[Pulmonary involvement in idiopathic inflammatory myopathies]

Area of Science:

• Rheumatology and Pulmonology
• Systemic Autoimmune Diseases
• Interstitial Lung Diseases

Context:

• Idiopathic inflammatory myopathies (IIM) are rare systemic diseases.
• Pulmonary manifestations, especially interstitial lung diseases (ILD), are common in IIM.
• Limited prospective studies exist for this heterogeneous patient group, particularly with ILD involvement.

Purpose:

• To highlight the diagnostic challenges and importance of multidisciplinary discussion in IIM-ILD.
• To emphasize the role of myositis-specific antibodies (e.g., Anti-ARS, MDA5) in diagnosis, phenotype, and prognosis.
• To review common ILD patterns (NSIP, OP) and current treatment strategies.

Summary:

• Diagnosis relies on ILD guidelines and expert multidisciplinary teams.
• Myositis antibodies are key diagnostic tools, correlating with specific phenotypes and disease progression.
• Common ILD patterns include non-specific interstitial pneumonia (NSIP) and organizing pneumonia (OP).
• Treatment involves systemic steroids and early immunosuppression, though evidence is largely retrospective.

Impact:

• Improved understanding of IIM-ILD heterogeneity and diagnostic approaches.
• Highlights the prognostic and therapeutic significance of myositis antibodies.
• Underscores the need for prospective studies to establish definitive treatment pathways for IIM-ILD.