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

Did you know?

Goodpasture's syndrome is an aggressive disease with a mortality rate as high as 95% earlier. Now it has a better prognosis with modern treatment.

Protein A immunoadsorption is successful in those who do not respond to to plasmapheresis.

Prognosis worst in those with DDRF, oliguria and serum creatinine > 5.7 mg/dl.