ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: TH-PO549

ESRD Secondary to Anti-Glomerular Basement Membrane Disease in a Child with Common Variable Immunodeficiency

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1600 Pediatric Nephrology

Authors

  • Mannemuddhu, Sai Sudha, UF Shands, Gainesville, Florida, United States
  • Upadhyay, Kiran K., UF Shands, Gainesville, Florida, United States
Introduction

Anti- glomerular basement membrane (GBM) disease is an uncommon autoimmune disorder that is characterized by rapidly progressive glomerulonephritis (GN) caused by autoantibodies against the α3-chain of type IV collagen in the GBM. Commom variable immunodeficiency (CVID) is a primary immunodeficiency manifested by hypogammaglobulinemia, inability to make functional antibody, and recurrent infections. We report an interesting association of anti-GBM disease with CVID.

Case Description

A 15-year-old Caucasian female with prior normal renal function presented with 2 weeks of nephrotic proteinuria, oliguria, AKI, and was found to have serum anti-GBM antibody. She had been diagnosed with CVID and West Nile meningoencephalitis at 3 and 12 yrs of age respectively. Her renal biopsy showed crescentic GN with at least 50% global glomerulosclerosis and immunofluorescence showed linear staining for IgG along the glomerular capillary wall. There was no clinical or radiological evidence of pulmonary hemorrhage. She was treated with pulse IV steroids, Cyclophosphamide, Rituximab and several sessions of plasmapheresis. Her serum anti-GBM antibody level decreased from 194 U/mL at presentation to 0 U/mL post therapy. However, she progressed to ESRD within weeks and required dialysis. Her clinical course was complicated by hypertensive encephalopathy, CMV meningoencephalitis, CMV viremia, status epilepticus, and she passed away a few months later from lower respiratory infection related complications.

Discussion

Anti-GBM disease is a rare autoimmune condition, which has not been reported in association with a primary immunodeficiency syndrome. ESRD secondary to anti-GBM disease in a patient with CVID is an interesting association and supports role of immune dysregulation in systemic autoimmune disease.

Kidney Biopsy images- Anti GBM disease