An Observational Study on the Clinical Characteristics and Prognosis of Patients With Interstitial Lung Disease Secondary to Dermatomyositis and Antisynthetase Syndrome.

IF 2.3 Q2 RHEUMATOLOGY International Journal of Rheumatology Pub Date : 2024-09-26 eCollection Date: 2024-01-01 DOI:10.1155/2024/9679944
Ling Lei, Zongbo Ma, Xuejia Ma, Dongmei Pan, Zhanrui Chen, Fang Qin, Fei Dong
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Abstract

Objective: Identify the clinical characteristics and prognostic factors in patients with idiopathic inflammatory myopathy (IIM) combined with interstitial lung disease (ILD). Methods: IIM-ILD patients who were hospitalized at Guangxi Medical University from January 2017 to December 2022 were retrospectively analyzed and classified as having dermatomyositis (DM)-ILD or -ILD. Clinical and laboratory results were analyzed. Results: There were 39 males and 111 females, the mean age of disease onset was 50.4 ± 12.3 years, and the median disease duration was 3 months (range: 1-6). Ninety-seven patients had DM-ILD, and 53 had ASS-ILD. The DM-ILD group had 72% positivity for the anti-MDA5 antibody and 5.2% positivity for the anti-Mi-2 antibody; the ASS-ILD group had 67.9% positivity for the anti-Jo-1 antibody and 17% positivity for the anti-EJ antibody. Muscle symptoms, skin ulcers, rash, rapidly progressing interstitial lung disease (RP-ILD), and elevated levels of serum carcinoembryonic antigen were more common in DM-ILD patients (all p < 0.05). However, pericardial effusion and pleural effusion, elevated creatinine kinase, and elevated C-reactive protein were more common in ASS-ILD patients. After a median follow-up of 15.5 months, there were more deaths in the DM-ILD group (42.3% vs. 13.2%, p < 0.001). Multivariate Cox regression analysis showed that RP-ILD, dyspnea, and the usual interstitial pneumonia type of ILD had negative associations with overall survival (OS), and arthralgia had a positive association with OS (all p < 0.05). Conclusion: DM-ILD patients were more prone to secondary RP-ILD and skin ulcers, had milder symptoms of myositis and less severe serositis, and had lower survival rates than the ASS-ILD patients. RP-ILD, dyspnea, and the usual interstitial pneumonia type of ILD had adverse effects on prognosis, but arthralgia was a protective factor.

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皮肌炎和抗肌酸激酶综合征间质性肺病患者临床特征和预后的观察性研究
目的确定特发性炎症性肌病(IIM)合并间质性肺病(ILD)患者的临床特征和预后因素。方法回顾性分析2017年1月至2022年12月在广西医科大学住院治疗的特发性炎症性肌病(IIM-ILD)患者,并将其分为皮肌炎(DM)-ILD或-ILD。对临床和实验室结果进行分析。结果男性39例,女性111例,平均发病年龄为(50.4±12.3)岁,中位病程为3个月(1-6个月)。97名患者为DM-ILD,53名患者为ASS-ILD。DM-ILD组的抗MDA5抗体阳性率为72%,抗Mi-2抗体阳性率为5.2%;ASS-ILD组的抗Jo-1抗体阳性率为67.9%,抗EJ抗体阳性率为17%。肌肉症状、皮肤溃疡、皮疹、快速进展的间质性肺病(RP-ILD)和血清癌胚抗原水平升高在 DM-ILD 患者中更为常见(所有数据均小于 0.05)。然而,心包积液和胸腔积液、肌酸激酶升高和 C 反应蛋白升高在 ASS-ILD 患者中更为常见。中位随访15.5个月后,DM-ILD组的死亡人数更多(42.3%对13.2%,P<0.001)。多变量 Cox 回归分析显示,RP-ILD、呼吸困难和常见的间质性肺炎类型的 ILD 与总生存期(OS)呈负相关,而关节痛与总生存期呈正相关(均 p < 0.05)。结论与 ASS-ILD 患者相比,DM-ILD 患者更容易继发 RP-ILD 和皮肤溃疡,肌炎症状较轻,血清炎症状较轻,生存率较低。RP-ILD、呼吸困难和常见的间质性肺炎型 ILD 对预后有不利影响,但关节痛是一个保护因素。
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来源期刊
CiteScore
4.40
自引率
0.00%
发文量
9
审稿时长
24 weeks
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