Objectives The aim of this study was to determine the association between different 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 histological findings. Three different patterns were observed: fibrosing myopathy (FM), inflammatory myopathy (IM) and necrotizing myopathy (NM). Rates of survival, muscle relapse, and cardiac and pulmonary events were compared between these three groups. Results Among 71 scleroderma patients with muscle biopsy specimens available for review, 33 (46.5%) were classified in the FM group, 18 (25.5%) in the IM group, and 20 (28%) in the NM 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-Raynaud’s disease 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 OM group, significantly lower than in the FM group (hazard ratio, 0.17; 95% CI, 0.04–0.67) and non-significantly lower than in the IMNM 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 of 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 患者的不同组织学模式和预后之间的关联以及经组织学证实的肌肉受累。方法对一组接受过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，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（FM）、炎性肌病（IM）和坏死性肌病（NM）。比较这三组之间的生存率、肌肉复发率以及心脏和肺部事件。结果 71 例有肌肉活检标本可供复查的硬皮病患者中，FM 组 33 例（46.5%），IM 组 18 例（25.5%），NM 组 20 例（28%）。中位随访时间为 6.4 年（四分位数间距，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。首次出现非雷诺氏病症状后的 10 年生存率为 80%，肌肉复发的累积发生率为 25%。这两个因素在三组之间都没有显着差异。 OM 组肺部事件的风险最低，显着低于 FM 组（风险比，0.17；95% CI，0.04-0.67），但不显着低于 IMNM 组（风险比，0.28；95） % CI，0.06–1.24）。三组之间心脏事件的风险没有显着差异。结论 肌肉受累的硬皮病患者的死亡率与其组织学类型无关。