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嗜酸性筋膜炎:临床、实验室及显微镜检查方面的考量

Eosinophilic fasciitis: clinical, laboratory, and microscopic considerations.

作者信息

Kent L T, Cramer S F, Moskowitz R W

出版信息

Arthritis Rheum. 1981 May;24(5):677-83. doi: 10.1002/art.1780240508.

DOI:10.1002/art.1780240508
PMID:6972219
Abstract

Two patients with clinical and pathologic features of eosinophilic fasciitis manifested serologic and systemic abnormalities that raised the question of the fundamental nature and relationship of eosinophilic fasciitis to scleroderma. In addition to the characteristic features of eosinophilic fasciitis, both patients exhibited arthritis, a predominantly mononuclear cell infiltration of muscles with normal serum muscle enzyme levels, weakly positive serum antinuclear factor, IgA deficiency, and abnormalities of pulmonary function. In addition, one patient had wide-mouthed colonic diverticulae and synovial deposits consistent with amyloid; the second patient had bone marrow hypoplasia. Although corticosteroid therapy was of benefit, hydroxychloroquine and potassium para-aminobenzoate were of further help in controlling the disorder. Biopsies from the two patients revealed inflammatory lesions to be heaviest deep in the skeletal muscle; fascia was only minimally inflamed with mild fibrosis. The findings suggest that striking fibroinflammatory lesions noted in the fascia in some patients with eosinophilic fasciitis may derive largely from spillover of lesions in neighboring skeletal muscle.

摘要

两名具有嗜酸性筋膜炎临床和病理特征的患者出现了血清学和全身异常,这引发了嗜酸性筋膜炎与硬皮病的本质及关系问题。除了嗜酸性筋膜炎的特征性表现外,两名患者均有关节炎、肌肉以单核细胞为主的浸润且血清肌肉酶水平正常、血清抗核因子弱阳性、IgA缺乏以及肺功能异常。此外,一名患者有宽口结肠憩室和与淀粉样蛋白一致的滑膜沉积物;另一名患者有骨髓发育不全。尽管皮质类固醇治疗有益,但羟氯喹和对氨基苯甲酸钾在控制病情方面有进一步帮助。两名患者的活检显示炎症病变在骨骼肌深层最为严重;筋膜仅有轻微炎症和轻度纤维化。这些发现表明,一些嗜酸性筋膜炎患者筋膜中显著的纤维炎性病变可能主要源于邻近骨骼肌病变的蔓延。

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1
Eosinophilic fasciitis: clinical, laboratory, and microscopic considerations.嗜酸性筋膜炎:临床、实验室及显微镜检查方面的考量
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[Eosinophilic fasciitis].
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