Autoimmune Autonomic Ganglionopathy in a Patient with Celiac Disease on Dialysis: A Rare Case of Refractory Orthostatic Hypotension

Introduction: Orthostatic hypotension (OH) is a frequent yet complex complication in hemodialysis patients. While commonly linked to ultrafiltration, volume depletion, or medications, persistent symptoms in autoimmune patients may signal an underlying autonomic dysfunction. I present a case of post-dialysis OH in a celiac patient diagnosed with Autoimmune Autonomic Ganglionopathy, managed through pharmacologic, immunologic, and lifestyle interventions. Case Description: A 56-year-old male with a history of celiac disease and hypertension was admitted with respiratory distress. Workup revealed ESRD secondary to chronic hypertension, and he was initiated on peritoneal dialysis. However, after a few sessions, he developed severe orthostatic symptoms, including dizziness and syncope, significantly impairing mobility. Due to intolerance, he was switched to in-center hemodialysis, where he exhibited resting hypertension with marked orthostatic hypotension and paradoxical bradycardia, suggesting autonomic dysfunction. Cardiac, endocrine, and medication-related causes were excluded. Despite monitored midodrine therapy, symptoms persisted. A strong clinical suspicion for autonomic dysfuntion was considered. Further evaluation revealed positive ganglionic (α3-AChR) autoantibodies, confirming the diagnosis of Autoimmune Autonomic Ganglionopathy. After the diagnosis was confired, the patient was treated with lifestyle modifications, including the use of an abdominal binder and postural precautions. Additionally, intravenous immunoglobulin (IVIG) therapy was initiated 3 times a week. Over time, his functional status and hemodynamic stability improved, allowing successful transition back to peritoneal dialysis. Discussion: This case highlights the diagnostic challenge of OH in dialysis patients. While OH is often attributed to volume shifts, autoimmune autonomic dysfunction should be considered in patients with severe symptoms, particularly those with autoimmune conditions. Treatment with IVIG or Plasma Exchange has been reported to be effective. There are also strong evidences that novel immunosuppressant agents such as Mycophenolate mofetil and Rituximab may be effective in certain patients who are unresponsive to IVIG or PE. Autoimmune Autonomic Ganglionopathy (AAG) is a rare but potentially treatable cause of OH, requiring a high index of clinical suspicion.