Recognition and Management of Myositis-Associated Rapidly Progressive Interstitial Lung Disease

Idiopathic inflammatory myopathies are autoimmune processes that are characterized by skeletal muscle inflammation. The lung is the most commonly involved extramuscular organ, and, when present, pulmonary disease drives morbidity and mortality. A subset of patients can present with rapidly progressive hypoxemic respiratory failure due to myositis-related interstitial lung disease. Confirmatory autoantibody testing requires sending samples to a reference laboratory; thus, diagnosis of rapidly progressive myositis-associated interstitial lung disease relies on a high index of suspicion and careful history and physical examination. Although the cornerstone of therapy for these patients remains multimodality immunosuppression, emerging data support a role for advanced therapies (including extracorporeal membrane oxygenation and lung transplantation) in appropriately selected patients. It is hoped that greater awareness of the clinical features of this syndrome will allow for appropriate diagnosis and treatment of these potentially treatable patients, as well as raise awareness of the need for multicenter collaboration to prospectively study how to manage this complex disease. Idiopathic inflammatory myopathies are autoimmune processes that are characterized by skeletal muscle inflammation. The lung is the most commonly involved extramuscular organ, and, when present, pulmonary disease drives morbidity and mortality. A subset of patients can present with rapidly progressive hypoxemic respiratory failure due to myositis-related interstitial lung disease. Confirmatory autoantibody testing requires sending samples to a reference laboratory; thus, diagnosis of rapidly progressive myositis-associated interstitial lung disease relies on a high index of suspicion and careful history and physical examination. Although the cornerstone of therapy for these patients remains multimodality immunosuppression, emerging data support a role for advanced therapies (including extracorporeal membrane oxygenation and lung transplantation) in appropriately selected patients. It is hoped that greater awareness of the clinical features of this syndrome will allow for appropriate diagnosis and treatment of these potentially treatable patients, as well as raise awareness of the need for multicenter collaboration to prospectively study how to manage this complex disease.