Peking University China-Japan Friendship School of clinical medicine, Beijing, China.
Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.
Semin Arthritis Rheum. 2024 Oct;68:152536. doi: 10.1016/j.semarthrit.2024.152536. Epub 2024 Aug 10.
To analyze the clinical features of idiopathic inflammatory myopathies (IIMs) patients with anti-PM/Scl antibodies.
In this retrospective cohort study, we compared the clinical manifestations between patients who were solely positive for anti-PM/Scl antibodies (isolated anti-PM/Scl group) and those with a coexistence of anti-PM/Scl antibodies and myositis-specific antibodies (MSAs) (double-positive group).
Sixty-five IIMs patients positive for anti-PM/Scl antibodies were included, among whom 51 (78.5 %) were females, with a mean age of 49.1 years. Thirty-four (52.3 %) patients coexisted with MSAs. Compared to the double-positive group, the isolated anti-PM/Scl group demonstrated a higher proportion of women (90.3 % vs 67.6 %, p = 0.026) and a higher incidence of sclerodactyly (16.1 % vs 0, p = 0.021). Although there were no differences in the incidence of muscular weakness, dysphagia, or creatine kinase levels, thigh magnetic resonance imaging (MRI) revealed less muscle edema, atrophy, and fatty replacement in the isolated anti-PM/Scl group (p < 0.05). Interstitial lung disease (ILD) occurred in 80 % of patients, more frequently in the double-positive group (90.6 % vs 67.9 %, p = 0.028). According to HRCT, non-specific interstitial pneumonia (NSIP) was the most common pattern among anti-PM/Scl antibodies positive IIMs patients. The double-positive group exhibited higher ferritin levels, and a lower peripheral lymphocyte count (p < 0.05). The mortality rate in the double-positive group was higher than that in the isolated anti-PM/Scl group (20.6 % vs 0, p = 0.034).
Among IIMs patients who tested positive for anti-PM/Scl antibodies, ILD emerged as the predominant clinical feature, particularly when combined with MSA. Notably, patients with isolated anti-PM/Scl antibodies exhibited a favorable prognosis following immunotherapy.
分析抗 PM/Scl 抗体阳性的特发性炎性肌病（IIM）患者的临床特征。
在这项回顾性队列研究中，我们比较了仅抗 PM/Scl 抗体阳性的患者（单纯抗 PM/Scl 组）和同时存在抗 PM/Scl 抗体和肌炎特异性抗体（MSA）的患者（双阳性组）的临床表现。
共纳入 65 例抗 PM/Scl 抗体阳性的 IIM 患者，其中 51 例（78.5%）为女性，平均年龄为 49.1 岁。34 例（52.3%）患者同时存在 MSA。与双阳性组相比，单纯抗 PM/Scl 组女性比例更高（90.3%比 67.6%，p=0.026），硬皮病发生率更高（16.1%比 0，p=0.021）。尽管两组肌肉无力、吞咽困难或肌酸激酶水平无差异，但大腿 MRI 显示单纯抗 PM/Scl 组肌肉水肿、萎缩和脂肪替代程度较低（p<0.05）。间质性肺病（ILD）在 80%的患者中发生，双阳性组更为常见（90.6%比 67.9%，p=0.028）。根据高分辨率 CT（HRCT），非特异性间质性肺炎（NSIP）是抗 PM/Scl 抗体阳性的 IIM 患者最常见的模式。双阳性组铁蛋白水平较高，外周淋巴细胞计数较低（p<0.05）。双阳性组的死亡率高于单纯抗 PM/Scl 组（20.6%比 0，p=0.034）。
在抗 PM/Scl 抗体阳性的 IIM 患者中，ILD 是主要的临床特征，尤其是与 MSA 同时存在时。值得注意的是，接受免疫治疗后，单纯抗 PM/Scl 抗体患者的预后较好。
