Autoinflammatory & rare systemic diseases
Symptomatic myopathies in sarcoidosis: disease spectrum and myxovirus resistance protein A expression
Authors' conclusion
Symptomatic myopathy occurred in 0.36% of sarcoidosis. IBM was the second most common cause of myopathies after ScM. Frequency of IBM in sarcoidosis is higher than in the general population.
Study context
- Population
- Sarcoidosis patients with myopathic symptoms and pathological evidence of myopathy, Mayo Clinic database (May 1980-December 2020)of 5885 sarcoidosis patients
- Participants
- 21 patients
- Exposure
- Sarcoid myopathy (ScM) vs non-sarcoid myopathies
- ScMn = 11
- Outcome
- Symptomatic myopathy spectrum
Methodological detailssingle-centre database review
- Single-centre database review
Main finding
As reported in the abstract
- Symptomatic myopathy among sarcoidosis patients
- 21 of 5885
- ScM
- 11
- IBM
- 5
- Immune-mediated necrotizing myopathy
- 1
- Non-specific myositis
- 1
- Non-specific myopathy
- 2
- Steroid myopathy
- 1
- Estimated frequency of IBM per 100 000 sarcoidosis patients
- 85
Observational data: associations, not effects.
Further findings
- Improvement in modified Rankin scale (mRS) ≥1 with immunosuppressive therapy · none of the five IBM improved — ScM
- 5/10 events
- Improvement in mRS ≥1 with immunosuppressive therapy: IBM 0 events
- Improvement in mRS ≥1 with immunosuppressive therapy: Remaining non-sarcoid myopathies 3/3 events
Features associated with non-sarcoid myopathies
- Predominant finger flexor and quadriceps weakness; mRS >2 at diagnosis; creatine kinase >500 U/l; absence of intramuscular granulomas
- P < 0.05
Sarcoplasmic MxA expression in scattered myofibres
- Patients with MxA expression
- 3
- Tested for DM-specific autoantibodies, negative
- 2
Sarcoidosis diagnosis prior to myopathy onset
- Patients with prior sarcoidosis diagnosis
- 8
- Median years before myopathy onset
- 5.5
Original publication
Symptomatic myopathies in sarcoidosis: disease spectrum and myxovirus resistance protein A expression. Rheumatology (Oxford). Published 28 November 2022. doi:10. 1093/ rheumatology/ keac668
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