全身紅斑性狼瘡很少在主要診斷徵候發生之前,以嚴重腹膜炎次發性?生大量腹水來表現。本文報告一個十三歲女孩,前後發生四次嚴重腹痛合併大量腹水。第一次發病前三個月有間歇性慢性腹痛進而在三天內轉?劇痛,超音波檢查發現有大量腹水,經保寧治療即改善。第二次發病,她的急性腹痛極似闌尾炎或婦科急症,差點要進行探勘性剖腹手術。因?血小板數目較低,延後手術半進一步檢查,結果自體免疫的檢查發現疑似全身紅斑性狼瘡,於是開始以類固醇治療,病人的腹痛與腹水很快改善,免除了不必要的手術。在腹膜炎合併腹水改善出院後,其他全身紅斑性狼瘡的主要臨床症狀才陸續出現。之後第三次發病,以一般劑量類固醇治療效果不佳,改以類固醇脈衝療法治療才得以改善。第四次腹水因嚴重腦出血而不幸死亡。就吾人所知,這是目前文獻上第一個小兒科病人以反覆性腹膜炎合併大量腹水?起始主要表現之全身紅斑性狼瘡病例。
Systemic lupus erythematosus (SLE) rarely presents with massive ascites secondary to severe peritonitis before the occurrence of major diagnostic features. We described a 13-year-old girl who developed four episodes of severe abdominal pain with massive ascites. During the first episode, the patient experienced deterioration with massive ascites over a 3-day period, after a 3-month history of intermittent abdominal pain. This episode resolved under conservative treatment. During the second episode, her acute abdominal findings mimicked appendicitis or gynecologic emergency. An exploratory laparotomy was initially planned, but not performed. Thrombocytopenia postponed the surgical intervention and led to further investigation. Autoimmune studies confirmed SLE and prompted steroid therapy, which rapidly improved her painful ascites and spared an unnecessary operation. After improvement of peritonitis with ascites, and following discharge, other major clinical features of SLE ensued. The third episode of peritonitis with massive ascites responded well to steroid pulse therapy. The fourth episode of ascites was accompanied by intracranial hemorrhage that led to her death. To the best of our knowledge, this is the first case report of recurrent peritonitis with massive ascites as the initial and major manifestation of SLE in a pediatric patient.