Muscle involvement in systemic sclerosis: high mortality not associated with nature of histological lesions

Noémie Le Gouellec; Louai Zaidan; Benjamin Chaigne; Baptiste Periou; Emeline Cailliau; Robin Dhote; Sebastien Riviere; Yurdagul Uzunhan; Christian Agard; Bertrand Godeau; Pierre Wolkenstein; Eric Hachulla; Luc Mouthon; Jerome Authier

doi:10.1093/rheumatology/keae407

Muscle involvement in systemic sclerosis: high mortality not associated with nature of histological lesions

Rheumatology (Oxford). 2024 Aug 13:keae407. doi: 10.1093/rheumatology/keae407. Online ahead of print.

Authors

Affiliations

¹ Department of nephrology and internal medicine, Hôpital de Valenciennes, Valenciennes, France.
² Department of histopathology, Hôpital Henri Mondor, Assistance Publique Hôpitaux de Paris (AP-HP) Paris Est-Creteil University, Paris, France.
³ Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, Cochin Hospital, AP-HP, CEDEX 14, Université Paris Cité, Paris, France.
⁴ Department of biostatistics, CHU de Lille, Lille, France.
⁵ Department of internal medicine, Hôpital Avicenne, AP-HP, Sorbonne Paris Nord University, Bobigny, France.
⁶ Department of internal medicine, Hôpital Saint Antoine, AP-HP-Sorbonne Université, Département Hospitalo-Universitaire Inflammation-Immunopathologie-Biotherapie (DMU i3), Paris, France.
⁷ Department of pulmonology, Hôpital Avicenne-APHP-Sorbonne Paris Nord University, Reference Center for Rare Pulmonary Diseases, Bobigny, France.
⁸ Department of internal medicine, National Referral Center for Rare Systemic Autoimmune and Autoinflammatory Diseases of Ile de France, East and West, University of Nantes, Nantes, France.
⁹ Department of internal medicine, Hôpital Henri Mondor, AP-HP, Paris Est-Creteil University, Paris, France.
¹⁰ Department of dermatology, Hôpital Henri Mondor, AP-HP, Paris Est-Creteil University, Paris, France.
¹¹ Department of internal medicine and Clinical Immunology, Referral Centre for Rare Systemic Autoimmune and Autoinflammatory Diseases, CHU Lille, University of Lille, Inserm, Lille, France.

PMID: 39137158
DOI: 10.1093/rheumatology/keae407

Abstract

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.

Keywords: fibrosing myopathy; immune-mediated necrotizing myopathy; inflammatory myopathy; mortality; muscle biopsy; systemic sclerosis.