Rolla Giovanni, Tartaglia Nicoletta, Motta Manuela, Ferrero Nicoletta, Bergia Roberta, Guida Giuseppe, Heffler Enrico
Allergologia e Immunologia Clinica, Università di Torino and Ospedale Mauriziano Umberto I, Torino, Italy.
Ann Allergy Asthma Immunol. 2007 Jun;98(6):595-7. doi: 10.1016/S1081-1206(10)60743-0.
Churg-Strauss syndrome (CSS) is a systemic vasculitis that occurs in the setting of asthma or allergic rhinitis with eosinophilia. The development of systemic manifestations in these allergic patients needs to be recognized as a likely sign of CSS.
To describe a patient with limb paresthesia and abdominal complaints related to CSS.
Blood leukocyte count, nerve conduction study, ultrasound and computed tomography of the abdomen, laparoscopic cholecystectomy and ileum resection, and histopathologic examination of ileum and gallbladder samples.
A 55-year-old man with chronic asthma and rhinosinusitis had acute acalculous cholecystitis after he experienced lower limb paresthesia subsequently recognized as being due to mononeuritis multiplex. His eosinophil count was 1,860/microL. Three days after laparoscopic cholecystectomy the patient developed sudden severe diffuse abdominal pain with hypotension due to perforation of the ileum. The peripheral eosinophil count increased to 14,000/microL. Ileal resection was performed. Histopathologic examination showed necrotizing vasculitis with eosinophilic infiltration of the ileum and granulomatous vasculitis with eosinophilic infiltration of the gallbladder. He was treated with pulse intravenous methylprednisolone, 1 g for 3 consecutive days, followed by pulse intravenous cyclophosphamide, 750 mg/m(2), and recovered uneventfully. He received 6 additional monthly infusions of cyclophosphamide, and oral prednisone was tapered. When last seen, 2 years later, the patient was in good clinical condition, continuing alternate-day use of oral prednisone (10 mg).
Nonrespiratory symptoms, such as paresthesia and acalculous cholecystitis, in a patient with asthma should alert the physician to consider CSS. If the neuropathic complaints had prompted the consideration of vasculitis, medical management might have avoided one or both surgical procedures.
变应性肉芽肿性血管炎（CSS）是一种系统性血管炎，发生于哮喘或伴有嗜酸性粒细胞增多的变应性鼻炎患者。这些过敏患者出现全身表现需被视为CSS的可能征象。
描述一名与CSS相关的肢体感觉异常和腹部不适患者。
进行血液白细胞计数、神经传导研究、腹部超声和计算机断层扫描、腹腔镜胆囊切除术和回肠切除术，以及回肠和胆囊样本的组织病理学检查。
一名55岁患有慢性哮喘和鼻窦炎的男性，在经历下肢感觉异常（随后被认定为多发性单神经炎所致）后发生急性非结石性胆囊炎。其嗜酸性粒细胞计数为1860/μL。腹腔镜胆囊切除术后3天，患者因回肠穿孔出现突发严重弥漫性腹痛并伴有低血压。外周嗜酸性粒细胞计数增至14000/μL。遂行回肠切除术。组织病理学检查显示回肠为坏死性血管炎伴嗜酸性粒细胞浸润，胆囊为肉芽肿性血管炎伴嗜酸性粒细胞浸润。给予患者静脉注射甲泼尼龙冲击治疗，连续3天每日1g，随后静脉注射环磷酰胺冲击治疗，剂量为750mg/m²，患者恢复顺利。此后每月额外给予环磷酰胺静脉输注6次，口服泼尼松逐渐减量。2年后最后一次随访时，患者临床状况良好，继续隔日口服泼尼松（10mg）。
哮喘患者出现感觉异常和非结石性胆囊炎等非呼吸道症状应提醒医生考虑CSS。如果神经性症状促使考虑血管炎，药物治疗或许可以避免一次或两次外科手术。
