Myositis-associated interstitial lung disease.

Abstract

PURPOSE OF REVIEW:

In idiopathic inflammatory myopathies (IIMs), interstitial lung disease (ILD) is common and the autoantibody profile, made up of myositis-specific and myositis-associated (MSA and MAA) antibodies, can predict the clinical phenotype and progression over time. This review will focus on the characteristics and management of antisynthetase syndrome related ILD and anti-MDA5 positive ILD, which are the most clinically relevant subtypes.

RECENT FINDINGS:

The prevalence of ILD in IIM has been estimated in Asia, North America and Europe at 50, 23 and 26%, respectively, and is increasing. In antisynthetase syndrome related ILD, the clinical presentation, progression and prognosis varies among anti-ARS antibodies. ILD is more common and severe in patients with anti-PL-7/anti-PL-12 antibodies when compared with anti Jo-1 patients. The prevalence of anti-MDA5 antibodies is higher in Asians (11-60%) than in whites (7-16%). Sixty-six percent of antisynthetase syndrome patients had 'chronic ILD' compared with the more rapidly progressive ILD (RP-ILD) seen in 69% of patients with anti-MDA5 antibodies.

SUMMARY:

ILD is most common in the antisynthetase subtype of IIM and can be a chronic indolent or RP- ILD. The MSA and MAAs are associated with different clinical phenotypes of ILD. Treatments typically involve combinations of corticosteroids and other immunosuppressants.

• Authors: Trang T T Vu, Kevin K Brown, Joshua J Solomon
• Journal: Current opinion in pulmonary medicine (Curr Opin Pulm Med) Vol. 29 Issue 5 Pg. 427-435 (09 01 2023) ISSN: 1531-6971 [Electronic] United States
• PMID: 37435671 (Publication Type: Review, Journal Article)
• Copyright: Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.

Chemical References

• Autoantibodies
• Immunosuppressive Agents

Topics

• Humans
• Myositis (complications, drug therapy)
• Autoantibodies
• Lung Diseases, Interstitial (etiology, complications)
• Immunosuppressive Agents