Abstract：
OBJECTIVE: 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.
CONCLUSIONS: The mortality rate of scleroderma patients with muscle involvement was not associated with their histological patterns.
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.
CONCLUSIONS: The mortality rate of scleroderma patients with muscle involvement was not associated with their histological patterns.
摘要：
目的：本研究的目的是确定SSc和组织学证实的肌肉受累患者的不同组织学模式与预后之间的关系。
方法：对接受过肌肉活检的硬皮病患者进行了一项多中心回顾性研究。活检以协调的方式进行审查，以根据组织学发现对患者进行分类。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）。生存率,肌肉复发，比较三组间的心脏和肺事件。
结果：在71例硬皮病患者中，肌肉活检标本可供检查，33人(46.5%)被归类为FM组，IM组中18人(25.5%)，NM组中有20人(28%)。中位随访时间为6.4年(四分位距，2.2-10.9年）和21例患者在随访期间死亡，主要来自心脏病和感染。首次出现非雷诺疾病症状后的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。OM组发生肺部事件的风险最低，显著低于FM组(风险比，0.17；95%CI，0.04-0.67）且非显著低于IMNM组（风险比，0.28；95%CI，0.06-1.24）。三组之间的心脏事件风险没有显着差异。
结论：肌肉受累硬皮病患者的死亡率与其组织学模式无关。
方法：对接受过肌肉活检的硬皮病患者进行了一项多中心回顾性研究。活检以协调的方式进行审查，以根据组织学发现对患者进行分类。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）。生存率,肌肉复发，比较三组间的心脏和肺事件。
结果：在71例硬皮病患者中，肌肉活检标本可供检查，33人(46.5%)被归类为FM组，IM组中18人(25.5%)，NM组中有20人(28%)。中位随访时间为6.4年(四分位距，2.2-10.9年）和21例患者在随访期间死亡，主要来自心脏病和感染。首次出现非雷诺疾病症状后的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。OM组发生肺部事件的风险最低，显著低于FM组(风险比，0.17；95%CI，0.04-0.67）且非显著低于IMNM组（风险比，0.28；95%CI，0.06-1.24）。三组之间的心脏事件风险没有显着差异。
结论：肌肉受累硬皮病患者的死亡率与其组织学模式无关。
