PT - JOURNAL ARTICLE AU - Dieudonné, Yannick AU - Allenbach, Yves AU - Benveniste, Olivier AU - Leonard-Louis, Sarah AU - Hervier, Baptiste AU - Mariampillai, Kuberaka AU - Nespola, Benoit AU - Lannes, Béatrice AU - Echaniz-Laguna, Andoni AU - Wendling, Daniel AU - Von Frenckell, Christian AU - Poursac, Nicolas AU - Mortier, Emmanuel AU - Lavigne, Christian AU - Hinschberger, Olivier AU - Magnant, Julie AU - Gottenberg, Jacques-Eric AU - Geny, Bernard AU - Sibilia, Jean AU - Meyer, Alain TI - Granulomatosis-associated myositis AID - 10.1212/WNL.0000000000008863 DP - 2020 Mar 03 TA - Neurology PG - e910--e920 VI - 94 IP - 9 4099 - http://n.neurology.org/content/94/9/e910.short 4100 - http://n.neurology.org/content/94/9/e910.full SO - Neurology2020 Mar 03; 94 AB - 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.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