Interstitial lung disease and idiopathic inflammatory myopathies: progress and pitfalls

To present the latest findings regarding interstitial lung disease (ILD) in idiopathic inflammatory myopathies, focusing on the phenotype of ILD and auto-antibodies, pathogenesis and treatment.

Interstitial lung disease is a common manifestation of myositis and different phenotypes of ILD associate with various clinical or serological phenotypes. Thus, antisynthetase antibodies are strongly associated with ILD, especially for non-Jo-1 (anti-PL-7 and anti-PL-12) where the association approaches 90-100%. A rare form of ILD with a very poor prognosis, acute and rapidly progressive lung disease, is strongly associated with clinically amyopathic dermatomyositis (CADM) and a novel - anti-CADM-140 auto-antibody. A combination of anti-Jo-1 and anti-SSA/Ro antibodies is another marker of severe pulmonary involvement. The use of immunossuppresive therapy in myositis-associated ILD is based on clinical experience, and a few new case reports indicate beneficial effect of tacrolimus in antisynthetase syndrome and rituximab in resistant or acute forms of lung disease but further studies are needed.

Interstitial lung disease is a common manifestation of myositis. Auto-antibody profile seems to determine the frequency, course and severity of lung disease. New biological therapies such as rituximab show promising results in resistant and acute lung disease but controlled trials are needed.