Rapidly progressive glomerulonephritis

Question 1

What is Rapidly progressive glomerulonephritis?

Answer 1

Rapidly progressive glomerulonephritis is a term used to describe a rapid loss of renal function associated with the formation of epithelial crescents in the majority of glomeruli.

Question 2

Causes?

Answer 2

• Goodpasture’s syndrome
• Wegener’s granulomatosis
• SLE, microscopic polyarteritis
• Henoch Schonlein purpura
• IgA neph
• Post Strep Glomerulonephritis
• Cryoglobulinaemia

Question 3

Features?

Answer 3

• nephritic syndrome: haematuria with red cell casts, proteinuria, hypertension, oliguria
• features specific to underlying cause (e.g. haemoptysis with Goodpasture’s, vasculitic rash or sinusitis with Wegener’s)
• uraemia
• pericardial rub
• bruising/petiechiae/rash

Question 4

Investigations?

Answer 4

• Urinalysis
• Blood protein/red cell casts
• FBC: anaemia if alveolar haemoorhage
• Creatinie/Urea raised
• CRP/ESR raised - systemic vasculitis
• Complement levels
• cANCA: Wegeners Granulamatosis
• pANCA: Microscopic polyangitis
• ANA DsDNA:SLE
• Anti GMB: Goodpasture’s syndrome
• Renal biopsies

Question 5

Management?

Answer 5

• Steroids
• Cyclophosphamide
• Plasma exchange
• Pulsed methylprednisolone

Prognosis poor, many go on to end stage renal failure