Abstract: PUB176
IgA Nephropathy in a Patient with Beta-Thalassemia Minor: A Case Report
Session Information
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Perez-Ortiz, Andric C., Universidad Panamericana, Ciudad de Mexico, Ciudad de México, Mexico
- Avila-Casado, Carmen, University Health Network, Toronto, Ontario, Canada
- Obrador, Gregorio T., Universidad Panamericana, Ciudad de Mexico, Ciudad de México, Mexico
Introduction
IgA nephropathy (IgAN) is the most prevalent type of primary glomerulonephritis worldwide. There are few reports on IgAN development in thalassemia, especially among beta-thalassemia trait (minor) carriers.
Case Description
A 52-year-old male Mexican patient was evaluated for hypertension, hematuria, proteinuria, and rising serum creatinine levels. His history included an ischemic cerebrovascular accident and beta-thalassemia minor (trait). He was treated initially, by another provider, with hydrochlorothiazide/irbesartan and carvedilol for hypertensive nephropathy. Despite treatment, he continued with rising creatinine levels. Then, he was switched to irbesartan only. During this period, the patient developed metabolic acidosis treated with NaHCO3. When referred to our care, we began our approach by completing biochemical and immunodiagnostics assessments (IF), ultrasound imaging (USG), and pathology for glomerulonephritis. On biopsy, we observed IgA nephropathy with advanced nodular glomerulosclerosis and grade II interstitial fibrosis. On IF, IgA, C3, lambda, and kappa chains (mild) were positive. IgG, IgM, C1q were negative. On USG, we documented renal replacement lipomatosis. The patient had microcytic hypochromic anemia, but GBM, C3, C4, ANA antibodies were negative. We managed successfully with irbesartan, amlodipine, NaHCO3 then furosemide.
Discussion
To our knowledge, there are only three additional cases of IgA nephropathy among beta-thalassemia minor. All, including ours, shared as chief complaint rising serum creatinine, hypertension, and persistent microscopic hematuria. However, one report noted bilateral sensorineural hearing loss and prior psychosis under treatment, which may be a syndromic disease presentation. A common feature also is negative antinuclear, anti-DNA, anti-neutrophil cytoplasmic, anti-glomerular basement membrane antibody, HBV, HIV, serum complements antibodies. Significantly, at the time of biopsy, all cases had fibrosis in various degrees ranging from partial to global sclerosis with fibrous crescents. Notably, prior cases occurred in Asian ethnicities, were IgAN is prevalent; our case is the first Hispanic affected patient.