Antiglomerular basement membrane (Goodpasture) disease

The diagnosis and treatment of patients with pulmonary-renal syndrome or any rapidly progressive glomerulonephritis is a medical emergency. Patients should be hospitalized to allow for rapid diagnosis and treatment. Urgent consultation with a nephrologist is essential to expedite diagnosis and treatment, as pulmonary hemorrhage can be rapidly fatal and earlier treatment may prevent irreversible renal destruction. If treatment is delayed, the chances of renal recovery are low; so, where clinical suspicion is high, it may be appropriate to begin treatment before the results of diagnostic testing are available.

History

Goodpasture disease should be suspected in any patient with rapidly progressive glomerulonephritis in whom renal function has deteriorated over the course of weeks. Symptoms may be minimal or include cough, SOB, hemoptysis, fatigue, malaise, dark urine, decreased urine output, and leg edema.

Potential risk factors that should be asked about include smoking and previous exposure to hydrocarbons, organic solvents and heavy metals. Goodpasture disease may also be suspected in patients who have a history of lithotripsy, have recently contracted a respiratory infection or been treated with immune checkpoint inhibitors or the monoclonal antibody alemtuzumab. It should also be considered post renal transplant in patients with Alport syndrome.

Testing

History and physical examination cannot reliably determine the etiology of a rapidly progressive glomerulonephritis. Thus, serologic testing and renal biopsy are required for definitive diagnosis. All patients suspected of having rapidly progressive glomerulonephritis should have the following tests ordered immediately: hepatitis panel, cryoglobulins, C3, C4, ANA, antineutrophil cytoplasmic antibodies, antistreptolysin O, antiglomerular basement membrane (anti-GBM), clotting screen, and, if not contraindicated, a renal biopsy. Contraindications to renal biopsy include uncontrolled bleeding disorder, uncontrolled hypertension, and an unwilling patient. If an immediate renal biopsy is not possible, treatment will need to proceed based on the anti-GBM serology until a confirmatory renal biopsy can be performed. The remaining tests listed above are needed to rule out other causes of rapidly progressive glomerulonephritis.