Abstract: FR-PO0794
Rapidly Progressive Glomerulonephritis Due to Noncrescentic Immune Complex Glomerulonephritis (GN) with Full-House Immunofluorescence
Session Information
- Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Kim, Dean Ki-Hyun, UPMC, Pittsburgh, Pennsylvania, United States
- Stephenoff, Kevin Michael, UPMC, Pittsburgh, Pennsylvania, United States
Introduction
Lupus nephritis is a well understood cause of rapidly progressive glomerulonephritis. Prompt treatment is required to prevent progression to ESRD. We present a unique case with RPGN and biopsy findings consistent with lupus nephritis, though serologic findings were negative and kidney function continued to deteriorate after initial lupus nephritis treatment.
Case Description
Patient is a 23-year-old male with a past medical history of antiphospholipid syndrome, ITP, myo/pericarditis, recurrent pneumonia, eosinophilic esophagitis, and hypogammaglobulinemia. Baseline creatinine was 0.9mg/dl and he had persistent blood and protein on urinalysis. He had progressively worsening kidney function over 4 months initially attributed to long-term NSAID use for recurrent pericarditis. Serologic workup revealed normal complements, low ANA titer, neg anti-dsDNA antibody, neg mpo and pr3 antibodies. A native kidney biopsy showed primarily immune complex glomerulonephritis with full house immunofluorescence and no crescents. Treatment was initiated with steroids, mycophenolate mofetil, and hydroxychloroquine. Despite treatment, kidney function rapidly deteriorated (cr increased to 5.7 mg/dl from 3.5 mg/dl) with ongoing severe proteinuria (24hr urine protein 13g). Alternative diagnoses were considered due to seronegative findings. Given other clinical findings (antiphospholipid syndrome, myocarditis), treatment for class IV lupus nephritis was escalated to obinutuzumab. Kidney function continued to decline requiring 3 sessions of hemodialysis after which his kidney function did recover. Proteinuria has improved but upcr remains 2.4 g/g and he has residual CKD stage 4.
Discussion
Seronegative lupus nephritis is a rare and diagnostically challenging condition that requires kidney biopsy to distinguish from other glomerulonephropathies. This case was diagnostically challenging due to normal serologic findings, no signs of lupus flare at time of declining kidney function, and no response to initial treatment. Other diagnoses were considered including nonlupus full house nephropathy and other glomerular disorders. Though no other clinical or serologic signs of acute lupus flare at the time of rapidly declining kidney function, treatment for lupus nephritis was continued due to history of other clinical lupus-related events and no clear alternative explanation for declining kidney function.