SScILD

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) refers to interstitial lung disease that occurs in the context of systemic sclerosis (scleroderma). It is a major contributor to illness and death in systemic sclerosis, particularly in diffuse cutaneous disease. The condition presents with symptoms such as new or worsening shortness of breath on exertion, dry cough, and fatigue. Physical examination may reveal crackles and, in advanced cases, signs of systemic sclerosis.

Diagnosis relies on clinical assessment, pulmonary function testing, and imaging. High-resolution CT scans commonly show fibrotic changes that resemble nonspecific interstitial pneumonia (NSIP) patterns, though UIP-like patterns may also occur. Pulmonary function tests typically show a restrictive pattern with reduced forced vital capacity (FVC) and decreased diffusing capacity for carbon monoxide (DLCO). Autoantibody profiles support the diagnosis and help characterize disease risk, with anti-Scl-70 (topoisomerase I) linked to ILD in systemic sclerosis.

Management aims to control systemic sclerosis activity and slow ILD progression. Immunosuppressive therapy, particularly mycophenolate mofetil, has been shown to stabilize or improve lung function in many patients. Cyclophosphamide has historical utility in selected cases. A newer antifibrotic, nintedanib, reduces the rate of FVC decline in SSc-ILD and is approved for this indication, often used in combination with MMF. Supportive care includes pulmonary rehabilitation, vaccination, oxygen as needed, and management of extrapulmonary disease manifestations. In advanced cases, lung transplantation may be considered. Regular follow-up with PFTs and clinical assessment guides treatment decisions and monitoring for complications.