Dabadie A, Gié S, Taque S, Babut J M, Roussey M
Pédiatrie génétique médicale, CHU Pontchaillou, Rennes, France.
Arch Pediatr. 1996 Sep;3(9):884-7. doi: 10.1016/0929-693x(96)87579-8.
Renal disease is an unfrequent extraintestinal manifestation of chronic inflammatory bowel disease.
A 12-year-old girl suffered from recurrent abdominal pain, diarrhea and growth impairment due to Crohn's disease of ileocaecal region. After six months of nutritional rehabilitation, an ileo-caecal resection was performed because of intestinal stenosis. The surgical procedure was followed by parietal abcess and cutaneous fistula. One year later, a purulent secretion came out of the fistula associated with fever, hematuria and acute renal failure. Renal biopsy confirmed IgA nephropathy. The course was favorable under parenteral nutrition and after surgical closure of the sigmoido-cutaneous fistula. The microscopic hematuria only persisted but the nephropathy did not relapse even during a further digestive exacerbation.
IgA nephropathy has been reported in association with chronic inflammatory bowel disease. Its mechanism remains unclear: increased mucosal IgA production in inflammatory bowel, increased serum IgA and/or immune complex deposition in the renal mesangium appear the most relevant hypotheses.
肾脏疾病是慢性炎症性肠病不常见的肠外表现。
一名12岁女孩因回盲部克罗恩病出现反复腹痛、腹泻及生长发育障碍。经过6个月的营养康复后,因肠道狭窄行回盲部切除术。术后出现腹壁脓肿及皮肤瘘。1年后,瘘口有脓性分泌物排出,并伴有发热、血尿及急性肾衰竭。肾活检确诊为IgA肾病。在肠外营养支持及乙状结肠-皮肤瘘手术闭合后病情好转。仅镜下血尿持续存在,但即使在消化系统再次发作时肾病也未复发。
IgA肾病已被报道与慢性炎症性肠病相关。其机制尚不清楚:炎症性肠病中黏膜IgA产生增加、血清IgA升高和/或免疫复合物在肾小球系膜沉积似乎是最相关的假说。