Scleroderma

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Carol M. Black - One of the best experts on this subject based on the ideXlab platform.

  • Pilot study of anti‐thymocyte globulin plus mycophenolate mofetil in recent‐onset diffuse Scleroderma
    Rheumatology, 2001
    Co-Authors: Richard Stratton, H. Wilson, Carol M. Black
    Abstract:

    OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse Scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse Scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, Scleroderma skin score, hand contractures, EuroQol score, Scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P

  • pilot study of anti thymocyte globulin plus mycophenolate mofetil in recent onset diffuse Scleroderma
    Rheumatology, 2001
    Co-Authors: Richard Stratton, H. Wilson, Carol M. Black
    Abstract:

    OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse Scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse Scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, Scleroderma skin score, hand contractures, EuroQol score, Scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P<0.01). Hand contractures worsened during the study. Mean measurements of systemic disease remained stable. One patient died after a Scleroderma renal crisis. Five patients developed serum sickness after ATG treatment, but this was controlled by corticosteroid therapy. MMF therapy was well tolerated. CONCLUSION: ATG and MMF appear safe in Scleroderma. The improvement in skin score and the apparent stability of systemic disease during the study period suggest that controlled studies of these agents are justified.

  • Scleroderma and fasciitis in children.
    Current Opinion in Rheumatology, 1995
    Co-Authors: Carol M. Black
    Abstract:

    : Scleroderma is a spectrum of disorders, all of which may occur in childhood. Childhood disease differs from adult disease in that localized forms predominate, with major problems confined to the skin and underlying soft tissues; generalized Scleroderma, whether diffuse or limited, is less common. Childhood eosinophilic fasciitis is rare, and the literature is scant. The pattern appears to be similar to that in adults, and as in the adult form, the overlap between eosinophilic fasciitis, morphea, and linear Scleroderma is blurred. The etiopathogenesis of juvenile-onset Scleroderma is unknown but almost certainly multifactorial. The heterogeneity of its clinical expression may argue for it being more than one disease. This review attempts to document the expression of Scleroderma in childhood and relate it to etiologic, immunologic, and pathogenic considerations.

Fredrick M. Wigley - One of the best experts on this subject based on the ideXlab platform.

  • autoantibodies and Scleroderma phenotype define subgroups at high risk and low risk for cancer
    Annals of the Rheumatic Diseases, 2018
    Co-Authors: Takeru Igusa, Fredrick M. Wigley, Laura K Hummers, Kala Visvanathan, Carrie Richardson, Livia Casciolarosen, Antony Rosen, Ami A Shah
    Abstract:

    Objectives Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in Scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic Scleroderma subsets and compared estimates with the general population. Methods Patients in the Johns Hopkins Scleroderma Center observational cohort were studied. Overall and site-specific cancer incidence was calculated in distinct autoantibody and Scleroderma phenotypic subsets, and compared with the Surveillance, Epidemiology and End Results registry, a representative sample of the US population. Results 2383 patients with Scleroderma contributing 37 686 person-years were studied. 205 patients (8.6%) had a diagnosis of cancer. Within 3 years of Scleroderma onset, cancer risk was increased in patients with RNA polymerase III autoantibodies (antipol; standardised incidence ratio (SIR) 2.84, 95% CI 1.89 to 4.10) and those lacking centromere, topoisomerase-1 and pol antibodies (SIR 1.83, 95% CI 1.10 to 2.86). Among antipol-positive patients, cancer-specific risk may vary by Scleroderma subtype; those with diffuse Scleroderma had an increased breast cancer risk, whereas those with limited Scleroderma had high lung cancer risk. In contrast, patients with anticentromere antibodies had a lower risk of cancer during follow-up (SIR 0.59, 95% CI 0.44 to 0.76). Conclusions Autoantibody specificity and disease subtype are biologically meaningful filters that may inform cancer risk stratification in patients with Scleroderma. Future research testing the value of targeted cancer screening strategies in patients with Scleroderma is needed.

  • examination of autoantibody status and clinical features associated with cancer risk and cancer associated Scleroderma
    Arthritis & Rheumatism, 2015
    Co-Authors: Ami A Shah, Laura K Hummers, Kala Visvanathan, Livia Casciolarosen, Antony Rosen, Fredrick M. Wigley
    Abstract:

    Introduction We previously reported a contemporaneous onset of cancer and Scleroderma in patients with RNA polymerase III (pol) antibodies and identified a biological link between cancer and Scleroderma. This investigation was designed to further evaluate whether autoantibody status and other characteristics associate with cancer and a clustering of cancer with Scleroderma onset.

  • Scleroderma in the Elderly Population
    Geriatric Rheumatology, 2011
    Co-Authors: Rebecca L. Manno, Fredrick M. Wigley
    Abstract:

    Scleroderma has a median age of onset in the fifth decade of life; however, there are many individuals who develop Scleroderma later or who are aging with this disease. It is important that clinicians are able to recognize features of Scleroderma in the elderly and distinguish these from well-established imitators. The age of Scleroderma onset can impact the course of disease and increase the risk for organ-specific complications such as pulmonary vascular disease. As individuals age with Scleroderma, physicians should focus on careful monitoring of each organ system and comprehensive medical care. Treatment principles should be customized to an individual’s disease, and the importance of nutrition, mobility, and social support emphasized.

  • abnormalities in the regulators of angiogenesis in patients with Scleroderma
    The Journal of Rheumatology, 2009
    Co-Authors: Laura K Hummers, Fredrick M. Wigley, Amy Hall, Michael Simons
    Abstract:

    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 < 0.0001). Levels of MMP were also higher in Scleroderma patients compared to controls (MMP-9 and pro-MMP-1) (p < 0.0001). Levels of the pro-angiogenic and anti-fibrotic factor, HGF, were noted to be lower in patients with Scleroderma, but had a positive correlation with right ventricular systolic pressure (RVSP) as measured by echocardiogram (p < 0.0001) and the Raynaud Severity Score (p = 0.05). Endostatin (an anti-angiogenic factor) was notably higher in patients with Scleroderma (p < 0.0001) and also correlated positively with RVSP (p = 0.023). Conclusion. These results demonstrate striking abnormalities in the circulating regulators of angiogenesis in patients with 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.

  • Bullous lesions in Scleroderma.
    International Journal of Dermatology, 2002
    Co-Authors: Adrienne Rencic, Fredrick M. Wigley, Supriya Goyal, Mona Z. Mofid, H. Carlos Nousari
    Abstract:

    Background The occurrence of bullous lesions in localized or systemic Scleroderma is rare. Three histologic patterns have been reported: lichen sclerosus et atrophicus-like, lymphangiectatic blisters and autoimmune blistering diseases. Objective  To investigate the frequency, clinical, and immunopathologic features of patients with Scleroderma and bullous eruptions and to review the literature regarding this rare condition. Methods  A retrospective study of 53 cases of Scleroderma (localized, generalized, and systemic) in the dermatology and rheumatology clinics at one institution over an 8-year span. Clinical, serologic, and immunopathologic findings were analyzed in four cases. Results Four of 53 patients exhibited bullous lesions in association with Scleroderma. The first case illustrates lymphangioma-like clinical and pathologic presentation. The second case demonstrates bullous lichen sclerosus et atrophicus-like pattern. The other two cases exemplify a superimposed autoimmune skin disease, epidermolysis bullosa acquisita and penicillamine induced pemphigus foliaceus after treatment for systemic Scleroderma. Conclusions  Of the 53 original patients, we have described four cases of bullous Scleroderma (7.5%) Illustrating several pathogenetic mechanisms of bulla formation. inflammatory (lichen sclerosus et atrophicus), fibrotic/obstructive (lymphangiomatous), autoimmune (epidermolysis bullosa acquisita), and pemphigus foliaceus. The final case illustrates bullae as a complication of therapy for the underlying Scleroderma.

Ami A Shah - One of the best experts on this subject based on the ideXlab platform.

  • Cancer and Scleroderma
    Rheumatic Diseases Clinics of North America, 2020
    Co-Authors: Emma Weeding, Livia Casciola-rosen, Ami A Shah
    Abstract:

    Individuals with Scleroderma have an increased risk of cancer compared with the general population. This heightened risk may be from chronic inflammation and tissue damage, malignant transformation provoked by immunosuppressive therapies, or a common inciting factor. In unique subsets of patients with Scleroderma, there is a close temporal relationship between the onset of cancer and Scleroderma, suggesting cancer-induced autoimmunity. This article discusses the potential mechanistic links between cancer and Scleroderma, the serologic and clinical risk factors associated with increased cancer risk in patients with Scleroderma, and implications for cancer screening.

  • Progress in Understanding, Diagnosing, and Managing Cardiac Complications of Systemic Sclerosis
    Current Rheumatology Reports, 2019
    Co-Authors: George Hung, Ami A Shah, Valentina Mercurio, Stephen C. Mathai, Monica Mukherjee
    Abstract:

    Purpose of the Review Systemic sclerosis (Scleroderma) is a complex autoimmune disease that commonly involves the cardiovascular system. Even if often subclinical, cardiac involvement is considered a poor prognostic factor as it is a leading cause of death in Scleroderma patients. We review the cardiac manifestations of Scleroderma, the diagnostic methods useful in detection, and current advances in therapeutic management. Recent Findings Beside the routine exams for the assessment of cardiac status (including EKG, standard echocardiography, provocative tests) novel techniques such as myocardial strain imaging on echocardiography, cardiac magnetic resonance imaging, invasive hemodynamic assessment, and endomyocardial biopsy have been demonstrated to be useful in understanding the cardiac alterations that typically affect Scleroderma patients. Summary Recent application of novel cardiac detection strategies is providing increased insight into the breadth and pathogenesis of cardiac complications of Scleroderma. Further studies coupling exercise provocation, invasive and imaging assessment, and mechanistic studies in Scleroderma cardiac tissue are needed to develop the optimal approach to early detection of cardiac disease in Scleroderma and targeted therapies.

  • autoantibodies and Scleroderma phenotype define subgroups at high risk and low risk for cancer
    Annals of the Rheumatic Diseases, 2018
    Co-Authors: Takeru Igusa, Fredrick M. Wigley, Laura K Hummers, Kala Visvanathan, Carrie Richardson, Livia Casciolarosen, Antony Rosen, Ami A Shah
    Abstract:

    Objectives Recent studies demonstrate autoantibodies are powerful tools to interrogate molecular events linking cancer and the development of autoimmunity in Scleroderma. Investigating cancer risk in these biologically relevant subsets may provide an opportunity to develop personalised cancer screening guidelines. In this study, we examined cancer risk in distinct serologic and phenotypic Scleroderma subsets and compared estimates with the general population. Methods Patients in the Johns Hopkins Scleroderma Center observational cohort were studied. Overall and site-specific cancer incidence was calculated in distinct autoantibody and Scleroderma phenotypic subsets, and compared with the Surveillance, Epidemiology and End Results registry, a representative sample of the US population. Results 2383 patients with Scleroderma contributing 37 686 person-years were studied. 205 patients (8.6%) had a diagnosis of cancer. Within 3 years of Scleroderma onset, cancer risk was increased in patients with RNA polymerase III autoantibodies (antipol; standardised incidence ratio (SIR) 2.84, 95% CI 1.89 to 4.10) and those lacking centromere, topoisomerase-1 and pol antibodies (SIR 1.83, 95% CI 1.10 to 2.86). Among antipol-positive patients, cancer-specific risk may vary by Scleroderma subtype; those with diffuse Scleroderma had an increased breast cancer risk, whereas those with limited Scleroderma had high lung cancer risk. In contrast, patients with anticentromere antibodies had a lower risk of cancer during follow-up (SIR 0.59, 95% CI 0.44 to 0.76). Conclusions Autoantibody specificity and disease subtype are biologically meaningful filters that may inform cancer risk stratification in patients with Scleroderma. Future research testing the value of targeted cancer screening strategies in patients with Scleroderma is needed.

  • examination of autoantibody status and clinical features associated with cancer risk and cancer associated Scleroderma
    Arthritis & Rheumatism, 2015
    Co-Authors: Ami A Shah, Laura K Hummers, Kala Visvanathan, Livia Casciolarosen, Antony Rosen, Fredrick M. Wigley
    Abstract:

    Introduction We previously reported a contemporaneous onset of cancer and Scleroderma in patients with RNA polymerase III (pol) antibodies and identified a biological link between cancer and Scleroderma. This investigation was designed to further evaluate whether autoantibody status and other characteristics associate with cancer and a clustering of cancer with Scleroderma onset.

Richard Stratton - One of the best experts on this subject based on the ideXlab platform.

  • Pilot study of anti‐thymocyte globulin plus mycophenolate mofetil in recent‐onset diffuse Scleroderma
    Rheumatology, 2001
    Co-Authors: Richard Stratton, H. Wilson, Carol M. Black
    Abstract:

    OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse Scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse Scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, Scleroderma skin score, hand contractures, EuroQol score, Scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P

  • pilot study of anti thymocyte globulin plus mycophenolate mofetil in recent onset diffuse Scleroderma
    Rheumatology, 2001
    Co-Authors: Richard Stratton, H. Wilson, Carol M. Black
    Abstract:

    OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse Scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse Scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, Scleroderma skin score, hand contractures, EuroQol score, Scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P<0.01). Hand contractures worsened during the study. Mean measurements of systemic disease remained stable. One patient died after a Scleroderma renal crisis. Five patients developed serum sickness after ATG treatment, but this was controlled by corticosteroid therapy. MMF therapy was well tolerated. CONCLUSION: ATG and MMF appear safe in Scleroderma. The improvement in skin score and the apparent stability of systemic disease during the study period suggest that controlled studies of these agents are justified.

H. Wilson - One of the best experts on this subject based on the ideXlab platform.

  • Pilot study of anti‐thymocyte globulin plus mycophenolate mofetil in recent‐onset diffuse Scleroderma
    Rheumatology, 2001
    Co-Authors: Richard Stratton, H. Wilson, Carol M. Black
    Abstract:

    OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse Scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse Scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, Scleroderma skin score, hand contractures, EuroQol score, Scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P

  • pilot study of anti thymocyte globulin plus mycophenolate mofetil in recent onset diffuse Scleroderma
    Rheumatology, 2001
    Co-Authors: Richard Stratton, H. Wilson, Carol M. Black
    Abstract:

    OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse Scleroderma. METHODS: A pilot study of 13 patients with recent-onset diffuse Scleroderma was carried out. Patients received ATG for 5 days, followed by MMF for 12 months. We recorded adverse events, Scleroderma skin score, hand contractures, EuroQol score, Scleroderma functional assessment, pulmonary function studies, echocardiogram and plasma creatinine concentration. RESULTS: Mean skin score decreased during the study from 28 at baseline to 17 after 12 months of MMF (P<0.01). Hand contractures worsened during the study. Mean measurements of systemic disease remained stable. One patient died after a Scleroderma renal crisis. Five patients developed serum sickness after ATG treatment, but this was controlled by corticosteroid therapy. MMF therapy was well tolerated. CONCLUSION: ATG and MMF appear safe in Scleroderma. The improvement in skin score and the apparent stability of systemic disease during the study period suggest that controlled studies of these agents are justified.