World journal of methodology · 2025
Need for combined therapy for a rare case of autoimmune autonomic ganglionopathy: A case report.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Angeloudi Elena, Xanthopoulou Maria, Lima Maria, Kalyvas Athanasios C, Kotoulas Serafeim C, Dimitriou Maria, Ioannidis Panagiotis, Liakos Aris, Gigi Eleni
Аннотация
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.
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.