Abstract: SA-PO254
Triple Hit – Case of IgA nephropathy with Thin Glomerular Basement Disease and Nutcracker Syndrome
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Savedchuk, Solomiia, Beaumont Health, Royal Oak, Michigan, United States
- Mansuri, Saima, Beaumont Hospital, Royal oak, Michigan, United States
Introduction
Included in the differential of hematuria are IgA nephropathy (IgAN) and thin glomerular basement membrane disease (TGBM). IgAN is the most common cause of idiopathic glomerulonephritis with slow progression to End Stage Renal Disease in up to 50% of patients. IgAN has been reported to be associated with TGBM. Recurrent gross hematuria could also be seen in nutcracker syndrome, the incidence of which is increasing with the use of CT/MR-angiography. We report a patient diagnosed concomitantly with nutcracker syndrome, IgAN, and TGBM
Case Description
We present a case of 33-year-old Caucasian female with hematuria. She reported microscopic hematuria since the age of 7 and strong family history of microscopic hematuria. Five years prior to presentation she started developing intermittent gross hematuria with periods of stress and URTIs. Her past medical history is significant for long-standing abdominal and pelvic pain. She also reported episodes of orthostatic hypotension. She was diagnosed with median arcuate ligament syndrome and underwent MAL release in 2015 without resolution of symptoms. Physical exam was unremarkable. Laboratory and other work up was unremarkable except for 3+ blood with numerous RBC/hpf without evidence of infection on urinalysis. Urine protein:creatinine ratio ranged from 0.62-2.04. She had orthostatic proteinuria. Her renal function was stable. Her prior MRA from 2015 revealed a focal narrowing of left renal vein (LRV) between aorta and SMA. Given this constellation of symptoms, not completely explained by one pathology, possibility of nutcracker syndrome was entertained and CTA was repeated which confirmed the LRV narrowing. However, due to her family history of hematuria, intermittent episodes of gross hematuria and proteinuria, a renal biopsy was performed with findings suggestive of IgAN and TGBM.
Discussion
There are published case reports about the association between IgAN and TGBM as well as IgAN and nutcracker syndrome. However, coexistence of all three diseases has not been reported. There are no distinguishing clinical or laboratory features. The causal relationship and prognosis remains unclear. The presence of multiple glomerular pathology poses a unique diagnostic and management challenge. This, combined with anatomic vascular abnormality, further complicates management.