Systemic Sclerosis-Related Interstitial Lung Disease

Authors: Dalal, Samay; Fischer, Aryeh; Swigris, Jeffrey J.

Source: Current Respiratory Medicine Reviews, Volume 4, Number 3, August 2008 , pp. 226-232(7)

Publisher: Bentham Science Publishers

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Abstract:

Systemic sclerosis is a multisystem autoimmune disease with potential lung manifestations that include pulmonary vascular and interstitial disease. Not only is interstitial lung disease the most common pulmonary manifestation, it is a major cause of morbidity and mortality in patients with systemic sclerosis. The risk of systemic sclerosis patients developing progressive interstitial lung disease is strongly associated with the presence of certain autoantibodies The most common lung injury pattern observed in systemic sclerosis-related interstitial lung disease is the fibrotic variant of nonspecific interstitial pneumonia. Because scleroderma renal crisis can now be effectively treated, recently, focus has turned toward how best to treat systemic sclerosis-related interstitial lung disease. Therapy usually involves an immunomodulatory regimen consisting of a steroid-sparing agent with or without glucocorticoids, and response to therapy is variable.

Keywords: Systemic sclerosis; scleroderma; pulmonary fibrosis; lung diseases; interstitial

Document Type: Research article

DOI: http://dx.doi.org/10.2174/157339808785161332

Publication date: 2008-08-01

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Current Respiratory Medicine Reviews publishes frontier reviews on all the latest advances on respiratory diseases and its related areas e.g. pharmacology, pathogenesis, clinical care, and therapy. The journal's aim is to publish the highest quality review articles dedicated to clinical research in the field. The journal is essential reading for all researchers and clinicians in respiratory medicine.