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一例青少年系统性红斑狼疮与多发性肌炎重叠综合征病例,后期出现抗Scl 70抗体和抗Ku抗体,并伴发系统性硬化症。

A juvenile case of overlap syndrome of systemic lupus erythematosus and polymyositis, later accompanied by systemic sclerosis with the development of anti-Scl 70 and anti-Ku antibodies.

作者信息

Nitta Y, Muramatsu M

机构信息

Department of Dermatology, Aichi Medical University, Japan.

出版信息

Pediatr Dermatol. 2000 Sep-Oct;17(5):381-3. doi: 10.1046/j.1525-1470.2000.017005381.x.

DOI:10.1046/j.1525-1470.2000.017005381.x
PMID:11085667
Abstract

We describe a 16-year-old girl with an overlap syndrome consisting of systemic lupus erythematosus (SLE) from the age of 7 and polymyositis (PM) from the age of 10, later accompanied by systemic sclerosis (SSc) from the age of 15. She was diagnosed as having SLE with exudative malar erythema, photosensitivity, and discoid rashes with positive antinuclear antibody (ANA) and anti-DNA antibody titers. The diagnosis of PM was also made in accordance with findings of a high titer of muscle enzymes and a muscle biopsy specimen demonstrating marked degeneration of the muscle fibers and perivascular infiltration of mononuclear cells. She developed Raynaud's phenomenon and pitting ulcers on her fingers with positive anti-Scl 70 and anti-Ku antibodies, leading to a diagnosis of SSc. The patient was treated with prednisolone. To our knowledge this is the youngest case of SLE-PM overlap syndrome later accompanied by SSc.

摘要

我们描述了一名16岁女孩,患有重叠综合征,7岁时患系统性红斑狼疮(SLE),10岁时患多发性肌炎(PM),15岁时又出现系统性硬化症(SSc)。她因出现渗出性颧部红斑、光过敏和盘状皮疹,抗核抗体(ANA)和抗DNA抗体滴度呈阳性而被诊断为SLE。PM的诊断也是根据高滴度的肌肉酶以及肌肉活检标本显示的肌纤维明显变性和单核细胞血管周围浸润得出的。她出现了雷诺现象,手指有凹陷性溃疡,抗Scl 70和抗Ku抗体呈阳性,从而诊断为SSc。该患者接受了泼尼松龙治疗。据我们所知,这是最年轻的先出现SLE - PM重叠综合征,随后又伴有SSc的病例。

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