| Title | The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients |
| Authors | Chen, Xixia Zhang, Lu Jin, Qiwen Lu, Xin Lei, Jieping Peng, Qinglin Wang, Guochun Ge, Yongpeng |
| Affiliation | Peking Univ, China Japan Friendship Sch Clin Med, Beijing, Peoples R China China Japan Friendship Hosp, Dept Rheumatol, Beijing, Peoples R China China Japan Friendship Hosp, Inst Clin Med Sci, Data & Project Management Unit, Beijing, Peoples R China China Japan Friendship Hosp, Dept Rheumatol, Beijing Key Lab Immune Mediated Inflammatory Dis, Beijing, Peoples R China |
| Keywords | INTERSTITIAL LUNG-DISEASE GENE 5 MACROPHAGE ACTIVATION POLYMYOSITIS ASSOCIATION PREDICTION CT |
| Issue Date | 30-Aug-2022 |
| Publisher | FRONTIERS IN IMMUNOLOGY |
| Abstract | ObjectiveTo 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. MethodsWe 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. ResultsSix 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. ConclusionPresentation 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. |
| URI | http://hdl.handle.net/20.500.11897/654527 |
| ISSN | 1664-3224 |
| DOI | 10.3389/fimmu.2022.987841 |
| Indexed | SCI(E) |
| Appears in Collections: | 中日友好医院 |
