Pasha Shabana F, Lunsford Tisha N, Lennon Vanda A
Department of Gastroenterology & Hepatology, Mayo Clinic College of Medicine, Scottsdale, Arizona, USA.
Gastroenterology. 2006 Nov;131(5):1592-6. doi: 10.1053/j.gastro.2006.06.018.
BACKGROUND & AIMS: Autoimmune gastrointestinal dysmotility (AGID) is a limited form of autoimmune autonomic neuropathy occurring idiopathically or in a paraneoplastic context. This disorder is considered rare, but is underrecognized as a cause for GI dysmotilities of varying anatomic extent, severity, and duration. We describe the diagnosis and management of an instructive case.
A 60-year-old (nondiabetic) woman presented with a 15-year history of severe isolated gastroparesis. Paraneoplastic autoantibody evaluation aided the diagnosis of AGID. This included indirect immunofluorescence (neuronal nuclear and cytoplasmic antibodies), radioimmunoprecipitation assays (neuronal and muscle plasma membrane cation channel antibodies), and enzyme-linked immunosorbent assay (muscle striational antibodies).
Serologic testing revealed both ganglionic neuronal acetylcholine receptor and N-type voltage-gated calcium channel autoantibodies. This profile was consistent with AGID and, despite the long history, raised the possibility of lung, breast, or ovarian carcinoma or thymoma. An underlying neoplasm was excluded by appropriate investigations. In a 1-month trial of oral pyridostigmine therapy, the patient's GI symptoms improved and her weight stabilized. Pyridostigmine was continued at a low dose, and was supplemented by tegaserod.
Autoimmune serology is a valuable adjunct to the diagnosis and guide to management of patients with AGID. The favorable response to acetylcholinesterase inhibitors, despite a 15-year history, suggests an immunopharmacologic rather than an inflammatory cytotoxic pathology. Immunomodulatory therapy may not always be required. Of numerous autoantibodies currently recognized as biomarkers of AGID, the ganglionic acetylcholine receptor autoantibody is the only proven pathophysiologic effector. Certain neuronal nuclear and cytoplasmic autoantibodies are highly predictive of an underlying malignancy.
自身免疫性胃肠动力障碍（AGID）是一种自身免疫性自主神经病变的局限性形式，可自发发生或在副肿瘤情况下出现。这种疾病被认为很罕见，但作为不同解剖范围、严重程度和病程的胃肠动力障碍的病因，它未得到充分认识。我们描述了一个具有指导意义的病例的诊断和治疗。
一名60岁（非糖尿病）女性，有15年严重孤立性胃轻瘫病史。副肿瘤自身抗体评估有助于AGID的诊断。这包括间接免疫荧光法（神经元核抗体和细胞质抗体）、放射免疫沉淀试验（神经元和肌细胞质膜阳离子通道抗体）和酶联免疫吸附测定（肌横纹抗体）。
血清学检测显示存在神经节神经元乙酰胆碱受体和N型电压门控钙通道自身抗体。这种情况与AGID一致，尽管病史较长，但仍提示有肺癌、乳腺癌或卵巢癌或胸腺瘤的可能。通过适当检查排除了潜在肿瘤。在为期1个月的口服吡啶斯的明治疗试验中，患者的胃肠道症状改善，体重稳定。继续以低剂量服用吡啶斯的明，并辅以替加色罗。
自身免疫血清学是AGID患者诊断的有价值辅助手段和治疗指导。尽管有15年病史，但对乙酰胆碱酯酶抑制剂的良好反应提示为免疫药理学而非炎症性细胞毒性病理。并非总是需要免疫调节治疗。在目前被认为是AGID生物标志物的众多自身抗体中，神经节乙酰胆碱受体自身抗体是唯一已证实的病理生理效应器。某些神经元核抗体和细胞质自身抗体高度预测潜在恶性肿瘤。
