Tanaka H, Waga S, Tateyama T, Nakahata T, Ito T, Sugimoto K, Kakizaki Y, Tomimoto K, Yokoyama M
Department of Pediatrics, Hirosaki University, School of Medicine, Japan.
Pediatr Nephrol. 2000 Aug;14(8-9):859-61. doi: 10.1007/s004679900285.
A girl aged 11 years presented with autoimmune hemolytic anemia with thrombocytopenia, and subsequently developed severe abdominal pain, vomiting, and pollakiuria. X-ray findings of her abdomen demonstrated paralytic ileus with intestinal wall thickening. Intravenous pyelography revealed bilateral hydroureter with mild hydronephrosis and contracted bladder. Pathological examination of her bladder revealed interstitial cystitis, with evidence of focal deposition of IgG and C3 in a granular pattern on small blood vessel walls. She was diagnosed as having systemic lupus erythematosus (SLE) associated with paralytic ileus and chronic interstitial cystitis. Although initiation of high-dose prednisolone therapy resulted in a gradual improvement in clinical symptoms, reducing the dosage of prednisolone caused a relapse. To our knowledge, the combination of paralytic ileus and chronic interstitial cystitis is quite uncommon in pediatric-onset SLE.
一名11岁女孩出现自身免疫性溶血性贫血伴血小板减少,随后出现严重腹痛、呕吐和尿频。她腹部的X线检查结果显示为麻痹性肠梗阻伴肠壁增厚。静脉肾盂造影显示双侧输尿管积水伴轻度肾积水和膀胱挛缩。对她的膀胱进行病理检查发现为间质性膀胱炎,在小血管壁上有IgG和C3呈颗粒状的局灶性沉积。她被诊断为患有与麻痹性肠梗阻和慢性间质性膀胱炎相关的系统性红斑狼疮(SLE)。尽管开始使用大剂量泼尼松龙治疗后临床症状逐渐改善,但减少泼尼松龙剂量会导致复发。据我们所知,麻痹性肠梗阻和慢性间质性膀胱炎的组合在儿童期发病的SLE中相当罕见。