Nonaka D, Higuchi M, Yoshizawa S, Horiuchi T, Nakashima H, Niho Y
First Department of Internal Medicine, Faculuty of Medicine, Kyushu University, Fukuoka-city.
Ryumachi. 1998 Aug;38(4):605-10.
Pneumatosis cystoides intestinalis (PCI) is an uncommon disease manifestation characterized by the presence of air in the bowel wall. PCI is sometimes observed in patients with progressive systemic sclerosis or mixed connective tissue disease but extremely rare in patients with systemic lupus erythematosus (SLE). We here report a patient with SLE who developed PCI after the treatment with intravenous cyclophosphamide (IVCY). This is the first case that association between IVCY and PCI was suggested. A 51-year-old woman with a 24-year history of SLE was admitted to our hospital because of skin ulcers in the lower legs. She had been receiving prednisolone orally. Laboratory findings on the present admission showed a elevated titer of anti-double stranded DNA antibody and positive LE test. She was successfully treated with three pulses of methylprednisolone followed by two IVCY together with vasodilators for her disease activity of SLE including skin manifestation. Just after the second IVCY, abdominal distention was gradually developed without any other abdominal symptoms, including abdominal pain. Abdominal radiography and computed tomography revealed pneumoperitoneum and multiple intramural air collections which involved the ascending colon primarily. Gastrointestinal series, however, showed no evidence of intestinal perforation. The diagnosis of PCI was made radiologically. After she was treated with a combined therapy with intravenous hyperalimentation and breathing with high concentration of oxygen for three weeks, PCI and pneumoperitoneum disappeared. It would be necessary that IVCY is carefully administrated, especially for the patients under the risk of PCI, such as collagen diseases.
肠气囊肿症（PCI）是一种罕见的疾病表现，其特征为肠壁内存在气体。PCI有时在进行性系统性硬化症或混合性结缔组织病患者中观察到，但在系统性红斑狼疮（SLE）患者中极为罕见。我们在此报告一例SLE患者，在接受静脉环磷酰胺（IVCY）治疗后发生了PCI。这是首次提示IVCY与PCI之间存在关联的病例。一名有24年SLE病史的51岁女性因小腿皮肤溃疡入院。她一直在口服泼尼松龙。本次入院时的实验室检查结果显示抗双链DNA抗体滴度升高且LE试验阳性。她通过三次甲泼尼龙冲击治疗，随后两次IVCY联合血管扩张剂成功治疗了包括皮肤表现在内的SLE疾病活动。就在第二次IVCY后不久，逐渐出现腹胀，无任何其他腹部症状，包括腹痛。腹部X线摄影和计算机断层扫描显示气腹和多个壁内积气，主要累及升结肠。然而，胃肠道造影未显示肠道穿孔的证据。PCI通过影像学诊断。在她接受静脉高营养和高浓度吸氧联合治疗三周后，PCI和气腹消失。对于PCI风险较高的患者，如胶原病患者，谨慎使用IVCY是必要的。
