Autoimmune enteropathy and colitis in an adult patient
Autoimmune enteropathy and colitis in an adult patient
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DOI：
10.1023/a:1024705032326
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发表时间：
2003-08-01
影响因子：
3.1
通讯作者：
Notarbartolo, A
中科院分区：
文献类型：
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作者：
Carroccio, A;Volta, U;Notarbartolo, A
在成人中，肠道细胞循环自身抗体的存在很少被描述，被认为是难治性口炎的可能原因。我们的目的是描述一个成年患者的情况下，血清抗肠上皮细胞自身抗体与临床图片的特点是参与小肠和结肠。一位50岁的女性，从20岁开始患有腹泻伴粘液和血液、腹痛、消瘦、贫血和白细胞减少症。她还患有丙型肝炎病毒感染和轻度慢性肝炎。家族史为自身免疫阳性。据报道，吃了含麸质的食物后症状加重，但抗转氨酶和抗肌内膜抗体呈阴性。肠组织学显示轻度、片状绒毛萎缩，上皮内淋巴细胞计数高，但携带γ/δ +受体的上皮内淋巴细胞数量正常。HLA：A11，A31（19），B52（5），DR 15（2），DR 14（6），DR 51，DR 52，DQ 1。结肠镜检查未显示溃疡或糜烂，结肠组织学显示中度炎症浸润，无轻微隐窝变形或肉芽肿。RAST试验呈阳性乳白蛋白，乳球蛋白，酪蛋白，鸡蛋，麦醇溶蛋白。开始寡抗原饮食后，大便频率最初下降，但粪便中的粘液持续存在，伴有血性腹泻。经过一年的饮食，营养参数低，贫血与低白细胞计数持续存在。上消化道和下消化道内镜检查以及小肠和结肠的组织学几乎无变化。因此，测定了天然自身抗体和肠上皮细胞自身抗体。患者IgG类肠上皮细胞自身抗体呈阳性，滴度为1：34。其他器官特异性或非器官特异性自身抗体均为阳性。开始泼尼松龙治疗，症状改善。经过一年的这种治疗加消除饮食，她重新评估。排便频率正常，体重增加，虚弱已完全消退。不存在IgG抗肠上皮细胞自身抗体。远端十二指肠的组织学显示绒毛/隐窝比例正常，IEL浸润减少。结肠组织学显示固有层炎性浸润减少。总之，我们报告了一例成人患者的全身性肠道疾病，影响小肠和结肠，其特征是存在循环抗肠上皮细胞自身抗体。肠上皮细胞自身抗体的系统检测不仅应在难治性口炎性腹泻患者中进行，还应在有上、下肠道症状但尚未确诊的受试者中进行。
The presence of circulating autoantibodies to gut enterocytes has been very rarely described in adults and is considered a possible cause of refractory sprue. Our aims was to describe the case of an adult patient with serum anti-enterocyte autoantibodies associated with a clinical picture characterized by involvement of both the small intestine and colon. A female, age 50, had suffered from diarrhea with mucus and blood, abdominal pain, thinness, anemia, and leukopenia since the age of 20. She also suffered from HCV infection and had mild chronic hepatitis. Family history was positive for autoimmunity. Symptoms were reported to worsen after eating gluten-containing foods, but anti-transglutaminase and anti-endomysial antibodies were negative. Intestinal histology showed mild, patch villous atrophy with a high intraepithelial lymphocyte count, but a normal number of intraepithelial lymphocytes carrying the gamma/delta+ receptor. HLA was: A11, A31 (19), B52 (5), DR 15 (2), DR 14 (6), DR 51, DR 52, DQ1. Colonoscopy did not show ulcerations or erosions and colon histology showed a moderate inflammatory infiltrate without minor crypt distortion or granuloma. RAST tests were positive for lactalbumin, lactoglobulin, casein, egg, and gliadin. After commencement of an oligoantigenic diet, stool frequency initially decreased, but the presence of mucus in the stools persisted, with episodes of bloody diarrhea. After one year of diet, nutritional parameters were low and anemia associated with a low leukocyte count persisted. Upper and lower gastrointestinal endoscopy and histology of the small intestine and colon were virtually unchanged. Consequently, natural autoantibodies and enterocyte autoantibodies were assayed. The patient was positive for IgG class enterocyte autoantibodies at a titer of 1: 34. No other organ-specific or non-organ-specific autoantibodies were positive. Prednisolone treatment was started and the symptoms improved. After one year of this treatment plus elimination diet she was reevaluated. Bowel movement frequency was normal, body weight increased, and the asthenia had completely regressed. IgG anti-enterocyte autoantibodies were absent. Histology of the distal duodenum showed a normal villus/crypt ratio and IEL infiltration was reduced. Colon histology showed a reduction in inflammatory infiltrate in the lamina propria. In conclusion, we report a case of generalized gut disorder in an adult patient, affecting both the small intestine and the colon and characterized by the presence of circulating anti-enterocyte autoantibodies. Systematic testing for enterocyte autoantibodies should be performed not only in patients with refractory sprue, but also in subjects with upper and lower intestinal symptoms who have not been definitively diagnosed.