目的:特发性炎性肌病主要由肌肉内的炎性浸润(淋巴细胞和巨噬细胞)定义。嗜酸性粒细胞肌肉浸润已在特发性嗜酸性粒细胞性肌炎(IEM)中描述,而在嗜酸性粒细胞性筋膜炎(EF)中很少描述。本研究旨在通过对IEM和EF患者的详尽分析,进一步描绘特发性嗜酸性粒细胞肌肉浸润的病理框架。
方法:此多中心回顾性病例系列包括2000年至2022年诊断的IEM患者。IEM纳入标准为嗜酸性肌层浸润伴肌炎病理特征,排除鉴别诊断后。构成了另一组在2016年至2022年之间诊断的EF患者。纳入标准是EF诊断和筋膜增厚伴炎症浸润。
结果:共20例IEM和10例EF。诊断时的中位[IQR]年龄为65[49-70]岁;有18名男性。数据分析描绘了4个亚组:局灶性EM(FEM,n=3),漫射EM(DEM,n=6),嗜酸性肌筋膜炎(EMF,n=11),和EF(n=10)。FEM代表一种有限的良性形式的肌炎。DEM病例表现为伴有嗜酸性粒细胞性肌肉浸润的客观肌肉损害。EMF患者表现出主观肌肉损伤(肌痛,55%),筋膜炎(组织学和/或影像学检查),嗜酸性粒细胞肌肉浸润,和频繁的嗜酸性粒细胞增多(55%)。EF患者出现肌痛(50%),组织学上的肌肉病变伴有筋膜限制性炎症浸润,伴有嗜酸性粒细胞(60%)或不伴有嗜酸性粒细胞(40%)。
结论:对IEM和EF患者特征的分析描绘了4个亚组(FEM,DEM,EMF,和EF)在临床方面,实验室,成像,病态,和结果的特殊性,并提出了一种适应性的诊断和护理管理方法。
Idiopathic inflammatory myopathies are mainly defined by inflammatory infiltrates within the muscle (lymphocytes and macrophages). Eosinophil muscle infiltration has been described in idiopathic eosinophilic myositis (IEM) and rarely in EF. This study aimed to further delineate the nosological frame of idiopathic eosinophil muscle infiltration through the exhaustive analysis of IEM and EF patients.
This multicentre retrospective case series included IEM patients diagnosed between 2000 and 2022. IEM inclusion criteria were eosinophilic muscle infiltration with myositis pathological features, after the exclusion of differential diagnoses. An additional group of EF patients diagnosed between 2016 and 2022 was constituted. Inclusion criteria were an EF diagnosis and fascia thickening with inflammatory infiltrate.
A total of 20 IEM cases and 10 EF cases were included. The median (interquartile range) age at diagnosis was 65 (49-70) years; there were 18 males. Data analysis delineated four subgroups: focal EM (FEM, n = 3), diffuse EM (DEM, n = 6), eosinophilic myofasciitis (EMF, n = 11) and EF (n = 10). FEM represented a limited and benign form of myositis. DEM cases presented objective muscle impairment with eosinophilic muscle infiltration. EMF patients presented subjective muscle impairment (myalgia, 55%), fasciitis (on histology and/or imaging), eosinophilic muscle infiltration and frequent hypereosinophilia (55%). EF patients presented myalgia (50%), muscle lesions on histology with fascia-restricted inflammatory infiltrates with (60%) or without (40%) eosinophils.
The analysis of IEM and EF patient characteristics delineates four subgroups (FEM, DEM, EMF and EF) in terms of clinical, laboratory, imaging, pathological and outcome specificities, and proposes an adapted diagnostic and care management approach.