Objectives The aim of this study was to determine the association between various histological patterns and prognosis in patients with SSc and histologically proven muscle involvement. Methods A multicentre retrospective study was conducted of a cohort of scleroderma patients who had undergone muscle biopsy. The biopsies were reviewed in a coordinated manner to classify patients based on the histological findings. Three different patterns were observed: fibrosing myopathy (FMy), inflammatory myopathy (IMy), and immune-mediated necrotizing myopathy (IMNMy). Rates of survival, muscle relapse, and cardiac and pulmonary events were compared between these three groups. Results Among the 71 scleroderma patients with muscle biopsy specimens available for review, 33 (46.5%) were classified in the FMy group, 18 (25.5%) in the IMy group and 20 (28%) in the NMy group. The median follow-up time was 6.4 years (interquartile range, 2.2–10.9 years), and 21 patients died during follow-up, primarily from heart disease and infections. The 10-year survival rate after the first non-RP symptom was 80%, and the cumulative incidence of muscle relapse was 25%. Neither factor differed significantly between the three groups. The risk of pulmonary events was lowest in the IMy group, significantly lower than in the FMy group (hazard ratio, 0.17; 95% CI, 0.04–0.67) and non-significantly lower than in the IMNMy group (hazard ratio, 0.28; 95% CI, 0.06–1.24). The risk of cardiac events did not differ significantly between the three groups. Conclusion The mortality rate in scleroderma patients with muscle involvement was not associated with their histological patterns.

中文翻译：

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系统性硬化症中的肌肉受累：高死亡率与组织学病变的性质无关


目的 本研究的目的是确定 SSc 患者各种组织学模式与预后之间的关联，组织学证实的肌肉受累。方法 对一组接受肌肉活检的硬皮病患者进行了多中心回顾性研究。以协调的方式审查活检，以根据组织学结果对患者进行分类。观察到三种不同的模式：纤维化肌病 （FMy） 、炎症性肌病 （IMy） 和免疫介导的坏死性肌病 （IMNMy）。比较这三组的生存率、肌肉复发率以及心肺事件。结果 71 例有肌肉活检标本的硬皮病患者中，33 例 （46.5%） 归类为 FMy 组，18 例 （25.5%） 归类为 IMy 组，20 例 （28%） 归为 NMy 组。中位随访时间为 6.4 年 （四分位距，2.2-10.9 年），21 名患者在随访期间死亡，主要死于心脏病和感染。首次非 RP 症状后的 10 年生存率为 80%，肌肉复发的累积发生率为 25%。三组之间没有显著差异。IMy 组发生肺部事件的风险最低，显著低于 FMy 组 （风险比，0.17;95% CI，0.04-0.67），无统计学意义低于 IMNMy 组 （风险比，0.28;95% CI，0.06-1.24）。三组之间发生心脏事件的风险无显著差异。结论 肌肉受累硬皮病患者的死亡率与其组织学模式无关。