Goodpasture's Syndrome

'GOODPASTURE'S SYNDROME'

It is an uncommon but aggressive disease with rapid progression

  • Genetic predisposition and strong association with HLADR2
  • Gross haematuria may be the presentation
  • Glomerulonephritis-RPGN
  • Oliguric renal failure carries worse prognosis
  • Organic solvents exposure as an etiological factor
  • Oral cyclophosphamide for treatment
  • Diffuse alveolar haemorrhage
  • DDRF (Dialysis-dependent renal failure)
  • DLCO for assessing alveolar haemorrhage
  • Pallor due to alvolar haemorrhage and gross haematuria
  • Protein A immunoadsorption and Prednisolone as treatment
  • AntiGBM antibodies as well as ANCA  for diagnosis
  • Skin rashes as clinical feature
  • Smoking as aetiology
  • Total volume plasmapheresis daily x 2weeks as successful therapeutic intervention
  • Unlike other autoimmune disease male are more prone to Goodpatures disease
  • Respiratory failure and death
  • Environmental exposure to hydrocarbons, Paraquat (weed killer), organic solvents and metal dust - all are considered to trigger anti GBM disease

 

RPGN: Rapidly progressive glomerulonephritis

DLCO: Diffusing capacity of the lung for carbon monoxide

 

 

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Did you know?


imageGoodpasture's syndrome is an aggressive disease with a mortality rate as high as 95% earlier. Now it has a better prognosis with modern treatment.
imageProtein A immunoadsorption is successful in those who do not respond to to plasmapheresis.
imagePrognosis worst in those with DDRF, oliguria and serum creatinine > 5.7 mg/dl.

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