Goodpasture syndrome

Goodpasture syndrome, also referred as antiglomerular basement membrane (anti-GBM) antibody disease, is an autoimmune disease characterised by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. It is a type of pulmonary-renal syndrome.

Goodpasture syndrome is defined by:

• pulmonary haemorrhage

• glomerulonephritis

• circulating antiglomerular basement membrane antibodies

Epidemiology

Goodpasture’s syndrome accounts for 1-2% of cases of rapidly progressive glomerulonephritis. It typicallycan occur at any age but most commonly affects young adult menmen aged 20 - 30 with an M:F of 3:1. Smoking commonly predates pulmonary haemorrhage. Other risk factors may include hydrocarbon exposure, infections such as influenza and HLA type.

Clinical presentation

Patients may present with cough, dyspnoea, haemoptysis and hypoxia, hypoxaemia, anaemia, fatigue, chest pain, uraemia or haematuria. Pulmonary manifestations are usually the cause of presentation although most also have evidence of renal disease. Affected patients may also present with hypertensionHypertension is rare.

Pathology

Goodpasture syndrome is a type II hypersensitivity reaction with antibodies primarily directed against type IV collagen of the renal glomerular basement membrane. There is further cross-reactivity with the alveolar basement membrane resulting in pulmonary injury. This is typically characterised by rapidly progressive glomerulonephritis (RPGN) and necrotising haemorrhagic interstitial pneumonitis respectively.

The diagnosis is made by immunofluorescent studies of renal or lung tissue, which show a smooth wavy line of fluorescent staining along the basement membrane. 

Radiographic features

Plain radiograph

• non-specific, bilateral, coalescent airspace opacities, which resolve in several days to give reticular opacities in the same distribution

• complete radiographic resolution is usually seen within 2-3 weeks

CT

• ground glass and airspace opacities that progress to reticular "crazy paving" pattern over a few weeks

• hilar lymphadenopathy may be seen

• no interlobular septal thickening in the acute phase

Treatment and prognosis

The overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.

Differential diagnosis

Often radiograph and CT findings are indistinguishable from pulmonary oedema. Other causes of pulmonary haemorrhage should also be considered. 

See also

• pulmonary vasculitis