Granulomatosis-associated myositis: high prevalence of s-IBM

In this study, the team of Belgian and French researchers including researchers from the Institute of Myology aimed to refine the predictive significance of muscle granuloma in patients with myositis.

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).

All but 2 granuloma-myositis patients had extramuscular involvement, including signs common in sarcoidosis that were systematically absent in the control-myositis and the control-sIBM groups. Almost half of granuloma-myositis patients 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 granuloma-myositis patients 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.

Patients with granuloma-myositis should be carefully screened for sIBM associated with sarcoidosis in order to best tailor their care.

 

Granulomatosis-associated myositis: High prevalence of sporadic inclusion body myositis. Dieudonné Y, Allenbach Y, Benveniste O, Leonard-Louis S, Hervier B, Mariampillai K, Nespola B, Lannes B, Echaniz-Laguna A, Wendling D, Von Frenckell C, Poursac N, Mortier E, Lavigne C, Hinschberger O, Magnant J, Gottenberg JE, Geny B, Sibilia J, Meyer A. Neurology. 2019 Dec 27. pii: 10.1212/WNL.0000000000008863. doi: 10.1212/WNL.0000000000008863. [Epub ahead of print]