Abstract: PUB408
Atypical Anti-glomerular Basement Membrane Disease: An Emerging Clinical Dilemma
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Vasquez-Rios, George, Glomerular Disease Center, Renal Medicine Associates, Albuquerque, New Mexico, United States
- Carias Martinez, Karla G., Renal Medicine Associates, Albuquerque, New Mexico, United States
- Thimmisetty, Ravi K., Renal Medicine Associates, Albuquerque, New Mexico, United States
- Garcia, Pablo, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, United States
Introduction
Atypical anti-glomerular basement membrane (anti-GBM) disease is a rare subtype of vasculitis that has been described as indolent and relatively benign at times. However, aggressive presentations might pose significant challenge specially in young individuals.
Case Description
We present a 35-year-old female with no relevant past medical history who presented to the hospital with anasarca, fatigue, and nausea. She was recently seen by her primary care provider, who recommended evaluation in the ED due to progressive functional deterioration. On examination, she was hypertensive (BP 150/90s), with HR of 80s, RR: 16, and T: 37°C. Additionally, she had evident anasarca and mild paleness. Laboratory markers revealed a serum Cr: 7 mg/dL (baseline: 0.7 mg/dL 3 months prior), BUN of 85 mg/dL, bicarbonate of 17 mmol/L, and hypoalbuminemia at 2 mg/dL. Urinalysis showed hematuria, RBC casts, and proteinuria, which was later quantified to be 8 gr in a 24-hour collection. An extensive GN and MGRS workup was pursued, which showed positive ANCA levels (MPO: 5.1 IU/mL, UNL>0.9 IU/mL) and negative serum anti-GBM antibodies. Imaging studies and comprehensive evaluation did not reveal significant pulmonary, bronchial, or extra-renal compromise.
Discussion
She received a pulse of steroids and started on hemodialysis due to oliguria, anasarca, and high BUN prior to kidney biopsy. Kidney biopsy demonstrated diffuse fibrotic crescents with extensive interstitial fibrosis and tubular atrophy (IFTA >70%). IF studies revealed moderate IgG dominance with no evidence of MPO or PR3 signals. Our team was concerned about kidney-limited atypical anti-GBM. After a careful multidisciplinary discussion considering marked IFTA, medication side-effect profile, and prognosis, the patient was treated with Rituximab. Three weeks later, we continue to monitor for potential kidney response, though we explained that the chances of significant improvement are low. Atypical anti-GBM disease is an emerging and obscure vasculitis presentation that is yet to be understood. Linear deposition with IgG on IF should also warrant careful evaluation for MGRS in the appropriate age group. Our case calls for caution within the nephrology community given the miriad of questions regarding prognosis, treatment, and recurrence risk after tranpslant.