Anti-glomerular Basement Membrane Disease (Goodpasture’s Syndrome)

The co-existence of acute glomerulonephritis and pulmonary alveolar haemorrhage

Aetiology

• More common in men

• Rare in children

Pathophysiology

• Specific autoimmune disease caused by a type II antigen-antibody reaction leading to diffuse pulmonary haemorrhage, glomerulonephritis (and often acute kidney injury and chronic kidney disease)

• There are circulating antiglomerular basement membrane (anti-GBM) antibodies

Clinical presentation

• Typically presents as acute kidney injury caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary haemorrhage that may be life-threatening

Symptoms

• Chills and fever, nausea and vomiting, weight loss, chest pain

• Anaemia

• Massive pulmonary haemorrhage, which can cause respiratory failure

• Haematuria

• Rapidly progressive glomerulonephritis that may lead to acute kidney injury and volume overload

• Arthralgia

Signs

• Tachypnoea

• Dyspnoea

• Inspiratory crackles over lung bases

• Cyanosis

Investigations

• Bloods - FBC, U+Es, anti-GBM antibodies (diagnostic)

• Urinalysis is typical of acute glomerulonephritis, with low-grade albuminuria, gross or microscopic haematuria, and red blood cell casts

• CXR - transient patchy shadows due to intrapulmonary haemorrhage

• Anti-GBM antibodies are diagnostic

Management

• Plasmapheresis to remove circulating antibodies

• Immunosuppression (prednisolone and cyclophosphamide) to prevent further antibody production