Autoimmune disorder with antibodies generated to components of the glomerular basement membrane, affecting the kidneys and the lungs

Aetiology

• Antibodies against alpha-3 subunit of type IV collagen produce a type II hypersensitivity reaction

Predisposing factors

• Association with HLA-DR2
• Exposure to organic solvents or hydrocarbons
• Smoking
• Infection, e.g. influenza A2
• Cocaine inhalation
• Exposure to metal dusts
• More common in men, rare in children

Pathophysiology

• Specific autoimmune disease caused by antibodies against alpha-3 subunit of type IV collagen (anti-GBM antibodies) produce a type II hypersensitivity reaction
• The reactio leads to diffuse pulmonary haemorrhage, glomerulonephritis, and often acute kidney injury and chronic kidney disease

Clinical features

• 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