Matsuda Masayuki, Nishikawa Noriko, Okano Tomomi, Hoshi Kenichi, Suzuki Akio, Ikeda Shu-ichi
Division of Rheumatology, Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.
Amyloid. 2003 Mar;10(1):42-6. doi: 10.3109/13506120308995257.
We report a 71-year-old man with reactive AA amyloidosis secondary to rheumatoid arthritis who developed spontaneous pneumoperitoneum with intestinal pseudo-obstruction as an initial symptom. Severe deposition of amyloid in the intestinal wall was considered to play an important role in the pathogenesis of this unusual symptom. The patient has been successfully treated with total parenteral alimentation and intermediate-dose prednisolone (30 mg/day). Although pneumoperitoneum usually suggests gastrointestinal perforation requiring emergency surgery, conservative therapy should be seriously considered in amyloidosis-related cases with no associated peritonitis, since multiple vital organs are probably involved by severe amyloid deposition, thus increasing the risks of surgery.
我们报告了一名71岁男性,患有类风湿性关节炎继发的反应性AA淀粉样变性,以自发性气腹伴肠假性梗阻为首发症状。肠道壁中淀粉样蛋白的严重沉积被认为在这种不寻常症状的发病机制中起重要作用。该患者已通过全胃肠外营养和中等剂量泼尼松龙(30毫克/天)成功治疗。虽然气腹通常提示需要紧急手术的胃肠道穿孔,但对于无相关腹膜炎的淀粉样变性相关病例,应认真考虑保守治疗,因为严重的淀粉样蛋白沉积可能累及多个重要器官,从而增加手术风险。