Shahedi Kamyar, Hanna Ramy Magdy, Melamed Oleg, Wilson James
Department of Medicine Olive-View UCLA Medical Center, Sylmar, CA, USA ; David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Int Med Case Rep J. 2013 Oct 7;6:65-9. doi: 10.2147/IMCRJ.S36546. eCollection 2013.
Wegener's granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener's granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener's granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener's granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener's granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener's granulomatosis, which mimicked inflammatory bowel disease.
韦格纳肉芽肿病,也称为抗中性粒细胞胞浆抗体(ANCA)相关性血管炎,是一种主要表现为肺部、肾脏和鼻窦疾病的小血管血管炎。韦格纳肉芽肿病的患病率为每10万名患者中有3例。在许多病例报告和病例系列中都报道过心血管、神经、皮肤和关节表现。韦格纳肉芽肿病中胃肠道表现较少被提及,尽管此前曾有肠穿孔和肠缺血的形式报道过。此外,小肠活动性韦格纳肉芽肿病有血管炎的特征性表现。我们报告一例老年患者,表现为体重减轻、腹泻和便血。他的症状是慢性的,在诊断前持续了1年多。最初怀疑其病因是由于接触爬行动物感染亚利桑那沙门氏菌导致的炎症性肠病或慢性肠炎。肾活检发现蛋白尿、肾衰竭和寡免疫性肾小球肾炎,同时c-ANCA滴度升高,确诊为韦格纳肉芽肿病合并肠道血管炎。该病例显示了韦格纳肉芽肿病继发慢性十二指肠和空肠炎的非典型表现,其类似于炎症性肠病。