Symptomatic muscular sarcoidosis

ObjectivesTo describe clinicopathologic features of muscular sarcoidosis and the associated sarcoidosis phenotype through a nationwide multicenter study.MethodsPatients were included if they had histologically proven sarcoidosis and symptomatic muscular involvement confirmed by biological, imaging, or histologic examinations.ResultsForty-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.ConclusionThis nationwide study of muscular sarcoidosis allowed the identification of 4 patterns of granulomatous myositis, which differed by phenotypes and the clinical course.

Sarcoidosis muscular en una paciente con hipercalcemia no mediada por PTH, reporte de un caso

La sarcoidosis es una enfermedad granulomatosa sistémica de etiología desconocida, cuyos síntomas iniciales pueden ser inespecíficos y de intensidad variable, o incluso en muchos casos es asintomática. Cualquier órgano puede estar afectado, sin embargo, se observa con mayor frecuencia compromiso pulmonar y de ganglios linfáticos, seguido por la afectación a nivel oftálmico, cutáneo y hepático, el compromiso muscular aislado es poco frecuente. La sarcoidosis suele ser un diagnóstico de exclusión y en ocasiones puede imitar lesiones malignas, por lo que se debe considerar como un diagnóstico diferencial en muchas patologías, dada la gran variedad de manifestaciones. Presentamos el caso de una mujer de 34 años quien consultó por oligomenorrea, documentándose de manera incidental hipercalcemia no mediada por PTH, finalmente llegando al diagnóstico de una sarcoidosis muscular por medio de los hallazgos en el PET con 18-FDG, lo que permitió la realización de biopsia de músculo glúteo mayor izquierdo para confirmación histopatológica del diagnóstico.AbstractSarcoidosis is a systemic granulomatous disease of unknown etiology, whose initial symptoms may be non-specific and variable in severity, or even asymptomatic in many cases. Any organ can be affected, but the lung and the lymphatic system are mostly involved, followed by the eyes, skin and liver, with isolated muscular involvement being rare. Sarcoidosis is an exclusion diagnosis and, in some cases, it could mimic malignancies, so it should be considered in the differential diagnosis of many conditions, given its diverse manifestations. We present the case of a 34 years old female who complained of oligomenorrhea, with the incidental discovery of a non-PTH-mediated hypercalcemia. Finally, the diagnosis of muscular sarcoidosis was made using 18 FDG-PET followed by histopathology confirmation.