Goodpasture’s Syndrome

Co-existence of acute glomerulonephritis and pulmonary alveolar haemorrhage caused by a type II antigen-antibody reaction involving anti-glomerular basement membrane (anti-GBM) antibodies.

Background

  • Rare (incidence 1-2/1000000); accounts for 1-2% of all rapidly progressive glomerulonephritides
  • Between 60-80% have both renal and pulmonary involvement; whilst 20-40% just have renal and 10% just have pulmonary

Aetiology/Risk factors

  • Association with HLA-DR2/DR15
  • Exposure to organic solvents or hydrocarbons
  • Smoking
  • Infection (influenza A2)
  • Cocaine (inhalation)
  • Exposure to metal dusts

Pathophysiology

  • Autoimmune reaction (type II- cytotoxic) occurs in the glomerulus basement membrane and alveolar basement membrane, which causes glomerulonephritis (crescentic) and pulmonary haemorrhage

Presentation

  • Constitutional symptoms
    • Malaise, chills, fever, arthralgia, nausea/vomiting, weight loss
    • Can precede or be concurrent with renal/pulmonary symptoms
  • Haemoptysis, cough, dyspnoea, shortness of breath
    • Massive pulmonary haemorrhage may cause respiratory failure
    • Chest pain is uncommon
  • Haematuria; oedema; high blood pressure
  • Other signs include tachypnoea; inspiratory crackles (po haemorrhage); cyanosis; oedema; pallor

Investigations

  • Ideally, renal biopsy for antiGBM fluorescence or anti-GBM ELISA
    • Check other antibodies too e.g. ANCA, dsDNA (in case of SLE as cause)
  • Other lab tests
    • FBC (anaemia)
    • U&Es/renal function- be weary of AKI
    • ESR/PV (often normal in GP; but would be raised in vasculitis)
    • Clotting/coagulation study
    • Urinalysis
  • CXR
    • Patchy consolidation, often bilateral, symmetrical, perihilar and bibasal
      • Apices/costphrenic angles are often normal
      • CXR can be completely normal in ~20%
  • Occasionally, lung biopsy may be required

Management

  • IV steroids and usually cyclophosphamide alongside plasmapheresis until anti-GBM undetectable
    • Usually with human albumin solution
    • +/- fresh frozen plasma if there is severe haemorrhage
    • If there is severe AKI, dialysis is required (poor prognosis)
    • Corticosteroids are tapered down over 6-12 months
    • Cyclophosphamide is usually given for 3 months
  • In severe kidney failure, transplant may be an option

Complications

  • Pulmonary haemorrhage is a significant cause of mortality in these patients (5-year survival is >80%)
  • Some patients will require long-term dialysis (20-30%)
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