Anti-NXP2 Antibody-positive Juvenile Dermatomyositis with Characteristic Fascial Thickening on Muscle Ultrasound and Improvement with Immunotherapy

被引：1

作者：

Fukushima, Koji ^{[1
,2
]}

Yoshida, Takeshi ^{[3
,4
]}

Yamazaki, Hiroki ^{[1
]}

Takamatsu, Naoko ^{[1
]}

Nagai, Takashi

Osaki, Yusuke ^{[1
]}

Harada, Masafumi ^{[5
]}

Nishino, Ichizo ^{[6
]}

Okiyama, Naoko ^{[7
]}

Sugie, Kazuma ^{[2
]}

Izumi, Yuishin ^{[1
]}

机构：

[1] Tokushima Univ Hosp, Dept Neurol, Tokushima, Japan

[2] Nara Med Univ, Dept Neurol, Sch Med, Kashihara, Japan

[3] Chikamori Hosp, Dept Rheumatol, Kochi, Japan

[4] Tokushima Univ Hosp, Dept Pediat, Tokushima, Japan

[5] Tokushima Univ Hosp, Dept Radiol, Tokushima, Japan

[6] Natl Inst Neurosci, Natl Ctr Neurol & Psychiat, Dept Neuromuscular Res, Tokyo, Japan

[7] Tokyo Med & Dent Univ, Grad Sch Med & Dent Sci, Dept Dermatol, Tokyo, Japan

来源：

INTERNAL MEDICINE | 2024年 / 63卷 / 12期

关键词：

nuclear matrix protein 2; juvenile dermatomyositis; dermatomyositis sine dermatitis; fasciitis; myxovirus resistant protein A; muscle ultrasound; IDIOPATHIC INFLAMMATORY MYOPATHIES; AUTOANTIBODIES; POLYMYOSITIS; DIAGNOSIS; FASCIITIS; DISEASE; TOOL;

D O I：

10.2169/internalmedicine.2720-23

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

We herein report a 12-year-old boy who presented with a fever, erythematous rash on the cheeks, back pain, and dysphagia. Blood tests revealed increased creatine kinase levels, and muscle ultrasonography (MUS) revealed characteristic fascial thickening in the lumbar paraspinal muscles, where myalgia was prominent. Sarcoplasmic expression of myxovirus-resistant protein A on a muscle biopsy and the presence of antinuclear matrix protein 2 (NXP2) antibodies confirmed the diagnosis of dermatomyositis. Prednisolone and intravenous immunoglobulin therapy improved the clinical and laboratory parameters as well as fascial thickening. MUS is useful for evaluating fasciitis associated with anti-NXP2 autoantibodies and monitoring therapeutic efficacy.

引用

页码：1813 / 1817

页数：5

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