Interstitial lung disease in patients with idiopathic inflammatory myopathy (IIM-ILD): Definitions, epidemiology, pathophysiology, clinical manifestations, complications, risk, and mortality factors (narrative review)

Abstract

Interstitial lung disease refers to a group of disorders generally characterized by progressive scarring of lung tissue due to a wide variety of causes and associated with a plethora of symptoms. Patients with this diagnosis can be asymptomatic or present severe symptoms that could lead to death. Its signs and symptoms are the same in patients with concomitant connective tissue disease and those without. Genetics and immunity play essential roles in patients with interstitial lung disease and idiopathic inflammatory myopathy. Alterations in genes and excessive production of specific cytokines can lead to the development of interstitial lung disease. Interstitial lung disease can have several complications, including chronic respiratory distress and infections, and can worsen the prognosis of patients with idiopathic inflammatory myopathy. Here, we present a narrative review describing the epidemiology, pathophysiology, clinical manifestations, risk factors, and complications of the population with interstitial lung disease and idiopathic inflammatory myopathy.