Grimbacher B, Huber M, von Kempis J, Kalden P, Uhl M, Köhler G, Blum H E, Peter H H
Division of Rheumatology and Clinical Immunology, Albert-Ludwigs-University Medical Centre Freiburg, Germany.
Br J Rheumatol. 1998 Sep;37(9):1023-8. doi: 10.1093/rheumatology/37.9.1023.
Gastrointestinal vasculitis in systemic lupus erythematosus (SLE) is quite rare and almost always accompanied by evidence of active disease in other organs, although occasionally it may be the presenting feature of the disease. Gastrointestinal involvement in SLE may present as lupus peritonitis, non-necrotizing pancreatitis, gastrointestinal vasculitis or surgical abdomen. Here we report a severe case of SLE which presented initially with fever of unknown origin. Severe distress, abdominal pain, the presence of occult blood in the stool and high acute-phase proteins were explained by a lupus peritonitis and intestinal vasculitis resembling inflammatory bowel disease. Whereas high-dose prednisone treatment did not prevent a severe relapse, we observed a sustained remission following i.v. cyclophosphamide pulse therapy. In the literature, only two similar cases are reported: one died despite a change in the therapy of a bowel perforation; our case was the second that improved under pulse cyclophosphamide. We suggest the use of cyclophosphamide after failure of steroids early in the course of SLE gastrointestinal vasculitis to prevent devastating complications.
系统性红斑狼疮（SLE）中的胃肠道血管炎相当罕见，几乎总是伴有其他器官活动性疾病的证据，尽管偶尔它可能是该疾病的首发特征。SLE的胃肠道受累可能表现为狼疮性腹膜炎、非坏死性胰腺炎、胃肠道血管炎或急腹症。在此，我们报告一例严重的SLE病例，最初表现为不明原因发热。严重不适、腹痛、大便潜血阳性和急性期蛋白升高是由狼疮性腹膜炎和类似炎症性肠病的肠道血管炎所致。尽管大剂量泼尼松治疗未能预防严重复发，但我们观察到静脉注射环磷酰胺脉冲治疗后病情持续缓解。在文献中，仅报道了两例类似病例：一例尽管改变了肠穿孔的治疗方法仍死亡；我们的病例是第二例在脉冲环磷酰胺治疗下病情改善的病例。我们建议在SLE胃肠道血管炎病程早期类固醇治疗失败后使用环磷酰胺，以预防严重并发症。
