Serologic profiles aiding the diagnosis of autoimmune gastrointestinal dysmotility
Serologic profiles aiding the diagnosis of autoimmune gastrointestinal dysmotility
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DOI：
10.1016/j.cgh.2008.04.009
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发表时间：
2008-09-01
影响因子：
12.6
通讯作者：
Lennon, Vanda A.
中科院分区：
文献类型：
--
作者：
Dhamija, Radhika;Tan, K. Meng;Lennon, Vanda A.
背景与目的：自身免疫性胃肠运动障碍是一种有限的自体自主神经功能障碍，发生在特发性或解剖学上远处的肿瘤中，以前有文献记载或未被怀疑。在这里，我们报告了24名梅奥诊所的患者，他们的血清自身抗体谱有助于这种诊断。方法：采用血清学方法对所有患者进行符合神经系统自身免疫的自身抗体服务评价。回顾他们的病史、运动研究和实验室结果显示，所有人都表现出亚急性胃肠运动障碍。结果：记录的运动异常包括食管运动障碍8（6例失弛缓症），胃排空延迟12，小肠运输缓慢7，结肠运输缓慢4，盆底协同作用障碍4。4例患者行腹部手术；2开始全肠外营养。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（n型5例，P/ q型1例）、乙酰胆碱受体（神经节型11例，肌肉型4例）、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（包括骨骼肌条纹和谷氨酸脱羧酶，65kd异构体）或其他器官特异性自身免疫抗体标记（甲状腺或胃壁细胞特异性）。11例（近期9例，远期2例）诊断为肿瘤：肺、乳腺、子宫内膜、胃肠道和胸腺瘤。4例接受免疫治疗的患者中有4例，2例接受吡哆斯的明治疗的患者中有2例胃肠道症状有中度至显著改善。结论：自身免疫血清学有助于诊断自身免疫性胃肠运动障碍，包括副肿瘤性和特发性，并可能指导治疗。
Background & Aims: Autoimmune gastrointestinal dysmotility is a limited autoinumme 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. Methods: 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. Results: 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. Conclusions: Autoimmune serology aids the diagnosis of autoimmune gastrointestinal dysmotility, both paraneo- plastic and idiopathic, and might guide management.