嗜酸细胞胃肠炎病例

Case Review,浙江大学医学院附属邵逸夫医院 消化科王侃2012-05-24,现病史,患者，男，48岁，杭州人，经商患者半月余前无明显诱因下出现上腹痛，不剧可忍，持续2小时后缓解，无肩背部放射痛，无发热畏寒，无恶心呕吐，无肛门停止排气排便，无胸闷心悸，无呕血黑便，此后上腹部隐痛每天发作2-3次，与进食、排便无明显相关，半月来患者自觉症状加重，上腹部持续隐痛不适，阵发性加重，有时较难忍，发作频率增加，遂来我院就诊。 自病以来，神清，精神可，胃纳可，睡眠一般，大小便无殊，体重无明显减轻。,既往史/个人史/家族史,既往体健否认食物药物过敏史否认长期服药史吸烟史：1包/天20年，否认饮酒史家人体健,体格检查,神清，精神偏软，生命体征平稳，浅表淋巴结未及肿大，两肺呼吸音清，未闻及啰音，心律齐，未闻及杂音，腹软，上腹部及脐上轻压痛，无反跳痛，肠鸣音6次/分，双下肢无水肿。,辅助检查,腹部CT：肝内小钙化灶，右肾小结石，右肾盂旁囊性灶，肾盂旁囊肿可能。胃镜：胃腔内大量食物残留，影响观察；病理：慢性炎，灶性活动性，灶性肠化，幽门螺杆菌：阴性,入院诊断,腹痛： 消化性溃疡？ 消化道肿瘤？ 胆管结石？,诊治经过,诊治经过,予屈他维林、泮托拉唑静滴，患者症状无缓解BUS：肝内钙化灶，右肾小结石,腹痛+嗜酸粒细胞增多=?,鉴别诊断,Yan and Shaffer. Gut 2009.,诊治经过,复查胃镜,诊治经过,术前免疫、甲状腺功能、 尿常规、PPD试验、大便培养、CXR无殊开瑞坦1# po qd，2天后症状缓解拒绝骨髓穿刺检查，带药出院,诊治经过,追问病史：起病前食用花生出院后2周门诊随访，症状未再发，嗜酸性粒细胞计数恢复正常,病理诊断,病理诊断,胃镜病理：送检“十二指肠降部”及“球部”组织，镜示小肠粘膜内见大量淋巴浆细胞聚集，并见较多嗜酸性粒细胞浸润，有处高倍达50个以上，可见脱颗粒现象，未见血管炎，未见肉芽肿，未见隐窝脓肿，间质水肿不明显。送检“胃窦”、“胃角”及“胃体”组织，镜示胃粘膜呈慢性炎改变伴淋巴细胞灶性聚集，可见部分嗜酸性粒细胞散在分布。HP阴性,最后诊断,原发性嗜酸性粒细胞性胃肠炎 Talley诊断标准：(1) 有胃肠道症状(2) 病理证实胃肠道一处或多处嗜酸性粒细胞浸润(3) 排除寄生虫感染和胃肠道外嗜酸性粒细胞增多疾病,原发性嗜酸性粒细胞性胃肠炎流行病学,过敏性胃肠病（Allergic gastroenteropathy）最早于1937年由Kaijser提出发病率约为1/10万好发年龄30-60岁发病男女比例1.4:1 25%-75%的患者有过敏病史,Yan and Shaffer. Gut 2009.,发病机制,分型与临床表现,辅助检查,75%-80%的患者外周血嗜酸粒细胞升高IgE水平可以升高或者正常胃镜下无特异性表现：粘膜充血、红斑、水肿、结节、蠕动异常影像学表现各异,病理表现,灶性分布，最常累及胃和十二指肠嗜酸性粒细胞浸润（20-50/HPF） 嗜酸性粒细胞可侵及粘膜上皮层、固有肌层、浆膜层 微脓肿形成 脱颗粒现象,诊断标准,Talley诊断标准： (1) 有胃肠道症状；(2) 病理证实胃肠道一处或多处嗜酸性粒细胞浸润；(3) 排除寄生虫感染和胃肠道外嗜酸性粒细胞增多疾病Leinbach诊断标准： (1) 进食特殊食物后出现胃肠道症状； (2)外周血嗜酸粒细胞增多；(3) 病理证实胃肠道嗜酸性粒细胞浸润除外继发性的胃肠道嗜酸粒细胞浸润,治疗,避免接触过敏原：花生、大豆、鸡蛋、 牛奶、花粉激素：泼尼松 20-40 mg/天，6-8周后减量抗过敏药物：色甘酸钠、酮替芬、孟鲁司特免疫抑制剂：硫唑嘌呤手术：胃肠道穿孔、梗阻,小结,THANKS,Although considered idiopathic, an allergic mechanism may beinvolved as most patients exhibit increased total IgE and food-specific IgE levels. Secondary disorders linked to EGE include the hypereosinophilic syndrome (HES), coeliac disease, Crohns disease, vasculitis (ChurgStrauss syndrome, polyarteritis nodosa), connective tissue disease (scleroderma), infection (Helicobacter pylori, parasites) and drug injury/hypersensitivity.,The diagnosis of EGE may be elusive. Symptoms are non-specific. Peripheral eosinophilia is variable; the eosinophil count is normal in 25% of patients.Atopy and allergies are associated in 2575% of cases.,Mucosal disease is the most readily diagnosed form of EGE, given that endoscopy can directly visualise any mucosal changes and acquire biopsies.Endoscopic features are nevertheless rather non-pecific: thickened folds, erythema, friability, nodularity, and abnormal peristalsis.,修正诊断,腹痛： 寄生虫感染？ 嗜酸粒细胞性胃肠炎？ 高嗜酸粒细胞综合征？,预后,容易复发,自然病程免疫抑制剂的疗程激素的减量法,As per the definition, patients with EGIDs and sustained blood eosinophilia exceeding 1500 cells/mm would have HES. Therefore, routine surveillance of the cardiorespiratory system is warranted,especially if the patients have extragastrointestinal manifestations.,They suggested the following diagnostic criteria:(1) the presence of gastrointestinal symptoms, (2) biopsies showing eosinophilic infiltration of one or more areas of the gastrointestinal tract from esophagus to colon, or characteristic radiologic findings with peripheral eosinophilia, (3) no evidence of parasitic or extraintestinal disease.,The gastrointestinal tract is the main nonhematopoietic organ where eosinophils reside in the healthy state. Eosinophils are normally present in the lamina propria, but the number of eosinophils regarded as pathological for various sites along the gastrointestinal tract is debated; the highest concentrations are found in the cecum and appendix. Within the gastrointestinal tract,In published reports, the most frequently affected organs were the small intestine and stomach.Many patients with EG have a history of seasonal allergies, food sensitivities, eczema, asthma, and atopy. Talley et al. reported a history of allergy in 20 of 40 patients with EG.The most common foods reported to be positive by SPT include common food allergenspeanuts, eggs, soy, cow milk, and wheatin addition to beans, rye, and beef.,HES is an idiopathic condition defined as marked peripheral eosinoilia exceeding 1500 cells/l that persists for at leas 6 months; there is organ dysfunction. Target-organ damage mediated by eosinophils is highly variable mong patients, with involvement of skin, heart, lung, central and peripheral nervous systems in more than 50% of cases.,therapeutic strategy. First, they recommended specifi allergy avoidance (airborne and dietary). If this failed to improve EGID pathology, then they recommended glucocorticoid therapy, first starting with topical delivery and then considering systemic delivery. They also advocated elemental diet trials aimed at avoiding all protein antigen exposure.,Most patients receive prednisolone in doses from 20 to 40 mg/day for 68 weeks with various schemes of dose tapering.Other drugs, such as sodium chromoglycate and ketotifen (antihistamine and mast cell-stabilizing agents), suplatast tosilate (antiallergic drug that suppresses cytokines production), and montelukast (leukotriene receptor antagonist), were found to be effective in the management as well as steroid-sparing agents in some case reports but not in others. Surgical treatment is required for patients with intestinal per