Systemic sclerosis–associated interstitial lung disease (SSc-ILD) is a collagen tissue disorder (CTD) characterized by autoimmunity, vasculopathy, and microvascular damage and progressive fibrosis of the skin and internal organs. Fibrosing ILD is a common manifestation of SSc, thought to be caused by repetitive epithelial injury, activation of the innate and adaptive immune mechanisms, and fibroblast recruitment and activation, which leads to extracellular matrix accumulation. Diagnosis of SSc-ILD is based on high-resolution CT (HRCT) findings in a patient with known SSc, along with normal or abnormal pulmonary function tests (PFTs).
Management strategies for SSc-ILD focus on disrupting the autoimmune-mediated pulmonary inflammation that is thought to play a key role in pathophysiology. Immunosuppressive therapy (whether steroids or steroid-sparing agents) is a mainstay in the treatment of severe and/or progressive disease. The goal of treatment in most cases is to achieve disease stabilization.
Are you up-to-date on the management of SSc-ILD? Test your knowledge with this quick quiz.
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Cite this: Zab Mosenifar. Fast Five Quiz: Management of Systemic Sclerosis Interstitial Lung Disease - Medscape - Apr 08, 2022.