症状性肌肉结节病：一项全国多中心研究的经验教训
Symptomatic muscular sarcoidosis: Lessons from a nationwide multicenter study.
作者信息
Cohen Aubart Fleur, Abbara Salam, Maisonobe Thierry, Cottin Vincent, Papo Thomas, Haroche Julien, Mathian Alexis, Pha Micheline, Gilardin Laurent, Hervier Baptiste, Soussan Michael, Morlat Philippe, Nunes Hilario, Benveniste Olivier, Amoura Zahir, Valeyre Dominique
机构信息
AP-HP (F.C.A., S.A, J.H., A.M., M.P., Z.A), Service de Médecine Interne 2, Institut e3m, Hôpital de la Pitié-Salpêtrière, Centre National de Référence Maladies Systémiques Rares, Lupus, Syndrome des anticorps antiphospholipides; Université Paris VI (F.C.A., J.H., O.B.), UPMC, Sorbonnes Universités; AP-HP (T.M.), Département de neurophysiologie et de neuropathologie, Hôpital de la Pitié-Salpêtrière, Paris; Service de Pneumologie (V.C.), Centre des maladies pulmonaires rares, Lyon; AP-HP (T.P.), Service de Médecine Interne, Hôpital Bichat; AP-HP (L.G., B.H., O.B., Z.A.), Service de Médecine Interne et immunologie clinique, Hôpital de la Pitié-Salpêtrière, Paris; AP-HP (M.S.), Service de Médecine Nucléaire, Hôpital Avicenne, Bobigny; Service de Médecine Interne (P.M.), CHU Bordeaux, Bordeaux; AP-HP (H.N., D.V.), Service de Pneumologie, Hôpital Avicenne, Bobigny, France.
出版信息
Neurol Neuroimmunol Neuroinflamm. 2018 Mar 16;5(3):e452. doi: 10.1212/NXI.0000000000000452. eCollection 2018 May.
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.
目的
通过一项全国性多中心研究描述肌肉结节病的临床病理特征及相关结节病表型。
方法
纳入经组织学证实为结节病且经生物学、影像学或组织学检查确认有症状性肌肉受累的患者。
结果
共研究了48例患者（20例男性），肌肉症状出现时的中位年龄为45岁（范围18 - 71岁）。确定了四种模式：结节型（27%）；隐匿型表型（29%）；急性、亚急性或进行性肌病型（35%）；以及肌病与神经源性混合型（10%）。在所有模式中，结节病多为多脏器受累，累及的肌肉外器官中位数为3个（主要是肺、淋巴结、眼和皮肤），病程较长，长期使用皮质类固醇和免疫抑制药物。肌肉模式根据临床表现（肌痛、结节或无力）、肌电图结果、肌肉MRI以及对结节病治疗的反应而有所不同。肌病型和神经肌肉型更为严重。
结论
这项关于肌肉结节病的全国性研究确定了肉芽肿性肌炎的4种模式，这些模式在表型和临床病程上有所不同。
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