Spectrum of interstitial lung disease in rheumatic diseases

Abstract

Among the diverse forms of lung involvement, interstitial lung disease (ILD) and pulmonary arterial hypertension are two important conditions in patients with rheumatic diseases that are associated with significant morbidity and mortality. The management of ILD is challenging and these challenges are associated with several mechanisms, particularly the inflammatory processes of immune cells and also fibrotic processes, which play a crucial role in disease progression, making immunosuppressive therapy less effective. However, recent advances in pharmacologic interventions have been shown to delay disease progression of these lung conditions and improve patient survival. Disease stratification and early identification of patients who are more likely to progress, and thus in need of more aggressive treatment, is important in the field of ILD. Early diagnosis, monitoring, and multidisciplinary team input are very important to improve overall outcomes. This review focuses on ILD in patients with various rheumatic diseases (rheumatoid arthritis, myositis, Sjogren' syndrome, and systemic sclerosis) and the challenges.

Keywords

• connective tissue disease
• interstitial lung disease
• myositis
• scleroderma
• vasculitis