TY - JOUR
T1 - Clinical characteristics and outcome in muscular sarcoidosis
T2 - a retrospective cohort study and literature review
AU - ten Dam, Leroy
AU - Raaphorst, Joost
AU - van der Kooi, Anneke J.
AU - Eftimov, Filip
AU - Aronica, Eleonora
AU - van de Beek, Diederik
AU - Brouwer, Matthijs C.
N1 - Funding Information: DvdB is supported by a grant from the Netherlands Organization for Health Research and Development (ZonMw; NWO-Vici grant 2019 [ 918.19.627 ]) and an Innovation grant by the board of directors of the Amsterdam UMC, Amsterdam, the Netherlands. MCB is supported by grants from the Netherlands Organization for Health Research and Development (ZonMw; NWO-Vidi grant 2017 [ 917.17.308 ]) and the European Research Council (ERC Consolidator Grant 101001237 ). No potential conflict of interest relevant for this article exist. Publisher Copyright: © 2022
PY - 2022/7/1
Y1 - 2022/7/1
N2 - We evaluated the clinical features and treatment response of patients with muscular sarcoidosis. A retrospective cohort of 12 patients showed muscle weakness in 11 and myalgia in seven. One had focal myositis. Four had a negative medical history for sarcoidosis. Muscle imaging showed muscle edema in all and replacement of muscle tissue by fat in half of patients. Muscle biopsy showed non-caseating granulomas in six of nine patients and inflammation without granulomas in three. None of the muscle biopsies showed features of inclusion body myositis. Imaging in three patients without muscle biopsy showed focal intramuscular masses or a ‘tiger man’ appearance typical for muscular sarcoidosis. Treatment consisted of glucocorticoids in 11, additional methotrexate or azathioprine in seven and infliximab in two patients. Half of the patients had symptoms leading to substantial disability (modified Rankin scale score >1) at last follow-up. A literature review of articles describing more than one muscular sarcoidosis patient published in the last 25 years identified 153 additional patients. We found muscular sarcoidosis to be a rare and often disabling disease which may be recognized by typical muscle imaging characteristics and add focal myositis to the muscular phenotypes of sarcoidosis.
AB - We evaluated the clinical features and treatment response of patients with muscular sarcoidosis. A retrospective cohort of 12 patients showed muscle weakness in 11 and myalgia in seven. One had focal myositis. Four had a negative medical history for sarcoidosis. Muscle imaging showed muscle edema in all and replacement of muscle tissue by fat in half of patients. Muscle biopsy showed non-caseating granulomas in six of nine patients and inflammation without granulomas in three. None of the muscle biopsies showed features of inclusion body myositis. Imaging in three patients without muscle biopsy showed focal intramuscular masses or a ‘tiger man’ appearance typical for muscular sarcoidosis. Treatment consisted of glucocorticoids in 11, additional methotrexate or azathioprine in seven and infliximab in two patients. Half of the patients had symptoms leading to substantial disability (modified Rankin scale score >1) at last follow-up. A literature review of articles describing more than one muscular sarcoidosis patient published in the last 25 years identified 153 additional patients. We found muscular sarcoidosis to be a rare and often disabling disease which may be recognized by typical muscle imaging characteristics and add focal myositis to the muscular phenotypes of sarcoidosis.
KW - Inflammatory myopathy
KW - Myopathies
KW - Myosarcoidosis
KW - Neuromuscular diseases
KW - Sarcoidosis
UR - http://www.scopus.com/inward/record.url?scp=85131432350&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.nmd.2022.05.012
DO - https://doi.org/10.1016/j.nmd.2022.05.012
M3 - Article
C2 - 35654706
SN - 0960-8966
VL - 32
SP - 557
EP - 563
JO - Neuromuscular disorders
JF - Neuromuscular disorders
IS - 7
ER -