GLOMERULONEPHRITIS-ANTI GBM GN

SUMMARY

1. Characterised by linear deposits of Ig, often IgG & C3 along the glomerular basement membrane on immunofluorescence. Light microscopy: severe necrotizing and crescentic pattern.

2. Dx: confirmed by detection of circulating anti-GBM antibodies.

3. Caused by small vessel vasculitis affecting the capillary beds of the kidneys and lungs.

4. Directly pathogenic autoantibodies: non-collagenous domain of the a3 chain of type IV collagen (Goodpasture’s auto-antigen).

5. Associations: smoking, hydrocarbons, alemtuzumab, genetic (HLA DR2).

6. Presentation: rapidly progressive GN, alveolar haemorrhage (40-60%).

7. Tx: rapidly remove the pathogenic auto-antibody via plasma exchange, steroids, cytotoxic therapy.

Reference(s)

Wilkinson, I. (2017). Oxford handbook of clinical medicine. Oxford: Oxford University Press.
Hannaman, R. A., Bullock, L., Hatchell, C. A., & Yoffe, M. (2016). Internal medicine review core curriculum, 2017-2018. CO Springs, CO: MedStudy.
Therapeutic Guidelines. Melbourne: Therapeutic Guidelines Limited. https://www.tg.org.au [Accessed 2021].