Abstract: SA-PO772
Rapidly Progressive Glomerulonephritis (RPGN) Due to Myeloperoxidase Anti-neutrophil Cytoplasmic Antibodies (MPO-ANCA)-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease
Session Information
- ANCA-Associated Vasculitis, Anti-GBM Disease, and Other RPGN
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Park, Ji-Min, Brooke Army Medical Center, Fort Sam Houston, Texas, United States
- Sancillo, Rafael, Brooke Army Medical Center, Fort Sam Houston, Texas, United States
- Wickham, Jesse M., Brooke Army Medical Center, Fort Sam Houston, Texas, United States
- Gilani, Sarwat, The University of Texas Health Science Center at San Antonio, San Antonio, Texas, United States
Introduction
RPGN is a type of nephritic syndrome characterized by rapid decline of kidney function with associated proteinuria, hematuria, and presence of urinary dysmorphic red blood cells/casts. RPGN caused by both MPO-ANCA associated vasculitis and anti-GBM disease (Goodpasture syndrome) is rarely reported and generally results in poorer outcomes. We present such a case in an otherwise healthy 32 year-old woman.
Case Description
A 32 year-old woman with no prior history of kidney disease presented with bilateral lower extremity edema and right flank pain of 1 day duration with associated gross hematuria. Physical exam showed 1+ bilateral lower extremity edema extending up to mid-shins. Initial labs revealed serum creatinine of 5.71 mg/dL (no prior baseline), ESR 62 mm/hr, CRP 68.9 mg/L, and urinalysis with >50 RBCs/HPF and 6-10 WBCs/HPF. Urine protein/creatinine ratio was 5.85 g/g. Additional labs showed elevated myeloperoxidase (MPO) antibody at 2.9 AI and positive IgG glomerular basement membrane antibody (anti-GBM) with negative serine protease 3 (PR3) antibody, cryoglobulins, anti-nuclear antibody, and double stranded DNA antibody. Infectious workup was negative for hepatitis, syphilis, HIV, and tuberculosis. Renal ultrasound showed bilateral echogenic kidneys. Computed tomography of abdomen and pelvis showed mild right renal hydronephrosis. Subsequent kidney biopsy showed crescentic, necrotizing, and sclerosing glomerulonephritis with linear glomerular basement membrane staining for IgG, consistent with anti-GBM nephritis. Interestingly, patient had an isolated renal involvement with no neurologic, cardiac, pulmonary, or gastrointestinal manifestations. She was ultimately diagnosed with RPGN secondary to anti-GBM disease with dual positivity for MPO-ANCA. She was treated aggressively with corticosteroids and cyclophosphamide followed by plasma exchange and rituximab, with reduction of antibody titers and disease regression.
Discussion
RPGN secondary to renal MPO-ANCA associated vasculitis and anti-GBM disease is extremely rare. Currently, there are only three reported cases in the literature. In the absence of signs suggestive of infection, clinicians should initiate prompt investigation of autoimmune causes of RPGN to guide optimal treatment.