PT  - JOURNAL ARTICLE
AU  - Cohen Aubart, Fleur
AU  - Abbara, Salam
AU  - Maisonobe, Thierry
AU  - Cottin, Vincent
AU  - Papo, Thomas
AU  - Haroche, Julien
AU  - Mathian, Alexis
AU  - Pha, Micheline
AU  - Gilardin, Laurent
AU  - Hervier, Baptiste
AU  - Soussan, Michael
AU  - Morlat, Philippe
AU  - Nunes, Hilario
AU  - Benveniste, Olivier
AU  - Amoura, Zahir
AU  - Valeyre, Dominique
TI  - Symptomatic muscular sarcoidosis
AID  - 10.1212/NXI.0000000000000452
DP  - 2018 May 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e452
VI  - 5
IP  - 3
4099  - http://nn.neurology.org/content/5/3/e452.short
4100  - http://nn.neurology.org/content/5/3/e452.full
SO  - Neurol Neuroimmunol Neuroinflamm2018 May 01; 5
AB  - Objectives To describe clinicopathologic features of muscular sarcoidosis and the associated sarcoidosis phenotype through a nationwide multicenter study.Methods Patients were included if they had histologically proven sarcoidosis and symptomatic muscular involvement confirmed by biological, imaging, or histologic examinations.Results Forty-eight patients (20 males) were studied, with a median age at muscular symptoms onset of 45 years (range 18–71). Four patterns were identified: a nodular pattern (27%); smoldering phenotype (29%); acute, subacute, or progressive myopathic type (35%); and combined myopathic and neurogenic pattern (10%). In all patterns, sarcoidosis was multivisceral with a median of 3 extramuscular organs involved (mostly lungs, lymph nodes, eyes, and skin) and a prolonged course with long-term use of corticosteroids and immunosuppressive drugs. Muscular patterns differed according to clinical presentation (myalgia, nodules, or weakness), electromyographic findings, muscular MRI, and response to sarcoidosis treatment. The myopathic and neuromuscular patterns were more severe.Conclusion This nationwide study of muscular sarcoidosis allowed the identification of 4 patterns of granulomatous myositis, which differed by phenotypes and the clinical course.mRS=modified Rankin score