Goodpasture syndrome (anti-GBM disease)

Introduction

Anti-GBM disease is a disease where autoantibodies against the glomerular basal membrane can be found in the circulation. The antibodies provoke a severe glomerulonephritis that often presents itself as a rapidly progressive glomerulonephritis. The autoantibodies can also react with the alveolar basal membrane causing a renopulmonar syndrome with bleeding from the lung and glomerulonephritis, this condition is called Goodpasture syndrome.

Causes

Unknown stimuli result in the production of autoantibodies.

Histopathology

Link to the pathology part

Clinical presentation

Presents typicallys with severe nephritic symptoms with rapidly progressing glomerulonephritis. Rarely high amounts of proteinuria. About 50% of patients also have symptoms in the lungs (Goodpasture syndrome) with hemoptysis and dyspnea.

Urinalysis

Link to nephritic sediment

Prognosis and treatment

The prognosis is quite bad if untreated. Quick diagnosis and treatment with strong immunosuppressants and plasmapheresis is crucial for protecting kidney function. If the patient does not start with treatment before dialysis is necessary, then the prognosis is bad. If adequate treatment is started early enough, it is continued until the patient is negative for anti-GBM, a minimum of 2-3 months of immunosuppressants. Chances for recurrence are relatively low after undergoing the acute disease.