Lia Yihua, Fana Yali, Wanga Yuanying, Yanga Shuqiao, Dua Xuqin, Yea Qiao
Clinical Center for Interstitial Lung Diseases, Department of Occupational Medicine and Toxicology, Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Sarcoidosis Vasc Diffuse Lung Dis. 2022;38(4):e2021047. doi: 10.36141/svdld.v38i4.11368. Epub 2022 Jan 13.
Idiopathic inflammatory myopathy (IIM) is highly combined with interstitial pneumonia (IP), often as the initial or solo presentation with positive myositis-specific autoantibodies (MSAs) but does not fulfill the diagnostic criteria.
We aimed to explore the phenotypic clusters and prognosis of the patients with IP and positive MSA, which is called MSA-IP in the present study.
A total of 178 patients with MSA-IP were prospectively enrolled for analysis. Serum MSAs were detected using Western blotting. Radiological patterns of IP were determined according to the classification of idiopathic IPs. Clusters of patients with MSA-IP were identified using cluster analysis. Predictors for acute/subacute onset, therapeutic response, IP progression and survival were also analyzed.
Patients with MSA-IP were classified into four distinct clusters. Cluster 1 were the elderly with chronic onset, nearly normal oxygenation and good survival. Cluster 2 had dyspnea on exertion and nonspecific IP pattern, with moderate survival. Patients in cluster 3 had chronic onset and were prone to IP progression (OR 2.885). Cluster 4 had multi-systemic involvements, positive anti-melanoma differentiation associated gene 5 antibody, and were prone to acute/subacute onset (OR 3.538) and IP progression (OR 5.472), with poor survival. Corticosteroids combined immunosuppressants showed therapeutic response in MSA-IP (OR 4.303) and had a protective effect on IP progression (OR 0.136).
Four clusters of the patients with MSA-IP suggested the distinct clinical, radiological and prognostic features.
特发性炎性肌病（IIM）与间质性肺炎（IP）高度相关，常以肌炎特异性自身抗体（MSA）阳性作为首发或唯一表现，但不符合诊断标准。
本研究旨在探讨IP伴MSA阳性患者（称为MSA-IP）的表型聚类和预后。
前瞻性纳入178例MSA-IP患者进行分析。采用蛋白质印迹法检测血清MSA。根据特发性IP的分类确定IP的影像学模式。采用聚类分析确定MSA-IP患者的聚类。还分析了急性/亚急性起病、治疗反应、IP进展和生存的预测因素。
MSA-IP患者分为四个不同的聚类。聚类1为老年患者，起病慢性，氧合近乎正常，生存良好。聚类2患者运动时呼吸困难，IP模式不特异，生存中等。聚类3患者起病慢性，易于IP进展（比值比2.885）。聚类4患者多系统受累，抗黑色素瘤分化相关基因5抗体阳性，易于急性/亚急性起病（比值比3.538）和IP进展（比值比5.472），生存较差。糖皮质激素联合免疫抑制剂对MSA-IP有治疗反应（比值比4.303），对IP进展有保护作用（比值比0.136）。
MSA-IP患者的四个聚类提示了不同的临床、影像学和预后特征。
