Idiopathic inflammatory myopathies related lung disease in adults

Abstract

Interstitial lung disease (ILD) is common in idiopathic inflammatory myopathies in adults, especially in patients with antisynthetase syndrome and anti-MDA5 antibody-associated dermatomyositis. Pulmonary manifestations can range from subclinical ILD to rapidly progressive respiratory failure. Coexistent myositis, characteristic skin lesions, arthritis, and Raynaud's phenomenon are common. However, 16–65% of patients present with isolated lung disease. Detection of myositis-specific and myositis-associated antibodies can aid in diagnosis and disease characterisation. Chest imaging and pathology most commonly show non-specific interstitial pneumonia and organising pneumonia patterns. Immunosuppression is the mainstay of management with aggressive combination treatment for rapidly progressive disease and incremental escalation as needed for chronic ILD. The addition of antifibrotic agents is an option in progressive fibrotic disease, and lung transplantation can be considered in severe, end-stage disease. Most patients respond to treatment, but short-term mortality remains high for patients with rapidly progressive disease associated with anti-MDA5 antibody ILD.