{Reference Type}: Journal Article
{Title}: Muscle involvement in systemic sclerosis: high mortality not associated with nature of histological lesions.
{Author}: Gouellec NL;Zaidan L;Chaigne B;Periou B;Cailliau E;Dhote R;Riviere S;Uzunhan Y;Agard C;Godeau B;Wolkenstein P;Hachulla E;Mouthon L;Authier J;
{Journal}: Rheumatology (Oxford)
{Volume}: 0
{Issue}: 0
{Year}: 2024 Aug 13
{Factor}: 7.046
{DOI}: 10.1093/rheumatology/keae407
{Abstract}: <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.