title: Systemic sclerosis complicated by arrhythmogenic right ventricular dysplasia that was misinterpreted as pulmonary arterial hypertension.
abstract: 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 However, the autopsy revealed marked right ventricular dilatation, and the myocardium had been replaced by fatty tissue.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.
authors.affiliation: Clinical Immunology, Dokkyo Medical University School of Medicine, 880 Kita-Kobayashi, Mibu, Tochigi 321-0293, Japan.
authors.forename: Satoko
authors.initials: S
authors.lastname: Arai
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Kurasawa
authors.forename: Reika
authors.initials: R
authors.lastname: Maezawa
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Hanaoka
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fukuda
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D065627:Familial Primary Pulmonary Hypertension; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis*; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis*