%0 Journal Article
%T Muscle involvement in systemic sclerosis: high mortality not associated with nature of histological lesions.
%A Gouellec NL
%A Zaidan L
%A Chaigne B
%A Periou B
%A Cailliau E
%A Dhote R
%A Riviere S
%A Uzunhan Y
%A Agard C
%A Godeau B
%A Wolkenstein P
%A Hachulla E
%A Mouthon L
%A Authier J
%J Rheumatology (Oxford)
%V 0
%N 0
%D 2024 Aug 13
%M 39137158
%F 7.046
%R 10.1093/rheumatology/keae407
%X <B>OBJECTIVE: </B>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.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>The mortality rate of scleroderma patients with muscle involvement was not associated with their histological patterns.