Hiraishi T, Tokuda M, Mitsunaka H, Dobashi H, Takahara J
First Department of Internal Medicine, Kagawa Medical University.
Ryumachi. 1999 Jun;39(3):580-5.
Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of gas-filled cysts in the submucosa or subserosa of gastrointestinal tract. PCI has been widely recognized as a late manifestation of systemic sclerosis but seldom reported to take place in patients with systemic lupus erythematosus (SLE). We reported here a 13-year-old female who had been diagnosed to have SLE based on the following findings; malar rash, discoid erythema, proteinuria, positive antinuclear antibody and anti-DNA antibody. She had been treated with various immunosuppressive drugs including pulse use of corticosteroid, cyclophosphamide and cyclosporin A. She was referred to our hospital because of proteinuria and numbness on her right fifth toe, refractory to above treatment. On admission, the activity of her disease was already low and she had no abdominal symptoms. Plain X-ray film showed multiple round translucencies along the wall of the ascending and transverse colon. Colonoscopy revealed multiple firm-walled cysts distributing in the terminal ileum as well. A diagnosis of PCI was made and she was successfully treated with oral antibiotics and laxatives. The association of PCI with SLE is reviewed briefly.
肠气囊肿症（PCI）是一种罕见的疾病，其特征是胃肠道黏膜下层或浆膜下层存在充满气体的囊肿。PCI已被广泛认为是系统性硬化症的晚期表现，但很少有报道称其发生在系统性红斑狼疮（SLE）患者中。我们在此报告一名13岁女性，根据以下表现被诊断为SLE：颧部红斑、盘状红斑、蛋白尿、抗核抗体和抗DNA抗体阳性。她曾接受过多种免疫抑制药物治疗，包括脉冲使用皮质类固醇、环磷酰胺和环孢素A。因蛋白尿和右足第五趾麻木，上述治疗无效，她被转诊至我院。入院时，她的疾病活动度已经较低，且无腹部症状。腹部平片显示升结肠和横结肠壁有多个圆形透光区。结肠镜检查发现多个壁厚的囊肿也分布在回肠末端。诊断为PCI，她通过口服抗生素和泻药成功治愈。本文简要回顾了PCI与SLE的关联。
