Abstract: FR-PO948
A Case of Lupus-Negative Full-House Staining on Kidney Biopsy, Causing Nephrotic Syndrome during Pregnancy
Session Information
- Glomerular Diseases: Potpourri
October 25, 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
- Simhadri, Prathap, Florida State University Daytona Beach Regional Medical School Campus, Daytona Beach, Florida, United States
- Contractor, Renish, Florida State University Daytona Beach Regional Medical School Campus, Daytona Beach, Florida, United States
- Filoramo, Michael R., Florida State University Daytona Beach Regional Medical School Campus, Daytona Beach, Florida, United States
- Singh, Prabhat, Christus Spohn Health System, Corpus Christi, Texas, United States
- Chandramohan, Deepak, The University of Alabama at Birmingham, Birmingham, Alabama, United States
Introduction
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease and is recognized by a constellation of clinical, laboratory, and histopathological findings. Lupus nephropathy is a common manifestation of SLE, with immune-mediated inflammatory damage to the glomerulus leading to end-stage renal disease. The literature has described a small subset of patients demonstrating renal limited "lupus-like nephropathy", defined by pathological biopsy findings typical of lupus nephropathy without systemic manifestations or laboratory biomarkers of the disease. A full-house stain, identified by positive immunofluorescence staining of all three isotypes of immunoglobulins, is a pathological finding highly associated with SLE. This pathological pattern has been associated with renal-limited lupus-like nephropathy. However, few studies have identified full-house renal-limited "lupus-like nephropathy" in pregnancy.
Case Description
A 24-year-old G1P0 woman at 14 weeks gestation was referred to nephrology for further evaluation of proteinuria. At 16 weeks gestation, 24-hour urine revealed 14 g of proteinuria and a serum albumin level of 1.52 mg/dL. The patient was ANA positive and had elevated C1q antibody titer but negative for other SLE autoantibodies, including anti-Sm, anti-dsDNA, normal C3, and C4 complements. Biopsy revealed membranous nephropathy with positive staining for IgG, IgA, IgM, C3, C1Q, and Lp-PLA2, consistent with full-house immunoglobulin staining.
She was started on 500 mg pulse dose methylprednisolone for 3 days, which was gradually tapered to 5 mg daily, and cyclosporine 75 mg BID. Successfully induced at 36 weeks, she delivered a baby with 50% percentile birth weight. Six-month follow-up revealed 1g protein on 24-hour urine collection and normal C3/C4 levels. She continues exhibiting no signs of SLE, and the C1q antibody titer improved.
Discussion
A 24-year-old pregnant female presented with pathological evidence of full-house renal limited "lupus-like nephropathy" without clinical or laboratory evidence consistent with SLE. This case adds to the limited literature describing this condition and more evidence is essential to guide proper treatment and management.