Ge Yongpeng, Shu Xiaoming, He Linrong, Li Chunjia, Lu Xin, Wang Guochun
Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.
Front Med (Lausanne). 2022 Jan 18;8:778211. doi: 10.3389/fmed.2021.778211. eCollection 2021.
This study aimed to analyze the clinical features of anti-PM/Scl antibodies in Chinese patients.
We reviewed the clinical data of anti-PM/Scl antibody-positive patients, including their long-term follow-up.
A total of 30 patients carried anti-PM/Scl antibodies, 21 (70%) were females, and the mean age was 55.4 years, 15 (50%) and 10 (33.3%) patients were positive for anti-PM/Scl-75 and anti-PM/Scl-100, respectively. Fifteen cases (50%) were diagnosed as inflammatory myopathy, namely, 11 dermatomyositis (DM) and 4 polymyositis (PM). Five (16.7%) patients were diagnosed with overlap syndrome, and only one (3.3%) was diagnosed as systemic sclerosis. The other 9 (30%) patients were classified as undifferentiated connective tissue disease. Twenty-six (86.7%) had interstitial lung disease (ILD) and was the sole manifestation in 8 (26.7%) patients, 15 (58.0%) showed non-specific interstitial pneumonia based on high-resolution CT or lung biopsy. The majority of patients (95%) with mild and moderate groups on basis of pulmonary function tests. Compared to the anti-PM/Scl-100 group, the occurrence of clinical characteristics was not significantly different from the anti-PM/Scl-75 group, except the levels of C-reactive protein and erythrocyte sedimentation rate in the anti-PM/Scl-75 antibody-positive group were higher ( < 0.05). All patients with positive Ro-52 antibodies had ILD and were more likely to develop skin rash in the group with Ro-52 ( = 0.024). With a follow-up of the present cohort, 70.8% improved with treatment, but 16.7% of patients are easy to relapse.
The anti-PM/Scl antibody occurred frequently in DM/PM patients, ILD was the major clinical feature, especially in patients combined with Ro-52. Some patients may complicate with ILD alone without extrapulmonary manifestations. Anti-PM/Scl antibodies positive patients were responsive to treatment.
本研究旨在分析中国患者抗PM/Scl抗体的临床特征。
我们回顾了抗PM/Scl抗体阳性患者的临床资料，包括他们的长期随访情况。
共有30例患者携带抗PM/Scl抗体，其中21例（70%）为女性，平均年龄为55.4岁，15例（50%）和10例（33.3%）患者分别抗PM/Scl-75和抗PM/Scl-100阳性。15例（50%）被诊断为炎性肌病，即11例皮肌炎（DM）和4例多发性肌炎（PM）。5例（16.7%）患者被诊断为重叠综合征，仅1例（3.3%）被诊断为系统性硬化症。其他9例（30%）患者被归类为未分化结缔组织病。26例（86.7%）有间质性肺疾病（ILD），其中8例（26.7%）患者以ILD为唯一表现，15例（58.0%）基于高分辨率CT或肺活检显示为非特异性间质性肺炎。根据肺功能测试，大多数患者（95%）为轻度和中度组。与抗PM/Scl-100组相比，抗PM/Scl-75组临床特征的发生率无显著差异，只是抗PM/Scl-75抗体阳性组的C反应蛋白和红细胞沉降率水平更高（<0.05）。所有Ro-52抗体阳性患者均有ILD，且Ro-52组更易出现皮疹（=0.024）。对本队列进行随访，70.8%的患者经治疗后病情改善，但16.7%的患者容易复发。
抗PM/Scl抗体在DM/PM患者中频繁出现，ILD是主要临床特征，尤其是合并Ro-52的患者。部分患者可能仅合并ILD而无肺外表现。抗PM/Scl抗体阳性患者对治疗有反应。
