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Antifibrotics in systemic sclerosis
Best Practice & Research Clinical Rheumatology, 2021Systemic sclerosis (SSc) is a rare and complex disease, involving multiple organs, with high morbidity and mortality. Fibrosis is the hallmark of SSc, although vascular and inflammatory mechanisms are also implicated in its pathogenesis. Disease management is challenging, due to its heterogeneous presentation, and to the limited number of controlled ...
Maria, Martin-Lopez +1 more
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SYSTEMIC SCLEROSIS (SCLERODERMA)
Acta Clinica Belgica, 2007Systemic sclerosis is a rare and often debilitating disorder characterized by a pathological triad: increased deposition of extracellular matrix and collagen in tissues, microvascular damage and dysfunction, and immune activation as evidenced by inflammation and frequent occurrence of autoantibodies.
Roufosse, Florence +1 more
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Monckeberg’s sclerosis in a patient with systemic sclerosis
Rheumatology International, 2009Monckeberg’s sclerosis (MS) is one of the non-inflammatory vascular diseases characterized by calcification of the media of small and medium-sized muscular arteries, but is distinct from atherosclerosis. We present a case of MS that was incidentally detected by plain X-ray in a patient with systemic sclerosis.
Chang-Nam, Son +3 more
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The complement system and systemic sclerosis
Immunologic Research, 1993Serum concentrations of the various complement components including the classical and the alternative pathways were determined in 58 control healthy subjects and 80 patients with systemic sclerosis (SSC). The mean concentrations of C1q, C2, C5, C6, C7, C9, and factor B were significantly increased in the SSC patients in comparison to controls, while ...
BENBASSAT C +5 more
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2009
Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. Owing to the recently established networks in Germany (DNSS) as well as
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Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. Owing to the recently established networks in Germany (DNSS) as well as
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The fibroblast in systemic sclerosis
Clinics in Dermatology, 1994T he pathogenesis of the dramatic changes seen in systemic scleroderma is still poorly understood; however, three events are supposed to be involved: (1) alterations of the vessel wall, (2) immunologic changes, and (3) disturbance in the production of extracellular matrix proteins (for review see Ref 1).
S, Sollberg +3 more
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Treatment of systemic sclerosis
Current Opinion in Rheumatology, 1991Systemic sclerosis is an extremely variable disease in its manifestations and consequently, treatment needs to be individualized depending on the specific problems that each patient has. Limited scleroderma patients have a prolonged duration of Raynaud's phenomenon and puffy fingers before they develop any skin thickening, digital ulcers or ...
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Geoepidemiology of systemic sclerosis
Autoimmunity Reviews, 2010Systemic sclerosis (SSc) is a rare and potentially severe connective-tissue disease, characterized by skin fibrosis and involvement of internal organs. Because of its rarity and heterogeneous clinical presentation, reliable epidemiological studies on SSc have been particularly difficult to carry out. SSc prevalence is estimated between 3 and 24 per 100,
Brigitte, Ranque, Luc, Mouthon
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Treatment of systemic sclerosis
Current Opinion in Rheumatology, 1993Guidelines for the conduct of clinical trials in progressive systemic sclerosis have been recommended to determine drug efficacy better. To date, the results of disease-modifying drugs in scleroderma have been disappointing. The treatment of esophagitis has been revolutionized by omeprazole.
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Biologics in Systemic Sclerosis
Current Pharmaceutical Biotechnology, 2014Systemic sclerosis (scleroderma) is a heterogeneous autoimmune disorder characterized by collagen overproduction that leads to cutaneous and internal organs sclerosis and pulmonary arterial hypertension. SSc has high morbidity and mortality. SSc pathogenesis is uncertain. At present most therapies of SSc are symptomatic.
Jingxiu, Xuan +3 more
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