The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients.

Abstract

Objective:

To explore the clinical features and prognoses of dermatomyositis (DM) associated with a double-positive anti-MDA5 and anti-aminoacyl-tRNA synthetase (anti-ARS) antibody presentation.

Methods:

We retrospectively analyzed 1280 consecutive patients with idiopathic inflammatory myopathy (IIM). Individuals with anti-MDA5 and anti-ARS antibodies (anti-MDA5+/ARS+) were compared to anti-MDA5-/ARS+ and anti-MDA5+/ARS- control individuals based on clinical, pulmonary radiological characteristics, treatment, and follow-up information.

Results:

Six individuals (0.47%) presented with anti-MDA5+/ARS+; of these, 2 (33.3%) were anti-PL-12+, 2 (33.3%) were anti-Jo-1+, 1 (16.7%) was anti-EJ+, and 1 (16.7%) was anti-PL-7+. Hallmark cutaneous manifestations, including Gottron's sign (100%), heliotrope rash (50%), mechanic's hand (66.7%), and skin ulcers (16.7%) were common. Anti-MDA5+/ARS+ patients tended to have higher ferritin levels (p = 0.038) than anti-MDA5-/ARS+ group, and higher CD4+ T-cell counts (p = 0.032) compared to the anti-MDA5+/ARS- group. Radiologically, NSIP with OP overlap was predominant (60%). Consolidation (60%), ground-glass attenuation (GGA) (80%), traction bronchiectasis (80%), and intralobular reticulation (100%) were common in anti-MDA5+/ARS+ individuals. All were diagnosed with ILD and 50% were categorized as RPILD. All patients received glucocorticoids combined with one or more immunosuppressants. Most (83.3%) had a good prognosis following treatment, but there was no difference in the survival rate between the three subgroups.

Conclusion:

Presentation with anti-MDA5+/ARS+ DM was rare. The clinical and radiological characteristics of anti-MDA5+/ARS+ DM combined the features of anti-MDA5+ and anti-ARS+ individuals. Individuals with anti-MDA5+/ARS+ antibodies may respond well to glucocorticoid therapy; glucocorticoids combined with one or more immunosuppressants may be considered a basic treatment approach.

• Authors: Xixia Chen, Lu Zhang, Qiwen Jin, Xin Lu, Jieping Lei, Qinglin Peng, Guochun Wang, Yongpeng Ge
• Journal: Frontiers in immunology (Front Immunol) Vol. 13 Pg. 987841 ( 2022) ISSN: 1664-3224 [Electronic] Switzerland
• PMID: 36110863 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2022 Chen, Zhang, Jin, Lu, Lei, Peng, Wang and Ge.

Chemical References

• Autoantibodies
• Glucocorticoids
• Immunosuppressive Agents
• Ferritins
• Amino Acyl-tRNA Synthetases

Topics

• Amino Acyl-tRNA Synthetases
• Autoantibodies
• Dermatomyositis (diagnosis, drug therapy)
• Ferritins
• Glucocorticoids
• Humans
• Immunosuppressive Agents
• Lung Diseases, Interstitial (complications, diagnosis, drug therapy)
• Prognosis
• Retrospective Studies