July 23, 2022
Autoimmune gastrointestinal dysmotility (AGID) is a limited form of dysautonomia that affects digestive tract motility. Although rare, the condition can be debilitating, with individuals experiencing nausea and dramatic weight loss. AGID can manifest as a gastrointestinal hypo- or hypermobility or as a generalized autonomic syndrome, with subacute onset and a wide range of symptoms.
"The presentation varies from person to person, and that probably leads to a lot of patients being misdiagnosed," says Divyanshu (Div) Dubey, M.B.B.S., an autoimmune neurologist at Mayo Clinic in Rochester, Minnesota. "Early diagnosis is important because immunotherapy can result in meaningful recovery."
The etiology of AGID can be idiopathic, post-viral or paraneoplastic. Patients might have an individual history or a family history of autoimmune disease or cancer.
Diagnosis requires close collaboration between autoimmune neurologists and gastroenterologists. Mayo Clinic's state-of-the-art testing includes GI transit studies, objective autonomic testing and screening for neural antibodies.
"Mayo Clinic experts were the first to report objective evidence that immunotherapy might reverse AGID. We are committed to identifying potential autoimmune mechanisms for the benefit of patients," says Sean J. Pittock, M.D., an autoimmune neurologist and director of the Center for Multiple Sclerosis and Autoimmune Neurology at Mayo Clinic's campus in Minnesota.
In addition to subacute onset of nausea and weight loss, the symptoms associated with AGID include early satiety, vomiting, bloating, diarrhea and constipation. Mayo Clinic's comprehensive GI transit studies include whole GI scintigraphy and manometry. Autonomic reflex testing and thermoregulatory sweat testing can provide additional evidence.
Testing for neural antibodies also is key. Mayo Clinic Laboratories offers a serum-based panel assay for AGID.
"We've learned that it's sometimes difficult to distinguish the specific antibodies a patient has. A panel-based approach that includes multiple antibodies increases the possibility of yielding a positive test result that can guide our management of patients," Dr. Dubey says.
Mayo Clinic recommends that antibody test results be considered in conjunction with individuals' clinical presentations. "A subset of patients are antibody negative," Dr. Pittock says. "At times we use a trial of immunotherapy given the reversible and treatable nature of this condition."
AGID and SARS-CoV-2
Mayo Clinic specialists have published the first report of AGID occurring after SARS-CoV-2 infection. A case study in Neurogastroenterology and Motility describes the successful treatment of a 17-year-old girl who developed debilitating nausea after having coronavirus disease 2019 (COVID-19).
The patient tested positive for SARS-CoV-2 in March 2020. Although her fever and cough resolved within a week, five weeks later she presented with intractable nausea that required hospitalization. Over 10 months, the patient progressed to total parenteral nutrition.
She had a past medical history of celiac disease and tested positive for neural antibodies. Based on those factors and her subacute course, Mayo Clinic specialists recommended a trial treatment with intravenous immunoglobulin. Within four months, her symptoms greatly improved and she was able to tolerate a full oral diet.
"It's difficult to conclude that this patient's AGID was specifically triggered by SARS-CoV-2 since we don't have specific molecular evidence of the virus in the autonomic GI system," Dr. Pittock says. "But the timing and evolution support the association. AGID should be considered as part of the differential diagnosis in patients with subacute GI dysmotility, a recent viral illness, and evidence of family or personal history of autoimmunity."
For more information
Center for Multiple Sclerosis and Autoimmune Neurology. Mayo Clinic.
Montalvo M, et al. Autoimmune gastrointestinal dysmotility following SARS-CoV-2 infection successfully treated with intravenous immunoglobulin. Neurogastroenterology and Motility. 2022;34:e14314.
Refer a patient to Mayo Clinic.