Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Vasc Health Risk Manag. 2022 Jul 28;18:595-601. doi: 10.2147/VHRM.S354548. eCollection 2022.
Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis affecting small- to medium-sized arteries. The most common gastrointestinal manifestation of PAN is postprandial abdominal pain from mesenteric arteritis causing bowel ischemia. When transmural ischemia develops, there may be ischemic necrosis and perforation of the bowel wall, which are life-threatening. Severe, life-threatening gastrointestinal involvement is relatively rare in pediatric PAN and may require different management in adult patients. We report a pediatric PAN case in a patient who presented with acute abdominal pain and superimposed cytomegalovirus enteritis with jejunoileal perforation. The patient improved with emergency small intestinal resection followed by conventional immunosuppressive drugs of a corticosteroid and cyclophosphamide, and anti-viral drugs. Before increasing the immunosuppressive drug dosage, initial screening of infectious cytomegalovirus and comprehensive evaluation for surgical conditions are essential in pediatric PAN with severe gastrointestinal involvement. Early aggressive treatment for acute abdomen is useful in reducing morbidity and mortality in pediatric PAN.
结节性多动脉炎（PAN）是一种罕见的系统性坏死性血管炎，影响小至中等大小的动脉。PAN 最常见的胃肠道表现为肠系膜动脉炎引起的餐后腹痛导致肠缺血。当发生透壁性缺血时，可能会出现肠壁的缺血性坏死和穿孔，这是危及生命的。严重的、危及生命的胃肠道受累在儿科 PAN 中相对少见，可能需要与成年患者不同的治疗方法。我们报告了一例儿科 PAN 病例，患者表现为急性腹痛，并伴有巨细胞病毒肠炎和空肠回肠穿孔。患者通过紧急小肠切除术，随后使用皮质类固醇和环磷酰胺等常规免疫抑制剂以及抗病毒药物治疗后得到改善。在增加免疫抑制剂剂量之前，对于严重胃肠道受累的儿科 PAN，初始筛查感染性巨细胞病毒并全面评估手术情况至关重要。早期积极治疗急性腹痛有助于降低儿科 PAN 的发病率和死亡率。
