Abstract: PUB238
Dysautonomia: A Nephrology Consult
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Ashfaq, Areeba, University of Rochester Medical Center, Rochester, New York, United States
- Drury, Erika, University of Rochester Medical Center, Rochester, New York, United States
- Lusco, Mark, University of Rochester Medical Center, Rochester, New York, United States
Introduction
Dysautonomia presents with dizziness and hypotension, while amyloidosis leads in organ dysfunction, causing fatigue and swelling. This case emphasizes the association between these conditions, highlighting the need for a comprehensive renal evaluation at initial presentation.
Case Description
A 62 year old female diagnosed with post-COVID dysautonomia 8 months prior, presented with recurrent syncope and orthostatic hypotension. Symptoms progressed to include fatigue with minimal exertion and lower extremity swelling. She was admitted for another syncopal episode, now with new-onset AKI. Workup was significant for serum creatitine of 2.14 mg/dL (baseline 1.2mg/dL), serum albumin of 2.9, and 24-hour urine protein of 7.1g. Serologies for hepatitis B, hepatitis C, HIV, and autoimmune markers were negative. SPEP with immunofixation showed a M-spike. Medical records review revealed urinalysis with 3+ protein a month prior to hospitalization with no furtherevaluation at that time.
Renal biopsy showed amorphous, acellular, Congo red–positive deposits with apple-green birefringence in glomeruli, arteries, and the interstitium. Immunoflorescence (IF) demonstrated monoclonal lambda staining, and electron microscopy showed randomly arranged, straight fibrils, consistent with AL amyloidosis, confirmed by mass spectrometry.
Discussion
This case highlights the clinical spectrum of AL amyloidosis including nephrotic syndrome and dysautonomia. Urinalysis should be included in dysautonomia workup to aide in early detection of nephropathy.
Image 1: Glomerulus with amorphous acellular material in mesangium and capillary loops (H&E).
Image 2: Strong lambda staining of amorphous acellular material in glomerulus and interstitium (IF).
Image 3a-b: Amorphous acellular material in glomeruli, arteries and interstitium shows congo staining (3a) with apple-green birefringence under polarized light (3b) (Congo red).