Introduction:

Systemic sclerosis is a chronic autoimmune disease, characterized by the triad of small vessel vasculopathy, immune system activation and increased deposits of extracellular matrix in the skin and internal organs. In the last few years, pulmonary involvement has gained relevance since the introduction of angiotensin enzyme converter inhibitors with the subsequent decline in scleroderma renal crisis mortality, transforming scleroderma lung disease in the leading cause of mortality. Pulmonary involvement can manifest as hypertension or interstitial lung disease, which usually occurs in patients with generalized scleroderma in the first three years of the disease. Its prognosis is poor without treatment which is aimed at stopping pulmonary function deterioration. Among treatment options, cyclophosphamide has the best evidence, and mycophenolate mofetil, rituximab, and stem cell and lung transplantation are currently under investigation with positive preliminary results.

Objective:

To describe, according to reports in the literature, epidemiology, pathophysiology, diagnostic methods, and treatment of interstitial lung disease in systemic sclerosis.

Methods:

Structured, non-systematic literature review, focused on the aforementioned aspects of interest. It included 52 articles.

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