Clinical Immunology, Dokkyo Medical University School of Medicine, 880 Kita-Kobayashi, Mibu, Tochigi 321-0293, Japan.
Mod Rheumatol. 2012 Feb;22(1):152-7. doi: 10.1007/s10165-011-0484-x. Epub 2011 Jun 25.
A 58-year old Japanese woman who had been diagnosed with and managed for systemic sclerosis (SSc) with pulmonary arterial hypertension died suddenly. However, the autopsy revealed marked right ventricular dilatation, and the myocardium had been replaced by fatty tissue. These findings were consistent with arrhythmogenic right ventricular dysplasia (ARVD). A literature search identified nine cases of SSc with ARVD in Japan, including this case; this number is significantly higher than the value estimated from the prevalences of ARVD and SSc in Japan, suggesting an association between these two rare diseases.
一位 58 岁的日本女性，患有系统性硬化症（SSc）合并肺动脉高压，经诊断和治疗后死亡。然而，尸检显示右心室明显扩张，心肌被脂肪组织取代。这些发现与致心律失常性右室心肌病（ARVD）相符。文献检索在日本共发现了 9 例 SSc 合并 ARVD，包括本病例；这一数字明显高于日本 ARVD 和 SSc 患病率的估计值，提示这两种罕见疾病之间存在关联。
