Clinical Improvement With Pyridostigmine in a Patient With Acetylcholine Receptor Antibody-Associated Autoimmune Gastrointestinal Dysmotility

Autoimmune gastrointestinal dysmotility (AGID) is a rare form of limited autoimmune dysautonomia caused by autoantibodies against the enteric nervous system. Our patient was a 53-year-old man with 1 year of bloating, intolerance of oral intake, and recurrent ileus. Esophageal manometry showed aperistalsis and hypotensive lower sphincter, consistent with scleroderma esophagus. However, because the patient had no other sequelae of this disease, AGID was considered. Serologic evaluation revealed ganglionic acetylcholine receptor autoantibodies. Treatment with pyridostigmine led to resolution of symptoms. Early recognition of AGID should be considered when manometry shows scleroderma esophagus in patients without other evidence of systemic sclerosis.