Kumar Ashok, Goel Anshul, Lapsiwala Mehul
Department of Rheumatology, Fortis Flt Lt Rajan Dhall Hospital , New Delhi 110070 , India.
Oxf Med Case Reports. 2016 Oct 5;2016(10):omw065. doi: 10.1093/omcr/omw065. eCollection 2016 Oct.
Polyarteritis nodosa is a medium artery vasculitis that can cause fatal complications. It commonly follows an acute monophasic course that may remit after treatment or cause serious morbidity or death. A 24-year-old patient described here had many vasculitic episodes in the past 16 years causing infarction of different organs. The last episode was most serious which caused mononeuritis multiplex, digital gangrene, bowel gangrene and subsequent perforations. There was strong clinical suspicion of this disease right from the beginning. However, diagnosis could not be proved objectively with multiple angiographies and biopsies till the end when CT angiography of abdomen revealed aneurysms in mesenteric vessels. He survived serious complications of disease and even surgery for bowel perforations but died of cerebellar abscess resulting from immunosuppressive therapy. Unfortunately he did not tolerate intravenous cyclophosphamide. Corticosteroid pulses and intravenous immunoglobulin also could not prevent the progression of digital gangrene and bowel infarction.
结节性多动脉炎是一种可导致致命并发症的中动脉血管炎。它通常呈急性单相病程，治疗后可能缓解，也可能导致严重的发病或死亡。本文描述的一名24岁患者在过去16年中有多次血管炎发作，导致不同器官梗死。最后一次发作最为严重，引起了多发性单神经炎、手指坏疽、肠坏疽及随后的穿孔。从一开始临床就高度怀疑此病。然而，多次血管造影和活检都无法客观证实诊断，直到最后腹部CT血管造影显示肠系膜血管有动脉瘤。他在疾病的严重并发症甚至肠穿孔手术后存活下来，但死于免疫抑制治疗引起的小脑脓肿。不幸的是，他不能耐受静脉注射环磷酰胺。皮质类固醇脉冲治疗和静脉注射免疫球蛋白也无法阻止手指坏疽和肠梗死的进展。
