Abstract

Antiglomerular basement membrane disease is a small vessel vasculitis which affects the lungs and kidneys, causing diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis respectively. It is extremely uncommon in children. Diagnosis requires the presence of specific anti-GBM autoantibodies. Presentation with renal involvement is much more common than pulmonary involvement. However, a small proportion of cases might have isolated pulmonary involvement. Minimal changes on renal biopsy could still be present in these patients. Treatment of antiglomerular basement membrane disease includes plasmapheresis and immunosuppression. We present a case where a 7 year old boy presented with a long history suggestive of pulmonary haemorrhage but no renal manifestations and was diagnosed on the basis of anti-GBM autoantibodies and renal biopsy. He responded well to immunosuppressive therapy alone.