RT Journal Article
SR Electronic
T1 Symptomatic muscular sarcoidosis
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e452
DO 10.1212/NXI.0000000000000452
VO 5
IS 3
A1 Cohen Aubart, Fleur
A1 Abbara, Salam
A1 Maisonobe, Thierry
A1 Cottin, Vincent
A1 Papo, Thomas
A1 Haroche, Julien
A1 Mathian, Alexis
A1 Pha, Micheline
A1 Gilardin, Laurent
A1 Hervier, Baptiste
A1 Soussan, Michael
A1 Morlat, Philippe
A1 Nunes, Hilario
A1 Benveniste, Olivier
A1 Amoura, Zahir
A1 Valeyre, Dominique
YR 2018
UL http://nn.neurology.org/content/5/3/e452.abstract
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