Dhamija Radhika, Tan K Meng, Pittock Sean J, Foxx-Orenstein Amy, Benarroch Eduardo, Lennon Vanda A
Department of Immunology, Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA.
Clin Gastroenterol Hepatol. 2008 Sep;6(9):988-92. doi: 10.1016/j.cgh.2008.04.009. Epub 2008 Jul 2.
BACKGROUND & AIMS: Autoimmune gastrointestinal dysmotility is a limited autoimmune dysautonomia occurring idiopathically or in the context of an anatomically remote neoplasm, previously documented or unsuspected. Here we report 24 Mayo Clinic patients in whom the profile of serum autoantibodies aided this diagnosis.
All patients were ascertained serologically in the course of service evaluation for autoantibodies consistent with neurologic autoimmunity. Review of their histories, motility studies, and laboratory findings revealed that all had presented with subacute gastrointestinal dysmotility.
Recorded motility abnormalities included esophageal dysmotility 8 (6 had achalasia), delayed gastric emptying 12, slow small intestinal transit 7, slow colonic transit 4, and pelvic floor dyssynergia 4. Four patients underwent abdominal surgery; 2 commenced total parenteral nutrition. Plasma membrane cation channel autoantibodies were detected in 23 patients: neuronal voltage-gated calcium channel (5 N-type and 1 P/Q-type), acetylcholine receptor (11 ganglionic-type and 4 muscle-type), and neuronal voltage-gated potassium channel autoantibodies (4). Two patients had antineuronal nuclear autoantibodies, type 1. Approximately half of the patients had neural autoantibodies (including skeletal muscle striational and glutamic acid decarboxylase, 65kd isoform) or other antibody markers of organ-specific autoimmunity (thyroid or gastric parietal cell specificities). Neoplasia was diagnosed in 11 patients (9 recent, 2 remote): lung, breast and endometrial, gastrointestinal and thymoma. Moderate to dramatic improvement in gastrointestinal symptoms was reported after immunotherapy in 4 of 4 patients treated and after pyridostigmine treatment in 2 of 2 patients treated.
Autoimmune serology aids the diagnosis of autoimmune gastrointestinal dysmotility, both paraneoplastic and idiopathic, and might guide management.
自身免疫性胃肠动力障碍是一种局限性自身免疫性自主神经功能障碍，可特发性发生，或在解剖学上远处存在既往有记录或未被怀疑的肿瘤的情况下发生。在此，我们报告24例梅奥诊所的患者，其血清自身抗体谱有助于该疾病的诊断。
所有患者均在服务评估过程中进行血清学检测，以查找与神经自身免疫相符的自身抗体。回顾他们的病史、动力研究和实验室检查结果发现，所有患者均表现为亚急性胃肠动力障碍。
记录的动力异常包括食管动力障碍8例（6例为贲门失弛缓症）、胃排空延迟12例、小肠传输缓慢7例、结肠传输缓慢4例和盆底协同失调4例。4例患者接受了腹部手术；2例开始接受全胃肠外营养。23例患者检测到质膜阳离子通道自身抗体：神经元电压门控钙通道（5例为N型，1例为P/Q型）、乙酰胆碱受体（11例为神经节型，4例为肌肉型）和神经元电压门控钾通道自身抗体（4例）。2例患者有1型抗神经元核自身抗体。大约一半的患者有神经自身抗体（包括骨骼肌横纹肌和谷氨酸脱羧酶65kd亚型）或其他器官特异性自身免疫的抗体标志物（甲状腺或胃壁细胞特异性）。11例患者诊断为肿瘤（9例为近期肿瘤，2例为远处肿瘤）：肺癌、乳腺癌和子宫内膜癌、胃肠道肿瘤和胸腺瘤。4例接受免疫治疗的患者中有4例以及2例接受吡啶斯的明治疗的患者中有2例报告胃肠道症状有中度至显著改善。
自身免疫血清学有助于诊断副肿瘤性和特发性自身免疫性胃肠动力障碍，并可能指导治疗。
