpression and mutagenesis experiments. Knockdown of Fox-2 was performed in mouse embryonic fibroblasts and in fibroblasts from SSc patients. Overexpression of Fox-2 enhanced the inclusion of exon 13A and increased the generation of LH2(long) mRNA, whereas knockdown of Fox-2 decreased LH2(long) transcripts. Mutational analysis of an LH2 minigene demonstrated that 2 of the 4 Fox binding motifs flanking LH2 exon 13A are required for inclusion of exon 13A. In early passage fibroblasts derived from patients with scleroderma, the knockdown of Fox-2 protein significantly decreased the endogenous levels of LH2(long) mRNA. Our findings indicate that Fox-2 plays an integral role in the regulation of LH2 splicing. Knockdown of Fox-2 and other methods to decrease

  17. Pigmentation abnormalities in systemic scleroderma examined by using a colorimeter (Choromo Meter CR-200).

    Science.gov (United States)

    Maeda, M; Kachi, H; Matubara, K; Mori, S; Kitajima, Y

    1996-03-01

    Cutaneous colors of the dorsum of the hands (A), the distal forearms (B; 5 cm from the wrists), the proximal forearm (C; proximal 1/3 from the elbow) and sternal skin region (D) in patients with systemic scleroderma (73 cases; M:F = 16:57) systemic lupus erythematosus (SLE) or dermatomyositis (27 cases; M:F = 7:20) and healthy controls (HC) (36 cases; M:F = 8:28) was characterized by a XYZ colorimetric system (CIE, 1931) using a colorimeter (Choromo Meter CR-200, Minolta Camera Co. Ltd., Osaka). The index Y, which means color value shows a lower value in male HC and in patients with systemic scleroderma, especially in the more severe type with hyperpigmentation (score 5-6; the system proposed by Ishikawa) than that of female HC. The values of indices x and y, which relate to reddish (erythema with hyperpigmentation) and greenish color (pale), respectively, were higher in the exposed portion of the severe type of systemic scleroderma with hyperpigmentation, especially male and older patients, and in unexposed portions of the female group without hyperpigmentation. Histopathologically, there was prominent pigmentation in the upper dermis of the forearm in the severe type of systemic scleroderma, so that melanin quantity may be closely related to the decrease in index Y. There was no statistical significance in the value of indices Y, x and y between HC, SLE and dermatomyositis. This method may contribute not only to diagnosis of systemic scleroderma and differentiation from other collagen diseases, but also studies of clinical follow-up and effects of medication.

  18. β-thymosins and interstitial lung disease: study of a scleroderma cohort with a one-year follow-up

    Directory of Open Access Journals (Sweden)

    Messana Irene

    2011-02-01

    Full Text Available Abstract Background β-thymosins play roles in cytoskeleton rearrangement, angiogenesis, fibrosis and reparative process, thus suggesting a possible involvement in the pathogenesis of systemic sclerosis. The aim of the study was to investigate the presence of thymosins β4, β4 sulfoxide, and β10 in bronchoalveolar lavage fluid of scleroderma patients with interstitial lung disease and the relation of these factors with pulmonary functional and radiological parameters. Methods β-thymosins concentrations were determined by Reverse Phase-High Performance Liquid Chromatography-Electrospray-Mass Spectrometry in the bronchoalveolar lavage fluid of 46 scleroderma patients with lung involvement and of 15 controls. Results Thymosin β4, β4 sulfoxide, and β10 were detectable in bronchoalveolar lavage fluid of patients and controls. Thymosin β4 levels were significantly higher in scleroderma patients than in controls. In addition, analyzing the progression of scleroderma lung disease at one-year follow-up, we have found that higher thymosin β4 levels seem to have a protective role against lung tissue damage. Thymosin β4 sulfoxide levels were higher in the smokers and in the scleroderma patients with alveolitis. Conclusions We describe for the first time β-thymosins in bronchoalveolar lavage fluid and their possible involvement in the pathogenesis of scleroderma lung disease. Thymosin β4 seems to have a protective role against lung tissue damage, while its oxidation product mirrors an alveolar inflammatory status.

  19. Occupational and environmental scleroderma. Systematic review and meta-analysis.

    Science.gov (United States)

    Rubio-Rivas, Manuel; Moreno, Rafael; Corbella, Xavier

    2017-03-01

    The etiology of systemic sclerosis (SSc) remains unknown; however, several occupational and environmental factors have been implicated. Our objective was to perform a meta-analysis of all studies published on SSc associated with occupational and environmental exposure. The review was undertaken by means of MEDLINE and SCOPUS from 1960 to 2014 and using the terms: "systemic," "scleroderma," or "systemic sclerosis/chemically induced" [MesH]. The Newcastle-Ottawa Scale was used for the qualifying assessment. The inverse variance-weighted method was performed. The meta-analysis of silica exposure included 15 case-control studies [overall OR 2.81 (95%CI 1.86-4.23; p < 0.001)] and 4 cohort studies [overall RR 17.52 (95%CI 5.98-51.37; p < 0.001)]; the meta-analysis of solvents exposure included 13 case-control studies (overall OR 2.00 [95%CI 1.32-3.02; p = 0.001); the meta-analysis of breast implants exposure included 4 case-control studies (overall OR 1.68 (95%CI 1.65-1.71; p < 0.001)) and 6 cohort studies (overall RR 2.13 (95%CI 0.86-5.27; p = 0.10)); the meta-analysis of epoxy resins exposure included 4 case-control studies (overall OR 2.97 (95%CI 2.31-3.83; p < 0.001)), the meta-analysis of pesticides exposure included 3 case-control studies (overall OR 1.02 (95%CI 0.78-1.32; p = 0.90)) and, finally, the meta-analysis of welding fumes exposure included 4 studies (overall OR 1.29 (95%CI 0.44-3.74; p = 0.64)). Not enough studies citing risks related to hair dyes have been published to perform an accurate meta-analysis. Silica and solvents were the two most likely substances related to the pathogenesis of SSc. While silica is involved in particular jobs, solvents are widespread and more people are at risk of having incidental contact with them.

  20. Comparing HLA shared epitopes in French Caucasian patients with scleroderma.

    Directory of Open Access Journals (Sweden)

    Doua F Azzouz

    Full Text Available Although many studies have analyzed HLA allele frequencies in several ethnic groups in patients with scleroderma (SSc, none has been done in French Caucasian patients and none has evaluated which one of the common amino acid sequences, (67FLEDR(71, shared by HLA-DRB susceptibility alleles, or (71TRAELDT(77, shared by HLA-DQB1 susceptibility alleles in SSc, was the most important to develop the disease. HLA-DRB and DQB typing was performed for a total of 468 healthy controls and 282 patients with SSc allowing FLEDR and TRAELDT analyses. Results were stratified according to patient's clinical subtypes and autoantibody status. Moreover, standardized HLA-DRß1 and DRß5 reverse transcriptase Taqman PCR assays were developed to quantify ß1 and ß5 mRNA in 20 subjects with HLA-DRB1*15 and/or DRB1*11 haplotypes. FLEDR motif is highly associated with diffuse SSc (χ(2 = 28.4, p<10-6 and with anti-topoisomerase antibody (ATA production (χ(2 = 43.9, p<10-9 whereas TRAELDT association is weaker in both subgroups (χ(2 = 7.2, p = 0.027 and χ(2 = 14.6, p = 0.0007 respectively. Moreover, FLEDR motif- association among patients with diffuse SSc remains significant only in ATA subgroup. The risk to develop ATA positive SSc is higher with double dose FLEDR than single dose with respectively, adjusted standardised residuals of 5.1 and 2.6. The increase in FLEDR motif is mostly due to the higher frequency of HLA-DRB1*11 and DRB1*15 haplotypes. Furthermore, FLEDR is always carried by the most abundantly expressed ß chain: ß1 in HLA DRB1*11 haplotypes and ß5 in HLA-DRB1*15 haplotypes.In French Caucasian patients with SSc, FLEDR is the main presenting motif influencing ATA production in dcSSc. These results open a new field of potential therapeutic applications to interact with the FLEDR peptide binding groove and prevent ATA production, a hallmark of severity in SSc.

  1. Scleroderma renal crisis in a case of mixed connective tissue disease

    Directory of Open Access Journals (Sweden)

    Mukul Vij

    2014-01-01

    Full Text Available Mixed connective tissue disease (MCTD is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma and polymyositis. Scleroderma renal crisis (SRC is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

  2. Scleroderma renal crisis in a case of mixed connective tissue disease.

    Science.gov (United States)

    Vij, Mukul; Agrawal, Vinita; Jain, Manoj

    2014-07-01

    Mixed connective tissue disease (MCTD) is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma) and polymyositis. Scleroderma renal crisis (SRC) is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.

  3. Netrin-1 regulates fibrocyte accumulation in the decellularized fibrotic scleroderma lung microenvironment and in bleomycin induced pulmonary fibrosis

    Science.gov (United States)

    Sun, Huanxing; Zhu, Yangyang; Pan, Hongyi; Chen, Xiaosong; Balestrini, Jenna L.; Lam, TuKiet T.; Kanyo, Jean E.; Eichmann, Anne; Gulati, Mridu; Fares, Wassim H.; Bai, Hanwen; Feghali-Bostwick, Carol A.; Gan, Ye; Peng, Xueyan; Moore, Meagan W.; White, Eric S.; Sava, Parid; Gonzalez, Anjelica L.; Cheng, Yuwei; Niklason, Laura E.; Herzog, Erica L.

    2017-01-01

    Objectives Fibrocytes are collagen-producing leukocytes that accumulate in Scleroderma-associated interstitial lung disease (SSc-ILD) via unknown mechanisms. The extracellular matrix (ECM) influences cellular phenotypes. However, a relationship between the lung ECM and fibrocytes in Scleroderma has not been explored. This study uses a novel translational platform based on decellularized human lungs to determine whether the scleroderma lung ECM controls fibrocyte development from peripheral blood mononuclear cells. Methods Decellularized scaffolds prepared from healthy and fibrotic Scleroderma lung explants underwent biomechanical evaluation using tensile testing and biochemical analysis using proteomics. Cells from healthy and SSc-ILD subjects were cultured on these scaffolds, and CD45+Pro-ColIα1+ cells meeting criteria for fibrocytes were quantified. The contribution of Netrin-1 to fibrosis was assessed using neutralizing antibodies in this system and via the inhalational administration of bleomycin to Netrin-1+/− mice. Results Compared to control lung scaffold, SSc-ILD lung scaffolds showed aberrant anatomy, enhanced stiffness, and abnormal extracellular matrix composition. Culture of control cells in Scleroderma scaffolds increased Pro-ColIα1+ production, which was stimulated by enhanced stiffness and abnormal ECM composition. SSc-ILD cells demonstrated increased Pro-ColIα1 responsiveness to Scleroderma lung scaffolds, but not enhanced stiffness. Enhanced Netrin-1 expression was seen on CD14lo SSc-ILD cells and antibody mediated Netrin-1 neutralization attenuated CD45+Pro-ColIα1+ detection in all settings. Netrin-1+/− mice were protected from bleomycin induced lung fibrosis and fibrocyte accumulation. Conclusion Factors present in Scleroderma lung matrices regulate fibrocyte accumulation via a Netrin-1-dependent pathway. Netrin-1 regulates bleomycin induced murine pulmonary fibrosis. Netrin-1 might be a novel therapeutic target in SSc-ILD. PMID:26749424

  4. Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis.

    Science.gov (United States)

    Ghossein, Cybele; Varga, John; Fenves, Andrew Z

    2016-01-01

    Scleroderma renal crisis (SRC) is an uncommon complication of systemic sclerosis. Despite the advent of angiotensin-converting inhibitor therapy, SRC remains a life-threatening complication. Recent studies have contributed to a better understanding of SRC, but much remains unknown regarding its pathophysiology, risk factors, and optimal management. Genetic studies provide evidence that immune dysregulation might be a contributing factor, providing hope that further research in this direction might illuminate pathogenesis and provide novel predictors for this complication.

  5. Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

    Directory of Open Access Journals (Sweden)

    Irina Khamaganova

    2018-01-01

    Full Text Available Similar clinical and histhopathological features in progressive hemifacial atrophy and linear scleroderma en coup de sabre are well known. Trauma may predispose to the development of both diseases. The lack of association with anti-Borrelia antibodies was shown in both cases as well. The otolaryngological and endocrine disorders may be associated findings in both diseases. However, there are certain differences in neurological and ophthalmological changes in the diseases.

  6. Mechanical properties of the gastro-esophageal junction in health, achalasia, and scleroderma.

    Science.gov (United States)

    Mearin, F; Fonollosa, V; Vilardell, M; Malagelada, J R

    2000-07-01

    Manometric assessment of the gastro-esophageal junction (GEJ) is deceptive in that ignores key dynamic properties of the junction, such as resistance to flow and compliance. Our aim was to investigate the mechanical properties of the GEJ comprising intraluminal pressure (measured by manometry), resistance to flow and compliance (measured by resistometry). We studied 8 healthy subjects, 11 patients with achalasia and 11 patients with scleroderma. We used a pneumatic resistometer, previously developed and validated in our laboratory. The resistometer consists of a flaccid polyurethane 5-cm cylinder connected to an electronically regulated nitrogen-injection system; the instrument records nitrogen flow through the cylinder while maintaining a constant pressure gradient between its proximal and distal ends. By placing the cylinder successively in the proximal stomach and along the GEJ we measured the GEJ-gastric resistance gradient (GEJ resistance minus gastric resistance) and were able to calculate the cumulative resistance (sum of resistance exerted at each pressure level), peak resistance (at any injection pressure), nil resistance point (injection pressure in mmHg at which GEJ resistance equals gastric resistance), and compliance slope (flow/pressure relationship). We found that GEJ resistance to flow (cumulative resistance, peak resistance, and nil resistance point) is significantly increased in achalasia and decreased in scleroderma (P < 0.05 versus health) while GEJ compliance is diminished in achalasia (P < 0.05 versus health) and normal in scleroderma. Achalasia is a disease characterized by increased GEJ resistance and rigidity. By contrast, although scleroderma is characterized by decreased GEJ resistance, GEJ compliance may be normal.

  7. Antitopoisomerase antibody positivity predates nailfold capillaroscopy abnormalities in scleroderma. Postulated classification of 'prescleroderma'.

    Science.gov (United States)

    Englert, H; Champion, D; Wu, J C; Giallussi, J; McGrath, M; Manolios, N

    2011-02-01

    In a patient with early topoisomerase antibody-positive scleroderma, antinuclear antibody positivity was fortuitously observed to predate nailfold capillaroscopy changes. Using this case as a template, the prediagnostic phase of the presumed multifactorial disease may be divided into 5 temporal phases--phase 1 representing conception and intrauterine environment, phase 2 representing the extrauterine environment predating environmental exposure; phase 3 representing the early post-environmental exposure interval with no detectable perturbed body status; phase 4 representing the post-environmental exposure interval characterized by autoantibody production and microvascular changes, and phase 5, the symptomatic clinical prediagnostic interval (Raynaud's, skin, musculoskeletal, gastrointestinal, cardiorespiratory) prompting scleroderma diagnosis. Temporal classification of prescleroderma aids in both the understanding and definition of scleroderma 'onset'. If altered nailfold capillaries and autoantibodies develop at comparable rates, and if the findings from this case--that autoantibody changes precede microvascular changes--are truly representative of the preclinical disease phase, then these findings argue that the evolution of the disease is from within the vessel outwards, rather than vice versa. © 2011 The Authors. Internal Medicine Journal © 2011 Royal Australasian College of Physicians.

  8. Radiotherapy for malignancy in patients with scleroderma: The Mayo Clinic experience

    International Nuclear Information System (INIS)

    Gold, Douglas G.; Miller, Robert C.; Petersen, Ivy A.; Osborn, Thomas G.

    2007-01-01

    Purpose: To determine the frequency of acute and chronic adverse effects in patients with scleroderma who receive radiotherapy for treatment of cancer. Methods and Materials: Records were reviewed of 20 patients with scleroderma who received radiotherapy. Acute and chronic toxic effects attributable to radiotherapy were analyzed, and freedom from radiation-related toxicity was calculated. Results: Of the 20 patients, 15 had acute toxic effects, with Grade 3 or higher toxicity for 3 patients. Seven patients had self-limited Grade 1 or 2 radiation dermatitis, and no patient had Grade 3 or higher radiation dermatitis. Thirteen patients had chronic toxic effects, with Grade 3 or higher chronic toxicity for 3 patients. The median estimated time to any grade chronic toxicity was 0.4 years, and the median estimated time to Grade 3 or higher chronic toxicity has not been reached. Conclusions: The results suggest that although some patients with scleroderma treated with radiation experience considerable toxic effects, the occurrence of Grade 3 or higher toxicity may be less than previously anticipated

  9. Autologous fat transplantation for depressed linear scleroderma-induced facial atrophic scars.

    Science.gov (United States)

    Roh, Mi Ryung; Jung, Jin Young; Chung, Kee Yang

    2008-12-01

    Facial linear scleroderma results in depressed atrophic scars. Autologous fat transplantation has been widely used, and fat appears to be an ideal material for filling depressed atrophic scars and contour deformities, but long-term results for autologous fat transplantation are controversial. To review the short- and long-term results of 20 patients who underwent multiple autologous fat transplantations for depressed atrophic scar correction. Twenty patients with clinically inactive facial linear scleroderma were included. They received at least two transplantations and had a 12-month follow-up evaluation. On the forehead, 51% to 75% improvement (average grading scale: 2.4) was achieved when observed at least 12 months after the last treatment. For the chin, correction was poor (average grading scale: 0.7) with less than 25% improvement. The infraorbital area showed fair correction, but the nose showed poor correction. Two of three patients with scalp reduction surgery showed excellent results, showing only slight scar widening. Autologous fat transplantation is an effective method for long-term correction of depressed atrophic scars left by linear scleroderma on the forehead but is less effective for corrections on the nose, infraorbital area, and chin.

  10. Paraneoplastic scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia: a report of 10 cases.

    Science.gov (United States)

    Magro, Cynthia M; Iwenofu, Hans; Nuovo, Gerard J

    2013-07-01

    Systemic plasma cell dyscrasias have diverse manifestations in the skin and include an inflammatory paraneoplastic process. We encountered cases of scleroderma and eosinophilic fasciitis in the setting of an underlying plasma cell dyscrasia. Ten cases of scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia were encountered. The biopsies were stained for Transforming growth factor (Transforming growth factor) beta, IgG4, kappa, and lambda. Patients presented with a sclerodermoid reaction represented by eosinophilic fasciitis (5 cases), morphea (3 cases), and systemic scleroderma (2 cases). The mean age of presentation was 70 years with a striking female predominance (4:1). Acral accentuation was noted in 8 cases. In 6 of the cases, the cutaneous sclerosis antedated (4 cases) by weeks to 2 years or occurred concurrently (2 cases) with the initial diagnosis of the plasma cell. The biopsies showed changes typical of eosinophilic fasciitis and/or scleroderma. In 5 cases, light chain-restricted plasma cells were present on the biopsy. There was staining of the plasma cells for Transforming growth factor beta in 3 out of 5 cases tested. In any older patient presenting with a sudden onset of eosinophilic fasciitis or scleroderma especially with acral accentuation, investigations should be conducted in regards to an underlying plasma cell dyscrasia.

  11. Evaluation of Peripheral Blood Circulation Disorder in Scleroderma Patients Using an Optical Sensor with a Pressurization Mechanism.

    Directory of Open Access Journals (Sweden)

    Yoshiki Yamakoshi

    Full Text Available Blood circulation function of peripheral blood vessels in skin dermis was evaluated employing an optical sensor with a pressurization mechanism using the blood outflow and reflow characteristics. The device contains a light source and an optical sensor. When applied to the skin surface, it first exerts the primary pressure (higher than the systolic blood pressure, causing an outflow of blood from the dermal peripheral blood vessels. After two heartbeats, the pressure is lowered (secondary pressure and blood reflows into the peripheral blood vessels. Hemoglobin concentration, which changes during blood outflow and reflow, is derived from the received light intensity using the Beer-Lambert law. This method was evaluated in 26 healthy female volunteers and 26 female scleroderma patients. In order to evaluate the blood circulation function of the peripheral blood vessels of scleroderma patients, pressurization sequence which consists of primary pressure followed by secondary pressure was adopted. Blood reflow during the first heartbeat period after applying the secondary pressure of 40mmHg was (mean±SD 0.059±0.05%mm for scleroderma patients and 0.173±0.104%mm for healthy volunteers. Blood reflow was significantly lower in scleroderma patients than in healthy volunteers (p<0.05. This result indicates that the information necessary for assessing blood circulation disorder of peripheral blood vessels in scleroderma patients is objectively obtained by the proposed method.

  12. Metabolism of carbon-14 labelled l-tryptophan, l-kynerenine and hydroxy-l-kynerenine in miners with scleroderma

    International Nuclear Information System (INIS)

    Hankes, L.V.; De Bruin, E.; Jansen, C.R.; Voster, L.; Schmaeler, M.

    1977-01-01

    Six South African white miners were studied with the 2-g l-tryptophan load test and tracer doses of L-tryptophan-7a-carbon-14, L-kynurenine-keto-carbon-14 and hydroxy-L-kynerenine-keto-carbon-14. The breath 14 CO 2 and 14 urinary metabolites were measured. When they were compared with a previous study of American women with scleroderma, similar 14 CO 2 and tryptophan metabolite excretion patterns were observed in the data from the miners. The labelled quinolinic acid excretion was more significantly elevated in the South African miners' urine than in the urine of the American women. The data from both studies suggest that some patients with scleroderma have an altered step in the tryptophan metabolic pathway after hydroxy-anthranilic acid. What relationship exists between the induction of pulmonary silicosis and the subsequent development of scleroderma, requires additional human studies

  13. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension.

    Science.gov (United States)

    Fox, Benjamin D; Shimony, Avi; Langleben, David; Hirsch, Andrew; Rudski, Lawrence; Schlesinger, Robert; Eisenberg, Mark J; Joyal, Dominique; Hudson, Marie; Boutet, Kim; Serban, Alexandrina; Masetto, Ariel; Baron, Murray

    2013-10-01

    Our study aimed to determine the prevalence of occult left-heart disease in patients with scleroderma and pulmonary hypertension. In patients with pulmonary hypertension (mean pulmonary artery pressure (mean PAP)≥25 mmHg), differentiation between pre- and post-capillary pulmonary hypertension has been made according to pulmonary artery wedge pressure (PAWP) less than or more than 15 mmHg, respectively. We performed a retrospective chart review of 107 scleroderma patients. All patients with suspected pulmonary hypertension had routine right or left heart catheterisation with left ventricular end-diastolic pressure (LVEDP) measurement pre-/post-fluid challenge. We extracted demographic, haemodynamic and echocardiographic data. Patients were classified into one of four groups: haemodynamically normal (mean PAP15 mmHg); occult PVH (mean PAP≥25 mmHg, PAWP≤15 mmHg, LVEDP>15 mmHg before or after fluid challenge); and pulmonary arterial hypertension (PAH) (mean PAP≥25 mmHg, PAWP≤15 mmHg and LVEDP≤15 mmHg before or after fluid challenge). 53 out of 107 patients had pulmonary hypertension. Based on the PAWP-based definition, 29 out of 53 had PAH and 24 out of 53 had PVH. After considering the resting and post-fluid-challenge LVEDP, 11 PAH patients were reclassified as occult PVH. The occult PVH group was haemodynamically, echocardiographically and demographically closer to the PVH group than the PAH group. PVH had high prevalence in our scleroderma-pulmonary hypertension population. Distinguishing PAH from PVH with only PAWP may result in some PVH patients being misclassified as having PAH.

  14. Scleroderma and pulmonary hypertension Esclerodermia e hipertensão pulmonar

    Directory of Open Access Journals (Sweden)

    Karen A. Fagan

    2003-10-01

    Full Text Available Patients with scleroderma are at increased risk for the development of pulmonary hypertension, and the development of unexplained dyspnea or an isolated decrease in diffusing capacity should prompt evaluation. Echocardiography is often helpful in this situation, with further testing being performed as indicated. Because the prognosis of untreated pulmonary hypertension occurring in the setting of scleroderma is generally quite poor, vigilance is required on the part of physicians following this "at risk" group of patients. The past decade has seen important advances in the treatment of pulmonary arterial hypertension, including intravenous epoprostenol, oral bosentan and subcutaneously infused treprostinil. As new therapies are developed for the treatment of pulmonary arterial hypertension, it is essential that patients with scleroderma-related disease are included in clinical trials.Pacientes com esclerodermia têm risco aumentado para desenvolver hipertensão pulmonar. O aparecimento de dispnéia e/ou a diminuição da capacidade de difusão devem levar à suspeita imediata dessa complicação. A ecodopplercardiografia é importante para o diagnóstico e o seguimento desses casos. Os casos não tratados de hipertensão pulmonar em esclerodermia têm mau prognóstico, daí a necessidade em manter sob vigilância estes pacientes. Na última década surgiram avanços para o tratamento da hipertensão arterial pulmonar, incluindo os medicamentos epoprostenol EV, bosentan VO e treprostinil SC. À medida que novas terapias vão sendo desenvolvidas, torna-se necessário a realização de estudos clínicos de maior validade.

  15. Scleroderma "en coup de sabre" With Epilepsy and Uveitis Successfully Treated With Tocilizumab.

    Science.gov (United States)

    Osminina, Maria; Geppe, Nathalia; Afonina, Elena

    2018-06-18

    Case history of a small girl outlet with epilepsy, followed by scleroderma skin damage and uveitis, neurovasculitis with white matter foci in brain on the side of skin lesion in two months, immunologic disease activity. Resistance to conventional immunosuppressive therapy forced us to initiate the treatment with tocilizumab. It was well tolerated and led to significant improvement of brain, ocular and skin manifestations. Copyright © 2018 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  16. The preliminary evaluation on sclerodermas of newborn baby with thyroid hormone and β2-MG ria

    International Nuclear Information System (INIS)

    Liu Junchi; Du Sujun

    1995-01-01

    The combined determination of thyroid hormone and β 2 -MG may be used as the sensitive indication of the early degeneration of liver and kidney function. It is probably due to the damage of liver and kidney by scleroderma in newborn baby. The observation of 68 cases show that the serum levels of T 3 ,FT 3 in the concentration of thyroid hormone decreased significantly, and syndrome of the low serum T 3 , high serum rT 3 are taken place. Whereas, the serum level of β 2 -MG is increased significantly

  17. Spinal subarachnoid hemorrhage caused by scleroderma-induced aneurysm: a case report

    International Nuclear Information System (INIS)

    Mueller, J.; Neidl, K.; Contier-Dippel, B.; Huber, G.; Ernst, E.

    1995-01-01

    We introduce a 58-year-old woman who suffered from progressive systemic scleroderma (PSS) associated with trigeminal sensory neuropathy for approximately 10 years. She then had a stroke from spinal subarachnoid hemorrhage (SSAH). Spinal digital subtraction angiography (DSA) revealed two aneurysms and smaller dilations of the afferent vessel that could also be seen by MRI. Three asymptomatic brain infarctions in different vascular regions could be revealed by CCT. The SSAH, ischemic lesions and aneurysms were probably caused by vasculitic affections induced by PSS. (orig.)

  18. Spinal subarachnoid hemorrhage caused by scleroderma-induced aneurysm: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, J. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Neidl, K. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Contier-Dippel, B. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Huber, G. [Inst. fuer Neuroradiologie der Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Ernst, E. [Neurologische Abt., Caritas Krankenhaus, Dillingen (Germany)

    1995-11-01

    We introduce a 58-year-old woman who suffered from progressive systemic scleroderma (PSS) associated with trigeminal sensory neuropathy for approximately 10 years. She then had a stroke from spinal subarachnoid hemorrhage (SSAH). Spinal digital subtraction angiography (DSA) revealed two aneurysms and smaller dilations of the afferent vessel that could also be seen by MRI. Three asymptomatic brain infarctions in different vascular regions could be revealed by CCT. The SSAH, ischemic lesions and aneurysms were probably caused by vasculitic affections induced by PSS. (orig.)

  19. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR)

    NARCIS (Netherlands)

    Kowal-Bielecka, O.; Landewé, R.; Avouac, J.; Chwiesko, S.; Miniati, I.; Czirjak, L.; Clements, P.; Denton, C.; Farge, D.; Fligelstone, K.; Földvari, I.; Furst, D. E.; Müller-Ladner, U.; Seibold, J.; Silver, R. M.; Takehara, K.; Toth, B. Garay; Tyndall, A.; Valentini, G.; van den Hoogen, F.; Wigley, F.; Zulian, F.; Matucci-Cerinic, Marco

    2009-01-01

    The optimal treatment of systemic sclerosis (SSc) is a challenge because the pathogenesis of SSc is unclear and it is an uncommon and clinically heterogeneous disease affecting multiple organ systems. The aim of the European League Against Rheumatism (EULAR) Scleroderma Trials and Research group

  20. Inhibition of collagen production in scleroderma fibroblast cultures by a connective tissue glycoprotein extracted from normal dermis

    International Nuclear Information System (INIS)

    Maquart, F.X.; Bellon, G.; Cornillet-Stoupy, J.; Randoux, A.; Triller, R.; Kalis, B.; Borel, J.P.

    1985-01-01

    It was shown in a previous paper that a connective tissue glycoprotein (CTGP) extracted from normal rabbit dermis was able to inhibit total protein and collagen syntheses by normal dermis fibroblast cultures. In the present study, the effects of CTGP on scleroderma fibroblasts were investigated. [ 14 C]Proline incorporation into total proteins of the supernatant was not significantly different from that found in controls. By contrast, the amount of collagen, expressed as percentage of total secreted protein, was far higher in scleroderma cultures than in normal ones (14.4% +/- 6.0% vs 4.6% +/- 0.9%). Addition of CTGP to the medium induced a concentration-dependent inhibition of [ 14 C]proline incorporation into proteins from both control and scleroderma cells. In control cultures, no significant decrease of the percentage of collagen was observed, but over 60 micrograms/ml, both cytotoxic effects and inhibition of protein synthesis occurred. In scleroderma cultures, the inhibition was twice as effective on collagen as on noncollagen protein synthesis. The inhibition of collagen secretion was not related either to changes in collagen hydroxylation or to the intracellular catabolism of newly synthesized procollagen

  1. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study

    DEFF Research Database (Denmark)

    Peytrignet, Sébastien; Denton, Christopher P; Lunt, Mark

    2018-01-01

    Objectives: Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods: Patients completed questionnaires at study entry, 12 and 24 months, including the HA...

  2. Validation of the Self-Efficacy for Managing Chronic Disease Scale: A Scleroderma Patient-Centered Intervention Network cohort study

    NARCIS (Netherlands)

    Riehm, K.E.; Kwakkenbos, C.M.C.; Carrier, M.E.; Bartlett, S.J.; Malcarne, V.L.; Mouthon, L.; Nielson, W.R.; Poiraudeau, S.; Nielsen, K.; Baron, M.; Frech, T.; Hudson, M.; Pope, J.; Sauvé, M.; Suarez-Almazor, M.E.; Wigley, F.M.; Thombs, B.D.

    2016-01-01

    Objective: Self-management programs for patients with chronic illnesses, including rheumatic diseases, seek to enhance self-efficacy for performing health management behaviors. No measure of self-efficacy has been validated for patients with systemic sclerosis (SSc; scleroderma). The objective of

  3. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma)

    International Nuclear Information System (INIS)

    Tagliafico, Alberto; Panico, Nicoletta; Resmini, Eugenia; Derchi, Lorenzo E.; Ghio, Massimo; Martinoli, Carlo

    2011-01-01

    Background: Patients affected by scleroderma may complain of sensory disturbances especially in the hands. Purpose: To study the imaging features of upper limb nerves in patients affected by scleroderma (SSc). Materials and method: Twenty-five patients affected only by SSc were prospectively evaluated with high-resolution US and magnetic resonance (MRI) or computer tomography (CT) when necessary (2 patients). Median and ulnar nerves were evaluated bilaterally. Nerve conduction studies were performed in the symptomatic patients (n = 10). Results of imaging studies were correlated with disease duration, autoimmunity and immunosuppression. Nerves of SSc patients were compared with a control group of 90 patients matched for age and body mass index. Results: The prevalence of sensory disturbances revealed by clinical examination was 40%. In symptomatic SSc patients (n = 10) US evaluation revealed nerve abnormalities in 70% of cases (n = 7/10). n = 2 had a carpal tunnel syndrome. n = 5 had cubital tunnel syndrome. In two of them CT and MR were necessary to identify the compressed nerve at the level of the elbow due to the presence of calcifications. There was no association between the presence of an entrapment neuropathy and disease duration, autoantibodies and immunosuppression. Conclusion: Ultrasound, CT and MR may detect nerve abnormalities in 70% of SSc patients complaining of neurologic disturbances in the hands. The results of imaging studies support the hypothesis of a vascular dependent neuropathy in SSc.

  4. Esophageal function scintigraphy as parameter for organ involvement of progressive systemic scleroderma

    International Nuclear Information System (INIS)

    Leisner, B.; Koenig, G.; Hundegger, K.; Luderschmidt, C.

    1986-01-01

    Involvement of internal organs such as lungs, heart and kidneys, is a life-threatening complication in progressive systemic scleroderma (PSS). However, the earliest and the most frequent internal manifestation of PSS is that of esophageal dysfunction. This study was undertaken to determine whether the new, sensitive and noninvasive esphageal function scintigraphy (EFS) enables us to identify patients likely to develop pulmonary interstitial fibrosis. 131 patients with PSS of different clinical types and courses underwent EFS. The esophageal clearance of a sup(99m)Tc-tagged 15 ml water bolus was measured. In normals, 91 +- 4.8% of the maximal rate were cleared from the ROI comprising the whole esophagus 10 sec after T max was reached. For comparison, chest X-rays and pulmonary function data were used. In the presence of interstitial lung disease EFS gave normal results in four patients only (sensitivity, 89%). All cases with either severely impaired lung function or signs of fibrosis on X-ray films showed esophageal clearance values below 40%. Moreover, esophageal motility disorders were seen in 32 among 43 patients with normal lung function. There was a correlationi between the stage and progression (reflected by unspecific signs of inflammation) and the incidence and severity of both lung function impairment and esophageal dysfunction. In conclusion, functional scintigraphy proved to be a very sensitive diagnostic procedure in the screening for internal manifestations of progressive systemic scleroderma. (Author)

  5. Physiologic abnormalities of cardiac function in progressive systemic sclerosis with diffuse scleroderma

    International Nuclear Information System (INIS)

    Follansbee, W.P.; Curtiss, E.I.; Medsger, T.A. Jr.; Steen, V.D.; Uretsky, B.F.; Owens, G.R.; Rodnan, G.P.

    1984-01-01

    To investigate cardiopulmonary function in progressive systemic sclerosis with diffuse scleroderma, we studied 26 patients with maximal exercise and redistribution thallium scans, rest and exercise radionuclide ventriculography, pulmonary-function testing, and chest roentgenography. Although only 6 patients had clinical evidence of cardiac involvement, 20 had abnormal thallium scans, including 10 with reversible exercise-induced defects and 18 with fixed defects (8 had both). Seven of the 10 patients who had exercise-induced defects and underwent cardiac catheterization had normal coronary angiograms. Mean resting left ventricular ejection fraction and mean resting right ventricular ejection fraction were lower in patients with post-exercise left ventricular thallium defect scores above the median (59 +/- 13 per cent vs. 69 +/- 6 per cent, and 36 +/- 12 per cent vs. 47 +/- 7 per cent, respectively). The authors conclude that in progressive systemic sclerosis with diffuse scleroderma, abnormalities of myocardial perfusion are common and appear to be due to a disturbance of the myocardial microcirculation. Both right and left ventricular dysfunction appear to be related to this circulatory disturbance, suggesting ischemically mediated injury

  6. The role of ultrasound imaging in the evaluation of peripheral nerve in systemic sclerosis (scleroderma)

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto, E-mail: atagliafico@sirm.org [Department of Radiology, University of Genova, Genova (Italy); Panico, Nicoletta [Division of Immunology, Department of Internal Medicine, University of Genoa, Genoa (Italy); Resmini, Eugenia [Department of Endocrinological and Medical Sciences (DiSEM), Center of Excellence for Biomedical Research, University of Genova, Genova (Italy); Derchi, Lorenzo E. [Department of Radiology, University of Genova, Genova (Italy); Ghio, Massimo [Division of Immunology, Department of Internal Medicine, University of Genoa, Genoa (Italy); Martinoli, Carlo [Department of Radiology, University of Genova, Genova (Italy)

    2011-03-15

    Background: Patients affected by scleroderma may complain of sensory disturbances especially in the hands. Purpose: To study the imaging features of upper limb nerves in patients affected by scleroderma (SSc). Materials and method: Twenty-five patients affected only by SSc were prospectively evaluated with high-resolution US and magnetic resonance (MRI) or computer tomography (CT) when necessary (2 patients). Median and ulnar nerves were evaluated bilaterally. Nerve conduction studies were performed in the symptomatic patients (n = 10). Results of imaging studies were correlated with disease duration, autoimmunity and immunosuppression. Nerves of SSc patients were compared with a control group of 90 patients matched for age and body mass index. Results: The prevalence of sensory disturbances revealed by clinical examination was 40%. In symptomatic SSc patients (n = 10) US evaluation revealed nerve abnormalities in 70% of cases (n = 7/10). n = 2 had a carpal tunnel syndrome. n = 5 had cubital tunnel syndrome. In two of them CT and MR were necessary to identify the compressed nerve at the level of the elbow due to the presence of calcifications. There was no association between the presence of an entrapment neuropathy and disease duration, autoantibodies and immunosuppression. Conclusion: Ultrasound, CT and MR may detect nerve abnormalities in 70% of SSc patients complaining of neurologic disturbances in the hands. The results of imaging studies support the hypothesis of a vascular dependent neuropathy in SSc.

  7. Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma

    International Nuclear Information System (INIS)

    Follansbee, W.P.; Curtiss, E.I.; Medsger, T.A. Jr.; Owens, G.R.; Steen, V.D.; Rodnan, G.P.

    1984-01-01

    Myocardial function and perfusion were evaluated in 22 patients with progressive systemic sclerosis with the CREST syndrome using exercise and radionuclide techniques, pulmonary function testing, and chest roentgenography. The results were compared with a similar study of 26 patients with progressive systemic sclerosis with diffuse scleroderma. The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01). Reperfusion thallium defects in the absence of extramural coronary artery disease were seen in 38 percent of patients with diffuse scleroderma. This finding was not seen in any of the patients with the CREST syndrome. In diffuse scleroderma, abnormalities of both right and left ventricular function were related to larger thallium perfusion defects. In the CREST syndrome, abnormalities of left ventricular function were minor, were seen only during exercise, and were unrelated to thallium perfusion defects. Abnormal resting right ventricular function was seen in 36 percent of the patients with the CREST syndrome and was associated with an isolated decrease in diffusing capacity of carbon monoxide. It is concluded that the cardiac manifestations of the CREST syndrome are distinct from those found in diffuse scleroderma. Unlike diffuse scleroderma, abnormalities of left ventricular function in the CREST syndrome are minor and are unrelated to abnormalities of coronary perfusion. Right ventricular dysfunction in the CREST syndrome appears to be primarily related to pulmonary vascular disease

  8. Do scleroderma patients look young?: Evaluation by using facial imaging system.

    Science.gov (United States)

    Sawamura, Soichiro; Jinnin, Masatoshi; Kajihara, Ikko; Makino, Katsunari; Aoi, Jun; Ichihara, Asako; Makino, Takamitsu; Fukushima, Satoshi; Ihn, Hironobu

    2017-01-01

    These days various collagen supplements have widely been marketed. However, it has not been scientifically proved whether increasing collagen can actually prevent skin aging. Systemic sclerosis (SSc) is an autoimmune disease that is characterized by thickening of the skin caused by accumulation of collagen. In this study, we tried to evaluate facial skin characteristics and skin aging of SSc patients by using digital imaging system. As the result, the severity of wrinkles, texture and pores were significantly lower in SSc patients than control subjects. Among them, wrinkles showed better correlation with skin thickness score. Therefore, increased amount of collagen in scleroderma skin may directly affect wrinkles. In conclusion, attempt on collagen induction itself is reasonable and effective strategy in order to keep young appearance, although oral collagen supplementation may not directly reach to the skin.

  9. Microcirculatory disorders in scleroderma systematica: an association with vascular wall stiffness

    Directory of Open Access Journals (Sweden)

    Ulyana Yuryevna Ruzhentsova

    2013-01-01

    Full Text Available Objective: to study the specific features of regulation of peripheral vascular tone and their association with the endothelial structure and function of large vessels in patients with scleroderma systematica (SDS. Subjects and methods. The investigation enrolled 25 patients with SDS (mean age, 47±2.6 years; mean disease duration, 8.3+1.7 years and 15 apparently healthy individuals matched for age and gender. Comprehensive examination involved laboratory and instrumental studies, laser Doppler study to evaluate endothelium-dependent and endothelium-independent vasodilation, as well as applanation tonometry calculating the pulse wave velocity and augmentation index. Results. All the patients were found to have impaired peripheral vascular responsiveness as compared to the controls. The examination established a relationship between the magnitude of endothelium-dependent vasodilation and the stiffness index of large vessels. There was no association between endothelium-independent vasodilation and vascular elasticity parameters.

  10. The expression of myocardial injury in cold induced myocardial imaging and echocardiography of systematic scleroderma

    International Nuclear Information System (INIS)

    Liang Jiugen; Zhu Xiaojun; Jiang Ningyi; Chen Shaoxiong

    1999-01-01

    The study was performed with cold-induced 99m Tc(MIBI) myocardial imaging (MI) in 23 patients with systematic scleroderma. The left ventricular function and wall motion were also observed by dimensional echocardiography (UCG). 14 patients had myocardial perfusion abnormalities visualized by MI, including 5 cases with fixed defects of 9 segments, 3 cases with reversible defects of 6 segments and 6 cases with both fixed and reversible one of 14 segments. The positive rate in myocardial imaging had no significant differences between patients with and without Raynaud's phenomenon (0.5>P>0.25). Compared with baseline, the ejection fraction, stroke volume, cardiac output were significantly decreased during cold-induced in patients with abnormal myocardial scintigraphy (P<0.05), and had significant difference compared with normal group (P<0.05). 4 cases with cold-induced reversible perfusion defects had anatomically correlated regional ventricular hypokinesia in UCG

  11. Scleroderma keratinocytes promote fibroblast activation independent of transforming growth factor beta.

    Science.gov (United States)

    McCoy, Sara S; Reed, Tamra J; Berthier, Celine C; Tsou, Pei-Suen; Liu, Jianhua; Gudjonsson, Johann E; Khanna, Dinesh; Kahlenberg, J Michelle

    2017-11-01

    SSc is a devastating disease that results in fibrosis of the skin and other organs. Fibroblasts are a key driver of the fibrotic process through deposition of extracellular matrix. The mechanisms by which fibroblasts are induced to become pro-fibrotic remain unclear. Thus, we examined the ability of SSc keratinocytes to promote fibroblast activation and the source of this effect. Keratinocytes were isolated from skin biopsies of 9 lcSSc, 10 dcSSc and 13 control patients. Conditioned media was saved from the cultures. Normal fresh primary fibroblasts were exposed to healthy control and SSc keratinocyte conditioned media in the presence or absence of neutralizing antibodies for TGF-β. Gene expression was assessed by microarrays and real-time PCR. Immunocytochemistry was performed for α-smooth muscle actin (α-SMA), collagen type 1 (COL1A1) and CCL5 expression. SSc keratinocyte conditioned media promoted fibroblast activation, characterized by increased α-SMA and COL1A1 mRNA and protein expression. This effect was independent of TGF-β. Microarray analysis identified upregulation of nuclear factor κB (NF-κB) and downregulation of peroxisome proliferator-activated receptor γ (PPAR-γ) pathways in both SSc subtypes. Scleroderma keratinocytes exhibited increased expression of NF-κB-regulated cytokines and chemokines and lesional skin staining confirmed upregulation of CCL5 in basal keratinocytes. Scleroderma keratinocytes promote the activation of fibroblasts in a TGF-β-independent manner and demonstrate an imbalance in NF-κB1 and PPAR-γ expression leading to increased cytokine and CCL5 production. Further study of keratinocyte mediators of fibrosis, including CCL5, may provide novel targets for skin fibrosis therapy. © The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com

  12. Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

    Directory of Open Access Journals (Sweden)

    Rohit Tewari

    2016-01-01

    Full Text Available Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.

  13. Litter-forager termite mounds enhance the ectomycorrhizal symbiosis between Acacia holosericea A. Cunn. Ex G. Don and Scleroderma dictyosporum isolates.

    Science.gov (United States)

    Duponnois, Robin; Assikbetse, Komi; Ramanankierana, Heriniaina; Kisa, Marija; Thioulouse, Jean; Lepage, Michel

    2006-05-01

    The hypothesis of the present study was that the termite mounds of Macrotermes subhyalinus (MS) (a litter-forager termite) were inhabited by a specific microflora that could enhance with the ectomycorrhizal fungal development. We tested the effect of this feeding group mound material on (i) the ectomycorrhization symbiosis between Acacia holosericea (an Australian Acacia introduced in the sahelian areas) and two ectomycorrhizal fungal isolates of Scleroderma dictyosporum (IR408 and IR412) in greenhouse conditions, (ii) the functional diversity of soil microflora and (iii) the diversity of fluorescent pseudomonads. The results showed that the termite mound amendment significantly increased the ectomycorrhizal expansion. MS mound amendment and ectomycorrhizal inoculation induced strong modifications of the soil functional microbial diversity by promoting the multiplication of carboxylic acid catabolizing microorganisms. The phylogenetic analysis showed that fluorescent pseudomonads mostly belong to the Pseudomonads monteillii species. One of these, P. monteillii isolate KR9, increased the ectomycorrhizal development between S. dictyosporum IR412 and A. holosericea. The occurrence of MS termite mounds could be involved in the expansion of ectomycorrhizal symbiosis and could be implicated in nutrient flow and local diversity.

  14. Juvenile Scleroderma

    Science.gov (United States)

    ... of the tongue. Obvious cases of en coup de sabre and Parry Romberg syndrome differ significantly, but many children present with cross-over manifestations, which can make it difficult to determine with certainty which form of ... en coup de sabre is mixed. If the lesions are confined ...

  15. Radiotherapy of early breast cancer in scleroderma patients: our experience with four cases and a short review of the literature

    Directory of Open Access Journals (Sweden)

    Kyrgias G

    2012-01-01

    Full Text Available George Kyrgias1,2, Kiki Theodorou3,4, Anna Zygogianni1, Konstantinos Tsanadis2, Stefanos Zervoudis5, John Tzitzikas6, Michael Koukourakis71Academic Radiotherapy, University of Thessaly, Medical School, Greece; 2Radiation Oncology Department, University Hospital of Larissa, Greece; 3Academic Medical Physics, University of Thessaly, Medical School, Greece; 4Medical Physics Department, University Hospital of Larissa, 5Breast Unit, REA Hospital, Athens, Greece; 6Radiation Oncology Department, AHEPA University Hospital of Thessaloniki, Greece; 7Radiotherapy-Oncology Department, University Hospital of Alexandroupolis, GreecePurpose: Connective vascular diseases (CVD, including scleroderma, are reported to represent for some researchers a relative contraindication and for others absolute contraindication for radiotherapy. The purpose of our study is to add four new cases to the existing body of international literature and to determine whether women with pre-existing scleroderma who have been surgically treated for early breast cancer could undergo postsurgical radiotherapy without serious early and late complications.Patients and methods: From May 1998 to November 2010, we irradiated for early breast cancer four patients suffering from pre-existing scleroderma; after conservative surgery, we performed whole breast postoperative radiotherapy of 50.4 Gy total dose to the whole breast plus a 9 Gy boost to the tumor bed. We reviewed the records of all four patients and evaluated the early and late reactions using acute radiation morbidity scoring criteria (Radiation Therapy Oncology Group [RTOG], American College of Radiology, Philadelphia, PA and late radiation morbidity scoring scheme (European Organisation for Research and Treatment of Cancer [EORTC], Brussels, Belgium and RTOG.Results: After a median follow-up of 105 months (range 12–155 months the early and late toxicity concerning the skin, the subcutaneous tissues, the lungs, and the heart have

  16. Autoantibodies in scleroderma and their association with the clinical profile of the disease. A study of 66 patients from southern Brazil.

    Science.gov (United States)

    Skare, Thelma Larocca; Fonseca, Adriano Erlon; Luciano, Alan Campos; Azevedo, Pedro Ming

    2011-01-01

    Scleroderma is a fairly rare connective tissue disease whose autoantibody profile is associated with different clinical manifestations. The prevalence of autoantibodies in scleroderma is influenced by race and genetics. To study the prevalence of anti-Scl-70, anti-centromere (ACA) and anti-U1-RNP antibodies in patients with scleroderma in southern Brazil and verify their association with clinical manifestations of the disease. A retrospective study involving 66 patients with scleroderma for the presence of anti-Scl-70, anti-centromere and anti-U1-RNP and of clinical manifestations such as Raynaud's phenomenon, digital micro scars, digital necrosis, telangiectasias, calcinosis, pulmonary fibrosis, pleuritis, pericarditis, cardiomyopathy, arthralgia and arthritis, skin sclerosis, joint contractures, tendon friction rubs, pulmonary hypertension, esophageal disorders and renal crisis. The prevalence of anti-Scl-70 was 17.8% , that of ACA was 33.3% and the prevalence of U1 RNP was 11.8%. Anti-Scl-70 was associated with the diffuse form of the disease (p = 0.015), presence of cardiomyopathies (p = 0.016) and digital micro scars (p = 0.05). Anti-centromere was more common in the limited form, although it was not statistically significant, and had a protective role associated with cardiomyopathies (p = 0.005). Anti-U1-RNP was more common in the overlap forms (p = 0.0004). The prevalence and profile of clinical associations of autoantibodies in Brazilian patients with scleroderma are similar to those found in the literature.

  17. The thickness of the A1 pulleys reflects the disability of hand mobility in scleroderma. A pilot study using high-frequency ultrasound

    Energy Technology Data Exchange (ETDEWEB)

    Tagliafico, Alberto, E-mail: atagliafico@sirm.org [Department of Radiology, National Institute for Cancer Research, Genova (Italy); Panico, Nicoletta [Division of Immunology, Department of Internal Medicine, University of Genova, Genova (Italy); Serafini, Giovanni [Department of Radiology, Santa Corona Hospital, Pietra Ligure (Italy); Ghio, Massimo [Division of Immunology, Department of Internal Medicine, University of Genova, Genova (Italy); Martinoli, Carlo [Department of Radiology, University of Genova, Genova (Italy)

    2011-02-15

    Background: Hand involvement in scleroderma is a serious concern. Clinical tests to asses hand dysfunction are based on the experience of the clinician. Objective: To asses if utrasonographic (US) measurement of A1 pulley thickness may be used as an indicator of hand mobility in scleroderma. Materials and methods: Institutional review board approval and patient informed consent was obtained. Twenty-eight patients affected suffering from scleroderma and 40 healthy controls were prospectively evaluated by two blinded radiologists with US, with a transducer operating at 17 MHz. A1 pulley thickness was measured and correlated with the Hand Mobility in Scleroderma Test (HAMIS) and disease duration. Results: The thickness of the A1 pulley was greater in sclerodermic patients than in controls (p < 0.05). Intra and inter-observer agreement were better for ultrasound (0.94;0.88) than for HAMIS tests (0.71;0.70). A good correlation between pulley thickness, hand mobility and disease duration was found (r = 0.78, p < 0.018; r = 0.54, p < 0.05). Conclusion: A1 pulley thickness measured on ultrasound correlates with hand mobility and disease duration. Ultrasound is an useful tool to evaluate hand disability in scleroderma.

  18. The thickness of the A1 pulleys reflects the disability of hand mobility in scleroderma. A pilot study using high-frequency ultrasound

    International Nuclear Information System (INIS)

    Tagliafico, Alberto; Panico, Nicoletta; Serafini, Giovanni; Ghio, Massimo; Martinoli, Carlo

    2011-01-01

    Background: Hand involvement in scleroderma is a serious concern. Clinical tests to asses hand dysfunction are based on the experience of the clinician. Objective: To asses if utrasonographic (US) measurement of A1 pulley thickness may be used as an indicator of hand mobility in scleroderma. Materials and methods: Institutional review board approval and patient informed consent was obtained. Twenty-eight patients affected suffering from scleroderma and 40 healthy controls were prospectively evaluated by two blinded radiologists with US, with a transducer operating at 17 MHz. A1 pulley thickness was measured and correlated with the Hand Mobility in Scleroderma Test (HAMIS) and disease duration. Results: The thickness of the A1 pulley was greater in sclerodermic patients than in controls (p < 0.05). Intra and inter-observer agreement were better for ultrasound (0.94;0.88) than for HAMIS tests (0.71;0.70). A good correlation between pulley thickness, hand mobility and disease duration was found (r = 0.78, p < 0.018; r = 0.54, p < 0.05). Conclusion: A1 pulley thickness measured on ultrasound correlates with hand mobility and disease duration. Ultrasound is an useful tool to evaluate hand disability in scleroderma.

  19. Antifibrotic effects of crocetin in scleroderma fibroblasts and in bleomycin-induced sclerotic mice

    Directory of Open Access Journals (Sweden)

    Yinghua Song

    2013-10-01

    Full Text Available OBJECTIVE: To investigate the antifibrotic effects of crocetin in scleroderma fibroblasts and in sclerotic mice. METHODS: Skin fibroblasts that were isolated from three systemic scleroderma (SSc patients and three healthy subjects were treated with crocetin (0.1, 1 or 10 μM. Cell proliferation was measured with an MTT assay. Alpha-smooth muscle actin was detected via an immunohistochemical method. Alpha 1 (I procollagen (COL1A1, alpha 1 (III procollagen (COL3A1, matrix metalloproteinase (MMP-1 and tissue inhibitor of matrix metalloproteinase (TIMP-1 mRNA levels were measured using real-time PCR. SSc mice were established by the subcutaneous injection of bleomycin. Crocetin (50 mg/kg/d was injected intraperitoneally for 14 days. Dermal thickness and lung fibrosis were assessed with Masson's trichrome staining. Plasma ET-1 was detected with an enzyme-linked immunosorbent assay (ELISA. Skin and lung ET-1 and COL1A1 mRNA levels were measured via real-time PCR. RESULTS: Crocetin inhibited the proliferation of SSc and normal fibroblasts, an effect that increased with crocetin concentration and incubation time. Crocetin decreased the expression of α-SMA and the levels of mRNA for COL1A1, COL3A1 and matrix metalloproteinase-1, while crocetin increased TIMP-1 mRNA levels in both SSc and normal fibroblasts. Skin and lung fibrosis was induced, and the levels of ET-1 in the plasma, skin and lungs were elevated in bleomycin-injected mice. Crocetin alleviated the thickening of the dermis and lung fibrosis; decreased COL1A1 mRNA levels in the skin and lung; and simultaneously decreased ET-1 concentrations in the plasma and ET-1 mRNA levels in the skin and lungs of the bleomycin-induced sclerotic mice, especially during the early phase (weeks 1-3. CONCLUSION: Crocetin inhibits cell proliferation, differentiation and collagen production in SSc fibroblasts. Crocetin alleviates skin and lung fibrosis in a bleomycin-induced SSc mouse model, in part due to a

  20. Determination of muscle microcirculation of the limbs of healthy persons and patients with scleroderma by means of 133Xe clearance. 2

    International Nuclear Information System (INIS)

    Poenitzsch, I.; Wiemers, U.; Haustein, U.F.; Schneider, G.

    1984-01-01

    By means of 133 Xe muscle clearance the blood flow of the musculus tibialis anterior and the musculus opponens pollicis was determined during nonischemic work and after 3 minutes ischemia in patients with progressive scleroderma and additionally of the ischemic musculus opponens pollicis following contrast baths. In relation to 53 patients with normal vessels the reactive hyperemia of the musculus tibialis anterior after ischemia and of the musculus opponens pollicis after heat, cold as well as arterial flow decreasing was significantly decreased in patients with scleroderma and was as a disturbance of the microcirculation realized. For scientific problems in progressive scleroderma the 133 Xe muscle clearance is suitable as to the musculus opponens pollicis. (author)

  1. Metabolism of /sup 14/C-labelled L-tryptophan, L-kynurenine, and hydroxy-L-kynurenine in miners with scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Hankes, L.V.; De Bruin, E.; Jansen, C.R.; Vorster, L.; Schmaeler, M.

    1977-03-19

    Six South African white miners were studied with the 2-g L-tryptophan load test and tracer doses of L-tryptophan-7a-/sup 14/C, L-kynurenine-keto-/sup 14/C and hydroxy-L-kynurenine-keto-/sup 14/C. The breath /sup 14/CO/sub 2/ and 14 urinary metabolites were measured. When they were compared with a previous study of American women with scleroderma, similar /sup 14/CO/sub 2/ and tryptophan metabolite excretion patterns were observed in the data from the miners. The labelled quinolinic acid excretion was more significantly elevated in the South African miners' urine than in the urine of the American women. The data from both studies suggest that some patients with scleroderma have an altered step in the tryptophan metabolic pathway after hydroxy-anthranilic acid. What relationship exists between the induction of pulmonary silicosis and the subsequent development of scleroderma, requires additional human studies.

  2. Soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) in scleroderma skin

    DEFF Research Database (Denmark)

    Søndergaard, Klaus; Deleuran, Mette; Heickendorff, Lene

    1998-01-01

    In order to investigate whether soluble intercellular adhesion molecule-1 (sICAM-1) and soluble interleukin-2 receptors (sIL-2R) were present in scleroderma skin, and to compare their levels to concentrations measured in plasma and clinical parameters, we examined suction blister fluid and plasma...... from 13 patients with systemic sclerosis and 11 healthy volunteers. Suction blisters and biopsies were from the transition zone between normal skin and scleroderma, and uninvolved abdominal skin. The levels of sICAM-1 and sIL-2R were significantly increased in both plasma and suction blister fluid from...

  3. Parasitization by Scleroderma guani influences expression of superoxide dismutase genes in Tenebrio molitor.

    Science.gov (United States)

    Zhu, Jia-Ying; Ze, Sang-Zi; Stanley, David W; Yang, Bin

    2014-09-01

    Superoxide dismutase (SOD) is an antioxidant enzyme involved in detoxifying reactive oxygen species. In this study, we identified genes encoding the extracellular and intracellular copper-zinc SODs (ecCuZnSOD and icCuZnSOD) and a manganese SOD (MnSOD) in the yellow mealworm beetle, Tenebrio molitor. The cDNAs for ecCuZnSOD, icCuZnSOD, and MnSOD, respectively, encode 24.55, 15.81, and 23.14 kDa polypeptides, which possess structural features typical of other insect SODs. They showed 20-94% identity to other known SOD sequences from Bombyx mori, Musca domestica, Nasonia vitripennis, Pediculus humanus corporis, and Tribolium castaneum. Expression of these genes was analyzed in selected tissues and developmental stages, and following exposure to Escherichia coli and parasitization by Scleroderma guani. We recorded expression of all three SODs in cuticle, fat body, and hemocytes and in the major developmental stages. Relatively higher expressions were detected in late-instar larvae and pupae, compared to other developmental stages. Transcriptional levels were upregulated following bacterial infection. Analysis of pupae parasitized by S. guani revealed that expression of T. molitor SOD genes was significantly induced following parasitization. We infer that these genes act in immune response and in host-parasitoid interactions. © 2014 Wiley Periodicals, Inc.

  4. Parasitization by Scleroderma guani influences protein expression in Tenebrio molitor pupae.

    Science.gov (United States)

    Zhu, Jia-Ying; Wu, Guo-Xing; Ze, Sang-Zi; Stanley, David W; Yang, Bin

    2014-07-01

    Ectoparasitoid wasps deposit their eggs onto the surface and inject venom into their hosts. Venoms are chemically complex and they exert substantial impact on hosts, including permanent or temporary paralysis and developmental arrest. These visible venom effects are due to changes in expression of genes encoding physiologically relevant proteins. While the influence of parasitization on gene expression in several lepidopterans has been reported, the molecular details of parasitoid/beetle relationships remain mostly unknown. This shortcoming led us to pose the hypothesis that envenomation by the ectoparasitic ant-like bethylid wasp Scleroderma guani leads to changes in protein expression in the yellow mealworm beetle Tenebrio molitor. We tested our hypothesis by comparing the proteomes of non-parasitized and parasitized host pupae using iTRAQ-based proteomics. We identified 41 proteins that were differentially expressed (32↑- and 9↓-regulated) in parasitized pupae. We assigned these proteins to functional categories, including immunity, stress and detoxification, energy metabolism, development, cytoskeleton, signaling and others. We recorded parallel changes in mRNA levels and protein abundance in 14 selected proteins following parasitization. Our findings support our hypothesis by documenting changes in protein expression in parasitized hosts. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Decreased interleukin-20 expression in scleroderma skin contributes to cutaneous fibrosis.

    Science.gov (United States)

    Kudo, Hideo; Jinnin, Masatoshi; Asano, Yoshihide; Trojanowska, Maria; Nakayama, Wakana; Inoue, Kuniko; Honda, Noritoshi; Kajihara, Ikko; Makino, Katsunari; Fukushima, Satoshi; Ihn, Hironobu

    2014-06-01

    To clarify the role of interleukin-20 (IL-20) in the regulatory mechanism of extracellular matrix expression and to determine the contribution of IL-20 to the phenotype of systemic sclerosis (SSc). Protein and messenger RNA (mRNA) levels of collagen, Fli-1, IL-20, and IL-20 receptor (IL-20R) were analyzed using polymerase chain reaction (PCR) array, immunoblotting, immunohistochemical staining, enzyme-linked immunosorbent assay, and real-time PCR. PCR array revealed that IL-20 decreased gene expression of α2(I) collagen (0.03-fold), Smad3 (0.02-fold), and endoglin (0.05-fold) in cultured normal dermal fibroblasts. Fli-1 protein expression was induced by IL-20 (~2-fold). The inhibition of collagen by IL-20, the induction of Fli-1 by IL-20, and the reduction of Smad3 and endoglin by IL-20 were also observed in SSc fibroblasts. Serum IL-20 levels were reduced only slightly in SSc patients but were significantly decreased in patients with scleroderma spectrum disorders (the prodromal stage of SSc) compared with those in normal subjects (111.3 pg/ml versus 180.4 pg/ml; P value of IL-20 and IL-20R, their function and expression in vivo should be further studied. Copyright © 2014 by the American College of Rheumatology.

  6. The Assessment of Health-Related Quality of Life in Scleroderma-Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Shahrzad M Lari

    2014-05-01

    Full Text Available Introduction: Pulmonary involvement is the most common cause of mortality and disability in patients with systemic sclerosis and it significantly affects the quality of life in these patients. Therefore, early diagnosis and treatment of pulmonary involvement seems necessary in patients with SSc. In this study, we aimed to assess the health-related quality of life (HRQoL in patients with Scleroderma-Interstitial Lung Disease (SSc-ILD and its relationship with pulmonary function parameters. Materials and Methods: Considering the inclusion and exclusion criteria, 25 patients with SSc-ILD were enrolled in this cross-sectional study from April 2012 to June 2013. Full tests of lung function, including body plethysmography and diffusing capacity of the lungs for carbon monoxide (DLCO, 6-minute walk distance (6MWD, and pulse oximetry were performed. The HRQoL was assessed using St. George’s and CAT questionnaires; also, dyspnea was evaluated for all the patients, using modified medical research council (MMRC scale. Afterwards, the relationship between the total scores of HRQoL questionnaires and the severity of lung disease was analyzed, based on the recorded variables. Results: The mean age of the patients was 40.36±9.50 years and the mean duration of the disease was 7.16±4.50 years. A statistically significant inverse correlation was observed between 6MWD (r=-0.50, P=0.01, DLCO (r=-0.67, P

  7. Assessment of extent of skin involvement in scleroderma using shear wave elastography

    Directory of Open Access Journals (Sweden)

    Anupam Wakhlu

    2017-01-01

    Full Text Available Introduction: Scleroderma (systemic sclerosis [SSc] is a rare autoimmune disease which manifests as fibrosis in the skin and other internal organs. Conventionally, the modified Rodnan skin score (MRSS has been used to quantify the extent of skin fibrosis (resulting in skin tightness in SSc. This technique, although widely validated, is limited by the requirement of a trained, experienced assessor. Recent literature suggests that utilization of the objective ultrasound-based assessment of skin fibrosis utilizing shear wave elastography (SWE may be a more robust technique to detect early skin tightness in SSc. Methods: We evaluated the use of SWE (assessed by an experienced radiologist in 24 patients with SSc compared with 16 healthy controls. Results: Our patients were predominantly females, with median disease duration of 1.5 years and median MRSS of 17. There was minimal intraobserver variation in the assessment of SWE. Patients with SSc had higher SWE values (mean elasticity [Emean] compared to healthy controls at most assessed sites for the MRSS. The Emeancorrelated significantly at all sites with the MRSS scores. At the sites where MRSS was scored as 0 (normal, the Emeanin patients with SSc was higher when compared with similarly clinical normal skin in patients with SSc, suggesting potential early involvement of these areas of the skin with fibrosis. Conclusion: SWE is a promising tool to objectively assess skin fibrosis in SSc and may be useful in detecting early, subclinical skin involvement in this disease.

  8. Mortality Risk Prediction in Scleroderma-Related Interstitial Lung Disease: The SADL Model.

    Science.gov (United States)

    Morisset, Julie; Vittinghoff, Eric; Elicker, Brett M; Hu, Xiaowen; Le, Stephanie; Ryu, Jay H; Jones, Kirk D; Haemel, Anna; Golden, Jeffrey A; Boin, Francesco; Ley, Brett; Wolters, Paul J; King, Talmadge E; Collard, Harold R; Lee, Joyce S

    2017-11-01

    Interstitial lung disease (ILD) is an important cause of morbidity and mortality in patients with scleroderma (Scl). Risk prediction and prognostication in patients with Scl-ILD are challenging because of heterogeneity in the disease course. We aimed to develop a clinical mortality risk prediction model for Scl-ILD. Patients with Scl-ILD were identified from two ongoing longitudinal cohorts: 135 patients at the University of California, San Francisco (derivation cohort) and 90 patients at the Mayo Clinic (validation cohort). Using these two separate cohorts, a mortality risk prediction model was developed and validated by testing every potential candidate Cox model, each including three or four variables of a possible 19 clinical predictors, for time to death. Model discrimination was assessed using the C-index. Three variables were included in the final risk prediction model (SADL): ever smoking history, age, and diffusing capacity of the lung for carbon monoxide (% predicted). This continuous model had similar performance in the derivation (C-index, 0.88) and validation (C-index, 0.84) cohorts. We created a point scoring system using the combined cohort (C-index, 0.82) and used it to identify a classification with low, moderate, and high mortality risk at 3 years. The SADL model uses simple, readily accessible clinical variables to predict all-cause mortality in Scl-ILD. Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

  9. Gammadelta receptor bearing T cells in scleroderma: enhanced interaction with vascular endothelial cells in vitro.

    Science.gov (United States)

    Kahaleh, M B; Fan, P S; Otsuka, T

    1999-05-01

    In view of the documented perivascular mononuclear cell infiltration in the involved organs in scleroderma (SSc) and the reported accumulation of gammadelta-T cells in SSc skin and lung, we evaluated gammadelta-T cell interaction with endothelial cells (EC) in vitro. gammadelta- and alphabeta-T cells were isolated from BPMN of SSc patients with early diffuse disease and of matched control subjects by an immunomagnetic method after stimulation with mycobacterium lysate and interleukin-2 for 2 weeks. Lymphocyte adhesion, proliferation, and cytotoxicity to EC were investigated. SSc gammadelta-T cells adhered to cultured EC and proliferated at higher rates than control cells. Furthermore, significant EC cytotoxicity by SSc gammadelta was seen. The cytotoxicity was blocked by addition of anti-gammadelta-TCR antibody and by anti-granzyme A antibody but not by anti-MHC class I and II antibodies. Expression of granzyme A mRNA was seen in five/five SSc gammadelta-T cells and in one/five control cells. alphabeta-T cells from both SSc and control subjects were significantly less interactive with EC than gammadelta-T cells. The data demonstrate EC recognition by SSc gammadelta-T cells and propose gammadelta-T cells as a possible effector cell type in the immune pathogenesis of SSc. Copyright 1999 Academic Press.

  10. Pulmonary clearance of sup 99m Tc-DTPA aerosol in patients with progressive systemic scleroderma

    Energy Technology Data Exchange (ETDEWEB)

    Tateno, Madoka; Nakano, Akihiko; Hasegawa, Akira; Watanabe, Naoyuki; Oriuchi, Noboru; Inoue, Tomio; Endo, Keigo (Gunma Univ., Maebashi (Japan). School of Medicine); Sasaki, Yasuhito

    1992-05-01

    Alveolar epithelial permeability was assessed in 32 patients with progressive systemic scleroderma (PSS), using {sup 99m}Tc-DTPA aerosol. Immediately after the inhalation of {sup 99m}Tc-DTPA aerosol for 3 to 6 minutes under normal tidal breathing, lung was imaged sequentially for 30 minutes from the posterior by a gamma camera and exponential fitting was processed on the time activity curve. T{sub 1/2} (min) was used as a parameter for the evaluation of permeability of alveolar epithelium. Patients with collagen disease showed shorter T{sub 1/2} (T{sub 1/2}=43.7{+-}23.8 min) than the normal volunteers (T{sub 1/2}=76.8{+-}8.7 min). No significant difference was observed between patients with or without interstitial changes on the chest CT. Significant correlation was not observed between T{sub 1/2} and %VC or %DLco. In 8 cases, studies were repeated in the interval of 3 to 19 months. Improvement of T{sub 1/2} was seen in 4 cases, independent of CT findings. These results suggest that {sup 99m}Tc-DTPA aerosol clearance study provides information independent from other lung examinations, and may be useful for the assessment of lung interstitial changes in patients with PSS. (author).

  11. Effects of bosentan on collagen type I synthesis on in vitro culture of scleroderma skin fibroblasts

    Directory of Open Access Journals (Sweden)

    S. Soldano

    2011-01-01

    Full Text Available The present study evaluated the effects of a non-selective endothelin (ETA/B receptors antagonist, on collagen type I (COLI synthesis on in vitro culture of scleroderma (SSc skin fibroblasts (Fb. Fb were obtained from skin biopsies of 6 female SSc patients (mean age 64. 1±6 years, after informed consent and Ethical Committee Approval. Cells were treated with endothelin-I [ET-I, 100nM] for 24 and 48 hrs, pre-treated for I hr with ETA/B receptors antagonist [10nM] alone or followed by ET-I for 24 and 48 hrs. Untreated Fb were used as controls. Immunocytochemistry and western blot analysis were performed to evaluate COLI synthesis. ET-I increased COLI synthesis both at 24 and 48 hrs when compared to controls. ETA/B receptor antagonost blocks the increased COLI synthesis ET-I-mediated both at 24 and 48 hrs vs. ET-I. Results showed that ET-I receptors blockage by ETA/B receptors antagonist might prevent the excessive synthesis of COLI, supporting its positive action in the management of skin fibrosis.

  12. Pulmonary clearance of 99mTc-DTPA aerosol in patients with progressive systemic scleroderma

    International Nuclear Information System (INIS)

    Tateno, Madoka; Nakano, Akihiko; Hasegawa, Akira; Watanabe, Naoyuki; Oriuchi, Noboru; Inoue, Tomio; Endo, Keigo; Sasaki, Yasuhito.

    1992-01-01

    Alveolar epithelial permeability was assessed in 32 patients with progressive systemic scleroderma (PSS), using 99m Tc-DTPA aerosol. Immediately after the inhalation of 99m Tc-DTPA aerosol for 3 to 6 minutes under normal tidal breathing, lung was imaged sequentially for 30 minutes from the posterior by a gamma camera and exponential fitting was processed on the time activity curve. T 1/2 (min) was used as a parameter for the evaluation of permeability of alveolar epithelium. Patients with collagen disease showed shorter T 1/2 (T 1/2 =43.7±23.8 min) than the normal volunteers (T 1/2 =76.8±8.7 min). No significant difference was observed between patients with or without interstitial changes on the chest CT. Significant correlation was not observed between T 1/2 and %VC or %DLco. In 8 cases, studies were repeated in the interval of 3 to 19 months. Improvement of T 1/2 was seen in 4 cases, independent of CT findings. These results suggest that 99m Tc-DTPA aerosol clearance study provides information independent from other lung examinations, and may be useful for the assessment of lung interstitial changes in patients with PSS. (author)

  13. Radiation-induced skin injury in the animal model of scleroderma: implications for post-radiotherapy fibrosis

    International Nuclear Information System (INIS)

    Kumar, Sanath; Kolozsvary, Andrew; Kohl, Robert; Lu, Mei; Brown, Stephen; Kim, Jae Ho

    2008-01-01

    Radiation therapy is generally contraindicated for cancer patients with collagen vascular diseases (CVD) such as scleroderma due to an increased risk of fibrosis. The tight skin (TSK) mouse has skin which, in some respects, mimics that of patients with scleroderma. The skin radiation response of TSK mice has not been previously reported. If TSK mice are shown to have radiation sensitive skin, they may prove to be a useful model to examine the mechanisms underlying skin radiation injury, protection, mitigation and treatment. The hind limbs of TSK and parental control C57BL/6 mice received a radiation exposure sufficient to cause approximately the same level of acute injury. Endpoints included skin damage scored using a non-linear, semi-quantitative scale and tissue fibrosis assessed by measuring passive leg extension. In addition, TGF-β1 cytokine levels were measured monthly in skin tissue. Contrary to our expectations, TSK mice were more resistant (i.e. 20%) to radiation than parental control mice. Although acute skin reactions were similar in both mouse strains, radiation injury in TSK mice continued to decrease with time such that several months after radiation there was significantly less skin damage and leg contraction compared to C57BL/6 mice (p < 0.05). Consistent with the expected association of transforming growth factor beta-1 (TGF-β1) with late tissue injury, levels of the cytokine were significantly higher in the skin of the C57BL/6 mouse compared to TSK mouse at all time points (p < 0.05). TSK mice are not recommended as a model of scleroderma involving radiation injury. The genetic and molecular basis for reduced radiation injury observed in TSK mice warrants further investigation particularly to identify mechanisms capable of reducing tissue fibrosis after radiation injury

  14. IMPORTANCE OF CYCLOPHOSPHANUM IN THE TREATMENT OF INTERSTITIAL LUNG LESION IN PATIENTS WITH SCLERODERMA SYSTEMATICA (A REVIEW OF LITERATURE

    Directory of Open Access Journals (Sweden)

    Olga Aleksandrova Koneva

    2010-01-01

    Full Text Available Interstitial lung diseases (ILD are a common manifestation of scleroderma systematica (SSD that along with pulmonary arterial hypertension remains the leading cause of death in this nosological entity. As of now, cyclophosphanum remains the only immunosuppressant recommended by the European League against Rheumatism for the treatment of ILD in SSD. The paper analyzes the papers providing evidence for the efficacy of cyclophosphanum in ILD in patients with SSD. It also considers the regimens and duration of treatment with cyclophosphanum, ways of evaluating its efficacy and effects on extrapulmonary manifestations of SSD. It is concluded that cyclophosphanum has a positive, predominantly stabilizing, effect on the course of ILD in SSD.

  15. Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.

    Science.gov (United States)

    Lazzaroni, Maria-Grazia; Cavazzana, Ilaria; Colombo, Enrico; Dobrota, Rucsandra; Hernandez, Jasmin; Hesselstrand, Roger; Varju, Cecilia; Nagy, Gabriella; Smith, Vanessa; Caramaschi, Paola; Riccieri, Valeria; Hachulla, Eric; Balbir-Gurman, Alexandra; Chatelus, Emmanuel; Romanowska-Próchnicka, Katarzyna; Araújo, Ana Carolina; Distler, Oliver; Allanore, Yannick; Airò, Paolo

    2017-05-01

    To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. (3) A Delphi exercise was performed by 82 experts to reach consensus for cancer screening in anti-RNAP3+ patients. In the EUSTAR registry, anti-RNAP3 were associated in multivariable analysis with renal crisis and diffuse cutaneous involvement. In the case-control study, anti-RNAP3 were associated with gastric antral vascular ectasia, rapid progression of skin involvement, and malignancies concomitant to SSc onset (OR 7.38, 95% CI 1.61-33.8). When compared with other anti-RNAP3+ patients, those with concomitant malignancies had older age (p < 0.001) and more frequent diffuse cutaneous involvement (p = 0.008). The Delphi exercise highlighted the need for malignancy screening at the time of diagnosis for anti-RNAP3+ patients and tight followup in the following years. Anti-RNAP3+ patients with SSc have a high risk of concomitant malignancy. These results have implications for clinical practice and suggest regular screening for cancer in anti-RNAP3+ patients.

  16. Renal Shielding and Dosimetry for Patients With Severe Systemic Sclerosis Receiving Immunoablation With Total Body Irradiation in the Scleroderma: Cyclophosphamide or Transplantation Trial

    International Nuclear Information System (INIS)

    Craciunescu, Oana I.; Steffey, Beverly A.; Kelsey, Chris R.; Larrier, Nicole A.; Paarz-Largay, Cathy J.; Prosnitz, Robert G.; Chao, Nelson; Chute, John; Gasparetto, Cristina; Horwitz, Mitchell; Long, Gwynn; Rizzieri, David; Sullivan, Keith M.

    2011-01-01

    Purpose: To describe renal shielding techniques and dosimetry in delivering total body irradiation (TBI) to patients with severe systemic sclerosis (SSc) enrolled in a hematopoietic stem cell transplant protocol. Methods and Materials: The Scleroderma: Cyclophosphamide or Transplantation (SCOT) protocol uses a lymphoablative preparative regimen including 800 cGy TBI delivered in two 200-cGy fractions twice a day before CD34 + selected autologous hematopoietic stem cell transplantation. Lung and kidney doses are limited to 200 cGy to protect organs damaged by SSc. Kidney block proximity to the spinal cord was investigated, and guidelines were developed for acceptable lumbar area TBI dosing. Information about kidney size and the organ shifts from supine to standing positions were recorded using diagnostic ultrasound (US). Minimum distance between the kidney blocks (dkB) and the lumbar spine region dose was recorded, and in vivo dosimetry was performed at several locations to determine the radiation doses delivered. Results: Eleven patients were treated at our center with an anteroposterior (AP)/posteroanterior (PA) TBI technique. A 10% to 20% dose inhomogeneity in the lumbar spine region was achieved with a minimum kidney block separation of 4 to 5 cm. The average lumbar spine dose was 179.6 ± 18.1 cGy, with an average dkB of 5.0 ± 1.0 cm. Kidney block shield design was accomplished using a combination of US and noncontrast computerized tomography (CT) or CT imaging alone. The renal US revealed a wide range of kidney displacement from upright to supine positions. Overall, the average in vivo dose for the kidney prescription point was 193.4 ± 5.1 cGy. Conclusions: The dose to the kidneys can be attenuated while maintaining a 10% to 20% dose inhomogeneity in the lumbar spine area. Kidneys were localized more accurately using both US and CT imaging. With this technique, renal function has been preserved, and the study continues to enroll patients.

  17. Transcriptomic immune response of Tenebrio molitor pupae to parasitization by Scleroderma guani.

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    Jia-Ying Zhu

    Full Text Available BACKGROUND: Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. METHODOLOGY/PRINCIPAL FINDINGS: In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26% showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. CONCLUSIONS/SIGNIFICANCE: obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular

  18. Transcriptomic immune response of Tenebrio molitor pupae to parasitization by Scleroderma guani.

    Science.gov (United States)

    Zhu, Jia-Ying; Yang, Pu; Zhang, Zhong; Wu, Guo-Xing; Yang, Bin

    2013-01-01

    Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE) analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26%) showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular understanding of the host-parasitoid interaction.

  19. Transcriptomic Immune Response of Tenebrio molitor Pupae to Parasitization by Scleroderma guani

    Science.gov (United States)

    Zhu, Jia-Ying; Yang, Pu; Zhang, Zhong; Wu, Guo-Xing; Yang, Bin

    2013-01-01

    Background Host and parasitoid interaction is one of the most fascinating relationships of insects, which is currently receiving an increasing interest. Understanding the mechanisms evolved by the parasitoids to evade or suppress the host immune system is important for dissecting this interaction, while it was still poorly known. In order to gain insight into the immune response of Tenebrio molitor to parasitization by Scleroderma guani, the transcriptome of T. molitor pupae was sequenced with focus on immune-related gene, and the non-parasitized and parasitized T. molitor pupae were analyzed by digital gene expression (DGE) analysis with special emphasis on parasitoid-induced immune-related genes using Illumina sequencing. Methodology/Principal Findings In a single run, 264,698 raw reads were obtained. De novo assembly generated 71,514 unigenes with mean length of 424 bp. Of those unigenes, 37,373 (52.26%) showed similarity to the known proteins in the NCBI nr database. Via analysis of the transcriptome data in depth, 430 unigenes related to immunity were identified. DGE analysis revealed that parasitization by S. guani had considerable impacts on the transcriptome profile of T. molitor pupae, as indicated by the significant up- or down-regulation of 3,431 parasitism-responsive transcripts. The expression of a total of 74 unigenes involved in immune response of T. molitor was significantly altered after parasitization. Conclusions/Significance obtained T. molitor transcriptome, in addition to establishing a fundamental resource for further research on functional genomics, has allowed the discovery of a large group of immune genes that might provide a meaningful framework to better understand the immune response in this species and other beetles. The DGE profiling data provides comprehensive T. molitor immune gene expression information at the transcriptional level following parasitization, and sheds valuable light on the molecular understanding of the host

  20. Quantitative nanohistological investigation of scleroderma: an atomic force microscopy-based approach to disease characterization

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    Strange AP

    2017-01-01

    Full Text Available Adam P Strange,1 Sebastian Aguayo,1 Tarek Ahmed,1 Nicola Mordan,1 Richard Stratton,2 Stephen R Porter,3 Susan Parekh,4 Laurent Bozec1 1Department of Biomaterials and Tissue Engineering, UCL Eastman Dental Institute, 2Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, UCL Medical School, 3UCL Eastman Dental Institute, 4Department of Pediatrics, UCL Eastman Dental Institute, London, UK Abstract: Scleroderma (or systemic sclerosis, SSc is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. A more detailed analysis of SSc may provide better future treatment options and information of disease progression. Recently, the histological stain picrosirius red showing collagen register has been combined with atomic force microscopy (AFM to study SSc. Skin from healthy individuals and SSc patients was biopsied, stained and studied using AFM. By investigating the crosslinking of collagen at a smaller hierarchical stage, the effects of SSc were more pronounced. Changes in morphology and Young’s elastic modulus were observed and quantified; giving rise to a novel technique, we have termed “quantitative nanohistology”. An increase in nanoscale stiffness in the collagen for SSc compared with healthy individuals was seen by a significant increase in the Young’s modulus profile for the collagen. These markers of stiffer collagen in SSc are similar to the symptoms experienced by patients, giving additional hope that in the future, nanohistology using AFM can be readily applied as a clinical tool, providing detailed information of the state of collagen. Keywords: rheumatology, adjunct diagnosis, picrosirius red, collagen, nanohistology

  1. Is occupational exposure to solvents associated with an increased risk for developing systemic scleroderma?

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    Drexler Hans

    2006-07-01

    Full Text Available Abstract Background Our study was aimed to investigate in a German collective if there are any hints for an increased occupational or environmental risk to develop systemic sclerosis, especially, focussing on work-related exposure to solvents. Moreover, we tried to evaluate the feasibility of a sampling method addressing support groups. Methods A standardised questionnaire was published in two journals subscribed by members of two different support groups and all members were asked to complete the questionnaire and to return it anonymously. The subjects were not informed on the scientific hypotheses, nor did they know who of them belonged to the case group (scleroderma or to the control group (multiple sclerosis. Results 175 questionnaires could be included in the statistical analysis. As expected, a female predominance was in our collective. In the male subpopulation, the occupational exposure to solvents was higher in the case group than in the control-group (70% versus 45.8%. Based only on the male subgroup, a tendency for an association between occupational exposure to solvents and the risk to develop systemic sclerosis was found. Conclusion According to our experience in this case-control-study exposure misclassification, qualitative or quantitative, was an eminent problem. Within such a setting, it is generally very difficult to establish an exact dose-response relationship due to incomplete, imprecise or missing data concerning duration of exposure, frequency of use and kind of solvent. Additionally, a well-known problem in studies based on self-reported questionnaires is the so-called volunteer bias. Unfortunately, but similar to other studies assessing epidemiologic factors in such a rare disease, our study was of limited power, especially in the subgroups defined by gender.

  2. Valuation of scleroderma and psoriatic arthritis health states by the general public

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    Hays Ron D

    2010-10-01

    Full Text Available Abstract Objective Psoriatic arthritis (PsA and scleroderma (SSc are chronic rheumatic disorders with detrimental effects on health-related quality of life. Our objective was to assess health values (utilities from the general public for health states common to people with PsA and SSc for economic evaluations. Methods Adult subjects from the general population in a Midwestern city (N = 218 completed the SF-12 Health Survey and computer-assisted 0-100 rating scale (RS, time trade-off (TTO, range: 0.0-1.0 and standard gamble (SG, range: 0.0-1.0 utility assessments for several hypothetical PsA and SSc health states. Results Subjects included 135 (62% females, 143 (66% Caucasians, and 62 (28% African-Americans. The mean (SD scores for the SF-12 Physical Component Summary scale were 52.9 (8.3 and for the SF-12 Mental Component Summary scale were 49.0 (9.1, close to population norms. The mean RS, TTO, and SG scores for PsA health states varied with severity, ranging from 20.2 to 63.7 (14.4-20.3 for the RS 0.29 to 0.78 (0.24-0.31 for the TTO, and 0.48 to 0.82 (0.24-0.34 for the SG. The mean RS, TTO, and SG scores for SSc health states were 25.3-69.7 (15.2-16.3 for the RS, 0.36-0.80 (0.25-0.31 for the TTO, and 0.50-0.81 (0.26-0.32 for the SG, depending on disease severity. Conclusion Health utilities for PsA and SSc health states as assessed from the general public reflect the severity of the diseases. These descriptive findings could have implications regarding comparative effectiveness research for tests and treatments for PsA and SSc.

  3. Scleroderma dermal microvascular endothelial cells exhibit defective response to pro-angiogenic chemokines

    Science.gov (United States)

    Rabquer, Bradley J.; Ohara, Ray A.; Stinson, William A.; Campbell, Phillip L.; Amin, M. Asif; Balogh, Beatrix; Zakhem, George; Renauer, Paul A.; Lozier, Ann; Arasu, Eshwar; Haines, G. Kenneth; Kahaleh, Bashar; Schiopu, Elena; Khanna, Dinesh; Koch, Alisa E.

    2016-01-01

    Objectives. Angiogenesis plays a critical role in SSc (scleroderma). The aim of this study was to examine the expression of growth-regulated protein-γ (Gro-γ/CXCL3), granulocyte chemotactic protein 2 (GCP-2/CXCL6) and their receptor CXCR2 in endothelial cells (ECs) isolated from SSc skin and determine whether these cells mount an angiogenic response towards pro-angiogenic chemokines. The downstream signalling pathways as well as the pro-angiogenic transcription factor inhibitor of DNA-binding protein 1 (Id-1) were also examined. Methods. Skin biopsies were obtained from patients with dcSSc. ECs were isolated via magnetic positive selection. Angiogenesis was measured by EC chemotaxis assay. Results. Gro-γ/CXCL3 and GCP-2/CXCL6 were minimally expressed in both skin types but elevated in SSc serum. Pro-angiogenic chemokine mRNA was greater in SSc ECs than in normal ECs. SSc ECs did not migrate to vascular endothelial growth factor (VEGF), Gro-γ/CXCL3, GCP-2/CXCL6 or CXCL16. The signalling pathways stimulated by these chemokines were also dysregulated. Id-1 mRNA in SSc ECs was lower compared with normal ECs, and overexpression of Id-1 in SSc ECs increased their ability to migrate towards VEGF and CXCL16. Conclusion. Our results show that SSc ECs are unable to respond to pro-angiogenic chemokines despite their increased expression in serum and ECs. This might be due to the differences in the signalling pathways activated by these chemokines in normal vs SSc ECs. In addition, the lower expression of Id-1 also decreases the angiogenic response. The inability of pro-angiogenic chemokines to promote EC migration provides an additional mechanism for the impaired angiogenesis that characterizes SSc. PMID:26705326

  4. Orofacial Manifestations and Temporomandibular Disorders of Systemic Scleroderma: An Observational Study

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    Vito Crincoli

    2016-07-01

    Full Text Available Scleroderma is a disorder involving oral and facial tissues, with skin hardening, thin lips, deep wrinkles, xerostomia, tongue rigidity, and microstomia. The aim of this study was to investigate the prevalence of oral manifestations and temporomandibular disorders (TMD in Systemic Sclerosis (SSc patients compared with healthy people. Eighty patients (6 men, 74 women fulfilling ACR/EULAR SSc Criteria were enrolled. A randomly selected group of 80 patients, matched by sex and age served as control group. The examination for TMD signs and symptoms was based on the standardized Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD through a questionnaire and clinical examination. SSc patients complained more frequently (78.8% of oral symptoms (Xerostomia, dysgeusia, dysphagia and stomatodynia than controls (28.7% (χ2 = 40.23 p = 0.001. TMD symptoms (muscle pain on chewing, difficulty in mouth opening, headaches were complained by 92.5% of SSc patients and by 76.2% of controls (χ2 = 8.012 p = 0.005. At the clinical examination, 85% of SSc patients showed restricted opening versus 20.0% of controls (χ2 = 67.77 p = 0.001, 81.2% of SSc showed reduced right lateral excursion versus 50% of controls (χ2 = 17.316 p = 0.001; 73.8% of SSc showed limited left lateral excursion versus 53.8% of controls (χ2 = 6.924 p = 0.009; and 73.8% of SSc had narrow protrusion versus 56.2% of controls (χ2 = 5.385 p = 0.02.

  5. Perineural inflammation in morphea (localized scleroderma): systematic characterization of a poorly recognized but potentially useful histopathological feature.

    Science.gov (United States)

    Dhaliwal, Catharine A; MacKenzie, Andrew I; Biswas, Asok

    2014-01-01

    The association between morphea and perineural inflammation has been reported sporadically but never studied systematically. To assess the prevalence and nature of perineural inflammation in various clinicopathologic stages of morphea and a cohort of other inflammatory dermatoses, 80 morphea and 36 control skin biopsies were studied using hematoxylin/eosin and S100 stains. Perineural inflammation was semiquantitatively analyzed (scored), which along with the pattern (concentric vs. marginal) and cellular composition was compared in the two groups. Perineural inflammation was identified in 84% and 61% of morphea and control cases, respectively. Examination of only routinely stained sections could still detect this feature in 58% of morphea and 33% of control biopsies. Mean perineural inflammation score in morphea (0.65) was significantly higher than in the control group (0.23) (p morphea cases. Although perineural inflammation is common in morphea, it is not unusual to find this feature in other inflammatory conditions. Nevertheless, perineural inflammation can serve as an important diagnostic adjunct in difficult cases of morphea if one considers its greater intensity, predominantly concentric pattern and the tendency to show plasma cell neurotropism. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Results of RNV studies and /sup 201/Tl myocardial scintigraphy in patients with progressive systemic scleroderma (PSS)

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    Ammari, B.; Hotze, A.; Gruenwald, F.; Biersack, H.J.; Biltz, H.; Kuester, W.

    1988-12-01

    Prognosis of progressive systemic scleroderma (PSS) depends directly on the extent of visceral organ involvement, and in particular, on the cardiac, renal and pulmonary appearance. Therapeutic approaches therefore require periodic followup with non-invasive methods to evaluate the actual course of disease and the success of therapy. Tl-201 scintigraphy showed pathologic heart abnormalities in 47% and RNV in 23% of the PSS patients. Our results and published data reveal the sensitivity of both myocardial scans and RNV in the evaluation of patients with PSS. Other current methods for the diagnosis of heart diseases, however, such as echocardiography should also be performed. In patients with PSS coronary angiography, however, usually shows normal coronary vessels.

  7. Reduction of regulatory T cells in skin lesions but not in peripheral blood of patients with systemic scleroderma.

    Science.gov (United States)

    Klein, S; Kretz, C C; Ruland, V; Stumpf, C; Haust, M; Hartschuh, W; Hartmann, M; Enk, A; Suri-Payer, E; Oberle, N; Krammer, P H; Kuhn, A

    2011-08-01

    To determine the frequency and suppressive capacity of regulatory T cells (T(reg)) and their association with clinical parameters in patients with systemic scleroderma (SSc). Peripheral blood from 25 patients with SSc, 15 patients with localised scleroderma (LS) and 29 healthy controls (HC) was studied. Analysis of CD4(+) forkhead box P3 (Foxp3)(+) and CD4(+)CD25(++)Foxp3(+) T(reg) subpopulations was carried out by flow cytometry and cell proliferation was quantified by (3)H-thymidine incorporation. Quantitative analysis of T(reg) was further performed in skin biopsies from 17 patients with SSc and 21 patients with LS using anti-CD4 and anti-Foxp3 monoclonal antibodies for immunohistochemistry. The frequency of CD4(+)Foxp3(+) and CD4(+)CD25(++)Foxp3(+) T(reg) in peripheral blood from patients with SSc was not significantly different from that of patients with LS or HC. The suppressive capacity of CD4(+)CD25(++) T(reg) in SSc was also found to be similar to that of HC. Phenotypic and functional data revealed no significant difference between the limited or diffuse form of SSc. Moreover, therapy with bosentan showed no significant effect on the frequency of T(reg) during the course of the disease. However, the frequency of T(reg) in skin lesions from patients with SSc or LS, determined as the percentage of CD4(+) cells expressing Foxp3 in the inflammatory infiltrate, was significantly reduced compared with other inflammatory skin diseases. These results indicate that although the authors found no defect in the frequency or function of peripheral T(reg) subpopulations, the reduction of CD4(+)Foxp3(+) T(reg) in the skin of patients with SSc may be important in the pathogenesis of the disease.

  8. First results of functional RES scintigraphy using sup(99m)-Tc-labeled human serum albumin millimicrospheres in progressive scleroderma: Possibility of early diagnosis of lung involvement

    International Nuclear Information System (INIS)

    Munz, D.L.; Altmeyer, P.; Ehrenheim, C.; Tuengerthal, S.; Holzmann, H.; Hoer, G.; Frankfurt Univ.

    1984-01-01

    Functional RES scintigraphy with sup(99m)Tc-labeled human serum albumin millimicrospheres (sup(99m)Tc-HSA-MM) was conducted in 11 female patients suffering from progressive scleroderma. The RES scan revealed abnormalities in the bone marrow in eight patients as well as pathological changes of the liver in 2 cases. 7 patients showed diffusely enhanced concentrations of sup(99m)Tc-HSA-MM in the lung, whereas X-ray picture and pulmonary function test revealed pathological findings in only 4 patients, respectively. Humoral inflammatory and immunologic parameters, too, indicated abnormalities less frequently than the lung scan. Thus functional RES scintigraphy seems to be a very sensitive approach to the assessment and possibly to the early diagnosis of lung involvement in progressive scleroderma. (orig.) [de

  9. First results of functional RES scintigraphy using sup(99m)-Tc-labeled human serum albumin millimicrospheres in progressive scleroderma: Possibility of early diagnosis of lung involvement

    Energy Technology Data Exchange (ETDEWEB)

    Munz, D.L.; Altmeyer, P.; Ehrenheim, C.; Tuengerthal, S.; Holzmann, H.; Hoer, G.

    1984-01-01

    Functional RES scintigraphy with sup(99m)Tc-labeled human serum albumin millimicrospheres (sup(99m)Tc-HSA-MM) was conducted in 11 female patients suffering from progressive scleroderma. The RES scan revealed abnormalities in the bone marrow in eight patients as well as pathological changes of the liver in 2 cases. 7 patients showed diffusely enhanced concentrations of sup(99m)Tc-HSA-MM in the lung, whereas X-ray picture and pulmonary function test revealed pathological findings in only 4 patients, respectively. Humoral inflammatory and immunologic parameters, too, indicated abnormalities less frequently than the lung scan. Thus functional RES scintigraphy seems to be a very sensitive approach to the assessment and possibly to the early diagnosis of lung involvement in progressive scleroderma.

  10. Cyclophosphamide-refractory scleroderma-associated interstitial lung disease: remarkable clinical and radiological response to a single course of rituximab combined with high-dose corticosteroids.

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-10-01

    We would like to report our experience of using rituximab in cyclophosphamide refractory, rapidly progressive interstitial lung disease (ILD) in a patient with limited scleroderma. A 40-year-old man presented with 10-week history of inflammatory polyarthritis, which responded to a short course of oral corticosteroids. However, 3 weeks later, he developed new onset of exertional dyspnoea. High-resolution CT of the thorax was suggestive of early ILD. Surgical lung biopsy showed features of fibrotic non-specific interstitial pneumonia. He was diagnosed with scleroderma on the basis of: presence of anticentromere antibodies, Raynaud\\'s phenomenon, pulmonary fibrosis, digital oedema and hypomotility along with a dilated oesophagus. He was treated aggressively with pulse doses of corticosteroids and cyclophosphamide; however, his ILD continued to deteriorate. At this stage, he received rituximab (two pulses of 1 g each), which led to a gradual clinical improvement. Now, 12 months since his rituximab infusion, he walks 2 miles daily without any exertional dyspnoea.

  11. Freqüência de alterações hepáticas em pacientes com esclerodermia Frequency of hepatic abnormalities in patients with scleroderma

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    Ariene Paixão

    2007-10-01

    Full Text Available OBJETIVO: avaliar a freqüência das doenças hepáticas em pacientes com esclerodermia e, secundariamente, estudar a freqüência de infecção pelos vírus B e C da hepatite nesses pacientes, assim como a freqüência de auto-anticorpos séricos. MATERIAL E MÉTODOS: estudaram-se pacientes com diagnóstico de esclerodermia, localizada ou sistêmica, acompanhados no Ambulatório de Reumatologia do Hospital Santa Izabel. Como grupo de comparação, foram estudados pacientes com diagnóstico de acne vulgar. RESULTADOS: dos 65 pacientes com diagnóstico de esclerodermia incluídos nesse trabalho, 35% apresentaram a gama-glutamiltransferase (gama-GT alterada, 30% tiveram a fosfatase alcalina aumentada e 17,1%, a alaninoaminotransferase (ALT acima dos valores de referência. A ALT apresentou-se mais alterada nos pacientes do que nos controles. Apenas um indivíduo dos 41 testados apresentou positividade para o anticorpo antimitocôndria enquanto 19% tinham anticorpo antimúsculo liso, não se observando diferença estatística na positividade desses anticorpos entre os dois grupos. Um paciente apresentou o HBsAg positivo e outro foi positivo para o anticorpo anti-HCV. Nenhum paciente apresentou manifestações clínicas de doença hepática. CONCLUSÕES: no presente estudo, embora as alterações de enzimas hepáticas em pacientes com esclerodermia não tenham sido incomuns, não se observou nenhum caso com manifestações clínicas de doença hepática.INTRODUCTION: to determine the frequency of hepatic disease in patients with scleroderma and, secondarily, to investigate the frequency of hepatitis B and C virus infection and determine the frequency of autoantibodies in this disease. MATERIAL AND METHODS: patients with scleroderma followed at Hospital Santa Izabel were included in the study and patients with acne vulgaris served as a comparison group. RESULTS: considering the 65 scleroderma patients, 35% had elevated gamma

  12. Functional and phenotypical comparison of myofibroblasts derived from biopsies and bronchoalveolar lavage in mild asthma and scleroderma

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    Hansson Lennart

    2006-01-01

    Full Text Available Abstract Background Activated fibroblasts, which have previously been obtained from bronchoalveolar lavage fluid (BALF, are proposed to be important cells in the fibrotic processes of asthma and scleroderma (SSc. We have studied the motility for BALF derived fibroblasts in patients with SSc that may explain the presence of these cells in the airway lumen. Furthermore, we have compared phenotypic alterations in activated fibroblasts from BALF and bronchial biopsies from patients with mild asthma and SSc that may account for the distinct fibrotic responses. Methods Fibroblasts were cultured from BALF and bronchial biopsies from patients with mild asthma and SSc. The motility was studied using a cell migration assay. Western Blotting was used to study the expression of alpha-smooth muscle actin (α-SMA, ED-A fibronectin, and serine arginine splicing factor 20 (SRp20. The protein expression pattern was analyzed to reveal potential biomarkers using two-dimensional electrophoresis (2-DE and sequencing dual matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF-TOF. The Mann-Whitney method was used to calculate statistical significance. Results Increased migration and levels of ED-A fibronectin were observed in BALF fibroblasts from both groups of patients, supported by increased expression of RhoA, Rac1, and the splicing factor SRp20. However, these observations were exclusively accompanied by increased expression of α-SMA in patients with mild asthma. Compared to BALF fibroblasts in mild asthma, fibroblasts in SSc displayed a differential protein expression pattern of cytoskeletal- and scavenger proteins. These identified proteins facilitate cell migration, oxidative stress, and the excessive deposition of extracellular matrix observed in patients with SSc. Conclusion This study demonstrates a possible origin for fibroblasts in the airway lumen in patients with SSc and important differences between fibroblast

  13. Esophageal Motor Abnormalities in Patients With Scleroderma: Heterogeneity, Risk Factors, and Effects on Quality of Life.

    Science.gov (United States)

    Crowell, Michael D; Umar, Sarah B; Griffing, W Leroy; DiBaise, John K; Lacy, Brian E; Vela, Marcelo F

    2017-02-01

    Systemic scleroderma (SSc) is associated with esophageal aperistalsis and hypotensive esophagogastric junction pressure, although there could be a gradation in esophageal motor dysfunction. We characterized esophageal motor function by high-resolution esophageal manometry (HRM) and assessed associations between SSc severity, health-related quality of life (HRQOL), and HRM findings in patients. We performed a prospective study of 200 patients with SSc and 102 patients without SSc (controls) who underwent HRM at Mayo Clinic Arizona from May 2006 through January 2015. We used data on integrated relaxation pressure, distal contractile integral, and distal latency to classify esophageal motility disorders according to the Chicago Classification v 3.0. A subset of subjects (n = 122) completed SSc-specific gastrointestinal symptom and HRQOL questionnaires. HRM findings, symptoms, and HRQOL data were compared among diffuse SSc, limited SSc, and control subjects. Categorical variables were compared by using the χ 2 or Fisher exact test; continuous variables were compared by using Mann-Whitney or Kruskal-Wallis test. Multivariable logistic regression was used to assess the association between severity of esophageal dysmotility and baseline clinical factors. Among patients with SSc, 83 had diffuse SSc (42%), and 117 had limited SSc (58%). Absent contractility was more frequent in patients with SSc than in controls (56% vs 13%; P esophagus (esophagogastric junction pressure with absent contractility) was only observed in 33% of patients (34% with diffuse SSc vs 32% limited SSc) (P = .880). Severe esophageal dysmotility was associated with disease duration, interstitial lung disease, and higher gastrointestinal symptom scores (P esophagus in only one-third of patients with SSc. Esophageal motor function appears to be heterogeneous in SSc. Esophageal dysmotility reduces HRQOL in patients with SSc. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

  14. Pressure and pain In Systemic sclerosis/Scleroderma - an evaluation of a simple intervention (PISCES: randomised controlled trial protocol

    Directory of Open Access Journals (Sweden)

    Alcacer-Pitarch Begonya

    2012-02-01

    Full Text Available Abstract Background Foot problems associated with Systemic Sclerosis (SSc/Scleroderma have been reported to be both common and disabling. There are only limited data describing specifically, the mechanical changes occurring in the foot in SSc. A pilot project conducted in preparation for this trial confirmed the previous reports of foot related impairment and reduced foot function in people with SSc and demonstrated a link to mechanical etiologies. To-date there have been no formal studies of interventions directed at the foot problems experienced by people with Systemic Sclerosis. The primary aim of this trial is to evaluate whether foot pain and foot-related health status in people with Systemic Sclerosis can be improved through the provision of a simple pressure-relieving insole. Methods The proposed trial is a pragmatic, multicenter, randomised controlled clinical trial following a completed pilot study. In four participating centres, 140 consenting patients with SSc and plantar foot pain will be randomised to receive either a commercially available pressure relieving and thermally insulating insole, or a sham insole with no cushioning or thermal properties. The primary end point is a reduction in pain measured using the Foot Function Index Pain subscale, 12 weeks after the start of intervention. Participants will complete the primary outcome measure (Foot Function Index pain sub-scale prior to randomisation and at 12 weeks post randomisation. Secondary outcomes include participant reported pain and disability as derived from the Manchester Foot Pain and Disability Questionnaire and plantar pressures with and without the insoles in situ. Discussion This trial protocol proposes a rigorous and potentially significant evaluation of a simple and readily provided therapeutic approach which, if effective, could be of a great benefit for this group of patients. Trial registration number ISRCTN: ISRCTN02824122

  15. Reliability of digital ulcer definitions as proposed by the UK Scleroderma Study Group: A challenge for clinical trial design.

    Science.gov (United States)

    Hughes, Michael; Tracey, Andrew; Bhushan, Monica; Chakravarty, Kuntal; Denton, Christopher P; Dubey, Shirish; Guiducci, Serena; Muir, Lindsay; Ong, Voon; Parker, Louise; Pauling, John D; Prabu, Athiveeraramapandian; Rogers, Christine; Roberts, Christopher; Herrick, Ariane L

    2018-06-01

    The reliability of clinician grading of systemic sclerosis-related digital ulcers has been reported to be poor to moderate at best, which has important implications for clinical trial design. The aim of this study was to examine the reliability of new proposed UK Scleroderma Study Group digital ulcer definitions among UK clinicians with an interest in systemic sclerosis. Raters graded (through a custom-built interface) 90 images (80 unique and 10 repeat) of a range of digital lesions collected from patients with systemic sclerosis. Lesions were graded on an ordinal scale of severity: 'no ulcer', 'healed ulcer' or 'digital ulcer'. A total of 23 clinicians - 18 rheumatologists, 3 dermatologists, 1 hand surgeon and 1 specialist rheumatology nurse - completed the study. A total of 2070 (1840 unique + 230 repeat) image gradings were obtained. For intra-rater reliability, across all images, the overall weighted kappa coefficient was high (0.71) and was moderate (0.55) when averaged across individual raters. Overall inter-rater reliability was poor (0.15). Although our proposed digital ulcer definitions had high intra-rater reliability, the overall inter-rater reliability was poor. Our study highlights the challenges of digital ulcer assessment by clinicians with an interest in systemic sclerosis and provides a number of useful insights for future clinical trial design. Further research is warranted to improve the reliability of digital ulcer definition/rating as an outcome measure in clinical trials, including examining the role for objective measurement techniques, and the development of digital ulcer patient-reported outcome measures.

  16. Quantification of differences between nailfold capillaroscopy images with a scleroderma pattern and normal pattern using measures of geometric and algorithmic complexity.

    Science.gov (United States)

    Urwin, Samuel George; Griffiths, Bridget; Allen, John

    2017-02-01

    This study aimed to quantify and investigate differences in the geometric and algorithmic complexity of the microvasculature in nailfold capillaroscopy (NFC) images displaying a scleroderma pattern and those displaying a 'normal' pattern. 11 NFC images were qualitatively classified by a capillary specialist as indicative of 'clear microangiopathy' (CM), i.e. a scleroderma pattern, and 11 as 'not clear microangiopathy' (NCM), i.e. a 'normal' pattern. Pre-processing was performed, and fractal dimension (FD) and Kolmogorov complexity (KC) were calculated following image binarisation. FD and KC were compared between groups, and a k-means cluster analysis (n  =  2) on all images was performed, without prior knowledge of the group assigned to them (i.e. CM or NCM), using FD and KC as inputs. CM images had significantly reduced FD and KC compared to NCM images, and the cluster analysis displayed promising results that the quantitative classification of images into CM and NCM groups is possible using the mathematical measures of FD and KC. The analysis techniques used show promise for quantitative microvascular investigation in patients with systemic sclerosis.

  17. Quantitative texture-based assessment of one-year changes in fibrotic reticular patterns on HRCT in scleroderma lung disease treated with oral cyclophosphamide

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun J.; Brown, Matthew S. [David Geffen School of Medicine, UCLA, Center for Computer Vision and Imaging Biomarker, Department of Radiological Sciences, Los Angeles, CA (United States); Elashoff, Robert [David Geffen School of Medicine, UCLA, Department of Biostatistics and Biomathematics, Los Angeles, CA (United States); Li, Gang [School of Public Health, UCLA, Department of Biostatistics, Los Angeles, CA (United States); Gjertson, David W. [School of Public Health and David Geffen School of Medicine, UCLA, Department of Biostatistics and Pathology, Los Angeles (United States); Lynch, David A. [National Jewish Health, Radiology Department, Denver, CO (United States); Strollo, Diane C. [UPMC Presbyterian, Radiology Department, Pittsburgh, PA (United States); Kleerup, Eric; Tashkin, Donald P. [David Geffen School of Medicine, UCLA, Department of Med-Pul and Critical Care, Los Angeles, CA (United States); Chong, Daniel; Shah, Sumit K.; Ahmad, Shama; Abtin, Fereidoun; Goldin, Jonathan G. [David Geffen School of Medicine, UCLA, Department of Radiological Sciences, Los Angeles, CA (United States)

    2011-12-15

    The Scleroderma Lung Study showed the efficacy of cyclophosphamide in modestly improving the forced vital capacity (FVC) compared with placebo over 1 year. Using changes in texture-based scores that quantify lung fibrosis as the percentage involvement of reticulation patterns, the effectiveness of cyclophosphamide was re-assessed by examining its impact on quantitative lung fibrosis (QLF). Axial HRCT images were acquired (1-mm slice thickness, 10-mm increments) in the prone position at inspiration. A validated model for quantifying interstitial disease patterns was applied to images from 83 subjects at baseline and 12 months. Scores were calculated for six zones (upper, mid, lower of the right/left lung) and the whole lung. Average changes were compared. Correlations were performed between QLF and physiological and clinical scores. From the most severe zones identified at baseline, QLF scores decreased by 2.6% in the cyclophosphamide group, whereas they increased by 9.1% in the placebo group, leading to {proportional_to}12% difference (p = 0.0027). Between-treatment difference in whole lung QLF was {proportional_to}5% (p = 0.0190). Significant associations were observed between changes in QLF and FVC (r = -0.33), dyspnea score (r = -0.29), and consensus visual score (p = 0.0001). QLF scores provide an objective quantitative tool for assessing treatment efficacy in scleroderma-related interstitial lung disease. (orig.)

  18. Quantitative texture-based assessment of one-year changes in fibrotic reticular patterns on HRCT in scleroderma lung disease treated with oral cyclophosphamide

    International Nuclear Information System (INIS)

    Kim, Hyun J.; Brown, Matthew S.; Elashoff, Robert; Li, Gang; Gjertson, David W.; Lynch, David A.; Strollo, Diane C.; Kleerup, Eric; Tashkin, Donald P.; Chong, Daniel; Shah, Sumit K.; Ahmad, Shama; Abtin, Fereidoun; Goldin, Jonathan G.

    2011-01-01

    The Scleroderma Lung Study showed the efficacy of cyclophosphamide in modestly improving the forced vital capacity (FVC) compared with placebo over 1 year. Using changes in texture-based scores that quantify lung fibrosis as the percentage involvement of reticulation patterns, the effectiveness of cyclophosphamide was re-assessed by examining its impact on quantitative lung fibrosis (QLF). Axial HRCT images were acquired (1-mm slice thickness, 10-mm increments) in the prone position at inspiration. A validated model for quantifying interstitial disease patterns was applied to images from 83 subjects at baseline and 12 months. Scores were calculated for six zones (upper, mid, lower of the right/left lung) and the whole lung. Average changes were compared. Correlations were performed between QLF and physiological and clinical scores. From the most severe zones identified at baseline, QLF scores decreased by 2.6% in the cyclophosphamide group, whereas they increased by 9.1% in the placebo group, leading to ∝12% difference (p = 0.0027). Between-treatment difference in whole lung QLF was ∝5% (p = 0.0190). Significant associations were observed between changes in QLF and FVC (r = -0.33), dyspnea score (r = -0.29), and consensus visual score (p = 0.0001). QLF scores provide an objective quantitative tool for assessing treatment efficacy in scleroderma-related interstitial lung disease. (orig.)

  19. MRI of the fingers in patients with systemic scleroderma. Early results of contrast-enhanced examinations on a dedicated MRI system

    International Nuclear Information System (INIS)

    Bonel, H.; Seemann, M.; Reiser, M.; Messer, G.; Walchner, M.; Roecken, M.

    1997-01-01

    Purpose. To estimate disease activity in patients with systemic sclerosis using contrast-enhanced MRI of the skin. Material and Methods. In a pre-study, sequences of a low-field (0.2 T) scanner (Artoscan, Esaote, Genova, Italy) were optimized for detection of intravenous contrast (0.1 mmol/l Gd-DTPA) in six patients with the autoimmune disease systemic scleroderma. Based on the results of the pre-study, 17 patients with scleroderma (7 sclerotic/10 active inflammatory disease) were scanned using gradient-spoiled 3D GRE sequences (FA 90 , TR 100 ms, TE 18 ms), which had been established as most sensitive for intravenous contrast. Contrast enhancement of the skin was determined quantitatively by contrast-to-noise ratios (CNR), comparing post- to pre-contrast and dynamic scans (for 6 min, 1 acquisition/min). Patients in the chronic state with sclerodactylia and active inflammation of the hands were considered separately and compared to a control group (n=10) matched according to age. Results. CNR increase after intravenous contrast was significantly higher in patients with active disease (86±16% increase) than sclerosing disease (29±3%, p [de

  20. THE REAL PREVALENCE OF EROSIVE ESOPHAGITIS AND BARRETT'S ESOPHAGUS IN SYSTEMIC SCLERODERMA: DATA FROM 12-MONTHS PROSPECTIVE STUDY

    Directory of Open Access Journals (Sweden)

    Andrey Evgenievich Karateev

    2012-01-01

    Full Text Available Complicated forms of reflux-esophagitis, i.e., erosive esophagitis and Barrett's esophagus (BE — are common types of visceral pathology in systemic scleroderma (SSD, which require adequate therapy and follow up. Although real prevalence of esophageal involvement in SSD in Russian patients remains uncertain. Objective — to identify prevalence of erosive esophagitis and BE, and to quantify gastro-intestinal (GI symptoms in patients with SSD. Material and methods. During 1 year (December 2009 — January 2011 all consecutive SSD patients, hospitalized to FSBI «SRIR» RAMS, after signing informed consent, were subjected to esophagogastroduodenoscopy with biopsy of esophageal mucosa in upper 1/3. Totally 123 patients were examined (96,8% females, 3,2% males, aged 50,5±13,1 years. Esophageal mucous was evaluated for presence of pathologic changes and BE (intestinal metaplasia in biopsy samples was a BE diagnostic criterion. SODA questionnaire was used to quantify GIT symptoms Results. Erosive esophagitis was detected in 30 (24,3% patients, BE — in 11 (8,9%. In 80% of patients marked changes in esophageal mucosa were associated with typical symptoms (heartburn, regurgitaion, dysphagia, while in some cases (in 3 patients erosive esophagitis and BE were asymptomatic. Quantitative evaluation of symptoms with SODA questionnaire demonstrated clear correlation between subjective assessment and severity of esophageal pathologic changes. In patients with erosive gastritis and BE the SODA «pain» and «non-pain» parameters scores were significantly higher and satisfaction in dyspepsia management was lower (p<0,05, then in individuals without erosions and mucosal inflammation. Here was no clear correlation between esophageal pathology and SSD type (limited, diffuse, age, duration of the disease, presence of pulmonary interstitial lesion and Sjogren's syndrome. Patients with erosive esophagitis were significantly more often (36,6% using proton pomp

  1. Defects in the Zeta Chain Expression (ζ in a Group of Patients with SLE, Scleroderma and Late-onset Arthritis, Colombia 2014

    Directory of Open Access Journals (Sweden)

    Heber Siachoque-Montañez

    2014-09-01

    Full Text Available Introduction: Systemic Lupus Erythematosus (SLE, Scleroderma and late-onset arthritis are autoimmune inflammatory diseases (EIA characterized by autoantibody production and presence of abnormal T cells which generate defective immune response. The abnormal expression of key signaling molecules in the defective function of T-lymphocytes plays a significant role in the pathogenesis of autoimmune disease. The T-cells exhibit numerous abnormalities TCRζ1 signaling complex, these aberrations result in altered expression of cytokines and some biochemical events involved in the expression of surface molecules. Defects in the complex may be associated TCRζ to steroids used in autoimmune disease patients due to their powerful anti-inflammatory activity and immunosuppressive properties. The synthetic corticosteroids such as examethasone inhibit the transcriptional activity of some factors such as NFKB and AP-1, which regulate the synthesis of certain cytokines and could be involved in the TCRζ synthesis. Material and Methods: A case-control study, with a 1:1 ratio of cases and controls (13:13. Cases were patients with active autoimmune disease (6 patients with SLE, 5 patients with scleroderma and 2 patients with lateonset arthritis, who have not started treatment with corticosteroids. Controls were patients with no autoimmune disease. The diagnosis was made by the criteria established by the American College of Rheumatology for patients with SLE, scleroderma and late-onset arthritis. A 10 mL sample was obtained by venipuncture whole blood. Total RNA was extracted and RT-PCR was performed using a set of primers flanking a region of 138 base pairs involving exons 2, 3 and 4 of the ζ chain. Results: The values of Z chain amplification showed significant differences in patients with autoimmune disease (0.8214 } 0.1787, med = 0.7368 compared with the control group (0.9225 } 0.1272, med = 0.9830 (p = 0.045, Mann-Withney non-parametric one tailed exact

  2. Scleroderma skin ulcers definition, classification and treatment strategies our experience and review of the literature.

    Science.gov (United States)

    Giuggioli, Dilia; Manfredi, Andreina; Lumetti, Federica; Colaci, Michele; Ferri, Clodoveo

    2018-02-01

    Skin ulcers (SU) are one of the most frequent manifestations of systemic sclerosis (SSc). SSc-SU are very painful, often persistent and recurrent; they may lead to marked impairment of patient's activities and quality of life. Despite their severe impact on the whole SSc patient's management, the proposed definition, classification criteria, and therapeutic strategies of SSc-SU are still controversial. The present study aimed to elaborate a comprehensive proposal of definition, classification, and therapeutic strategy of SSc-SU on the basis of our long-term single center experience along with a careful revision of the world literature on the same topic. A series of 282 SSc patients (254 females and 28 males; 84% with limited and 16% diffuse cutaneous SSc; mean age of 51.5±13.9SD at SSc onset; mean follow-up 5.8±4.6SDyears) enrolled during the last decade at our Rheumatology Unit were retrospectively evaluated with specific attention to SSc-SU. The SSc-SU were classified in 5 subtypes according to prominent pathogenetic mechanism(s) and localization, namely 1. digital ulcers (DU) of the hands or feet, 2. SU on bony prominence, 3. SU on calcinosis, 4. SU of lower limbs, and 5. DU presenting with gangrene. This latter is a very harmful evolution of both DU of the hands and feet needing a differential diagnosis with critical limb ischemia. During the follow up period, one or more episodes of SSc-SU were recorded in over half patients (156/282, 55%); skin lesions were often recurrent and difficult-to-heal because of local complications, mainly infections (67.3%), in some cases associated to osteomyelitis (19.2%), gangrene (16%), and/or amputation (11.5%). SSc-SU were significantly associated with lower patients' mean age at the disease onset (p=0.024), male gender (p=0.03), diffuse cutaneous subset (p=0.015), calcinosis (p=0.002), telangiectasia (p=0.008), melanodermia (pTIME (necrotic Tissue, Infection/Inflammation, Moisture balance, and Epithelization). The updated

  3. Antibodies against human cytomegalovirus late protein UL94 in the pathogenesis of scleroderma-like skin lesions in chronic graft-versus-host disease.

    Science.gov (United States)

    Pastano, Rocco; Dell'Agnola, Chiara; Bason, Caterina; Gigli, Federica; Rabascio, Cristina; Puccetti, Antonio; Tinazzi, Elisa; Cetto, Gianluigi; Peccatori, Fedro; Martinelli, Giovanni; Lunardi, Claudio

    2012-09-01

    Human cytomegalovirus (hCMV) infection and its reactivation correlate both with the increased risk and with the worsening of graft-versus-host disease (GVHD). Because scleroderma-like skin lesions can occur in chronic GVHD (cGVHD) in allogeneic stem-cell transplant (HCT) patients and hCMV is relevant in the pathogenesis of systemic sclerosis (SSc), we evaluated the possible pathogenetic link between hCMV and skin cGVHD. Plasma from 18 HCT patients was tested for anti-UL94 and/or anti-NAG-2 antibodies, identified in SSc patients, by direct ELISA assays. Both donors and recipients were anti-hCMV IgG positive, without autoimmune diseases. Patients' purified anti-UL94 and anti-NAG-2 IgG binding to human umbilical endothelial cells (HUVECs) and fibroblasts was performed by FACS analysis and ELISA test. HUVECs apoptosis and fibroblasts proliferation induced by patients' anti-NAG-2 antibodies were measured by DNA fragmentation and cell viability, respectively. About 11/18 patients developed cGVHD and all of them showed skin involvement, ranging from diffuse SSc-like lesions to limited erythema. Eight of eleven cGVHD patients were positive for anti-UL94 and/or anti-NAG-2 antibodies. Remarkably, 4/5 patients who developed diffuse or limited SSc-like lesions had antibodies directed against both UL94 and NAG-2; their anti-NAG-2 IgG-bound HUVECs and fibroblasts induce both endothelial cell apoptosis and fibroblasts proliferation, similar to that induced by purified anti-UL94 and anti-NAG-2 antibodies obtained from SSc patients. In conclusion, our data suggest a pathogenetic link between hCMV infection and scleroderma-like skin cGVHD in HCT patients through a mechanism of molecular mimicry between UL94 viral protein and NAG-2 molecule, as observed in patients with SSc.

  4. 2013 American College of Rheumatology/European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group.

    Science.gov (United States)

    Alhajeri, Hebah; Hudson, Marie; Fritzler, Marvin; Pope, Janet; Tatibouet, Solène; Markland, Janet; Robinson, David; Jones, Niall; Khalidi, Nader; Docherty, Peter; Kaminska, Elzbieta; Masetto, Ariel; Sutton, Evelyn; Mathieu, Jean-Pierre; Ligier, Sophie; Grodzicky, Tamara; LeClercq, Sharon; Thorne, Carter; Gyger, Geneviève; Smith, Douglas; Fortin, Paul R; Larché, Maggie; Baron, Murray

    2015-04-01

    The goal of this study was to determine the sensitivity of the new 2013 classification criteria for systemic sclerosis (SSc; scleroderma) in an independent cohort of SSc subjects and to assess the contribution of individual items of the criteria to the overall sensitivity. SSc subjects from the Canadian Scleroderma Research Group cohort were assessed. Sensitivity was determined in several subgroups of patients. In patients without the criterion of skin thickening proximal to the metacarpophalangeal (MCP) joints, we recalculated sensitivity after removing the individual criterion. A total of 724 SSc patients were included. Most were women (86%), mean age was 55.8 years, mean disease duration was 10.9 years, and 59% had limited cutaneous SSc (lcSSc). Overall, the sensitivity of the 2013 criteria was 98.3% compared to 88.3% for the 1980 criteria. This pattern was consistent among those with lcSSc (98.8% versus 85.6%), anticentromere antibodies (98.9% versus 79.8%), disease duration ≤3 years (98.7% versus 84.7%), and no skin involvement proximal to the MCP joints (97% versus 60%). In the latter subgroup, removing Raynaud's phenomenon and sclerodactyly from the criteria reduced the sensitivity to 77% and 79%, respectively. Removing both sclerodactyly and puffy fingers reduced the sensitivity to 62%. The 2013 SSc classification criteria classify more SSc patients than the 1980 criteria. The improvement in sensitivity is most striking in those with lcSSc, especially those without skin involvement proximal to the MCP joints. The addition of Raynaud's phenomenon and puffy fingers to the 2013 criteria accounts for important gains in sensitivity. Copyright © 2015 by the American College of Rheumatology.

  5. Papel da lidocaína por via venosa no tratamento da dor na esclerodermia: relato de caso Papel de la lidocaína por vía venosa en el tratamiento del dolor en la esclerodermia: relato de un caso Intravenous lidocaine to treat scleroderma pain: case report

    Directory of Open Access Journals (Sweden)

    Durval Campos Kraychete

    2003-12-01

    , continua, diaria, acompañada de alteraciones tróficas, de color y de temperatura y pequeñas úlceras en las extremidades. La paciente fue sometida a una sesión semanal de lidocaína a 2% (400 mg sin vasoconstrictor por vía venosa durante 10 semanas con alivio del dolor, del turgor, de la elasticidad de la piel y de la perfusión periférica. CONCLUSIONES: El alivio del dolor y de otros síntomas después de la administración de lidocaína por vía venosa sugiere que los anestésicos locales pueden modular la respuesta inflamatoria en varios aprendizajes de la esclerodermia.BACKGROUND AND OBJECTIVES: Scleroderma or progressive systemic sclerosis is a systemic connective tissue disease of unknown origin, which normally courses with microangiopathy, extremities ischemia and severe pain. This report aimed at describing a case of intravenous lidocaine to treat ischemic pain and at emphasizing potential anti-inflammatory action of local anesthetics in scleroderma patients. CASE REPORT: Female patient, clear mulatto 34 years old, nursing assistant, with scleroderma for approximately 8 years, presented with severe continuous, daily pain (numeric scale = 10 in upper and lower limbs, followed by trophic, color and temperature changes, and small ulcers on extremities. Patient was submitted to 1 weekly session of intravenous 2% lidocaine (400 mg without vasoconstrictor for 10 weeks with pain, turgor, skin elasticity and peripheral perfusion improvement. CONCLUSIONS: Pain and other symptoms relief after intravenous lidocaine suggests that local anesthetics are able to modulate inflammatory response in different scleroderma stages.

  6. The cannabinoid quinol VCE-004.8 alleviates bleomycin-induced scleroderma and exerts potent antifibrotic effects through peroxisome proliferator-activated receptor-γ and CB2 pathways.

    Science.gov (United States)

    del Río, Carmen; Navarrete, Carmen; Collado, Juan A; Bellido, M Luz; Gómez-Cañas, María; Pazos, M Ruth; Fernández-Ruiz, Javier; Pollastro, Federica; Appendino, Giovanni; Calzado, Marco A; Cantarero, Irene; Muñoz, Eduardo

    2016-02-18

    Scleroderma is a group of rare diseases associated with early and transient inflammation and vascular injury, followed by fibrosis affecting the skin and multiple internal organs. Fibroblast activation is the hallmark of scleroderma, and disrupting the intracellular TGFβ signaling may provide a novel approach to controlling fibrosis. Because of its potential role in modulating inflammatory and fibrotic responses, both PPARγ and CB2 receptors represent attractive targets for the development of cannabinoid-based therapies. We have developed a non-thiophilic and chemically stable derivative of the CBD quinol (VCE-004.8) that behaves as a dual agonist of PPARγ and CB2 receptors, VCE-004.8 inhibited TGFβ-induced Col1A2 gene transcription and collagen synthesis. Moreover, VCE-004.8 inhibited TGFβ-mediated myofibroblast differentiation and impaired wound-healing activity. The anti-fibrotic efficacy in vivo was investigated in a murine model of dermal fibrosis induced by bleomycin. VCE-004.8 reduced dermal thickness, blood vessels collagen accumulation and prevented mast cell degranulation and macrophage infiltration in the skin. These effects were impaired by the PPARγ antagonist T0070907 and the CB2 antagonist AM630. In addition, VCE-004.8 downregulated the expression of several key genes associated with fibrosis, qualifying this semi-synthetic cannabinoid as a novel compound for the management of scleroderma and, potentially, other fibrotic diseases.

  7. Eating Well with Scleroderma

    Science.gov (United States)

    ... Add antioxidant rich, anti-inflam- matory herbs and spices, such as basil, rosemary, oregano, cin- namon, ginger, ... or Culturelle ® ) and/or eat yogurt with active cultures regularly. Remember to increase your fluid intake. • Inflammation: ...

  8. Hemodynamic effects of a prostacyclin analog (Prostavasin) in systemic sclero-derma patients; Effetti di un analogo della prostaciclina (Prostavasin) sui parametri Doppler nei pazienti con sclerodermia

    Energy Technology Data Exchange (ETDEWEB)

    Salera, Diego; Argalia, Giulio; Giuseppetti, Gian Marco [Univ. Politecnica delle Marche, Ancona (Italy). Istituto di radiologia

    2005-07-15

    Purpose. We examined the effects of a prostacyclin analogue (Prostavasin) on the circulation of upper extremity, cerebral, ocular and visceral districts such as portal vein, hepatic artery, superior mesenteric artery, and inter lobar renal artery in scleroderma patients. Materials and methods. peripheral vasculature was evaluated by the brachial artery flow-mediated dilatation by the high resolution ultrasound cross-sectional measurement, splenic arterial pulsatility index (PI) resistance index (RI) of the middle cerebral artery, the central retinal artery, the visceral arteries and the portal vein flow were assessed by colour Doppler sonography in an experimental group (EG) of 50 scleroderma patients, not affected by cerebrovascular, ocular, hepatic diseases or nephropathy, before and after 3 days of Prostavasin infusion and before and after 3 days in a control group (CG) of 10 patients not receiving any treatment. Results. EG patients showed significant increasement in the brachial artery flow-mediated dilatation, in the portal vein velocity and in the splenic arterial PI (preProstavasin vs post-Prostavasin treatment, p < 0.001) whereas CG patients had no significant changes. Values of the middle cerebral artery, the central retinal artery, the inter lobar renal artery, the superior mesenteric artery and the hepatic artery RI were reduced after treatment in the majority of EG patients although the difference did not achieve a satisfactory statistical significance. Conclusions. our results indicate that Prostavasin has a powerful effect in improving the peripheral circulation of scleroderma patients. Prostavasin significantly increases the portal vein flow but also the splenic arterial PI not supporting the hypothesis of its direct and specific action on relaxation of the hepatic micro circle. [Italian] Scopo. Sono stati studiati gli effetti di un analogo della prostaciclina (Prostavasin) sul circolo periferico degli arti superiori e sulla vascolarizzazione

  9. Desfecho da gravidez em portadoras de esclerodermia difusa e limitada Pregnancy outcome in patients with diffuse and limited scleroderma

    Directory of Open Access Journals (Sweden)

    Marina Celli Francisco

    2005-10-01

    with scleroderma (SSc when compared with women without the disease, between the two clinical variants of the disease and when the expression of SSc occurred before or after the gestation. METHODS: twenty-six patients were analyzed regarding the number of pregnancies, miscarriages, sex, and birth weight of the children, studying the clinical variant of the disease and the temporal relationship between diagnosis and gestation. The patients had not used either medications that could interfere in gestation or had any other disease that could do it. For control, twenty-six healthy women, without diseases that might alter the gestation, of the same ages and socioeconomic status were studied. For the statistical analysis study, tables of frequency, tables of contingency, and Fisher, chi2 and Mann-Whitney tests were used. Statistical significance was considered when p<0.05. RESULTS: among the patients with SSc, there were 96 gestations with 13.5% (n=13 of miscarriages. In the control group, there were 94 gestations with 9.6% (n=9 of miscarriages. There were no differences in the number of newborns with low weight between the two groups (cases, n=8 and controls, n=6, with p=0.54, nor in the number of miscarriages (p=0.46. However, the number of newborn babies with low weight was significantly higher among the cases with the diffuse disease (diffuse form, n=4 and limited, n=4, with p=0.04 and among patients that became pregnant after the diagnosis of SSc (37.5% in women known to be sick and 6.7% in women who became pregnant before getting ill, with p=0.03. The number of male newborns was higher in the women with SSc (p=0.002. CONCLUSIONS: the women with SSc showed a higher number of low-weight newborns in the group with diffuse disease and when pregnancy occurred after the clinical diagnosis of the disease.

  10. Local food:

    DEFF Research Database (Denmark)

    Sundbo, Donna Isabella Caroline

    2013-01-01

    are identified and then categorised according to whether they pertain to the food product itself or the production methods and facilities and whether they describe physical or social properties of local food. From this a model with four categories is developed. It is found that properties of the product are more......Recently there has been more focus on food in general and local food in particular. But what is local food? And what are the perceptions of this concept according to theory and to providers and consumers of local food? This article first summarises and compares three different theoretical...... perspectives on local food, namely experience economy, local food systems and what is termed pro-industrialism. These have differing and sometimes opposite conceptualisations and aims for the concept of local food. Using the perspective of experience economy as theoretical background, the concept of local food...

  11. Local Content

    CSIR Research Space (South Africa)

    Gibberd, Jeremy

    2016-10-01

    Full Text Available Local content refers to materials and products made in a country as opposed those that are imported. There is an increasing interest in the concept of local content as a means of supporting local economies and providing jobs (Belderbos & Sleuwaegen...

  12. A novel DNMT1 mutation associated with early onset hereditary sensory and autonomic neuropathy, cataplexy, cerebellar atrophy, scleroderma, endocrinopathy, and common variable immune deficiency.

    Science.gov (United States)

    Fox, Robin; Ealing, John; Murphy, Helen; Gow, David P; Gosal, David

    2016-09-01

    DNA methyltransferase 1 (DNMT1) is an enzyme which has a role in methylation of DNA, gene regulation, and chromatin stability. Missense mutations in the DNMT1 gene have been previously associated with two neurological syndromes: hereditary sensory and autonomic neuropathy type 1 with dementia and deafness (HSAN1E) and autosomal dominant cerebellar ataxia, deafness, and narcolepsy (ADCA-DN). We report a case showing overlap of both of these syndromes plus associated clinical features of common variable immune deficiency, scleroderma, and endocrinopathy that could also be mutation associated. Our patient was found to be heterozygous for a previously unreported frameshift mutation, c.1635_1637delCAA p.(Asn545del) in the DNMT1 gene exon 20. This case displays both the first frameshift mutation described in the literature which is associated with a phenotype with a high degree of overlap between HSAN1E and ADCA-DN and early age of onset (c. 8 years). Our case is also of interest as the patient displays a number of new non-neurological features, which could also be DNMT1 mutation related. © 2016 Peripheral Nerve Society.

  13. Aberrant immune response with consequent vascular and connective tissue remodeling - causal to scleroderma and associated syndromes such as Raynaud phenomenon and other fibrosing syndromes?

    Science.gov (United States)

    Durmus, Nedim; Park, Sung-Hyun; Reibman, Joan; Grunig, Gabriele

    2016-11-01

    Scleroderma and other autoimmune-induced connective tissue diseases are characterized by dysfunctions in the immune system, connective tissue and the vasculature. We are focusing on systemic sclerosis (SSc)-associated pulmonary hypertension, which remains a leading cause of death with only a 50-60% of 2-year survival rate. Much research and translational efforts have been directed at understanding the immune response that causes SSc and the networked interactions with the connective tissue and the vasculature. One of the unexpected findings was that in some cases the pathogenic immune response in SSc resembles the immune response to helminth parasites. During coevolution, means of communication were developed which protect the host from over-colonization with parasites and which protect the parasite from excessive host responses. One explanation for the geographically clustered occurrence of SSc is that environmental exposures combined with genetic predisposition turn on triggers of molecular and cellular modules that were once initiated by parasites. Future research is needed to further understand the parasite-derived signals that dampen the host response. Therapeutic helminth infection or treatment with parasite-derived response modifiers could be promising new management tools for autoimmune connective tissue diseases.

  14. Polysaccharides from the fungus Scleroderma nitidum with anti-inflammatory potential modulate cytokine levels and the expression of Nuclear Factor kB

    Directory of Open Access Journals (Sweden)

    Marília S. Nascimento

    2012-02-01

    Full Text Available Several pharmacological properties are attributed to polysaccharides and glucans derived from fungi such as tumor, anti-inflammatory, and immunomodulatory activity. In this work, the anti-inflammatory potential of polysaccharides from the fungus Scleroderma nitidum and their possible action mechanism were studied. The effect of these polymers on the inflammatory process was tested using the carrageenan and histamine-induced paw edema model and the sodium thioglycolate and zymosan-induced model. The polysaccharides from S. nitidum were effective in reducing edema (73% at 50 mg/kg and cell infiltrate (37% at 10 mg/kg in both inflammation models tested. Nitric oxide, a mediator in the inflammatory process, showed a reduction of around 26% at 10 mg/kg of body weight. Analysis of pro-and anti-inflammatory cytokines showed that in the groups treated with polysaccharides from S. nitidum there was an increase in cytokines such as IL-1ra, IL-10, and MIP-1β concomitant with the decrease in INF-γ (75% and IL-2 (22%. We observed the influence of polysaccharides on the modulation of the expression of nuclear factor κB. This compound reduced the expression of NF-κB by up to 64%. The results obtained suggest that NF-κB modulation an mechanisms that explain the anti-inflammatory effect of polysaccharides from the fungus S. nitidum.

  15. Pulsed versus continuous wave low-level light therapy on osteoarticular signs and symptoms in limited scleroderma (CREST syndrome): a case report

    Science.gov (United States)

    Barolet, Daniel

    2014-11-01

    Limited cutaneous systemic sclerosis (lcSSc) was formerly known as CREST syndrome in reference to the associated clinical features: calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. The transforming growth factor beta has been identified as a major player in the pathogenic process, where low-level light therapy (LLLT) has been shown to modulate this cytokine superfamily. This case study was conducted to assess the efficacy of 940 nm using millisecond pulsing and continuous wave (CW) modes on osteoarticular signs and symptoms associated with lcSSc. The patient was treated two to three times a week for 13 weeks using a sequential pulsing mode on one elbow and a CW mode on the other. Efficacy assessments included inflammation, symptoms, pain, health scales, patient satisfaction, clinical global impression, and adverse effects monitoring. Considerable functional and morphologic improvements were observed after LLLT, with the best results seen with the pulsing mode. No adverse effects were noted. Pulsed LLLT represents a treatment alternative for osteoarticular signs and symptoms in limited scleroderma (CREST syndrome).

  16. Chitinase 1 Is a Biomarker for and Therapeutic Target in Scleroderma-Associated Interstitial Lung Disease That Augments TGF-β1 Signaling

    Science.gov (United States)

    Lee, Chun Geun; Herzog, Erica L.; Ahangari, Farida; Zhou, Yang; Gulati, Mridu; Lee, Chang-Min; Peng, Xueyan; Feghali-Bostwick, Carol; Jimenez, Sergio A.; Varga, John; Elias, Jack A.

    2014-01-01

    Interstitial lung disease (ILD) with pulmonary fibrosis is an important manifestation in systemic sclerosis (SSc, scleroderma) where it portends a poor prognosis. However, biomarkers that predict the development and or severity of SSc-ILD have not been validated, and the pathogenetic mechanisms that engender this pulmonary response are poorly understood. In this study, we demonstrate in two different patient cohorts that the levels of chitotriosidase (Chit1) bioactivity and protein are significantly increased in the circulation and lungs of SSc patients compared with demographically matched controls. We also demonstrate that, compared with patients without lung involvement, patients with ILD show high levels of circulating Chit1 activity that correlate with disease severity. Murine modeling shows that in comparison with wild-type mice, bleomycin-induced pulmonary fibrosis was significantly reduced in Chit1−/− mice and significantly enhanced in lungs from Chit1 overexpressing transgenic animals. In vitro studies also demonstrated that Chit1 interacts with TGF-β1 to augment fibroblast TGF-β receptors 1 and 2 expression and TGF-β–induced Smad and MAPK/ERK activation. These studies indicate that Chit1 is potential biomarker for ILD in SSc and a therapeutic target in SSc-associated lung fibrosis and demonstrate that Chit1 augments TGF-β1 effects by increasing receptor expression and canonical and noncanonical TGF-β1 signaling. PMID:22826322

  17. Evaluation of the Satisfaction with Appearance Scale and Its Short Form in Systemic Sclerosis: Analysis from the UCLA Scleroderma Quality of Life Study.

    Science.gov (United States)

    Mills, Sarah D; Fox, Rina S; Merz, Erin L; Clements, Philip J; Kafaja, Suzanne; Malcarne, Vanessa L; Furst, Daniel E; Khanna, Dinesh

    2015-09-01

    Changes in appearance are common in patients with systemic sclerosis (SSc) and can significantly affect well-being. The Satisfaction with Appearance Scale (SWAP) measures body image dissatisfaction in persons with visible disfigurement; the Brief-Satisfaction with Appearance Scale (Brief-SWAP) is its short form. The present study evaluated the reliability and validity of SWAP and Brief-SWAP scores in SSc. A sample of 207 patients with SSc participating in the University of California, Los Angeles Scleroderma Quality of Life Study completed the SWAP. Brief-SWAP scores were derived from the SWAP. The structural validity of both measures was investigated using confirmatory factor analysis. Internal consistency reliability of total and subscale scores was assessed with Cronbach's alpha coefficients. Convergent and divergent validity was evaluated using the Center for Epidemiological Studies Depression Scale, the Health Assessment Questionnaire-Disability Index, and the Medical Outcomes Study Short Form-36 questionnaire. SWAP and Brief-SWAP total scores were highly correlated (r = 0.97). The 4-factor structure of the SWAP fit well descriptively; the 2-factor structure of the Brief-SWAP fit well descriptively and statistically. Internal consistencies for total and subscale scores were good, and results supported convergent and divergent validity. Both versions are suitable for use in patients with SSc. The Brief-SWAP is most efficient; the full SWAP yields additional subscales that may be informative in understanding body image issues in patients with SSc.

  18. Localized superconductors

    International Nuclear Information System (INIS)

    Ma, M.; Lee, P.A.

    1985-01-01

    We study the effects of Anderson localization on superconductivity by using a Bardeen-Cooper-Schrieffer (BCS)-type trial wave function which pairs electrons in exact time-reversed eigenstates of the single-particle Hamiltonian. Within this approximation, and neglecting localization effects on the effective Coulomb repulsion and the electron-phonon coupling, we find that superconductivity persists below the mobility edge. In fact, Anderson's theorem is valid in the localized phase as long as rhoΔ 0 L/sup d/ > 1 (rho is the density of states averaged over +- Δ 0 of the Fermi energy, Δ 0 the BCS gap parameter, and L the localization length). Hence the gap order parameter Δ(r) remains uniform in space at the BCS value Δ 0 . The superfluid density and response to electromagnetic perturbations, however, show marked differences from the ''dirty superconductor'' regime. For rhoΔ 0 L/sup d/ < 1, Δ(r) fluctuates spatially and eventually drops to zero. In the limit when states are site localized, the system crosses over into the ''Anderson negative-U glass.'' Considerations beyond the trial wave-function approximation will speed up the destruction of superconductivity. The superconductor formed from localized states has the property that its quasiparticle excitations are also localized. Such excitations can be probed by observing the normal current in a tunneling junction

  19. Validation of the Social Appearance Anxiety Scale in Patients with Systemic Sclerosis: A Scleroderma Patient-centered Intervention Network Cohort Study.

    Science.gov (United States)

    Mills, Sarah D; Kwakkenbos, Linda; Carrier, Marie-Eve; Gholizadeh, Shadi; Fox, Rina S; Jewett, Lisa R; Gottesman, Karen; Roesch, Scott C; Thombs, Brett D; Malcarne, Vanessa L

    2018-01-17

    Systemic sclerosis (SSc) is an autoimmune disease that can cause disfiguring changes in appearance. This study examined the structural validity, internal consistency reliability, convergent validity, and measurement equivalence of the Social Appearance Anxiety Scale (SAAS) across SSc disease subtypes. Patients enrolled in the Scleroderma Patient-centered Intervention Network Cohort completed the SAAS and measures of appearance-related concerns and psychological distress. Confirmatory factor analysis (CFA) was used to examine the structural validity of the SAAS. Multiple-group CFA was used to determine if SAAS scores can be compared across patients with limited and diffuse disease subtypes. Cronbach's alpha was used to examine internal consistency reliability. Correlations of SAAS scores with measures of body image dissatisfaction, fear of negative evaluation, social anxiety, and depression were used to examine convergent validity. SAAS scores were hypothesized to be positively associated with all convergent validity measures, with correlations significant and moderate to large in size. A total of 938 patients with SSc were included. CFA supported a one-factor structure (CFI: .92; SRMR: .04; RMSEA: .08), and multiple-group CFA indicated that the scalar invariance model best fit the data. Internal consistency reliability was good in the total sample (α = .96) and in disease subgroups. Overall, evidence of convergent validity was found with measures of body image dissatisfaction, fear of negative evaluation, social anxiety, and depression. The SAAS can be reliably and validly used to assess fear of appearance evaluation in patients with SSc, and SAAS scores can be meaningfully compared across disease subtypes. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  20. Locals Collection

    Directory of Open Access Journals (Sweden)

    Stephen Hastings-King

    2010-03-01

    Full Text Available A locals collection is a set of parameters that are used to delimit data-mining operations. This piece uses a collection of locals from around Essex Massachusetts to shape and delimit an interrogation of post-reality in contemporary America. It explores the notion of crisis, the possibility of a crisis of empire that may or may not emerge in a media-space that does not allow crisis of empire to be mentioned and relations this maybe-crisis to the various levels of economic dysfunction that have become evident since late 2008. But mostly this piece explores ways in which particular stories about particular people do and do not link/link to these larger-scale narratives. This is the first of a potential series of locals collections that will mine the American post-real.

  1. Local equilibrium

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    1984-12-15

    From 3-6 September the First International Workshop on Local Equilibrium in Strong Interaction Physics took place in Bad-Honnef at the Physics Centre of the German Physical Society. A number of talks covered the experimental and theoretical investigation of the 'hotspots' effect, both in high energy particle physics and in intermediate energy nuclear physics.

  2. Net Locality

    DEFF Research Database (Denmark)

    de Souza e Silva, Adriana Araujo; Gordon, Eric

    Provides an introduction to the new theory of Net Locality and the profound effect on individuals and societies when everything is located or locatable. Describes net locality as an emerging form of location awareness central to all aspects of digital media, from mobile phones, to Google Maps......, to location-based social networks and games, such as Foursquare and facebook. Warns of the threats these technologies, such as data surveillance, present to our sense of privacy, while also outlining the opportunities for pro-social developments. Provides a theory of the web in the context of the history...... of emerging technologies, from GeoCities to GPS, Wi-Fi, Wiki Me, and Google Android....

  3. The relationship between skin symptoms and the scleroderma modification of the health assessment questionnaire, the modified Rodnan skin score, and skin pathology in patients with systemic sclerosis.

    Science.gov (United States)

    Ziemek, Jessica; Man, Ada; Hinchcliff, Monique; Varga, John; Simms, Robert W; Lafyatis, Robert

    2016-05-01

    To determine how well skin symptoms considered specific to SSc are captured by patient reported outcomes currently used for assessing patients with SSc, the SHAQ, or skin disease, the Skindex-29; and how well these symptoms correlate with the extent of skin disease on physical exam and skin pathology. SSc patients completed the scleroderma modification of the Health Assessment Questionnaire (SHAQ), Skindex-29 and a Skin Symptom Assessment questionnaire developed for this study. Correlations were assessed between the Skin Symptom Assessment and SHAQ, Skindex-29, modified Rodnan skin score, and skin pathological features including myofibroblast staining completed on the same date. Tight, hard and rigid/stiff skin symptoms correlated moderately highly with the modified Rodnan skin score (r = 0.445, P = 0.0008; r = 0.486, P = 0.0002; and r = 0.488, P = 0.0002, respectively). Tight skin symptoms correlated moderately with myofibroblast infiltration (r = 0.544, P = 0.0023) and hyalinized collagen (r = 0.442, P = 0.0164), while both hard and rigid/stiff skin correlated moderately with inflammation (r = 0.401, P = 0.0310 and r = 0.513, P = 0.0045), myofibroblast infiltration(r = 0.480, P = 0.0084 and r = 0.527, P = 0.0033) and hyalinized collagen (r = 0.453, P = 0.0137 and r = 0.478, P = 0.0087), while the SHAQ was not found to correlate with any of these pathological changes. In contrast, painful skin symptoms correlated moderately with the SHAQ (r = 0.413, P = 0.0073), and with the three domains of Skindex-29: Symptoms, Emotions and Functioning. Skindex-29 indicates that dcSSc patient skin symptoms are nearly as severe as those of patients with psoriasis or atopic dermatitis. Patient reported skin symptoms correlate with clinical and pathological measures in the skin. A validated patient reported skin symptom instrument might considerably improve evaluation of SSc skin disease. © The Author 2016. Published by Oxford University Press on behalf of the British Society for

  4. Local supertwistors

    International Nuclear Information System (INIS)

    Merkulov, S.A.

    1987-01-01

    Geometry of local supertwistors is investigated. It is proved that the Yang-Mills equations for the introduced ansatz for supertwistor connection are equivalent to free bach equations, describing the dynamics of N=1 conformal supergravity. Analogous interpretation of the dynamics of N=1 conformal supergravity coupled to a vector superfield is proposed. It is proved that any complex conformally right or left flat superspace automatically satisfies the Bach equations

  5. Local supertwistors

    International Nuclear Information System (INIS)

    Merkulov, S.A.

    1987-01-01

    The geometry of local supertwistors is investigated. An ansatz on the form of the supertwistor superconnection is introduced. Because of this restriction on the form of such a superconnection the Yang-Mills equations for the superconnection turn out to be equivalent to the free Bach equations describing the dynamics of simple conformal supergravity. It is shown that the equations of motion of conformal supergravity interacting with a vector superfield admit an analogous interpretation. It is proved that an arbitrary conformally right-flat or left-flat superspace is automatically a solution of the Bach equations

  6. Local Sustainability

    International Nuclear Information System (INIS)

    Carrizosa Umana, Julio

    1998-01-01

    The current polemic about the possibilities of sustainable development has led to a renovated interest for the topic of the sustainability of the communities and the local sustainability. In front of the global sustainability whose conditions have been exposed by systemic ecologists and for macro economists, the sustainability of specific places arises in the planet whose conditions are object of study of the ecology of landscapes, of the ecological economy, of the cultural anthropology, of the environmental sociology and naturally, of the integral environmentalism. In this discussion the Colombian case charges unusual interest to be one of the few countries of Latin America, where a very dense net of municipalities exists, each one with its urban helmet and with a position and some functions defined by the political constitution of the nation. This net of municipalities and of urban helmets it also constitutes net of alternative to the current macro-cephalic situation. As well as Bogota grew, in a hundred years, of less than a hundred thousand inhabitants to six million inhabitants, each one of these municipalities contains a potential of growth that depends on the characteristics of its ecological, social, economic and politic sustainability

  7. Quantum Locality?

    Science.gov (United States)

    Stapp, Henry P.

    2012-05-01

    , in response to Griffiths' challenge, why a putative proof of locality that he has described is flawed.

  8. Local Foods, Local Places Summary Reports

    Science.gov (United States)

    These reports summarize community projects done with Local Foods, Local Places assistance, including farmers markets, cooperatives, community gardens, and other food-related enterprises that can boost local economies and drive revitalization.

  9. Quantum Locality?

    Energy Technology Data Exchange (ETDEWEB)

    Stapp, Henry

    2011-11-10

    vagaries that he cites do not upset the proof in question. It is show here in detail why the precise statement of this theorem justifies the specified application of CQT. It is also shown, in response to his challenge, why a putative proof of locality that he has proposed is not valid.

  10. Progressive Hemifacial Atrophy with Morphea of Cheek

    Directory of Open Access Journals (Sweden)

    Ajit Auluck

    2006-01-01

    Full Text Available Scleroderma is a rare collagen disorder in which fibrosis of skin, subcutaneous tissues and muscles can occur with occasional involvement of bones. Localized scleroderma is a benign condition but can cause significant deformity when it affects the face. We report a case of localized scleroderma of the face causing progressive hemifacial atrophy.

  11. Local Stereo Matching Using Adaptive Local Segmentation

    NARCIS (Netherlands)

    Damjanovic, S.; van der Heijden, Ferdinand; Spreeuwers, Lieuwe Jan

    We propose a new dense local stereo matching framework for gray-level images based on an adaptive local segmentation using a dynamic threshold. We define a new validity domain of the fronto-parallel assumption based on the local intensity variations in the 4-neighborhood of the matching pixel. The

  12. Prognostic model based on nailfold capillaroscopy for identifying Raynaud's phenomenon patients at high risk for the development of a scleroderma spectrum disorder: PRINCE (prognostic index for nailfold capillaroscopic examination).

    Science.gov (United States)

    Ingegnoli, Francesca; Boracchi, Patrizia; Gualtierotti, Roberta; Lubatti, Chiara; Meani, Laura; Zahalkova, Lenka; Zeni, Silvana; Fantini, Flavio

    2008-07-01

    To construct a prognostic index based on nailfold capillaroscopic examinations that is capable of predicting the 5-year transition from isolated Raynaud's phenomenon (RP) to RP secondary to scleroderma spectrum disorders (SSDs). The study involved 104 consecutive adult patients with a clinical history of isolated RP, and the index was externally validated in another cohort of 100 patients with the same characteristics. Both groups were followed up for 1-8 years. Six variables were examined because of their potential prognostic relevance (branching, enlarged and giant loops, capillary disorganization, microhemorrhages, and the number of capillaries). The only factors that played a significant prognostic role were the presence of giant loops (hazard ratio [HR] 2.64, P = 0.008) and microhemorrhages (HR 2.33, P = 0.01), and the number of capillaries (analyzed as a continuous variable). The adjusted prognostic role of these factors was evaluated by means of multivariate regression analysis, and the results were used to construct an algorithm-based prognostic index. The model was internally and externally validated. Our prognostic capillaroscopic index identifies RP patients in whom the risk of developing SSDs is high. This model is a weighted combination of different capillaroscopy parameters that allows physicians to stratify RP patients easily, using a relatively simple diagram to deduce the prognosis. Our results suggest that this index could be used in clinical practice, and its further inclusion in prospective studies will undoubtedly help in exploring its potential in predicting treatment response.

  13. Locally Finite Root Supersystems

    OpenAIRE

    Yousofzadeh, Malihe

    2013-01-01

    We introduce the notion of locally finite root supersystems as a generalization of both locally finite root systems and generalized root systems. We classify irreducible locally finite root supersystems.

  14. Local duality for 2-dimensional local ring

    Indian Academy of Sciences (India)

    dimensional complete local ring whose residue field is an n-dimensional local field in the sense of. Kato–Parshin. Our results generalize the Saito works in the case n = 0 and are applied to study the Bloch–Ogus complex for such rings in various cases.

  15. Local food and tourism

    DEFF Research Database (Denmark)

    Boesen, Morten; Sundbo, Donna; Sundbo, Jon

    2017-01-01

    This article investigates the question: Why local food networks succeed or fail in collaborating with local tourism actors to create more tourism based on local food? The article focuses on entrepreneurial local food networks and their collaboration with local tourism actors. Emphasis...... is on the actions and attitude logics of local food networks and tourism actors, and whether their respective logics fit as a factor to explain why or why not development of local food concepts lead to increased local tourism. Six local food networks and their collaboration with local tourism actors are studied...... by using observation supplemented with other qualitative methods. Analysis of these networks reveals that successful collaboration is characterised by the food networks and tourism actors having at least one logic in common. The fitting logics that lead to success are primarily celebrity and civic logics...

  16. Defining local food

    DEFF Research Database (Denmark)

    Eriksen, Safania Normann

    2013-01-01

    Despite evolving local food research, there is no consistent definition of “local food.” Various understandings are utilized, which have resulted in a diverse landscape of meaning. The main purpose of this paper is to examine how researchers within the local food systems literature define local...... food, and how these definitions can be used as a starting point to identify a new taxonomy of local food based on three domains of proximity....

  17. MRI of the fingers in patients with systemic scleroderma. Early results of contrast-enhanced examinations on a dedicated MRI system; MRT der Finger bei systemischer Sklerodermie. Erste Ergebnisse mit kontrastverstaerkten Untersuchungen an einem dedizierten MRT-System

    Energy Technology Data Exchange (ETDEWEB)

    Bonel, H.; Seemann, M.; Reiser, M. [Inst. fuer Radiologische Diagnostik, Klinikum Grosshadern, Univ. Muenchen (Germany); Messer, G.; Walchner, M.; Roecken, M. [Dermatologische Klinik und Poliklinik, Univ. Muenchen (Germany)

    1997-10-01

    Purpose. To estimate disease activity in patients with systemic sclerosis using contrast-enhanced MRI of the skin. Material and Methods. In a pre-study, sequences of a low-field (0.2 T) scanner (Artoscan, Esaote, Genova, Italy) were optimized for detection of intravenous contrast (0.1 mmol/l Gd-DTPA) in six patients with the autoimmune disease systemic scleroderma. Based on the results of the pre-study, 17 patients with scleroderma (7 sclerotic/10 active inflammatory disease) were scanned using gradient-spoiled 3D GRE sequences (FA 90 , TR 100 ms, TE 18 ms), which had been established as most sensitive for intravenous contrast. Contrast enhancement of the skin was determined quantitatively by contrast-to-noise ratios (CNR), comparing post- to pre-contrast and dynamic scans (for 6 min, 1 acquisition/min). Patients in the chronic state with sclerodactylia and active inflammation of the hands were considered separately and compared to a control group (n=10) matched according to age. Results. CNR increase after intravenous contrast was significantly higher in patients with active disease (86{+-}16% increase) than sclerosing disease (29{+-}3%, p<0.05) and the control group (4{+-}2%, p<0.05). The dynamic examination showed a significantly slower decrease after the peak rise in the first minute in patients with active disease (CNR 15.4{+-}0.7 to 14.2{+-}1.4) than in those with chronic disease (14.1{+-}0.5 to 11.3{+-}0.9, p<0.05). (orig.) [Deutsch] Zielsetzung der Arbeit war die Quantifizierung der Entzuendungsreaktion der Haut bei an systemischer Sklerodermie erkrankten Patienten mittels kontrastverstaerkter MRT. Material und Methoden. In einer Vorstudie mit 6 Patienten wurden die Sequenzen eines dedizierten Niederfeldmagnetresonanztomographen (Artoscan, Esaote, Genua, Italien) fuer den Kontrastmittelnachweis (0,1 mmol/l Gd-DTPA i.v.) optimiert. Basierend auf dieser Sequenzoptimierung wurden 17 Patienten mit systemischer Sklerodermie (7 Patienten mit sklerosierender, 10

  18. Local perturbations perturb—exponentially–locally

    International Nuclear Information System (INIS)

    De Roeck, W.; Schütz, M.

    2015-01-01

    We elaborate on the principle that for gapped quantum spin systems with local interaction, “local perturbations [in the Hamiltonian] perturb locally [the groundstate].” This principle was established by Bachmann et al. [Commun. Math. Phys. 309, 835–871 (2012)], relying on the “spectral flow technique” or “quasi-adiabatic continuation” [M. B. Hastings, Phys. Rev. B 69, 104431 (2004)] to obtain locality estimates with sub-exponential decay in the distance to the spatial support of the perturbation. We use ideas of Hamza et al. [J. Math. Phys. 50, 095213 (2009)] to obtain similarly a transformation between gapped eigenvectors and their perturbations that is local with exponential decay. This allows to improve locality bounds on the effect of perturbations on the low lying states in certain gapped models with a unique “bulk ground state” or “topological quantum order.” We also give some estimate on the exponential decay of correlations in models with impurities where some relevant correlations decay faster than one would naively infer from the global gap of the system, as one also expects in disordered systems with a localized groundstate

  19. Isolation and characterization of cDNA encoding the 80-kDa subunit protein of the human autoantigen Ku (p70/p80) recognized by autoantibodies from patients with scleroderma-polymyositis overlap syndrome

    International Nuclear Information System (INIS)

    Mimori, Tsuneyo; Ohosone, Yasuo; Hama, Nobuaki; Suwa, Akira; Akizuki, Masashi; Homma, Mitsuo; Griffith, A.J.; Hardin, J.A.

    1990-01-01

    Anti-Ku (p70/p80) autoantibodies in patients with scleroderma-polymyositis overlap syndrome recognize a 70-kDa/80-kDa protein heterodimer which binds to terminal regions of double-stranded DNA. In the present study, the authors isolated full-length cDNAs that encode the 80-kDa Ku subunit. Initial screening of a human spleen cDNA library with anti-Ku antibodies yielded a cDNA of 1.0 kilobase (kb) (termed K71) encoding a portion of the 80-kDa Ku polypeptide (identification based on immunological criteria). In RNA blots, this cDNA hybridized with two mRNAs of 3.4 and 2.6 kb. In vitro transcription and translation experiments produced an immunoprecipitable polypeptide which comigrated with the 80-kDa Ku subunit. The Ku80-6 cDNA proved to be 3304 nucleotides in length, with an additional poly(A) tail, closely approximating the size of the larger mRNA. It contains a single long open reading frame encoding 732 amino acids. The putative polypeptide has a high content of acidic amino acids and a region with periodic repeat of leucine in every seventh position which may form the leucine zipper structure. In genomic DNA blots, probes derived from the opposite ends of cDNA Ku80-6 hybridized with several nonoverlapping restriction fragments from human leukocyte DNA, indicating that the gene encoding the 80-kDa Ku polypeptide is divided into several exons by intervening sequences

  20. New model. Local financing for local energy

    International Nuclear Information System (INIS)

    Detroy, Florent

    2015-01-01

    While evoking the case of the VMH Energies company in the Poitou-Charentes region, and indicating the difference between France and Germany in terms of wind and photovoltaic energy production potential, of number of existing local companies, and of citizen-based funding, this article shows that renewable energies could put the energy production financing in France into question again, with a more important participation of local communities and of their inhabitants. The author describes how the law on energy transition makes this possible, notably with the strengthening of citizen participation. The author evokes some French local experiments and the case of Germany where this participation is already very much developed

  1. Estudo de associação entre anticorpos anticardiolipinas e fenômenos vasculares periféricos em pacientes com esclerodermia sistêmica Study about the association between anticardiolipin antibodies and peripheral vascular phenomena in patients suffering from systemic scleroderma

    Directory of Open Access Journals (Sweden)

    Ana Paula Torres Liberati

    2010-06-01

    Full Text Available Isquemia é comum em esclerodermia sistêmica e é causada por vasoespasmo e trombose. As autoras analisaram a associação de eventos vasculares periféricos e anticorpos anticardiolipinas (aCl em 54 esclerodérmicos. Em 100% deles existia Raynaud; 59,2% apresentaram cicatrizes estelares; 43,3%, telangiectasias; 14,8%, fenômenos tromboembólicos periféricos. ACl IgG foram positivos em 9,2% dos casos e o IgM, em 7,4%. Fenômenos embólicos periféricos estão associados a aCl IgG (p=0,03, não se encontrando associação com demais manifestações.Ischemia is common in systemic scleroderma and it is caused by vasospasm and thrombosis. In the present study we analyzed the association of peripheral vascular events and anticardiolipin (aCl antibodies in 54 patients suffering from systemic scleroderma. The results showed that 100% of the patients presented Raynaud; 59.2% presented digital micro scars; 43.3%, presented teleangiectasies and 14.8%, presented peripheral thromboembolism. ACl IgG were positive in 9.2% and IgM, in 7.4%. Peripheral tromboembolic phenomena had a positive association with aCl IgG (p=0.03. No other associations were found.

  2. Locally analytic vectors in representations of locally

    CERN Document Server

    Emerton, Matthew J

    2017-01-01

    The goal of this memoir is to provide the foundations for the locally analytic representation theory that is required in three of the author's other papers on this topic. In the course of writing those papers the author found it useful to adopt a particular point of view on locally analytic representation theory: namely, regarding a locally analytic representation as being the inductive limit of its subspaces of analytic vectors (of various "radii of analyticity"). The author uses the analysis of these subspaces as one of the basic tools in his study of such representations. Thus in this memoir he presents a development of locally analytic representation theory built around this point of view. The author has made a deliberate effort to keep the exposition reasonably self-contained and hopes that this will be of some benefit to the reader.

  3. Autoanticorpos em esclerodermia e sua associação ao perfil clínico da doença: estudo em 66 pacientes do sul do Brasil Autoantibodies in scleroderma and their association with the clinical profile of the disease: a study of 66 patients from southern Brazil

    Directory of Open Access Journals (Sweden)

    Thelma Larocca Skare

    2011-12-01

    Full Text Available FUNDAMENTOS: A esclerodermia é uma colagenose relativamente rara, cujo perfil de autoanticorpos está associado a diferentes manifestações clínicas. A prevalência de autoanticorpos na esclerodermia sofre influência racial e genética. OBJETIVO: Estudar a prevalência dos anticorpos anti-Scl-70, anticentrômero e anti-U1-RNP em pacientes com esclerodermia do sul do Brasil e verificar suas associações às manifestações clínicas. MÉTODOS: Estudo retrospectivo de análise de 66 pacientes com esclerodermia para presença de anti-Scl-70, anticentrômero (ACA e anti-U1-RNP e de manifestações clínicas como: Raynaud, cicatrizes estelares, necrose digital, telangiectasias, calcinose, fibrose pulmonar, pleurites, pericardites, miocardiopatias, artralgias e artrites, grau de esclerose da pele, contraturas articulares e atritos de tendão, hipertensão pulmonar, manifestações esofágicas e crise renal. RESULTADOS: A prevalência do anti-Scl-70 foi de 17,8%, a do ACA, de 33,3%, e a do U1 RNP foi de 11,8 %. O anti-Scl-70 estava associado à forma difusa da doença (p=0,015, presença de miocardiopatias (p=0,016 e de cicatrizes estelares (p=0,05; o anticentrômero foi mais comum na forma limitada, embora sem significância estatística e mostrou-se protetor para as miocardiopatias (p=0,005. O anti-U1-RNP foi mais comum nas formas de superposição (p=0,0004. CONCLUSÃO: A prevalência e o perfil de associações clínicas dos autoanticorpos em esclerodermia de pacientes brasileiros assemelham-se aos da literatura mundial.BACKGROUND: Scleroderma is a fairly rare connective tissue disease whose autoantibody profile is associated with different clinical manifestations. The prevalence of autoantibodies in scleroderma is influenced by race and genetics. OBJECTIVE: To study the prevalence of anti-Scl-70, anti-centromere (ACA and anti-U1-RNP antibodies in patients with scleroderma in southern Brazil and verify their association with clinical

  4. Active Neural Localization

    OpenAIRE

    Chaplot, Devendra Singh; Parisotto, Emilio; Salakhutdinov, Ruslan

    2018-01-01

    Localization is the problem of estimating the location of an autonomous agent from an observation and a map of the environment. Traditional methods of localization, which filter the belief based on the observations, are sub-optimal in the number of steps required, as they do not decide the actions taken by the agent. We propose "Active Neural Localizer", a fully differentiable neural network that learns to localize accurately and efficiently. The proposed model incorporates ideas of tradition...

  5. Representative of local committee

    International Nuclear Information System (INIS)

    Barcelo, C.

    2007-01-01

    Carlos Barcelo, spoke about the involvement of local companies in the dismantling project. Local companies realised that they could not undertake the whole project, and informed ENRESA of the tasks in which they would be able to co-operate. On the whole, during the course of the 5-year project the local and regional firms were given more work than they had originally expected. Mr. Barcelo praised the good partnership that evolved between the local firms and the implementer. (author)

  6. Local network assessment

    Science.gov (United States)

    Glen, D. V.

    1985-04-01

    Local networks, related standards activities of the Institute of Electrical and Electronics Engineers the American National Standards Institute and other elements are presented. These elements include: (1) technology choices such as topology, transmission media, and access protocols; (2) descriptions of standards for the 802 local area networks (LAN's); high speed local networks (HSLN's) and military specification local networks; and (3) intra- and internetworking using bridges and gateways with protocols Interconnection (OSI) reference model. The convergence of LAN/PBX technology is also described.

  7. Local quantum thermal susceptibility

    Science.gov (United States)

    de Pasquale, Antonella; Rossini, Davide; Fazio, Rosario; Giovannetti, Vittorio

    2016-09-01

    Thermodynamics relies on the possibility to describe systems composed of a large number of constituents in terms of few macroscopic variables. Its foundations are rooted into the paradigm of statistical mechanics, where thermal properties originate from averaging procedures which smoothen out local details. While undoubtedly successful, elegant and formally correct, this approach carries over an operational problem, namely determining the precision at which such variables are inferred, when technical/practical limitations restrict our capabilities to local probing. Here we introduce the local quantum thermal susceptibility, a quantifier for the best achievable accuracy for temperature estimation via local measurements. Our method relies on basic concepts of quantum estimation theory, providing an operative strategy to address the local thermal response of arbitrary quantum systems at equilibrium. At low temperatures, it highlights the local distinguishability of the ground state from the excited sub-manifolds, thus providing a method to locate quantum phase transitions.

  8. Local quantum thermal susceptibility

    Science.gov (United States)

    De Pasquale, Antonella; Rossini, Davide; Fazio, Rosario; Giovannetti, Vittorio

    2016-01-01

    Thermodynamics relies on the possibility to describe systems composed of a large number of constituents in terms of few macroscopic variables. Its foundations are rooted into the paradigm of statistical mechanics, where thermal properties originate from averaging procedures which smoothen out local details. While undoubtedly successful, elegant and formally correct, this approach carries over an operational problem, namely determining the precision at which such variables are inferred, when technical/practical limitations restrict our capabilities to local probing. Here we introduce the local quantum thermal susceptibility, a quantifier for the best achievable accuracy for temperature estimation via local measurements. Our method relies on basic concepts of quantum estimation theory, providing an operative strategy to address the local thermal response of arbitrary quantum systems at equilibrium. At low temperatures, it highlights the local distinguishability of the ground state from the excited sub-manifolds, thus providing a method to locate quantum phase transitions. PMID:27681458

  9. [Local anaesthesia in ruminants].

    Science.gov (United States)

    Nuss, Karl; Schwarz, Andrea; Ringer, Simone

    2017-06-20

    The use of local anaesthesia in ruminants allows many surgical procedures to be conducted free of pain, efficiently and inexpensively in the field. Local anaesthesia combined with sedation and immobilisation of the animal can replace general anaesthesia for many procedures (e. g. castration, claw amputation). The level of difficulty differs among various local anaesthetic techniques: local infiltration of tissue or anaesthesia of the cornual nerve are easily performed, whereas local anaesthesia of the eye, regional anaesthesia in limbs or anaesthesia for umbilical surgery are more difficult to carry out. This article presents an illustrated overview of the most common local anaesthetic procedures in cattle as well as in small ruminants and serves as a practical guide for veterinarians in the field. In principle, these techniques can likewise be applied in other ruminants or artiodactyls.

  10. Local Worlds of Activation

    DEFF Research Database (Denmark)

    Jacobsson, Kerstin; Hollertz, Katarina; Garsten, Christina

    2017-01-01

    arrangements and the role of private services and actors in service delivery differed significantly too, ranging from strictly market-based forms of governance to classical public administration. The article moreover shows how the different activation approaches were reflected in the radically different usages...... from local politics, established local traditions, patterns of networking and modes of collaborating, as the notion of ‘local words of activation’ intends to capture....

  11. Quantification of local mobilities

    DEFF Research Database (Denmark)

    Zhang, Y. B.

    2018-01-01

    A new method for quantification of mobilities of local recrystallization boundary segments is presented. The quantification is based on microstructures characterized using electron microscopy and on determination of migration velocities and driving forces for local boundary segments. Pure aluminium...... is investigated and the results show that even for a single recrystallization boundary, different boundary segments migrate differently, and the differences can be understood based on variations in mobilities and local deformed microstructures. The present work has important implications for understanding...

  12. Síndrome de Werner associada a quadro esclerodermiforme: relato de caso e revisão da literatura Werner's syndrome associated with scleroderma-like syndrome: case report and literature revision

    Directory of Open Access Journals (Sweden)

    Cristiane Kayser

    2008-04-01

    Full Text Available A síndrome de Werner é uma doença autossômica recessiva rara associada a envelhecimento precoce, cujo quadro cutâneo deve ser distinguido daquele encontrado na esclerose sistêmica (ES. Descrevemos aqui o caso de uma paciente de 39 anos de idade, portadora de síndrome de Werner, encaminhada ao nosso serviço com hipótese diagnóstica inicial de ES. A paciente apresentava várias manifestações associadas à síndrome de Werner, incluindo cabelos precocemente grisalhos, voz estridente, baixa estatura, alterações cutâneas esclerodermiformes, diabetes melito, catarata, hipogonadismo, hipotireoidismo e hiperlipidemia. Não apresentava fenômeno de Raynaud, manifestações viscerais típicas da ES, alterações capilaroscópicas periungueais ou auto-anticorpos. O diagnóstico de síndrome de Werner, apesar de raro, deve ser lembrado no diagnóstico diferencial de ES, principalmente na presença de manifestações atípicas e na ausência de alterações típicas da ES.Werner's syndrome is a rare autosomal recessive disease associated with premature ageing. Skin alteration must be distinguished from cutaneous manifestation of systemic sclerosis (SSc. We describe a case of a 39 years old patient with Werner's syndrome admitted with an initial diagnostic hypothesis of SSc. The patient had many characteristic features associated with Werner's syndrome including gray hair, hoarseness, short stature, scleroderma-like skin changes, diabetes mellitus, cataracts, hypogonadism, hypothyroidism, and hyperlipidemia. There was no Raynaud's phenomenon, other typical visceral manifestation of SSc, nailfold capillary alterations or autoantibodies. Werner's syndrome diagnosis notwithstanding rare, should be remember in the differential diagnosis of SSc, mainly in the presence of atypical manifestations and in the absence of typical features of SSc.

  13. Validation of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument 2.0 in English- and Chinese-speaking patients in a multi-ethnic Singapore systemic sclerosis cohort.

    Science.gov (United States)

    Low, Andrea Hsiu Ling; Xin, Xiaohui; Law, Weng Giap; Teng, Gim Gee; Santosa, Amelia; Lim, Anita; Chan, Grace; Ng, Swee Cheng; Thumboo, Julian

    2017-07-01

    The aim of this study was to (1) translate the Gastrointestinal Tract Instrument (GIT) 2.0 from English to Chinese and (2) validate both versions in a multi-ethnic systemic sclerosis cohort in Singapore (SCORE). The English GIT2.0 was translated to Chinese using a standard forward-backward translation approach. Psychometric evaluation of the GIT2.0 included internal consistency reliability (using Cronbach's alpha), test-retest reliability (using intra-class correlation coefficient (ICC)), scale level factor analysis, and construct validity (using Spearman correlation) against the modified Scleroderma Health Assessment Questionnaire (S-HAQ) and the SF-36 v2. Most of the patients were females (88.6%) and Chinese (78.2%), with mean (SD) age of 51.0 (13.0) years and median disease duration of 4.5 years. We administered English (n = 146) and Chinese (n = 74) GIT2.0. The mean (SD) total GIT score was 0.29 (0.37). There was good internal consistency (Cronbach's alpha >0.70 for all subscales) and good test-retest reliability for the scale and all subscales (ICC 0.71-0.92) except for "diarrhoea" (ICC = 0.54). Our hypothesised a priori construct validity was supported by moderate correlations between the total GIT score and S-HAQ GI subscale (r = 0.446), and the social functioning subscale and SF36v2 role-social domain (r = 0.337), and weak-to-moderate correlation between the emotional subscale and SF-36v2 role-emotional (r = 0.295) and mental health (r = 0.298) domains and mental component summary (r = 0.356). Exploratory factor analysis of the seven subscales yielded a two-factor solution explaining 69.63% of the total variance. This study provides evidence for the reliability and validity of the English and Chinese GIT2.0 to be used in Singapore for research and routine practice.

  14. Local Social Networks

    DEFF Research Database (Denmark)

    Sapuppo, Antonio; Sørensen, Lene Tolstrup

    2011-01-01

    Online social networks have become essential for many users in their daily communication. Through a combination of the online social networks with opportunistic networks, a new concept arises: Local Social Networks. The target of local social networks is to promote social networking benefits...... in physical environment in order to leverage personal affinities in the users' surroundings. The purpose of this paper is to present and discuss the concept of local social networks as a new social communication system. Particularly, the preliminary architecture and the prototype of local social networks...

  15. Local Estuary Programs

    Science.gov (United States)

    This page provides information about Local Individual Estuary Programs including links to their NEP homepages, social media, Comprehensive Conservation and Management Plans, and state of the bay reports.

  16. Local Exhaust Ventilation

    DEFF Research Database (Denmark)

    Madsen, Ulla; Breum, N. O.; Nielsen, Peter V.

    Capture efficiency of a local exhaust system, e.g. a kitchen hood, should include only contaminants being direct captured. In this study basic concepts of local exhaust capture efficiency are given, based on the idea of a control box. A validated numerical model is used for estimation of the capt......Capture efficiency of a local exhaust system, e.g. a kitchen hood, should include only contaminants being direct captured. In this study basic concepts of local exhaust capture efficiency are given, based on the idea of a control box. A validated numerical model is used for estimation...

  17. Locally orderless registration code

    DEFF Research Database (Denmark)

    2012-01-01

    This is code for the TPAMI paper "Locally Orderless Registration". The code requires intel threadding building blocks installed and is provided for 64 bit on mac, linux and windows.......This is code for the TPAMI paper "Locally Orderless Registration". The code requires intel threadding building blocks installed and is provided for 64 bit on mac, linux and windows....

  18. Local anaesthetic toxicity

    African Journals Online (AJOL)

    Local anaesthetic toxicity has been known since the introduction of local anaesthetic drugs into anaesthetic practice more than a hundred ... was the first to think of cocaine as a narcotic. ..... anaesthetics act as Na+ channel-blocking agents, they slow down .... all neurons, leading to global CNS depression, slowing and.

  19. LOCAL ORGANIZING COMMITTEE

    Indian Academy of Sciences (India)

    Prof. B. B. P. Gupta

    INDIAN ACADEMY OF SCIENCES. Bengaluru. 83rd ANNUAL MEETING. 3–5 November 2017, NEHU, Shillong. LOCAL ORGANIZING COMMITTEE. Local Organizing Committee. 1. Prof. S. K. Srivastava. Chairman. Vice-Chancellor, NEHU, Shillong. 2. Prof. B. B. P. Gupta. Organising Secretary. Department of Zoology ...

  20. Transverse Localization of Light

    NARCIS (Netherlands)

    Raedt, Hans De; Lagendijk, Ad; Vries, Pedro de

    1989-01-01

    We study the propagation of light through a semi-infinite medium with transverse disorder (that is, disorder in two directions only). We show that such a system exhibits strong two-dimensional localization by demonstrating that on propagation a beam expands until the transverse localization length

  1. Local instant conservation equations

    International Nuclear Information System (INIS)

    Delaje, Dzh.

    1984-01-01

    Local instant conservation equations for two-phase flow are derived. Derivation of the equation starts from the recording of integral laws of conservation for a fixed reference volume, containing both phases. Transformation of the laws, using the Leibniz rule and Gauss theory permits to obtain the sum of two integrals as to the volume and integral as to the surface. Integrals as to the volume result in local instant differential equations, in particular derivatives for each phase, and integrals as to the surface reflect local instant conditions of a jump on interface surface

  2. Localizing Expression of Ambiguity

    National Research Council Canada - National Science Library

    Bear, John; Hobbs, Sr, Jerry R

    1987-01-01

    In this paper we describe an implemented program for localizing the expression of many types of syntactic ambiguity, in the logical forms of sentences, in a manner convenient for subsequent inferential processing...

  3. RNA Localization in Astrocytes

    DEFF Research Database (Denmark)

    Thomsen, Rune

    2012-01-01

    , regulation of the blood brain barrier and glial scar tissue formation. Despite the involvement in various CNS functions only a limited number of studies have addressed mRNA localization in astrocytes. This PhD project was initially focused on developing and implementing methods that could be used to asses mRNA......Messenger RNA (mRNA) localization is a mechanism by which polarized cells can regulate protein synthesis to specific subcellular compartments in a spatial and temporal manner, and plays a pivotal role in multiple physiological processes from embryonic development to cell differentiation...... localization in astrocyte protrusions, and following look into the subcellular localization pattern of specific mRNA species of both primary astrocytes isolated from cortical hemispheres of newborn mice, and the mouse astrocyte cell line, C8S. The Boyden chamber cell fractionation assay was optimized, in a way...

  4. Nilpotent -local finite groups

    Science.gov (United States)

    Cantarero, José; Scherer, Jérôme; Viruel, Antonio

    2014-10-01

    We provide characterizations of -nilpotency for fusion systems and -local finite groups that are inspired by known result for finite groups. In particular, we generalize criteria by Atiyah, Brunetti, Frobenius, Quillen, Stammbach and Tate.

  5. Stereotype locally convex spaces

    International Nuclear Information System (INIS)

    Akbarov, S S

    2000-01-01

    We give complete proofs of some previously announced results in the theory of stereotype (that is, reflexive in the sense of Pontryagin duality) locally convex spaces. These spaces have important applications in topological algebra and functional analysis

  6. Stereotype locally convex spaces

    Energy Technology Data Exchange (ETDEWEB)

    Akbarov, S S

    2000-08-31

    We give complete proofs of some previously announced results in the theory of stereotype (that is, reflexive in the sense of Pontryagin duality) locally convex spaces. These spaces have important applications in topological algebra and functional analysis.

  7. Stereotype locally convex spaces

    Science.gov (United States)

    Akbarov, S. S.

    2000-08-01

    We give complete proofs of some previously announced results in the theory of stereotype (that is, reflexive in the sense of Pontryagin duality) locally convex spaces. These spaces have important applications in topological algebra and functional analysis.

  8. Focus on Local Reaction.

    Science.gov (United States)

    Howe, Rod

    1999-01-01

    Discusses how the student newspaper "The Lance" (at Westside High School in Omaha, Nebraska) covered the shootings at Columbine High School. Notes that the staff localized the event and brought the student body into the story. (RS)

  9. Local variances in biomonitoring

    International Nuclear Information System (INIS)

    Wolterbeek, H.Th; Verburg, T.G.

    2001-01-01

    The present study was undertaken to explore possibilities to judge survey quality on basis of a limited and restricted number of a-priori observations. Here, quality is defined as the ratio between survey and local variance (signal-to-noise ratio). The results indicate that the presented surveys do not permit such judgement; the discussion also suggests that the 5-fold local sampling strategies do not merit any sound judgement. As it stands, uncertainties in local determinations may largely obscure possibilities to judge survey quality. The results further imply that surveys will benefit from procedures, controls and approaches in sampling and sample handling, to assess both average, variance and the nature of the distribution of elemental concentrations in local sites. This reasoning is compatible with the idea of the site as a basic homogeneous survey unit, which is implicitly and conceptually underlying any survey performed. (author)

  10. EPR and Bell Locality

    OpenAIRE

    Norsen, Travis

    2004-01-01

    A new formulation of the EPR argument is presented, one which uses John Bell's mathematically precise local causality condition in place of the looser locality assumption which was used in the original EPR paper and on which Niels Bohr seems to have based his objection to the EPR argument. The new formulation of EPR bears a striking resemblance to Bell's derivation of his famous inequalities. The relation between these two arguments -- in particular, the role of EPR as part one of Bell's two-...

  11. Local Governance and Corruption

    OpenAIRE

    Marius PROFIROIU; Tudorel ANDREI; Gheorghe POPESCU; Alina PROFIROIU

    2006-01-01

    This paper attempts to examine, from the Romanian perspective, the degree to which decentralization process and improvement of local governance contributes to the reduction of corruption in the short and medium term. Through the methodology that is used the paper is consistent with the international trend that endeavors to analyze the impact of corruption on economic and social processes at the local level. In addition, recent research on corruption issues has focused upon the measurement of ...

  12. Local E11

    International Nuclear Information System (INIS)

    Riccioni, Fabio; West, Peter

    2009-01-01

    We give a method of deriving the field-strengths of all massless and massive maximal supergravity theories in any dimension starting from the Kac-Moody algebra E 11 . Considering the subalgebra of E 11 that acts on the fields in the non-linear realisation as a global symmetry, we show how this is promoted to a gauge symmetry enlarging the algebra by the inclusion of additional generators. We show how this works in eleven dimensions, and we call the resulting enlarged algebra E 11 local . Torus reduction to D dimensions corresponds to taking a subalgebra of E 11 local , called E 11,D local , that encodes the full gauge algebra of the corresponding D-dimensional massless supergravity. We show that each massive maximal supergravity in D dimensions is a non-linear realisation of an algebra E-tilde 11,D local . We show how this works in detail for the case of Scherk-Schwarz reduction of IIB to nine dimensions, and in particular we show how E-tilde 11,9 local arises as a subalgebra of the algebra E 11,10B local associated to the ten-dimensional IIB theory. This subalgebra corresponds to taking a combination of generators which is different to the massless case. We then show that E-tilde 11,D local appears as a deformation of the massless algebra E 11,D local in which the commutation relations between the E 11 and the additional generators are modified. We explicitly illustrate how the deformed algebra is constructed in the case of massive IIA and of gauged five-dimensional supergravity. These results prove the naturalness and power of the method.

  13. Desempleo y justicia local

    Directory of Open Access Journals (Sweden)

    Elster, Jon

    2003-04-01

    Full Text Available In this paper Jon Elster presents a set of criteria of local justice that regulate dismissals. In situations where work is limited and companies adopt means of employment redundancy, the decision of dismissing certain workers —when this one tries not to be arbitrary— can be based on local criteria of fairness like merit, efficiency, age or other analysed in this work. So, after defining the concept of local justice and presenting some examples, the paper focuses on the importance of the work to organize and structure the life of people. Unemployment has serious consequences for the persons who suffer it and the author advises that it is necessary to subordinate dismissals to criteria of local justice.

    En este artículo se presentan una serie de criterios de justicia local para regular los despidos. En situaciones en las que el trabajo es escaso y las empresas adoptan medidas de regulación de empleo, la decisión de despedir a unos trabajadores o a otros se puede basar, cuando se intenta que no sea arbitraria, en criterios locales de equidad como el mérito, la eficacia, la edad u otros que se analizan en este trabajo. Así, tras definir el concepto de justicia local y presentar algunos ejemplos, el artículo se centra en la importancia del trabajo para organizar y estructurar la vida de la gente. Las graves consecuencias que tiene, por ello, el desempleo para quien lo sufre, aconseja que los despidos se sometan a criterios de justicia local.

  14. Oleotourism: Local Actors for Local Tourism Development

    Directory of Open Access Journals (Sweden)

    Marco Tregua

    2018-05-01

    Full Text Available Olive oil consumption has grown substantially in recent years, due in part to the fact that olive oil is healthy. Much of the global olive oil production comes from the rural areas of Jaén in southern Spain. Surrounding this industry, services such as oleotourism are increasing. This paper aims to identify the key elements supporting the development of oleotourism. After a preliminary exploratory analysis of the existing data, a qualitative analysis was performed with actors directly involved in the industry in Jaén. The results helped us define the level of the stakeholders’ involvement and the sustainability, the opportunities, and the constraints affecting oleotourism in this area. The practical implications of this investigation can be useful for governing agencies, local firms, and the tourism industry in support of oleotourism development.

  15. Tackling Health Inequalities Locally

    DEFF Research Database (Denmark)

    Diderichsen, Finn; Scheele, Christian Elling; Little, Ingvild Gundersen

    of this study. It is based on three sources: 1. Interviews with policymakers (administrators and politicians) within healthcare administrations, childhood/education, and labour market administrations from September 2014 to March 2015*. 2. Textual analysis of available policy documents from regions...... of translating small inequalities in wealth into small inequalities in health. Denmark, Norway and Sweden all have legislation that indifferent ways offers local governments key roles in public health. This is partly due to local governments’ responsibility for many policy areas of great relevance to health...... state model, including its health policy, as an area of Nordic collaboration (104). However, realising the principle of health (equity) in all policiesis no simple matter. The national authorities and local government federations in Denmark, Norway and Sweden have therefore initiated various activities...

  16. Local homotopy theory

    CERN Document Server

    Jardine, John F

    2015-01-01

    This monograph on the homotopy theory of topologized diagrams of spaces and spectra gives an expert account of a subject at the foundation of motivic homotopy theory and the theory of topological modular forms in stable homotopy theory. Beginning with an introduction to the homotopy theory of simplicial sets and topos theory, the book covers core topics such as the unstable homotopy theory of simplicial presheaves and sheaves, localized theories, cocycles, descent theory, non-abelian cohomology, stacks, and local stable homotopy theory. A detailed treatment of the formalism of the subject is interwoven with explanations of the motivation, development, and nuances of ideas and results. The coherence of the abstract theory is elucidated through the use of widely applicable tools, such as Barr's theorem on Boolean localization, model structures on the category of simplicial presheaves on a site, and cocycle categories. A wealth of concrete examples convey the vitality and importance of the subject in topology, n...

  17. Localization of relativistic particles

    International Nuclear Information System (INIS)

    Omnes, R.

    1997-01-01

    In order to discuss localization experiments and also to extend the consistent history interpretation of quantum mechanics to relativistic properties, the techniques introduced in a previous paper [J. Math. Phys. 38, 697 (1997)] are applied to the localization of a photon in a given region of space. An essential requirement is to exclude arbitrarily large wavelengths. The method is valid for a particle with any mass and spin. Though there is no proper position operator for a photon, one never needs one in practice. Causality is valid up to exponentially small corrections. copyright 1997 American Institute of Physics

  18. Control of dynamical localization

    International Nuclear Information System (INIS)

    Gong Jiangbin; Woerner, Hans Jakob; Brumer, Paul

    2003-01-01

    Control over the quantum dynamics of chaotic kicked rotor systems is demonstrated. Specifically, control over a number of quantum coherent phenomena is achieved by a simple modification of the kicking field. These include the enhancement of the dynamical localization length, the introduction of classical anomalous diffusion assisted control for systems far from the semiclassical regime, and the observation of a variety of strongly nonexponential line shapes for dynamical localization. The results provide excellent examples of controlled quantum dynamics in a system that is classically chaotic and offer opportunities to explore quantum fluctuations and correlations in quantum chaos

  19. Local versus national

    DEFF Research Database (Denmark)

    Milan, Christian; Nielsen, Mads Pagh; Bojesen, Carsten

    2014-01-01

    of individual energy supply systems based on on-site weather and building conditions, as well as considering the expected energy consumption profile. However, local planning processes are problematic if they do not take regional or national impacts into account. Given the grid connection, the local building...... solution also has an impact on a national scale by exchanging electricity. Therefore it is important to implement respective grid loads into the planning process in order to avoid technology choices, which might counteract grid stability or cost inefficiencies at other sites. The aim of this paper...

  20. Locality and quantum mechanics.

    Science.gov (United States)

    Unruh, W G

    2018-07-13

    It is argued that it is best not to think of quantum mechanics as non-local, but rather that it is non-realistic.This article is part of a discussion meeting issue 'Foundations of quantum mechanics and their impact on contemporary society'. © 2018 The Author(s).

  1. Extent of local participation

    International Nuclear Information System (INIS)

    Albisu, F.

    1977-01-01

    After a brief historical comment on national participation on past nuclear projects, a description is made of the desirable situation to be achieved as regards local content. The reasons, the procedures and the areas for that participation (i.e., the why, how and where) are suggested, as well as the means to promote it. (orig.) [de

  2. Local Area Networks.

    Science.gov (United States)

    Marks, Kenneth E.; Nielsen, Steven

    1991-01-01

    Discusses cabling that is needed in local area networks (LANs). Types of cables that may be selected are described, including twisted pair, coaxial cables (or ethernet), and fiber optics; network topologies, the manner in which the cables are laid out, are considered; and cable installation issues are discussed. (LRW)

  3. Local government in Ethiopia

    African Journals Online (AJOL)

    LAW

    accompanied by the policy and practice of using local authorities for ... labour and revenue in the form of taxation and tribute for the centre. .... Menilik put under his direct administration the regions which resisted his expansionist move.25 This ...

  4. Local control room

    CERN Multimedia

    CERN PhotoLab

    1972-01-01

    Local control room in the ejection building : all electronics pertaining to proton distribution and concomitants such as beam gymnastics and diagnostics at high energies will eventually be gathered here. Shown is the first of two rows of fast ejection electronic racks. It includes only what is necessary for operation.

  5. Test of Einstein locality

    International Nuclear Information System (INIS)

    Roos, Matts

    1980-11-01

    Einstein locality imples that spacelike separated coherent quantum systems do not interfere, and that all interferences must propagate with at most the speed of light. It then follows that the reaction e + e - →K 0 antiK 0 must yield some Ksub(s)Ksuu(s) decays in apparent violation of P and C conservation. (author)

  6. Beyond local climate

    DEFF Research Database (Denmark)

    D'haen, Sarah Ann Lise; Nielsen, Jonas Østergaard; Lambin, Eric F.

    2014-01-01

    At the household level, nonfarm activities are thought to help rural poor households buffer against agricultural risks related to local climate variability by providing them with cash to buy food in the case of harvest shortfalls. Over the recent decades, households in rural Sub-Sahara have been...

  7. Statistics of Local Extremes

    DEFF Research Database (Denmark)

    Larsen, Gunner Chr.; Bierbooms, W.; Hansen, Kurt Schaldemose

    2003-01-01

    . A theoretical expression for the probability density function associated with local extremes of a stochasticprocess is presented. The expression is basically based on the lower four statistical moments and a bandwidth parameter. The theoretical expression is subsequently verified by comparison with simulated...

  8. Local youth policy

    NARCIS (Netherlands)

    R. Gilsing

    1999-01-01

    Original title: Lokaal jeugdbeleid. Local authorities have been given an important role in youth policy in the Netherlands. They are expected to develop preventive youth policy to increase the opportunities of young people and prevent them dropping out from society. At the request of the

  9. Stroke? Localized, otogenic meningitis!

    DEFF Research Database (Denmark)

    Ingolfsdottir, Harpa Maria; Thomasen, Per Caye

    2011-01-01

    We report the case of a patient admitted with aphasia, treated for a stroke. Subsequently, it was revealed that the symptoms were caused by complicated otitis media with localized meningitis. This case draws attention to the possible intracranial spread of infection when neurological symptoms occur...

  10. Evaluation de la rigidité artérielle par la vitesse de progression de l’onde pouls doigt-orteil mesuré par pOpmetre® chez des sujets noirs africains atteints de sclérodermie systémique [Evaluation of arterial stiffness by finger-toe pulse wave velocity measured by pOpmetre® in black africans patients with systemic scleroderma

    Directory of Open Access Journals (Sweden)

    Moussa Diallo

    2017-11-01

    Full Text Available Background: The purpose of this study was to assess the arterial stiffness (AS in black africans patients with systemic scleroderma. Patients and Methodology: A prospective cross-sectional hospital survey has been performed over a 6-month period, including 55 black african individuals, aged over 16 years and consenting, consisting of 29 patients with systemic scleroderma and 26 hospitalized controls. The finger-toe pulse wave velocity (ft-PWV was recorded by popmetre® and comparaison between the 2 groups has been performed with statistical analysis. Results: The mean ft-PWV was 9,56 m/s ± 3,09 in the patient group and 7,71 m/s ± 2,63 in control group. The ft-PWV was significantly higher in patients with scleroderma compared to controls (p<0.0145. The study of the relationship between AS and independent variables in multivariate analysis demonstrated that having scleroderma increase by 1.81 times the value of the ft-PWV after adjustment for age and systolic blood pressure. Discussion: To our knowledge, this is the first assessment of the AS in black african patients with scleroderma. It shows that ft-PWV was significantly higher in patients with scleroderma compared to controls. Also, this high AS was imputable to the scleroderma. Conclusion: In Africa, the measurement of the AS by popmetre® could be a simple, rapid, non invasive and pratical early detection and follow-up of cardio-vascular involvments in the course of scleroderma. RÉSUMÉ Introduction: L’objectif de notre étude était d’évaluer la résistance artérielle (RA chez les patients noirs africains atteints de SS. Patients et Méthode: Une enquête hospitalière prospective transversale a été réalisée sur une période de 6 mois, portant sur 55 sujets noirs africains, âgés de plus de 18 ans, consentants, composé de 29 malades atteints de SS et 26 témoins hospitalisés. La vitesse de progression de l’onde pouls doigt-orteil (VOPdo a été mesuré par Popmètre dans

  11. Serum homocystein level in patients with scleroderma.

    Science.gov (United States)

    Nazarinia, Mohammadali; Shams, Mesbah; Kamali Sarvestani, Eskandar; Shenavande, Saeede; Khademalhosseini, Maryam; Khademalhosseini, Zeinab

    2013-01-01

    Systemic Sclerosis (SSc) is a systemic connective tissue disease. In this study, we compared the serum Homocystein (Hcy) level between patients with SSc and normal control group. The current study was conducted to determine whether serum Hcy levels are elevated in SSc patients and whether there is any correlation between Hcy levels and RP, Gastro intestinal and lung involvement. Forty one patients who fulfilled the diagnostic criteria for SSc (39 females and 5 males) and Forty four community-based healthy individuals (sex and age matched) were enrolled in to the study. Serum Hcy, vitamin B12, and folate levels were determined. Thirty three patients (70.45%) had GI involvement, twenty two patients (50%) had lung involvement and twenty seven patients (61.36%) had Raynaud's phenomena. Mean serum Hcy level in control group was 22.78 ± 6.018 μmol/L and in case group was 19.43 ± 7.205 μmol/L, shows that the serum Hcy level in control group was significantly higher than patients (P = 0.020). Serum Hcy level is significantly lower in SSc patients than in control group. There is no statistically significant correlation between serum Hcy level and organ involvements.

  12. Current Treatments Available for Scleroderma Patients

    Science.gov (United States)

    ... constipation. Be aware of the increased risk of fractures of the hip, wrist and spine seen in ... agonist targeting the prostacyclin pathway in PAH. It lowers blood pressure in ... jaw pain, limb pain, myalgia, nausea, vomiting, and flushing. Other side ...

  13. Mycobacterium intracellulare Infection Mimicking Progression of Scleroderma

    DEFF Research Database (Denmark)

    Krabbe, Simon; Engelhart, Merete; Thybo, Sören

    2017-01-01

    nail fold capillaries, interstitial lung disease, Raynaud's phenomenon, esophageal dysmotility, and positivity for rheumatoid factor and anti-SSA antibodies. She developed massive inflammatory changes of the cutis, the subcutis, and the muscle fasciae of the right leg, that after several failed...

  14. Local Governance and Corruption

    Directory of Open Access Journals (Sweden)

    Marius PROFIROIU

    2006-06-01

    Full Text Available This paper attempts to examine, from the Romanian perspective, the degree to which decentralization process and improvement of local governance contributes to the reduction of corruption in the short and medium term. Through the methodology that is used the paper is consistent with the international trend that endeavors to analyze the impact of corruption on economic and social processes at the local level. In addition, recent research on corruption issues has focused upon the measurement of the level of corruption level and on its impact on the growing rate of the GDP (Mauro [1995]1, Abed and Davoodi2, Krueger [1974]3, on the impact upon some of the national economical sectors (Tanzi [1998]4, Shang-Jin Wei [2001]5, or on the decentralization processes (Shah [2006]6.

  15. Cerebral localization in antiquity.

    Science.gov (United States)

    Rose, F Clifford

    2009-07-01

    Fragments of neurology can be found in the oldest medical writings in antiquity. Recognizable cerebral localization is seen in Egyptian medical papyri. Most notably, the Edwin Smith papyrus describes hemiplegia after a head injury. Similar echoes can be seen in Homer, the Bible, and the pre-Hippocratic writer Alcmaeon of Croton. While Biblical writers thought that the heart was the seat of the soul, Hippocratic writers located it in the head. Alexandrian anatomists described the nerves, and Galen developed the ventricular theory of cognition whereby mental functions are classified and localized in one of the cerebral ventricles. Medieval scholars, including the early Church Fathers, modified Galenic ventricular theory so as to make it a dynamic model of cognition. Physicians in antiquity subdivided the brain into separate areas and attributed to them different functions, a phenomenon that connects them with modern neurologists.

  16. Are quasars local

    International Nuclear Information System (INIS)

    Terrell, J.

    1974-01-01

    The problems of interpreting quasars as galaxies, at distances of billions of light-years, seem to be increasing with time and with observational knowledge. The incredibly large energy and brightness requirements, the very small size and thus high surface brightness required by their rapid fluctuations in luminosity, the recently-discovered radio-source separation speeds apparently much greater than the speed of light, their general lack of association with distant galaxies, and many other properties are all very difficult to explain on the basis of cosmological distance. The very local quasar model, involving much less massive and bright objects--perhaps similar to Type O stars--emitted at relativistic speeds by the center of our own galaxy, greatly eases these difficulties. Since such ejected objects also seem necessary to explain the similarly strange properties of radio galaxies, the emission of local quasars from some galaxies might be deduced on this basis alone. (6 figures) (U.S.)

  17. Locality of Temperature

    Science.gov (United States)

    Kliesch, M.; Gogolin, C.; Kastoryano, M. J.; Riera, A.; Eisert, J.

    2014-07-01

    This work is concerned with thermal quantum states of Hamiltonians on spin- and fermionic-lattice systems with short-range interactions. We provide results leading to a local definition of temperature, thereby extending the notion of "intensivity of temperature" to interacting quantum models. More precisely, we derive a perturbation formula for thermal states. The influence of the perturbation is exactly given in terms of a generalized covariance. For this covariance, we prove exponential clustering of correlations above a universal critical temperature that upper bounds physical critical temperatures such as the Curie temperature. As a corollary, we obtain that above the critical temperature, thermal states are stable against distant Hamiltonian perturbations. Moreover, our results imply that above the critical temperature, local expectation values can be approximated efficiently in the error and the system size.

  18. Brane-Localized Gravity

    CERN Document Server

    Mannheim, Philip D

    2005-01-01

    This timely and valuable book provides a detailed pedagogical introduction and treatment of the brane-localized gravity program of Randall and Sundrum, in which gravitational signals are able to localize around our four-dimensional world in the event that it is a brane embedded in an infinitely-sized, higher dimensional anti-de Sitter bulk space. A completely self-contained development of the material needed for brane-world studies is provided for both students and workers in the field, with a significant amount of the material being previously unpublished. Particular attention is given to issues not ordinarily treated in the brane-world literature, such as the completeness of tensor gravitational fluctuation modes, the causality of brane-world propagators, and the status of the massless graviton fluctuation mode in brane worlds in which it is not normalizable.

  19. Local area networking handbook

    OpenAIRE

    O'Hara, Patricia A.

    1990-01-01

    Approved for public release; distribution is unlimited. This thesis provides Navy shore based commands with sufficient information on local area networking to (1) decide if they need a LAN, (2) determine what their networking requirements are, and (3) select a LAN that satisfies their requirements. LAN topologies, transmission media, and medium access methods are described. In addition, the OSI reference model for computer networking and the IEEE 802 LAN standards are explained in detail. ...

  20. Rationalising localism and brokerage

    DEFF Research Database (Denmark)

    Kusche, Isabel

    2017-01-01

    . The article employs the notion of global scripts and the concept of domestication of global trends in order to analyse how the question of the appropriate relationship between deputies and voters is informed both by a general trend towards the professionalisation of politicians and a domestic tradition...... of the domestic setting on the other. In this way the article contributes to a better understanding of the entanglement of the global and the local....

  1. Local dependency in networks

    Directory of Open Access Journals (Sweden)

    Kudĕlka Miloš

    2015-06-01

    Full Text Available Many real world data and processes have a network structure and can usefully be represented as graphs. Network analysis focuses on the relations among the nodes exploring the properties of each network. We introduce a method for measuring the strength of the relationship between two nodes of a network and for their ranking. This method is applicable to all kinds of networks, including directed and weighted networks. The approach extracts dependency relations among the network’s nodes from the structure in local surroundings of individual nodes. For the tasks we deal with in this article, the key technical parameter is locality. Since only the surroundings of the examined nodes are used in computations, there is no need to analyze the entire network. This allows the application of our approach in the area of large-scale networks. We present several experiments using small networks as well as large-scale artificial and real world networks. The results of the experiments show high effectiveness due to the locality of our approach and also high quality node ranking comparable to PageRank.

  2. Local orbit feedback

    International Nuclear Information System (INIS)

    Anon.

    1991-01-01

    Critically aligned experiments are sensitive to small changes in the electron beam orbit. At the NSLS storage rings, the electron beam and photon beam motions have been monitored over the past several years. In the survey conducted in 1986 by the NSLS Users Executive Committee, experimenters requested the vertical beam position variation and the vertical angle variation, within a given fill, remain within 10 μm and 10 μr, respectively. This requires improvement in the beam stability by about one order of magnitude. At the NSLS and SSRL storage rings, the beam that is originally centered on the position monitor by a dc orbit correction is observed to have two kinds of motion: a dc drift over a storage period of several hours and a beam bounce about its nominal position. These motions are a result of the equilibrium orbit not being held perfectly stable due to time-varying errors introduced into the magnetic guide field by power supplies, mechanical vibration of the magnets, cooling water temperature variations, etc. The approach to orbit stabilization includes (1) identifying and suppressing as many noise sources on the machine as possible, (2) correcting the beam position globally (see Section 6) by controlling a number of correctors around the circumference of the machine, and (3) correcting the beam position and angle at a given source location by position feedback using local detectors and local orbit bumps. The third approach, called Local Orbit Feedback will be discussed in this section

  3. Local variances in biomonitoring

    International Nuclear Information System (INIS)

    Wolterbeek, H.T.

    1999-01-01

    The present study deals with the (larger-scaled) biomonitoring survey and specifically focuses on the sampling site. In most surveys, the sampling site is simply selected or defined as a spot of (geographical) dimensions which is small relative to the dimensions of the total survey area. Implicitly it is assumed that the sampling site is essentially homogeneous with respect to the investigated variation in survey parameters. As such, the sampling site is mostly regarded as 'the basic unit' of the survey. As a logical consequence, the local (sampling site) variance should also be seen as a basic and important characteristic of the survey. During the study, work is carried out to gain more knowledge of the local variance. Multiple sampling is carried out at a specific site (tree bark, mosses, soils), multi-elemental analyses are carried out by NAA, and local variances are investigated by conventional statistics, factor analytical techniques, and bootstrapping. Consequences of the outcomes are discussed in the context of sampling, sample handling and survey quality. (author)

  4. Late onset ‘en coup de sabre’ following trauma: Rare presentation of a rare disease

    OpenAIRE

    Tasleem Arif; Imran Majid; Mir Laieq Ishtiyaq Haji

    2015-01-01

    En coup de sabre (linear scleroderma of face) is a rare type of morphea (localized scleroderma) involving frontoparietal area of the forehead and scalp. Many triggering factors have been implicated in the development of morphea like trauma, immobilization, bacille Calmette–Guérin (BCG) vaccination, injections of vitamin K, mechanical compression from clothing, etc. Linear scleroderma primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age....

  5. New Communication Technologies, Local Journalism and the Perception of Locality

    Directory of Open Access Journals (Sweden)

    Ali ÖZCAN

    2016-12-01

    Full Text Available Studies on local journalism as an important element of participatory democracy generally focus on the history of the local press, the financial insufficiencies of local newspapers as well as technical/ technological incompetency that occurs as a result of these factors. This research analyzes local newspapers with a new scientific point of view by focusing on the perception of locality as a mental projection of geographical constraint. Perspectives regarding the transformations on perceptions of locality as a result of the disappearance of time and space constraints due to new communication technologies, and the opportunities that new communication environments offer to local newspapers will be provided. Moreover, the ways in which local newspapers reflect on locality with their printed versions under time and space constraints will be analyzed. The research scope of the study has been limited to 15 newspapers in Konya. Structured interviews and content analysis methods were used for data collection and analysis.

  6. Particle localization detector

    International Nuclear Information System (INIS)

    Allemand, R.

    1976-01-01

    A proportional detector for the localization of particles comprises a leak-tight chamber filled with fluid and fitted with an electrode of a first type consisting of one or more conducting wires and with an electrode of a second type consisting of one or more conducting plates having the shape of a portion of cylindrical surface and a contour which provides a one-to-one correspondence between the position of a point of the wires and the solid angle which subtends the plate at that point, means being provided for collecting the electrical signal which appears on the plates. 12 Claims, 10 Drawing Figures

  7. 'Think globally, act locally'

    DEFF Research Database (Denmark)

    Hoff, Jens Villiam

    2015-01-01

    This chapter argues that citizen participation in environmental and climate policies has moved through four phases from the early 1960s until today. The most recent phase is characterised by a ‘reorientation towards the local’, a move which is increasingly supported by actors at both national...... and international levels. For local governments, the task of initiating or facilitating citizen action concerning climate change mitigation has generated the development of new policy instruments that increasingly target people’s everyday lives, homes and workplaces. Examples of the use of these new policy...

  8. Localization of CANDU technology

    Energy Technology Data Exchange (ETDEWEB)

    Alizadeh, Ala

    2010-09-15

    The CANDU pressurized heavy water reactor's principal design features suit it particularly well for technology transfer and localization. When the first commercial CANDU reactors of 540 MWe entered service in 1971, Canada's population of less than 24 million supported a 'medium' level of industrial development, lacking the heavy industrial capabilities of larger countries like the USA, Japan and Europe. A key motivation for Canada in developing the CANDU design was to ensure that Canada would have the autonomous capacity to build and operate nuclear power reactors without depending on foreign sources for key components or enriched fuel.

  9. Localized Acoustic Surface Modes

    KAUST Repository

    Farhat, Mohamed

    2015-08-04

    We introduce the concept of localized acoustic surface modes (ASMs). We demonstrate that they are induced on a two-dimensional cylindrical rigid surface with subwavelength corrugations under excitation by an incident acoustic plane wave. Our results show that the corrugated rigid surface is acoustically equivalent to a cylindrical scatterer with uniform mass density that can be represented using a Drude-like model. This, indeed, suggests that plasmonic-like acoustic materials can be engineered with potential applications in various areas including sensing, imaging, and cloaking.

  10. Local analytic geometry

    CERN Document Server

    Abhyankar, Shreeram Shankar

    1964-01-01

    This book provides, for use in a graduate course or for self-study by graduate students, a well-motivated treatment of several topics, especially the following: (1) algebraic treatment of several complex variables; (2) geometric approach to algebraic geometry via analytic sets; (3) survey of local algebra; (4) survey of sheaf theory. The book has been written in the spirit of Weierstrass. Power series play the dominant role. The treatment, being algebraic, is not restricted to complex numbers, but remains valid over any complete-valued field. This makes it applicable to situations arising from

  11. Pattern Driven Stress Localization

    Science.gov (United States)

    Croll, Andrew; Crosby, Alfred

    2010-03-01

    The self-assembly of patterns from isotropic initial states is a major driver of modern soft-matter research. This avenue of study is directed by the desire to understand the complex physics of the varied structures found in Nature, and by technological interest in functional materials that may be derived through biomimicry. In this work we show how a simple striped phase can respond with significant complexity to an appropriately chosen perturbation. In particular, we show how a buckled elastic plate transitions into a state of stress localization using a simple, self-assembled variation in surface topography. The collection of topographic boundaries act in concert to change the state from isotropic sinusoidal wrinkles, to sharp folds or creases separated by relatively flat regions. By varying the size of the imposed topographic pattern or the wavelength of the wrinkles, we construct a state diagram of the system. The localized state has implications for both biological systems, and for the control of non-linear pattern formation.

  12. Locality and reality

    International Nuclear Information System (INIS)

    Stapp, H.P.

    1980-01-01

    Einstein's principle that no signal travels faster than light suggests that observations in one spacetime region should not depend on whether or not a radioactive decay is detected in a spacelike-separated region. This locality property is incompatible with the predictions of quantum theory, and this incompatibility holds independently of the questions of realism, objective reality, and hidden variables. It holds both in the pragmatic quantum theory of Bohr and in realistic frameworks. It is shown here to hold in a completed realistic quantum theory that reconciles Einstein's demand for a description of reality itself with Bohr's contention that quantum theory is complete. This completed realistic quantum theory has no hidden variables, and no objective reality in which observable attributes can become definite independently of observers. The theory is described in some detail, with particular attention to those aspects related to the question of locality. This completed realistic quantum theory is in principle more comprehensive than Bohn's pragmatic quantum theory because it is not limited in principle by the requirement that the observed system be physically separated from the observing one. Applications are discussed

  13. Act local, think global

    International Nuclear Information System (INIS)

    Lewis, Chris; McRoberts, Doug

    2002-01-01

    Tip O'Neill, one of the grand old men of modem US politics, once famously remarked that all politics is local. Like most politicians who succeed on the national stage - and not just in the US - it was a truth he never lost sight of. What is true for politicians is equally true in the communications business. We may increasingly live in a global village, but successful companies - even multi-nationals - forget the importance of local and regional public relations at their peril. Think of Douglas Ivester, the CEO of Coca-Cola at the time of the 1999 Belgian contamination scandal, who allegedly reacted to first reports of the crisis by asking: 'Where the hell is Belgium?' A more appropriate question today - several years after Coke's share price toppled and the CEO was unceremoniously sacked - might be: 'Who the hell is Douglas Nester?' But - to adapt another famous phrase - the fact that communications (and marketing) professionals still need to 'act local' as much as ever before should not blind us to the growing need to 'think global'. In the nuclear industry, as in the world economy generally, increasing global integration is a reality, as are the international nature of the news media and the increasingly global nature of the anti-nuclear pressure groups. Indeed, it was the growing need for a truly global information network to counter these trends, by increasing the overall speed and accuracy of the worldwide nuclear information flow, that led the nuclear community to establish NucNet in 1991. So where exactly is the line between local and regional nuclear communications on the one hand, and global communications on the other? Is there one spin for a regional audience, and another for a global audience? This presentation proposes some guiding principles, by examining the response of nuclear communicators world-wide to the new communications agenda imposed in the wake of the September 11th suicide attacks in the US. NucNet President Doug McRoberts and Executive

  14. A digital local studies collection

    Directory of Open Access Journals (Sweden)

    Simona Resman

    2005-01-01

    Full Text Available Local studies and local studies departments reflect the entire spectrum of knowledge within the local community. Transition to the new digital environment means just continuing the basic functions of local studies collections: acquisition, cataloguing and preservation of materials in different formats on different media. The background of each digital library is the technical architecture of system that enables interaction between the user and the library, saving digital material and building a platform for searching and indexing digital objects. Using advanced ICT requests from librarians, designers of digital local studies collections a reflection about traditional roles. A digital local studies collection with new technology enlarges local boarders, local contents become more and more interesting for a wider sphere of people. In collecting of local materials a collaboration with archives and museums, with academic community, with community groups and with individuals comes in forefront. Digital local studies collections with their contents support local diversity, lifelong learning and social inclusion. The crucial elements of a digital local studies collection are attractive local contents with fast and simple access from one place – a portal. In the digital age public libraries become managers of knowledge also by establishing digital local studies collections.

  15. Local Agenda 21 - from global idea to local action

    DEFF Research Database (Denmark)

    Hoffmann, Birgitte

    1999-01-01

    The article give a status of the Danish works with Local Agenda 21 and discusses Local Agenda 21 as a planning tool. It describes the idea of Local Agenda 21 as a large meeting, which everybody attends. This picture is elaborated and discussed form different angles: the items on the agenda...

  16. Natural resources management by local associations in Ifedore local ...

    African Journals Online (AJOL)

    Management of natural resource by local associations not only in its socio ecological but also in its socio economic context will go a long way in reducing environmental degradation in some local communities. This study examined the operational capacity for natural resource management by local associations in Ifedore ...

  17. Local control stations

    International Nuclear Information System (INIS)

    Brown, W.S.; Higgins, J.C.; Wachtel, J.A.

    1993-01-01

    This paper describes research concerning the effects of human engineering design at local control stations (i.e., operator interfaces located outside the control room) on human performance and plant safety. The research considered both multifunction panels (e.g. remote shutdown panels) as well as single-function interfaces (e.g., valves, breakers, gauges, etc.). Changes in performance shaping factors associated with variations in human engineering at LCSs were estimated based on expert opinion. By means of a scaling procedure, these estimates were used to modify the human error probabilities in a PRA model, which was then employed to generate estimates of plant risk and scoping-level value/impact ratios for various human engineering upgrades. Recent documentation of human engineering deficiencies at single-function LCSs was also reviewed, and an assessment of the current status of LCSs with respect to human engineering was conducted

  18. Local Democracy in Myanmar

    DEFF Research Database (Denmark)

    Kyed, Helene Maria; Harrisson, Annika Pohl; McCarthy, Gerard

    Myanmar is undergoing a comprehensive political transition. In April this year the first democratically elected government in six decades came into power under the leadership of NLD, the pro-democracy party headed by Aung San Suu Kyi. The largest peace conference in the country’s history was held...... in early September, and Myanmar again made the international headlines a week later when Kofi Annan visited the country to head a commission for addressing communal violence. These national events are crucial to the democratization process in Myanmar and for ending the almost seven decades of civil war....... However, often overlooked in this larger picture of transition is the state of local democracy, including village level governance and everyday state-citizen engagements. Political changes at this level are equally crucial for the wider democratization process. This roundtable summary discusses the 2016...

  19. Localized folicular lymphomas

    International Nuclear Information System (INIS)

    Soubeyran, P.; Eghbali, H.; Bonichon, F.; Coindre, J.M.; Richaud, P.; Hoerni, B.

    1988-01-01

    From 1966 to 1985, 103 patients with a localized follicular lymphoma were treated at the Fondation Bergonie. Clinical staging was performed using, after physical examination, chest X-rays, bipedal lymphangiography and unilateral bone marrow biopsy (BMB). The patients were then treated by radiotherapy with or without chemotherapy. Overall survival (OS) at 5 and 10 years is 69 and 56.3%, respectively. Relapse-free survival (RFS) is 53.7 and 49%. Unifactorial analysis shows three prognostic parameters to be independently significant in terms of OS: age, stage and B symptons. In terms of RFS, only 2 factors are significant: age and B symptons. Multivariate analysis (Cox model) shows that age is a more important prognostic factor than stage. 40 refs.; 3 figs.; 3 tabs

  20. Changing local land systems

    DEFF Research Database (Denmark)

    Friis, Cecilie; Reenberg, Anette; Heinimann, Andreas

    2016-01-01

    . Combining the conceptual lenses of land systems and livelihood approaches, this paper demonstrates how the land use system has changed substantially because of the establishment of the rubber plantation by the company, notably in the linkages between livestock rearing, upland shifting cultivation......This paper investigates the direct and cascading land system consequences of a Chinese company's land acquisition for rubber cultivation in northern Laos. Transnational land acquisitions are increasingly acknowledged as an important driver of direct land use conversion with implications for local...... land-based livelihoods. The paper presents an empirical case study of the village of Na Nhang Neua in Nambak District, Luang Prabang Province, using a mixed methods approach to investigate the positive and negative implications for household agricultural strategies, income generation and food security...

  1. The Local Fractional Bootstrap

    DEFF Research Database (Denmark)

    Bennedsen, Mikkel; Hounyo, Ulrich; Lunde, Asger

    We introduce a bootstrap procedure for high-frequency statistics of Brownian semistationary processes. More specifically, we focus on a hypothesis test on the roughness of sample paths of Brownian semistationary processes, which uses an estimator based on a ratio of realized power variations. Our...... new resampling method, the local fractional bootstrap, relies on simulating an auxiliary fractional Brownian motion that mimics the fine properties of high frequency differences of the Brownian semistationary process under the null hypothesis. We prove the first order validity of the bootstrap method...... and in simulations we observe that the bootstrap-based hypothesis test provides considerable finite-sample improvements over an existing test that is based on a central limit theorem. This is important when studying the roughness properties of time series data; we illustrate this by applying the bootstrap method...

  2. Document Management in Local Government.

    Science.gov (United States)

    Williams, Bernard J. S.

    1998-01-01

    The latest in electronic document management in British local government is discussed. Finance, revenues, and benefits systems of leading vendors to local authorities are highlighted. A planning decisions archive management system and other information services are discussed. (AEF)

  3. Inference for local autocorrelations in locally stationary models.

    Science.gov (United States)

    Zhao, Zhibiao

    2015-04-01

    For non-stationary processes, the time-varying correlation structure provides useful insights into the underlying model dynamics. We study estimation and inferences for local autocorrelation process in locally stationary time series. Our constructed simultaneous confidence band can be used to address important hypothesis testing problems, such as whether the local autocorrelation process is indeed time-varying and whether the local autocorrelation is zero. In particular, our result provides an important generalization of the R function acf() to locally stationary Gaussian processes. Simulation studies and two empirical applications are developed. For the global temperature series, we find that the local autocorrelations are time-varying and have a "V" shape during 1910-1960. For the S&P 500 index, we conclude that the returns satisfy the efficient-market hypothesis whereas the magnitudes of returns show significant local autocorrelations.

  4. Local democracy in large municipalities

    DEFF Research Database (Denmark)

    Thuesen, Annette Aagaard

    2017-01-01

    Municipal amalgamations in Denmark in 2007 led to concern for local rural democracy, as the number of politicians from rural areas dropped after the reform. To preserve rural democracy, local councils at the village level were established in some municipalities, and they have begun to prepare local...

  5. String-localized quantum fields

    International Nuclear Information System (INIS)

    Mund, Jens; Santos, Jose Amancio dos; Silva, Cristhiano Duarte; Oliveira, Erichardson de

    2009-01-01

    Full text. The principles of physics admit (unobservable) quantum fields which are localized not on points, but on strings in the sense of Mandelstam: a string emanates from a point in Minkowski space and extends to infinity in some space-like direction. This type of localization might permit the construction of new models, for various reasons: (a) in general, weaker localization implies better UV behaviour. Therefore, the class of renormalizable interactions in the string-localized has a chance to be larger than in the point-localized case; (b) for certain particle types, there are no point-localized (free) quantum fields - for example Anyons in d = 2 + 1, and Wigner's massless 'infinite spin' particles. For the latter, free string-localized quantum fields have been constructed; (c) in contrast to the point-localized case, string-localization admits covariant vector/tensor potentials for fotons and gravitons in a Hilbert space representation with positive energy. We shall present free string-localized quantum fields for various particle types, and some ideas about the perturbative construction of interacting string-localized fields. A central point will be an analogue of gauge theories, completely within a Hilbert space and without ghosts, trading gauge dependence with dependence on the direction of the localization string. In order to discuss renormalizability (item (a)), methods from microlocal analysis (wave front set and scaling degree) are needed. (author)

  6. [Local treatment of liver tumors

    DEFF Research Database (Denmark)

    Pless, T.K.; Skjoldbye, Bjørn Ole

    2008-01-01

    Local treatment of non-resectable liver tumors is common. This brief review describes the local treatment techniques used in Denmark. The techniques are evaluated according to the evidence in literature. The primary local treatment is Radiofrequency Ablation of both primary liver tumors and liver...

  7. Local strategies for decontamination

    Energy Technology Data Exchange (ETDEWEB)

    Hubert, P [Institut de Protection et de Surete Nucleaire, Fontenay-aux-Roses cedex (France); Ramzaev, V [Branch of Institute of Radiation Hygiene, Novozybkov, Bryansk region (Russian Federation); Antsypov, G [Chernnobyl State Committee of the Republic of Belarus, Minsk (Belarus); Sobotovich, E [Institute of Geochemistry, Mineralogy and Ore formation, Kiev (Ukraine); Anisimova, L [EMERCOM, Moscow (Russian Federation)

    1996-07-01

    The efficiencies of a great number of techniques for decontamination or dose reduction in contaminated areas have been investigated by several teams of E.C. and CIS scientists (ECP4 project). Modelling, laboratory and field experiments, t and return from experience allowed to assess radiological efficiencies (e.g. 'decontamination factor') and requirements for the operation of numerous practical solutions. Then, those data were supplemented with data on cost and waste generation in order to elaborate all the information for the optimization of decontamination strategies. Results will be presented for about 70 techniques. However, a technique cannot be compared to another from a generic point of view. Rather it is designed for a specific target and the best technology depends on the objectives. It has been decided to implement decision analyses on case studies, and the local conditions and objectives have been investigated. Individual doses ranged from 1 to 5 mSv, with contrasted contributions of internal and external doses. The desire to restore a normal activity in a partially depopulated settlement, and concerns about the recent increase in internal doses were typical incentives for action. The decision aiding analysis illustrated that actions can be usually recommended. Results are outlined here.

  8. Local supervision of solariums

    International Nuclear Information System (INIS)

    2004-01-01

    In Norway, new regulations on radiation protection and application of radiation came into force on the first of January 2004. Local authorities may now perform the supervision of solariums. There are over 500 solar studios in Norway, with over 5000 solariums accessible to the public. An unknown number of solariums are in private homes, on workplaces, and in hotels and fitness studios. Norway currently has the highest frequency of skin cancer in Europe. The frequency of mole cancer has increased sixfold during the last 30 years, and 200 people die each year of this type of cancer. The Nordic cancer registers estimate that 95 per cent of the skin cancer incidences would have been avoided by limiting sunbathing. It is unknown how many cases are due to the use of solariums. But several studies indicate increased risk of mole cancer caused by solariums. It was found in previous inspection of 130 solariums that only 30 per cent had correct tubes and lamps. Only one solarium satisfied all the requirements of the regulations. But this has since improved. With the new regulations, all solarium businesses offering cosmetic solariums for sale, renting out or use have an obligation to submit reports to the Radiation Protection Authority

  9. Local strategies for decontamination

    International Nuclear Information System (INIS)

    Hubert, P.; Ramzaev, V.; Antsypov, G.; Sobotovich, E.; Anisimova, L.

    1996-01-01

    The efficiencies of a great number of techniques for decontamination or dose reduction in contaminated areas have been investigated by several teams of E.C. and CIS scientists (ECP4 project). Modelling, laboratory and field experiments, t and return from experience allowed to assess radiological efficiencies (e.g. 'decontamination factor') and requirements for the operation of numerous practical solutions. Then, those data were supplemented with data on cost and waste generation in order to elaborate all the information for the optimization of decontamination strategies. Results will be presented for about 70 techniques. However, a technique cannot be compared to another from a generic point of view. Rather it is designed for a specific target and the best technology depends on the objectives. It has been decided to implement decision analyses on case studies, and the local conditions and objectives have been investigated. Individual doses ranged from 1 to 5 mSv, with contrasted contributions of internal and external doses. The desire to restore a normal activity in a partially depopulated settlement, and concerns about the recent increase in internal doses were typical incentives for action. The decision aiding analysis illustrated that actions can be usually recommended. Results are outlined here

  10. On the local equilibrium condition

    International Nuclear Information System (INIS)

    Hessling, H.

    1994-11-01

    A physical system is in local equilibrium if it cannot be distinguished from a global equilibrium by ''infinitesimally localized measurements''. This should be a natural characterization of local equilibrium, but the problem is to give a precise meaning to the qualitative phrase ''infinitesimally localized measurements''. A solution is suggested in form of a Local Equilibrium Condition (LEC), which can be applied to linear relativistic quantum field theories but not directly to selfinteracting quantum fields. The concept of local temperature resulting from LEC is compared to an old approach to local temperature based on the principle of maximal entropy. It is shown that the principle of maximal entropy does not always lead to physical states if it is applied to relativistic quantum field theories. (orig.)

  11. Localization of primary aldosteronism

    International Nuclear Information System (INIS)

    Pagny, J.Y.; Chatellier, G.; Raynaud, A.; Plouin, P.F.; Corvol, P.

    1988-01-01

    After diagnosis of primary aldosteronism on the basis of biochemical evidence, the detection of the tumour is of crucial importance in the management of the disease. The efficacy of CT-Scan, Iodo-Cholesterol Scintigraphy, digitalized phlebography, adrenal vein sampling for steroid measurements (AVS), and Nuclear Magnetic Resonance (NMR) in 160 hypertensive patients with primary aldosteronism was reviewed. Diagnosis of Conn's adenoma (n=96) or Adrenal Hyperplasia (n=40) was confirmed by surgery or at least two concordant tumour localization tests. Scintigraphy gave a correct diagnosis in 53% of the 51 exams, CT-Scan in 82% of the 85 exams, and phlebography in 79% of 61 exams. Plasma Aldosterone/ Cortisol ratio was 5 times higher on the side of adenoma in 55% of the 47 cases but this ratio was also present in 23% of 22 patients with adrenal hyperplasia. Each procedure exhibited few false positive and false negative cases. NMR performed in 15 patients with Conn's adenoma identified all the cases. But tumours displayed a signal close to the liver signal and identical to the normal adrenal. These results and the risk of invasive procedure (failure of catheterization of the right adrenal vein (n=6) and adrenal haematoma (n=2) lead to propose a schema of exploration of patients with primary aldosteronism. The CT-Scan could be performed at the first step once the biological diagnosis confirmed. Phlebography and AVS will be performed only if tumour was less than 1 cm at the CT-Scan despite important biological abnormalities. This schema requires to be validated by a prospective evaluation [fr

  12. Indigeneity: global and local.

    Science.gov (United States)

    Merlan, Francesca

    2009-06-01

    The term indigenous, long used to distinguish between those who are "native" and their "others" in specific locales, has also become a term for a geocultural category, presupposing a world collectivity of "indigenous peoples" in contrast to their various "others." Many observers have noted that the stimuli for internationalization of the indigenous category originated principally from particular nation-states-Anglo-American settler colonies and Scandinavia. All, I argue, are relevantly political cultures of liberal democracy and weighty (in different ways) in international institutional affairs. However, international indigeneity has not been supported in any unqualified way by actions taken in the name of several nation-states that were among its main points of origin. In fact, staunch resistance to the international indigenous project has recently come from four of them. In 2007, the only four voting countries to reject the main product of international indigenist activity over the past 30 years, the Declaration on the Rights of Indigenous Peoples, were Australia, the United States, Canada, and New Zealand. In these locations, forms of "indigenous relationship" emerged that launched international indigeneity and that strongly influenced international perceptions of what "indigeneity" is and who "indigenous peoples" may be. Some other countries say the model of indigenous relationship that they see represented by the "establishing" set is inapplicable to themselves (but have nonetheless had to take notice of expanding internationalist indigenism). The apparently paradoxical rejection of the draft declaration by the establishing countries is consistent with the combination of enabling and constraining forces that liberal democratic political cultures offer.

  13. All strategy is local.

    Science.gov (United States)

    Greenwald, Bruce; Kahn, Judd

    2005-09-01

    The aim of strategy is to master a market environment by understanding and anticipating the actions of other economic agents, especially competitors. A firm that has some sort of competitive advantage-privileged access to customers, for instance--will have relatively few competitors to contend with, since potential competitors without an advantage, if they have their wits about them, will stay away. Thus, competitive advantages are actually barriers to entry and vice versa. In markets that are exposed, by contrast, competition is intense. If the incumbents have even brief success in earning greater than normal returns on investments, new entrants will swarm in to grab a share of the profits. Sooner or later, the additional competition will push returns as far down as the firms' costs of capital. For firms operating in such markets, the only choice is to forget about strategy and run the business as efficiently as possible. Barriers to entry are easier to maintain in a competitive arena that is "local", either in the geographic sense or in the sense of being limited to one product or a handful of related ones. The two most powerful competitive advantages-customer captivity and economies of scale-are more achievable and sustainable in circumscribed markets of this kind. Their opposites are the open markets and host of rivals that are features of globalization. Compapies entering such markets risk frittering away the advantages they secured on smaller playing fields.., Ifa company wants to grow but still obtain superior returns, the authors argue, the best strategy is to dominate a series of discrete but preferably contiguous markets and then expand only at their edges. WalMart's diminishing margins over the past 15 years are strong evidence of the danger of proceeding otherwise.

  14. Performing a local barrier operation

    Science.gov (United States)

    Archer, Charles J; Blocksome, Michael A; Ratterman, Joseph D; Smith, Brian E

    2014-03-04

    Performing a local barrier operation with parallel tasks executing on a compute node including, for each task: retrieving a present value of a counter; calculating, in dependence upon the present value of the counter and a total number of tasks performing the local barrier operation, a base value, the base value representing the counter's value prior to any task joining the local barrier; calculating, in dependence upon the base value and the total number of tasks performing the local barrier operation, a target value of the counter, the target value representing the counter's value when all tasks have joined the local barrier; joining the local barrier, including atomically incrementing the value of the counter; and repetitively, until the present value of the counter is no less than the target value of the counter: retrieving the present value of the counter and determining whether the present value equals the target value.

  15. Local fields and their extensions

    CERN Document Server

    Fesenko, I B

    2002-01-01

    This book offers a modern presentation of local fields whose spectacular development was initiated almost one hundred years ago by K. Hensel. The volume consists of nine chapters divided into four parts: arithmetic properties of local fields, class field theory for various types of local fields and generalizations, explicit formulas for the Hilbert pairing, and Milnor K-groups of fields and of local fields. The first three parts essentially simplify, revise, and update the first edition. This second edition contains about sixty additional pages reflecting several aspects of the developments in local number theory in the last ten years. Exercises point to many other paths for exploration. The book is designed for graduate students and research mathematicians interested in local number theory and its applications in arithmetic algebraic geometry.

  16. To renew local energy policies

    International Nuclear Information System (INIS)

    Bailleul, Esther; Alfano, Patrick; Ballan, Etienne; Bosboeuf, Pascale; Braun, Nicolas; Budin, Jacques-Olivier; Caron, Jean-Francois; Couturier, Christian; Dantec, Ronan; Ducolombier, Alexandre; Durand, Lucas; Haeringer, Nicolas; Izard, Charlotte; Jadot, Yannick; Joos, Marine; Landel, Pierre-Antoine; Le Du, Mathieu; Lucas, Guillaume; Maya, Michel; Moisan, Marie; Peullemeulle, Justine; Pin, Pascaline; Poize, Noemie; Regnier, Yannick; Rudinger, Andreas; Saultier, Patrick; Serne, Pierre; Zeroual, Bouchr; Arevalo, Henri; Bregeon, Anne; Vauquois, Victor; Aussavy, Gregoire

    2016-08-01

    Presented as a guide, this book proposes an operational and transverse approach to local action in the field of energy. It is illustrated by many field examples which show how actors have been able to (re)gain control of the energy issue. In the first part in which they present the main issues, the authors outline that local authorities are in front line for a European energy transition, recall the French legal framework for local energy transition, describe how to address local energy self-sufficiency, and comment stakes and levers for energy transition financing. The second part deals with action. The authors there describe how to manage a local energy transition, how to act transversally, how to get citizen involved, and how to relate energy transition and local development

  17. Local government and climate policy

    International Nuclear Information System (INIS)

    Burger, H.; Menkveld, M.; Coenen, F.H.J.M.

    2000-01-01

    Local government in the Netherlands could play a greater role than they currently do in reducing greenhouse gas emissions in the country. This fact formed the motivation for initiating the research project on Local Government and Climate Policy. Many local climate options are known in theory. Options for reduction that lie within the sphere of influence of Dutch local government are, for example, sustainable building or encouraging the utilisation of sustainable energy. But actual practice turns out to be rather refractory. If such options are to be implemented, then one has to overcome institutional barriers, such as the co-operation between different departments in the same local authority. The objective of this research is to improve the contribution that local government makes to climate policy

  18. Observations of Local Seychelles Circulation

    Science.gov (United States)

    2015-09-30

    1 DISTRIBUTION STATEMENT A. Approved for public release; distribution is unlimited. Observations of Local Seychelles Circulation Geno...goal for the proposed work is to develop predictive capabilities for physical oceanography for the Seychelles region in support of locally relevant...observations in the Seychelles region that will lead to long-term data collection efforts. In collaboration with local partnerships, we will carry out

  19. Local Agenda 21 and poverty

    OpenAIRE

    Palmans, Eva; Marysse, Stefaan

    2003-01-01

    Poverty, the increasing urbanisation of poverty and the environmental degradation are major problems facing the actual world. This is reflected in international conferences and agendas, such as Local Agenda 21. This agenda is responding to the current problems by promoting sustainable development through local action and by using participatory methods. Our major concern is to reflect on the impact of the Local Agenda 21 on the reduction of poverty in a Third World context.

  20. A Parameter Study of Localization

    Directory of Open Access Journals (Sweden)

    Sandor Stephen Mester

    1996-01-01

    Full Text Available Extensive work has been done on the vibration characteristics of perfectly periodic structures. Disorder in the periodic pattern has been found to lead to localization in one-dimensional periodic structures. It is important to understand localization because it causes energy to be concentrated near the disorder and may cause an overestimation of structural damping. A numerical study is conducted to obtain a better understanding of localization. It is found that any mode, even the first, can localize due to the presence of small imperfections.

  1. Local Government System in Japan

    Directory of Open Access Journals (Sweden)

    Vladimir V. Redko

    2016-12-01

    Full Text Available The article is devoted to the issues of the activities of the local government of Japan. Particular attention is drawn to the legal framework and the material basis for the functioning of local self-government bodies. The system of local self-government is considered as a special form of self-government with a specific functional and meaning; system of municipal management and delegation of authority, as well as features of interaction between civil and imperious levels. The allocation of the city with a special status, as well as the financial structure of the local government of Japan, is considered in detail.

  2. Radioprotective effect of local hypothermia

    International Nuclear Information System (INIS)

    Hong, Seong-Su; Ogawa, Yoshihiro; Higano, Shuichi; Nakamura, Mamoru; Hoshino, Fumihiko

    1985-01-01

    We attempted local hypothermia to prevent radiation dermatitis and stomatitis. With regard to parasternal skin reactions postoperatively irradiated breast cancer, dry and moist desquamation, which occasionally occurred with conventional irradiation was not observed in combination with local cooling. As for head and neck tumors, patients who complained of stomatitis decreased with the local cooling, and no one wanted a pause in irradiation before 40 Gy. As local hypothermia is free from danger and does not require special equipment, it was considered to be widely applicable. (author)

  3. Globalization vs. localization: global food challenges and local sollutions

    NARCIS (Netherlands)

    Quaye, W.; Jongerden, J.P.; Essegbey, G.; Ruivenkamp, G.T.P.

    2010-01-01

    The objective of this study was to examine the effect of global-local interactions on food production and consumption in Ghana, and identify possible local solutions. Primary data were collected using a combination of quantitative-qualitative methods, which included focus group discussions and

  4. String-localized quantum fields and modular localization

    Energy Technology Data Exchange (ETDEWEB)

    Mund, J. [Juiz de Fora Univ., MG (Brazil). Dept. de Fisica; Schroer, B. [FU-Berlin, Berlin (Germany). Inst. fuer Theoretische Physik; Yngvason, J. [Erwin Schroedinger Institute for Mathematical Physics, Vienna (Austria)

    2005-12-15

    We study free, covariant, quantum (Bose) fields that are associated with irreducible representations of the Poincare group and localized in semi-infinite strings extending to spacelike infinity. Among these are fields that generate the irreducible representations of mass zero and infinite spin that are known to be incompatible with point-like localized fields. For the massive representation and the massless representations of finite helicity, all string-localized free fields can be written as an integral, along the string, of point-localized tensor or spinor fields. As a special case we discuss the string-localized vector fields associated with the point-like electromagnetic field and their relation to the axial gauge condition in the usual setting. (author)

  5. String-localized quantum fields and modular localization

    International Nuclear Information System (INIS)

    Mund, J.

    2005-12-01

    We study free, covariant, quantum (Bose) fields that are associated with irreducible representations of the Poincare group and localized in semi-infinite strings extending to spacelike infinity. Among these are fields that generate the irreducible representations of mass zero and infinite spin that are known to be incompatible with point-like localized fields. For the massive representation and the massless representations of finite helicity, all string-localized free fields can be written as an integral, along the string, of point-localized tensor or spinor fields. As a special case we discuss the string-localized vector fields associated with the point-like electromagnetic field and their relation to the axial gauge condition in the usual setting. (author)

  6. Morphea profunda presenting as a neuromuscular mimic.

    NARCIS (Netherlands)

    Voermans, N.C.; Pillen, S.; Jong, E.M.G.J. de; Creemers, M.C.W.; Lammens, M.M.Y.; Alfen, N. van

    2008-01-01

    Localized scleroderma is characterized by idiopathic fibrosis of the skin and adjacent structures, and muscle involvement occurs predominantly in deep morphea. We report a patient with linear scleroderma who presented with slowly progressive atrophy, muscle weakness, and loss of function of her

  7. Local cohomology and superselection structure

    International Nuclear Information System (INIS)

    Roberts, J.E.

    1976-02-01

    A novel quantum analogue of the classical problem of cohomology incorporating locality is introduced and is shown to generate those superselection sectors whose charge can be strictly localized. In a 2-dimensional space-time there are further possibilities; in particular, soliton sectors can be generated by this procedure [fr

  8. Building Local Governance in Denmark

    DEFF Research Database (Denmark)

    Damgaard, Bodil

    local level actors through partnerships and networks and discuss how such involvement may improve societal regulation. The paper analyses the apparent paradox that local partnerships are mandated though hierarchical structures (law) and discusses the consequences hereof with regard to central features...

  9. A survey of local cohomology

    International Nuclear Information System (INIS)

    Roberts, J.E.

    1977-06-01

    Local cohomology is discussed in Wightman field theory and algebraic field theory. Applications are made to superselection structure, solitons, spontaneously broken gauge symmetries and quantum electrodynamics. A simplified picture of the probable relationship between gauge theories and the local 2-cohomology is presented

  10. Local coexistance of different phases

    International Nuclear Information System (INIS)

    Narnhofer, H.

    1982-01-01

    Under intuitively reasonable assumptions it is shown that in two dimensions different phases cannot exist locally. In three dimensions we discuss the possibility of local coexistance of districts with different magnetization for the Heisenberg ferromagnet and show that an interaction that breaks rotational invariance is necessary for this phenomenon. (Author)

  11. Applications of locally orderless images

    NARCIS (Netherlands)

    Ginneken, van B.; Haar Romenij, ter B.M.

    2000-01-01

    In a recent work, J. J. Koenderink and A. J. Van Doorn considered a family of three intertwined scale-spaces coined the locally orderless image (LOI) (1999, J. Comput. Vision, 31 (2/3), 159–168). The LOI represents the image, observed at inner scale ó, as a local histogram with bin-width â, at each

  12. A geometrical perspective on localization

    NARCIS (Netherlands)

    Dulman, S.O.; Baggio, A.; Havinga, Paul J.M.; Langendoen, K.G.; Zhang, Ying; Ye, Yinyu

    2008-01-01

    A large number of localization algorithms for wireless sensor networks (WSNs) are evaluated against the Cramer-Rao Bound (CRB) as an indicator of how good the algorithm performs. The CRB defines the lower bound on the precision of an unbiased localization estimator. The CRB concept, borrowed from

  13. BOOK REVIEW: Improving Local Government

    Directory of Open Access Journals (Sweden)

    Jaap de Visser

    2009-11-01

    Full Text Available This book is a compilation of ten essays on local government with an introductory and concluding chapter. The themes discussed include metropolitan governance, the role of community leadership, the value of best practice as an administration technique, indigenous knowledge, intergovernmental relations, public-private partnerships and local management.

  14. Is Local Community the Answer?

    DEFF Research Database (Denmark)

    Bruun, Ole; Olwig, Mette Fog

    2015-01-01

    This article critically examines claims that “local community” and “local/traditional knowledge” are vital contributions to safeguarding socio-economic stability and securing sustainable resource uses in times of stress. The empirical focus is on Central Vietnam, but the argument is relevant...

  15. Meet the local policy workers

    DEFF Research Database (Denmark)

    Wimmelmann, Camilla L.; Vallgårda, Signild; Jensen, Anja MB

    2018-01-01

    Reporting on an interview and observation based study in Danish municipalities, this article deals with local policy workers, and takes departure in the great variation we observed in implementation of centrally issued health promotion guidelines. We present five types of local policy workers, ea...

  16. Global Policies and Local Practice

    DEFF Research Database (Denmark)

    Rasche, Andreas; Gilbert, Dirk Ulrich

    networks that are embedded in a wider global network, I argue that tighter couplings (within local networks) and looser couplings (between local networks) coexist. Second, I suggest that this coexistence of couplings enables MSIs to generate policy outcomes which address the conditions of a transnational...

  17. Global Policies and Local Practice

    DEFF Research Database (Denmark)

    Rasche, Andreas

    2012-01-01

    networks that are embedded in a wider global network, I argue that tighter couplings (within local networks) and looser couplings (between local networks) coexist. Second, I suggest that this coexistence of couplings enables MSIs to generate policy outcomes which address the conditions of a transnational...

  18. Local initiative extrapolated to nation

    DEFF Research Database (Denmark)

    Wittchen, Kim Bjarne; Kragh, Jesper; Brøgger, Morten

    In the municipality of Sønderborg, in the southern part of Jutland, there is a shining example initiated in 2007, ProjectZero, of a local initiative that have resulted in extensive energy savings in residential buildings and at the same time created local workplaces. The intension with the pilot...

  19. Local community, mobility and belonging

    DEFF Research Database (Denmark)

    Jørgensen, Anja; Arp Fallov, Mia; Knudsen, Lisbeth B.

    2011-01-01

    ,recent developments in the understandings of mobility and local communities,and presents different theoretical views on local belonging.These questions highlight the necessity to discuss and investigate two overall narratives in social theory about the connection between space and social relations.Namely,1...

  20. Representations of locally symmetric spaces

    International Nuclear Information System (INIS)

    Rahman, M.S.

    1995-09-01

    Locally symmetric spaces in reference to globally and Hermitian symmetric Riemannian spaces are studied. Some relations between locally and globally symmetric spaces are exhibited. A lucid account of results on relevant spaces, motivated by fundamental problems, are formulated as theorems and propositions. (author). 10 refs