Granulomatosis with Polyangiitis GPA (Wegener's) |
Goodpasture's syndrome (Anti GBM disease) |
Respiratory Failure |
Acute Kidney Injury |
Prognosis is very much related to the speed of initiation of treatment. Many labs can do a quick test for ANCA/Anti-GBM. Speak to renal unit as soon as suspected.
- Very rare with 1 case per million per year
- Induces a rapidly progressive Glomerulonephritis (GN)
- Diffuse proliferative GN with crescent formation
- Inflammatory response leads to collagen damage
- Goodpasture's affects Kidneys and Lungs.
- If kidney/lungs alone called Anti GBM disease
- Production of an antibody against Alpha 3 chain of Type 4 collagen
- Found in lungs and GBM
- Lung haemorrhage - dyspnoea, haemoptysis
- Haematuria, Proteinuria
- Rarely and acute nephritic syndrome and RPGN
- Progressive renal failure over weeks
- FBC: raised WCC, anaemia, elevated ESR/CRP
- U&E: AKI picture with Urea and Creatinine
- Urine: protein, blood, red cell casts.
- CXR : alveolar shadowing from haemorrhage
- Check Anti-GBM antibody and ANCA
- Renal biopsy for Anti-GBM nephritis with Immunofluorescence shows linear ribbon-like deposition of IgG along GBM. Diffuse crescentic GN.
- Wegener's Granulomatosis / Granulomatosis with Polyangiitis
Management (Urgent early renal consult)
- Treatment: steroids, cyclophosphamide and plasma exchange
- Urgent Plasmapheresis / Prednisolone 1 mg/kg, Cyclophosphamide, Azathioprine
- Renal transplant in end-stage renal disease (recurrence in graft possible but unusual except for Alport's syndrome. Anti GBM antibodies need to be reduced to prevent disease in a transplant kidney.