Academic Journal
Fibrosing myopathy in systemic sclerosis: Treatment response and clinical outcomes in a distinct subset.
| Τίτλος: | Fibrosing myopathy in systemic sclerosis: Treatment response and clinical outcomes in a distinct subset. |
|---|---|
| Συγγραφείς: | Cameron S; Division of Rheumatology, University of Manitoba, Canada., Mohassel P; Division of Neuromuscular Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Shah AA; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Wigley F; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Hummers LK; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA., Paik JJ; Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address: jpaik1@jhmi.edu. |
| Πηγή: | Seminars in arthritis and rheumatism [Semin Arthritis Rheum] 2026 Oct; Vol. 80, pp. 153024. Date of Electronic Publication: 2026 Jun 20. |
| Τύπος έκδοσης: | Journal Article |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: W.B. Saunders Country of Publication: United States NLM ID: 1306053 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-866X (Electronic) Linking ISSN: 00490172 NLM ISO Abbreviation: Semin Arthritis Rheum Subsets: MEDLINE |
| Imprint Name(s): | Publication: Philadelphia Pa : W.B. Saunders Original Publication: New York, Stratton. |
| Ιατρικοί όροι (MeSH): | Scleroderma, Systemic*/complications , Scleroderma, Systemic*/drug therapy , Muscular Diseases*/drug therapy , Muscular Diseases*/etiology , Muscular Diseases*/pathology , Muscular Diseases*/mortality, Immunoglobulins, Intravenous/therapeutic use ; Mycophenolic Acid/therapeutic use ; Rituximab/therapeutic use ; Muscle, Skeletal/pathology ; Muscle, Skeletal/diagnostic imaging ; Humans ; Female ; Male ; Retrospective Studies ; Middle Aged ; Treatment Outcome ; Fibrosis ; Adult ; Magnetic Resonance Imaging ; Aged ; Electromyography ; Registries |
| Περίληψη: | Objective: To characterize the clinical features, treatment response, and mortality of patients with systemic sclerosis (SSc) associated fibrosing myopathy. Methods: In this retrospective study, we identified patients enrolled in the Johns Hopkins Scleroderma Center Research Registry with histopathological evidence of fibrosing myopathy. Data were collected regarding SSc features, treatment exposures, and outcomes. Muscle involvement was characterized using muscle enzyme levels, muscle magnetic resonance imaging (MRI) findings, electromyography (EMG) data, and histopathologic features. Results: 16 patients were included in the analysis. 14/16 patients (87.5%) received treatment specifically for muscle disease. Response based on muscle strength testing was evaluated at 6-12 months after treatment initiation. 9/16 patients (56.3%) had significant improvement, 4/16 (25%) showed no significant improvement, and 3/16 (18.8%) had inadequate follow-up. The most commonly used treatments were intravenous immunoglobulin, mycophenolate, and rituximab. After a mean follow-up time of 5.5 ± 4.7 years, 8/16 patients (50%) were deceased, with a cardiopulmonary cause of death in 4/8 (50%) of these patients. The baseline median CK value was 97 U/L (interquartile range 46-300 U/L). Myopathic changes were identified in 14/15 patients (93.3%) who had an EMG performed, while muscle edema was identified in 11/12 patients (91.7%) who underwent an MRI. A notable histopathological finding was myofiber atrophy in the perifascicular region in 9/16 patients (56.3%). Conclusion: SSc-associated fibrosing myopathy is a distinct entity associated with a high mortality rate in our cohort of patients. Early recognition and initiation of treatment is important due to the potential for improvement in muscle strength. (Copyright © 2026 Elsevier Inc. All rights reserved.) |
| Competing Interests: | Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. |
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| Grant Information: | K23 AR073927 United States AR NIAMS NIH HHS; K24 AR080217 United States AR NIAMS NIH HHS; P30 AR070254 United States AR NIAMS NIH HHS |
| Contributed Indexing: | Keywords: Myopathy; Scleroderma; Systemic sclerosis |
| Substance Nomenclature: | 0 (Immunoglobulins, Intravenous) HU9DX48N0T (Mycophenolic Acid) 4F4X42SYQ6 (Rituximab) |
| Entry Date(s): | Date Created: 20260630 Date Completed: 20260907 Latest Revision: 20260923 |
| Update Code: | 20260923 |
| PubMed Central ID: | PMC13518087 |
| DOI: | 10.1016/j.semarthrit.2026.153024 |
| PMID: | 42378828 |
| Βάση Δεδομένων: | MEDLINE |
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