Autoinflammatory & rare systemic diseases

Symptomatic myopathies in sarcoidosis: disease spectrum and myxovirus resistance protein A expression

Rheumatology (Oxford)Published Retrospective cohort study

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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