©The Author(s) 2025. Published by Baishideng Publishing Group Inc. All rights reserved.
Need for combined therapy for a rare case of autoimmune autonomic ganglionopathy: A case report
Eleni Gigi, Aris Liakos, Panagiotis Ioannidis, Maria Dimitriou, Serafeim C Kotoulas, Athanasios C Kalyvas, Maria Lima, Maria Xanthopoulou, Elena Angeloudi
Elena Angeloudi, Maria Xanthopoulou, Aris Liakos, Eleni Gigi, Second Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece
Maria Lima, Athanasios C Kalyvas, Maria Dimitriou, Panagiotis Ioannidis, Second Neurology Department, AHEPA University Hospital, Thessaloniki 54636, Greece
Serafeim C Kotoulas, Adult CF Unit, Pulmonary Department, General Hospital of Thessaloniki "G. Papanikolaou", Aristotle University of Thessaloniki, Thessaloniki 57010, Greece
Author contributions: Angeloudi E, Xanthopoulou M, Liakos A, and Gigi E conceptualized the study; Angeloudi E, Xanthopoulou M, Liakos A, and Gigi E developed the methodology; Ioannidis P, Lima M, and Gigi E validated the findings; Angeloudi E, Xanthopoulou M, Liakos A, Gigi E, Kalyvas AC, and Ioannidis P conducted the investigation; Kalyvas AC, Dimitriou M, and Kotoulas SC provided resources; Angeloudi E, Xanthopoulou M, Kalyvas AC, Lima M, Kotoulas SC, and Dimitriou M curated the data; Angeloudi E prepared the original draft; Angeloudi E, Xanthopoulou M, Liakos A, Ioannidis P, and Gigi E reviewed and edited the manuscript; Liakos A, Ioannidis P, and Gigi E supervised the study.
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Elena Angeloudi, MD, MSc, Research Scientist, Second Department of Internal Medicine, Hippokration Hospital, Aristotle University of Thessaloniki, Kon
stantinoupoleos 49, Thessaloniki 54642, Greece.
elena-angeloudi@hotmail.com
Received: September 18, 2024
Revised: February 9, 2025
Accepted: February 24, 2025
Published online: December 20, 2025
Processing time: 323 Days and 16.8 Hours
BACKGROUND
Autoimmune autonomic ganglionopathy (AAG), formerly known as acute pandysautonomia, is a rare, acquired, antibody-mediated, potentially curable autonomic disorder that presents with diffuse autonomic failure. High levels of anti-ganglionic nicotinic acetylcholine receptor (gAChR) serum antibodies are detected in approximately 50% of AAG cases, confirming the diagnosis.
CASE SUMMARY
We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period. Recently, the patient was unable to stand upright for more than a few seconds before fainting. Additionally, he presented with decreased sweating, dry mouth, urinary retention, early satiety, weight loss, bloating, constipation, and erectile dysfunction. Neurological examination revealed dilated pupils that were unresponsive to light. Deep tendon reflexes were absent or diminished. Serologic evaluation revealed the presence of gAChR autoantibodies. An orthostatic hypotension test yielded a positive result. The patient did not respond to symptomatic therapy, including midodrine, fludrocortisone and atomoxetine. Second-line therapy with immunoadsorption produced a noticeable clinical improvement; however, orthostatic hypotension persisted. Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.
CONCLUSION
Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.
Core Tip: This case highlights the potential benefit of combined immunoadsorption and rituximab therapy in refractory autoimmune autonomic ganglionopathy. Despite first-line therapy failure, the patient achieved significant long-term improvement, suggesting that a stepwise immunomodulatory approach may be necessary in resistant cases.
