Idiopathic inflammatory myopathies and the lung

Area of Science:

• Rheumatology
• Pulmonology
• Immunology

Background:

• Idiopathic inflammatory myositis (IIM) encompasses rare connective tissue diseases characterized by muscular and extramuscular manifestations.
• Pulmonary involvement, primarily interstitial lung disease (ILD), is a significant challenge in IIM, contributing to substantial morbidity and mortality.
• ILD affects up to 65% of IIM patients and is associated with various extramuscular signs common in other connective tissue diseases.

Purpose of the Study:

• To provide a comprehensive review of myositis-associated lung disease in both adult and juvenile populations.
• To detail the heterogeneity of pulmonary involvement across different IIM subsets and associated autoantibodies.
• To discuss therapeutic strategies for chronic and rapidly progressive ILD in the context of IIM.

Main Methods:

• Critical review of current knowledge on IIM-associated lung disease.
• Analysis of the prevalence and subtypes of pulmonary involvement in IIM.
• Examination of the role of autoantibodies in IIM-related ILD.

Main Results:

• Interstitial lung disease (ILD) is a hallmark of pulmonary involvement in IIM, leading to increased mortality.
• ILD prevalence varies across IIM subtypes, being more frequent in dermatomyositis and overlap myositis.
• Heterogeneity in IIM lung disease is linked to specific autoantibodies, including anti-synthetase, anti-MDA5, and anti-PM/Scl.

Conclusions:

• Myositis-associated lung disease, particularly ILD, is a critical determinant of outcomes in IIM.
• Understanding the specific IIM subset and associated autoantibodies is crucial for predicting and managing lung involvement.
• Effective therapeutic approaches for chronic and rapidly progressive ILD are essential for improving patient prognosis.