rapidly progressive glomerulonephritis

Question 1

types of pathophysiological mechanisms leading to RPGN

Answer 1

type 1: anti-GBM glomerulonephritis (goodpasture syndrome)
type 2: immune complex mediated GN eg. IgA nephropathy, membranoproliferative nephropathy, IgA vasculitis, lupus nephritis, PSGN
type 3: glomerulonephritis associated with vasculitis eg. granulomatosis with polyangiitis, microscopic polyangiitis, eosinophillis granulomatosis with polyangiitis

Question 2

pathophysiology

Answer 2

breaks in the glomerular capillary wall and dysfunction of the GBM causes leakage of plasma proteins and passage of inflammatory cells into the bowman space
damage of the membrane of the bowman space
formation of fibrin clots and proliferation of macrophages, fibroblasts, neutrophils

Question 3

clinical features

Answer 3

nephritic syndrome
decrease in urine output within days to weeks - possibly anuria
fatigue
pulmonary symptoms eg. haemoptysis

Question 4

general rules of treatment

Answer 4

glucocorticoids and cyclophosphamide
if therapy is initiated rely, full recovery of renal function in >50% of cases
without proper treatment, prognosis is unfavourable
rapid progression to end stage renal disease and high mortality

Question 5

treatment for goodpasture syndrome rapidly progressive GN

Answer 5

plasmaphoresis in addition to immunosuppression

Question 6

RPGN due to ANCA associated vasculitis

Answer 6

glomerulonephritis with polyangiitis, microscopic polyangiitis
combination therapy with glucocorticoids and rituximab is an effective alternative