| Title | Peripheral lymphocyte count defines the clinical phenotypes and prognosis in patients with anti-MDA5-positive dermatomyositis |
| Authors | Jin, Qiwen Fu, Lijun Yang, Hongxia Chen, Xixia Lin, Sang Huang, Zhenguo Gao, Baoxiang Tian, Xiaolan Jiang, Wei Shu, Xiaoming Lu, Xin Wang, Guochun Peng, Qinglin |
| Affiliation | Peking Univ, China Japan Friendship Sch Clin Med, Beijing, Peoples R China China Japan Friendship Hosp, Dept Rheumatol, Key Lab Myositis, Beijing, Peoples R China Southwestern Univ Finance & Econ, Sch Finance, Chengdu, Peoples R China Chinese Acad Med Sci, Peking Union Med Coll, Grad Sch, Beijing, Peoples R China China Japan Friendship Hosp, Dept Radiol, Beijing, Beijing, Peoples R China China Japan Friendship Hosp, Beijing Key Lab Immune Mediated Inflammatory Dis, Beijing, Peoples R China Peking Univ China, China Japan Friendship Hosp, China Japan Friendship Sch Clin Med, Key Lab Myositis,Dept Rheumatol, Yinghua East Rd, Beijing 100029, Peoples R China |
| Keywords | INTERSTITIAL LUNG-DISEASE GENE 5 ANTIBODY CLASSIFICATION POLYMYOSITIS MIGRATION PATTERNS |
| Issue Date | Feb-2023 |
| Publisher | JOURNAL OF INTERNAL MEDICINE |
| Abstract | ObjectiveTo explore the role of peripheral lymphocyte count in phenotyping and prognosis prediction in dermatomyositis (DM) patients with anti-MDA5 antibodies. MethodsIn total, 1669 patients with idiopathic inflammatory myopathy (IIM) were retrospectively enrolled. Clinical characteristics and prognosis of patients with anti-MDA5+ DM were analyzed in association with peripheral lymphocyte counts and clusters determined by unsupervised machine learning. ResultsThe peripheral lymphocyte count was significantly lower in the anti-MDA5+ DM group (N = 421) than in the other IIM serotype groups. The anti-MDA5+ DM patients were divided into three groups; the severe lymphopenia group had skin ulcers and rapidly progressive interstitial lung disease (RP-ILD); patients with a normal lymphocyte count had a younger age of onset, more frequent arthritis, and normal serum ferritin levels, whereas mild lymphopenia group showed a moderate increase of serum ferritin and intermediate incidence of RP-ILD. Survival analysis revealed that the 3- and 6-month mortality rates were significantly higher in the severe lymphopenia group (29.0% and 42.1%, respectively) than in the mild lymphopenia group and normal lymphocyte count group (p value <0.001). Consistently, unsupervised machine learning identified three similar groups; the arthritis cluster shows the highest lymphocyte counts and best prognosis; the RP-ILD cluster presents the lowest peripheral lymphocyte, high incidence of RP-ILD, and poor prognosis; the typical DM rash cluster had a moderate peripheral lymphocyte count and an intermediate prognosis. ConclusionsLymphopenia is a unique manifestation of anti-MDA5+ DM. Peripheral lymphocyte count can define clinical phenotypes and predict prognosis in anti-MDA5+ DM. |
| URI | http://hdl.handle.net/20.500.11897/670339 |
| ISSN | 0954-6820 |
| DOI | 10.1111/joim.13607 |
| Indexed | SCI(E) |
| Appears in Collections: | 中日友好医院 |
