Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital (CMCH), Vellore, Tamil Nadu, 632002, India.
Department of Bio-statistics, CMCH, Vellore, Tamil Nadu, 632002, India.
Clin Rheumatol. 2021 Jul;40(7):2805-2819. doi: 10.1007/s10067-021-05590-1. Epub 2021 Jan 27.
Consensus on treatment of idiopathic inflammatory myositis (IIM), particularly with regard to flares and interstitial lung disease (ILD), does not exist. We studied the long-term outcome and treatment response in our large, retrospective cohort of adult South-Asian patients exclusively with IIM.
Electronic records of IIM patients satisfying inclusion and exclusion criteria were studied longitudinally at presentation, at 3, 6, 12, 18 and 24 months and thereafter yearly till their last follow up (F/u) visit. Depending on clinical, imaging, and muscle enzyme profile during the F/u period, patients were categorised as complete (CR) and partial responders (PRs). Parameters favouring CR were assessed using multivariate logistic regression analysis. Outcome parameters and flares on immunosuppressants (IS) were then assessed in patients with/without ILD.
Two hundred thirty-two patients with median F/u duration of 44.5 months (25-80.25) were included. ILD was seen in 40.1%. Patients with non-Jo1 anti-synthetase antibodies (n=26) were numerically more than those with Jo-1 antibody (n=24). CR status was attained by 50.9% patients. Absence of pericardial effusion (p=0.042, OR 4.223, 95% CI: 1.05-16.9) and presence of Gottron's rash (p=0.044, OR 1.78, 95% CI 1.017-3.121) at baseline predicted CR by multivariate regression. Majority received mycophenolate during the entire F/u period. Discontinuation of steroids was feasible in 51.7% after a median duration of 24 months (18-42). After excluding patients with ILD, flares were numerically lesser in patients only on mycophenolate compared with those only on methotrexate (p=0.06). Further flares were curtailed when switched from other agents to mycophenolate.
Mycophenolate is an effective treatment option in IIM patients with and without co-existing ILD. Presence of Gottron's rash and absence of pericardial effusion were found to be predictors of favourable clinical outcome in this largest single-centre study.
特发性炎性肌病(IIM)的治疗共识,特别是关于发作和间质性肺病(ILD)的共识尚不存在。我们研究了我们的大型回顾性成年南亚患者队列中特发性炎性肌病患者的长期结果和治疗反应。
对符合纳入和排除标准的特发性炎性肌病患者的电子病历进行纵向研究,在就诊时、3、6、12、18 和 24 个月以及之后每年进行随访,直至最后一次随访(F/u)就诊。根据 F/u 期间的临床、影像学和肌肉酶谱,将患者分为完全缓解(CR)和部分缓解(PR)。使用多变量逻辑回归分析评估有利于 CR 的参数。然后评估有无ILD 的患者的缓解参数和免疫抑制剂(IS)发作情况。
共纳入 232 例患者,中位随访时间为 44.5 个月(25-80.25)。ILD 发生率为 40.1%。非 Jo1 抗合成酶抗体(n=26)患者数量多于 Jo-1 抗体(n=24)患者。50.9%的患者达到 CR 状态。多变量回归分析显示,基线时无心包积液(p=0.042,OR 4.223,95%CI:1.05-16.9)和存在戈特龙皮疹(p=0.044,OR 1.78,95%CI 1.017-3.121)预测 CR。大多数患者在整个 F/u 期间接受霉酚酸酯治疗。中位时间为 24 个月(18-42)后,51.7%的患者可停用类固醇。排除ILD 患者后,与仅接受甲氨蝶呤的患者相比,仅接受霉酚酸酯的患者的发作次数更少(p=0.06)。从其他药物转换为霉酚酸酯后,进一步减少了发作次数。
霉酚酸酯是一种有效的治疗特发性炎性肌病的药物,无论是否存在合并的ILD。在这项最大的单中心研究中,发现存在戈特龙皮疹和无心包积液是有利临床结局的预测因素。