Rapidly progressing glomerulonephritis

Question 1

What is special about this form of GN?

Answer 1

It is the most aggressive.

Its has the potential to cause end stage renal failure over the course of days.

Question 2

What is shown on biopsy?

Answer 2

Various causes, all have the same biopsy finding of crescents affecting most glomeruli.

Question 3

How is the pathology categorised?

Answer 3

1) Immune complex disease (45%) - e.g. post infective, SLE, IgA/HSP
2) Pauci-immune disease (50%, 80-90%of which are ANCA positive) - granulomatosis with polyangiitis GPA (formerly Wegener’s), microscopic polyangiitis, Churg-straus syndrome
3) Anti GBM disease (3%) - Goodpasture’s disease

Question 4

How do they present clinically?

Answer 4

AKI +/- systemic features (fever, weightloss, myalgia, haemoptysis)

Pulmonary haemorrhage is the most common cause of death in ANCA positive patients.

Question 5

How is it treated?

Answer 5

Aggressive immunosuppression with high dose IV steroids and cyclophosphamide +/- plasma exchange