嗜酸細胞胃腸炎病例

1、Case Review浙江大學醫(yī)學院附屬邵逸夫醫(yī)院 消化科王侃2012-05-24現病史 患者，男，48歲，杭州人，經商 患者半月余前無明顯誘因下出現上腹痛，不劇可忍，持續(xù)2小時后緩解，無肩背部放射痛，無發(fā)熱畏寒，無惡心嘔吐，無肛門停止排氣排便，無胸悶心悸，無嘔血黑便，此后上腹部隱痛每天發(fā)作2-3次，與進食、排便無明顯相關，半月來患者自覺癥狀加重，上腹部持續(xù)隱痛不適，陣發(fā)性加重，有時較難忍，發(fā)作頻率增加，遂來我院就診。 自病以來，神清，精神可，胃納可，睡眠一般，大小便無殊，體重無明顯減輕。 既往史/個人史/家族史 既往體健 否認食物藥物過敏史 否認長期服藥史 吸煙史：1包/天20年，否認飲酒史

2、 家人體健體格檢查 神清，精神偏軟，生命體征平穩(wěn)，淺表淋巴結未及腫大，兩肺呼吸音清，未聞及啰音，心律齊，未聞及雜音，腹軟，上腹部及臍上輕壓痛，無反跳痛，腸鳴音6次/分，雙下肢無水腫。 輔助檢查腹部CT：肝內小鈣化灶，右腎小結石，右腎盂旁囊性灶，腎盂旁囊腫可能。胃鏡：胃腔內大量食物殘留，影響觀察；病理：慢性炎，灶性活動性，灶性腸化，幽門螺桿菌：陰性 入院診斷 腹痛： 消化性潰瘍？ 消化道腫瘤？ 膽管結石？ 診治經過診治經過 予屈他維林、泮托拉唑靜滴，患者癥狀無緩解 BUS：肝內鈣化灶，右腎小結石腹痛腹痛+嗜酸粒細胞增多嗜酸粒細胞增多=?鑒別診斷Yan and Shaffer. Gut 2009.

3、診治經過 復查胃鏡診治經過術前免疫、甲狀腺功能、 尿常規(guī)、PPD試驗、大便培養(yǎng)、CXR無殊開瑞坦1# po qd，2天后癥狀緩解拒絕骨髓穿刺檢查，帶藥出院診治經過 追問病史：起病前食用花生 出院后2周門診隨訪，癥狀未再發(fā)，嗜酸性粒細胞計數恢復正常病理診斷病理診斷 胃鏡病理：送檢“十二指腸降部”及“球部”組織，鏡示小腸粘膜內見大量淋巴漿細胞聚集，并見較多嗜酸性粒細胞浸潤，有處高倍達50個以上，可見脫顆?，F象，未見血管炎，未見肉芽腫，未見隱窩膿腫，間質水腫不明顯。送檢“胃竇”、“胃角”及“胃體”組織，鏡示胃粘膜呈慢性炎改變伴淋巴細胞灶性聚集，可見部分嗜酸性粒細胞散在分布。HP陰性最后診斷原發(fā)性嗜酸

4、性粒細胞性胃腸炎原發(fā)性嗜酸性粒細胞性胃腸炎 Talley診斷標準：(1) 有胃腸道癥狀(2) 病理證實胃腸道一處或多處嗜酸性粒細胞浸潤(3) 排除寄生蟲感染和胃腸道外嗜酸性粒細胞增多疾病原發(fā)性嗜酸性粒細胞性胃腸炎流行病學 過敏性胃腸病（Allergic gastroenteropathy） 最早于1937年由Kaijser提出 發(fā)病率約為1/10萬 好發(fā)年齡30-60歲 發(fā)病男女比例1.4:1 25%-75%的患者有過敏病史Yan and Shaffer. Gut 2009.發(fā)病機制分型與臨床表現輔助檢查 75%-80%的患者外周血嗜酸粒細胞升高 IgE水平可以升高或者正常 胃鏡下無特異性表現

5、：粘膜充血、紅斑、水腫、結節(jié)、蠕動異常 影像學表現各異病理表現 灶性分布，最常累及胃和十二指腸 嗜酸性粒細胞浸潤（20-50/HPF） 嗜酸性粒細胞可侵及粘膜上皮層、固有肌層、漿膜層 微膿腫形成 脫顆?，F象診斷標準 Talley診斷標準：診斷標準： (1) 有胃腸道癥狀；(2) 病理證實胃腸道一處或多處嗜酸性粒細胞浸潤；(3) 排除寄生蟲感染和胃腸道外嗜酸性粒細胞增多疾病 Leinbach診斷標準：診斷標準： (1) 進食特殊食物后出現胃腸道癥狀； (2)外周血嗜酸粒細胞增多；(3) 病理證實胃腸道嗜酸性粒細胞浸潤 除外繼發(fā)性的胃腸道嗜酸粒細胞浸潤除外繼發(fā)性的胃腸道嗜酸粒細胞浸潤治療 避免接觸

6、過敏原：花生、大豆、雞蛋、 牛奶、花粉 激素：潑尼松 20-40 mg/天，6-8周后減量 抗過敏藥物：色甘酸鈉、酮替芬、孟魯司特 免疫抑制劑：硫唑嘌呤 手術：胃腸道穿孔、梗阻小結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 hypereosinoph

7、ilic 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. Periph

8、eral 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

9、are nevertheless rather non-pecific: thickened folds, erythema, friability, nodularity, and abnormal peristalsis.修正診斷 腹痛： 寄生蟲感染？ 嗜酸粒細胞性胃腸炎？ 高嗜酸粒細胞綜合征？預后 容易復發(fā) 自然病程 免疫抑制劑的療程 激素的減量法 As per the definition, patients with EGIDs and sustained blood eosinophilia exceeding 1500 cells/mm would have HES. There

10、fore, 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

11、 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. Eosi

12、nophils 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

13、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 i

14、nclude 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 eosino

15、phils 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 recomme

16、nded 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 do

17、se 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