Systemic sclerosis & Raynaud's
Fibrosing myopathy in systemic sclerosis: Treatment response and clinical outcomes in a distinct subset
Authors' conclusion
SSc-associated fibrosing myopathy is a distinct entity associated with a high mortality rate in our cohort of patients.
Study context
- Population
- Patients in the Johns Hopkins Scleroderma Center Research Registry with histopathological evidence of systemic sclerosis (SSc)-associated fibrosing myopathy
- Participants
- 16 patients
- Outcome
- Treatment response based on muscle strength testing
- Follow-up
- 6-12 months after treatment initiation
Methodological detailssingle-centre registry
- Single-centre registry
Main finding
As reported in the abstract
Significant improvement in muscle strength at 6-12 months after treatment initiation
9/16 patients
56.3%
No significant improvement
4 of 16
25%
Inadequate follow-up
3 of 16
18.8%
Observational data: associations, not effects.
Further findings
Received treatment specifically for muscle disease
- Treated for muscle disease
- 14/16 patients (87.5%)
- Most common treatments: IV immunoglobulin, mycophenolate, rituximab
Mortality · mean follow-up 5.5 ± 4.7 years
- Deceased
- 8/16 patients (50%)
- Cardiopulmonary cause of death among deceased
- 4/8 (50%)
Baseline CK
- Median CK (U/L), IQR 46-300 U/L
- 97
Muscle investigations
- Myopathic changes on electromyography (EMG) (of 15 with EMG)
- 14/15 patients (93.3%)
- Muscle edema on magnetic resonance imaging (MRI) (of 12 with MRI)
- 11/12 patients (91.7%)
- Perifascicular myofiber atrophy on histopathology
- 9/16 patients (56.3%)
Original publication
Fibrosing myopathy in systemic sclerosis: Treatment response and clinical outcomes in a distinct subset. Semin Arthritis Rheum. Published 20 June 2026. doi:10.
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