Test Your Knowledge: Anti-GBM Disease

Anti–glomerular basement membrane (anti-GBM) disease is a devastating disease that leads to a rapidly progressive glomerular disease. Anti-GBM antibodies generally are of immunoglobulin G subclass 1 (IgG1), and in most cases can readily be detected in the circulation using enzyme-linked immunosorbent assays (ELISAs). A recent article in AJKD by Ohlsson et al report 4 cases in which anti-GBM ELISA yielded negative or borderline results despite life-threatening disease.  The following questions will refresh your knowledge on anti-GBM-related kidney disease.

1. What is the epitope that is the principal target for anti-GBM autoantibodies?

A. Amino terminal 7S domain of the alpha-3 chain of type IV collagen

B. NC1 domain of the alpha-3 chain of type IV collagen

C. Middle triple helical domain of the alpha-3 chain of type IV collagen

D. Middle triple helical domain of the alpha-5 chain of type IV collagen

2. Which allele is associated with increased genetic susceptibility to anti-GBM antibody disease?

A. HLA-DR1

B. HLA-DR15

C. HLA-DR7

D. HLA-DP1

3. Which of the following statements is NOT true?

A. Presence of ANCA positivity in patients with anti-GBM glomerulonephritis (double positive) alters treatment outcomes.

B. Double positive patients more often present with c-ANCA than p-ANCA.

C. Double positive patients are more likely to have systemic manifestations of vasculitis on presentation.

D. Patients who present with anti-GBM disease and 100% crescents on kidney biopsy requiring dialysis have a lower likelihood of kidney recovery than patients who present without requiring dialysis when treated aggressively with plasmapharesis, steroids, and cyclophosphamide.

 4. De novo anti-GBM glomerulonephritis post-transplantation may occur in patients who had ESRD from which of the following diseases?

A. Membranous Nephropathy

B. IgA Nephropathy

C. Alport syndrome

D. Diabetes Mellitus

Post prepared by Dr. Aditya Kadiyala, Nephrology Fellow, Hofstra NSLIJ School of Medicine, and Dr. Kenar D. Jhaveri, eAJKD Blog Editor.

To see answers, please click here.

To view the article abstract or full-text (subscription required), please visit AJKD.org.

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