Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan.
World J Gastroenterol. 2013 Jun 21;19(23):3693-8. doi: 10.3748/wjg.v19.i23.3693.
Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification II-IV. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.
这里，我们报告了一例 74 岁日本男性患者，他在接受脉冲甲基强的松龙治疗多发性单神经炎后出现节段性肠坏死。该患者髓过氧化物酶（MPO）-抗中性粒细胞胞质抗体（ANCA）弱阳性。计算机断层扫描和手术结果与非闭塞性肠系膜缺血（NOMI）相符。他接受了急诊小肠切除术，并进行了肠造口术。病理上，中等至小动脉存在伴有纤维蛋白样坏死的坏死性血管炎，相当于 Arkin 分类 II-IV 级。大多数动脉有纤维内膜增厚，这被认为会阻塞动脉，从而导致节段性肠坏死。诊断为结节性多动脉炎（PAN），并在治疗方案中添加了静脉注射环磷酰胺脉冲治疗。该患者通过多学科治疗成功治愈，他的造口最终得以关闭。这是一例非常罕见且具有指示意义的 MPO-ANCA 弱阳性的 PAN 病例，临床表现类似于 NOMI，甚至在接受脉冲甲基强的松龙治疗后也会发生。
