Chérin P, Laforet P, Ghérardi R K, Authier F J, Coquet M, Maisonobe T, Mussini J M, Pellissier J F, Herson S
Service de médecine interne I, hôpital de la Pitié-Salpêtrière, Paris.
Rev Med Interne. 1999 Jun;20(6):483-9. doi: 10.1016/s0248-8663(99)80083-6.
A new type of inflammatory myopathy of unknown etiology has recently been described in France. The myopathy, called macrophagic myofasciitis, had never been described in the literature.
In December 1998, 35 cases of macrophagic myofasciitis were reported, showing an increase in its incidence since the description of the first case in 1993. The first 22 cases are described.
The 22 patients were each referred with a presumptive diagnosis of either polymyositis (11 patients), polymyalgia rheumatica (5 patients), mitochondrial cytopathy (4 patients), or congenital myopathy or muscle dystrophy (1 patient for each). Clinical symptoms included myalgias (91%), arthralgias (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%). Laboratory findings included elevated CK levels (50%) and a marked increased in the erythrocyte sedimentation rate (37%). Electromyographic recordings showed the existence of myopathy (35%). Muscle biopsy showed a unique pattern characterized by: (i) centripetal infiltration of the epimysium, perimysium and perifascicular endomysium by non epitheloid, cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-) with both large cytoplasm and PAS-positive content; (ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; (iii) occasional CD8+ T-cells; and, (iiii) minimal myocyte suffering. The disease symptoms were easily distinguishable from those of sarcoid myopathy and fasciitis-panniculitis syndromes. Infectious diseases known to be associated with reactive histiocytosis, including Whipple's disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients' condition improved under corticosteroid therapy, associated or not with non-specific antibiotic therapy.
A new inflammatory muscle disorder of unknown etiology, characterized by a distinctive pathological pattern of macrophagic myofasciitis, is emerging in France. Diagnosis is based on muscular biopsy. Numerous clinical, epidemiological and etiopathologic studies initiated by the GERMMAD (Groupe d'études et de recherche sur les maladies musculaires acquises) are in progress.
法国最近描述了一种病因不明的新型炎性肌病。这种肌病被称为巨噬细胞性肌筋膜炎，此前文献中从未有过描述。
1998年12月，报告了35例巨噬细胞性肌筋膜炎病例，自1993年首例病例被描述以来，其发病率有所上升。本文描述了前22例病例。
这22例患者最初的诊断推测分别为多发性肌炎（11例）、风湿性多肌痛（5例）、线粒体细胞病（4例），以及先天性肌病或肌肉营养不良（各1例）。临床症状包括肌痛（91%）、关节痛（68%）、明显乏力（55%）、肌无力（45%）和发热（32%）。实验室检查结果包括肌酸激酶水平升高（50%）和红细胞沉降率显著升高（37%）。肌电图记录显示存在肌病（35%）。肌肉活检显示出一种独特的模式，其特征为：（i）肌外膜、肌束膜和束周肌内膜被单核细胞/巨噬细胞谱系的非上皮样细胞（CD68+、CD1a-、S100-）向心性浸润，这些细胞具有大的细胞质且富含PAS阳性物质；（ii）不存在坏死，不存在上皮样细胞和巨细胞，也不存在有丝分裂象；（iii）偶尔可见CD8+ T细胞；以及（iiii）极少的肌细胞受累。该疾病症状很容易与结节病性肌病和筋膜炎-脂膜炎综合征的症状区分开来。已知与反应性组织细胞增多症相关的传染病，包括惠普尔病、鸟分枝杆菌胞内感染和软斑病，均未得到证实。患者在接受皮质类固醇治疗（无论是否联合非特异性抗生素治疗）后病情有所改善。
在法国，一种病因不明的新型炎性肌肉疾病正在出现，其特征为独特的巨噬细胞性肌筋膜炎病理模式。诊断基于肌肉活检。由GERMMAD（获得性肌肉疾病研究与研究小组）发起的众多临床、流行病学和病因病理学研究正在进行中。
