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Abstract
Objective To refine the predictive significance of muscle granuloma in patients with myositis.
Methods A group of 23 patients with myositis and granuloma on muscle biopsy (granuloma-myositis) from 8 French and Belgian centers was analyzed and compared with (1) a group of 23 patients with myositis without identified granuloma (control-myositis) randomly sampled in each center and (2) a group of 20 patients with sporadic inclusion body myositis (sIBM) without identified granuloma (control-sIBM).
Results All but 2 patients with granuloma-myositis had extramuscular involvement, including signs common in sarcoidosis that were systematically absent in the control-myositis and the control-sIBM groups. Almost half of patients with granuloma-myositis matched the diagnostic criteria for sIBM. In these patients, other than the granuloma, the characteristics of the myopathy and its nonresponse to treatment were similar to the control-sIBM patients. Aside from 1 patient with myositis overlapping with systemic sclerosis, the remaining patients with granuloma-myositis did not match the criteria for a well-defined myositis subtype, suggesting pure sarcoidosis. Matching criteria for sIBM was the sole feature independently associated with nonresponse to myopathy treatment in patients with granuloma-myositis.
Conclusion Patients with granuloma-myositis should be carefully screened for sIBM associated with sarcoidosis in order to best tailor their care.
Glossary
- CK=
- creatine kinase;
- DM=
- dermatomyositis;
- ENMC=
- European Neuromuscular Centre;
- EULAR/ACR=
- European League Against Rheumatism/American College of Rheumatology;
- NAM=
- necrotizing autoimmune myopathy;
- OM=
- overlap myositis;
- PM=
- polymyositis;
- sIBM=
- sporadic inclusion body myositis
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received November 8, 2018.
- Accepted in final form August 30, 2019.
- © 2019 American Academy of Neurology
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- Author response: Granulomatosis-associated myositis: High prevalence of sporadic inclusion body myositis
- Reader response: Granulomatosis-associated myositis: High prevalence of sporadic inclusion body myositis
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