Review Question - QID 105867

The patient is presenting with hemoptysis and hematuria/proteinuria which is concerning for Goodpasture syndrome. Given his acute worsening of symptoms and hypoxia, treatment with methylprednisolone, plasmapheresis, and cyclophosphamide is indicated.

Anti-glomerular basement membrane (GBM) syndrome (Goodpasture syndrome) is a glomerular disorder marked by the clinical triad of crescentic glomerulonephritis, pulmonary hemorrhage, and IgG anti-glomerular basement membrane antibodies. Common presentations include fever, muscle pain, renal failure, hemoptysis, cough, and dyspnea. Initial treatment in anti-GBM syndrome is emergent plasmapheresis in conjunction with steroids and cyclophosphamide. Plasmapharesis clears circulating auto-antibodies and decreases the risk of progression to dialysis. Steroids and cyclophosphamide are used to suppress antibody production, but the effect is less immediate than that with plasmapheresis. The diagnosis can be confirmed with renal biopsy and immunofluorescence in anti-GBM syndrome which reveals linear IgG deposits along the basement membrane.

Incorrect Answers:
Answer 1: Methylprednisolone has a slow onset of action and would not improve this patient's symptoms and hypoxia immediately.

Answer 2: Methylprednisolone and cyclophosphamide both can treat the acute flare of Goodpasture syndrome and prevent future episodes; however, they would not rapidly reverse his current symptoms without plasmapheresis.

Answer 3: Methylprednislone, IVIG, and cyclophosphamide are incorrect as IVIG is not preferred in the treatment of this condition when compared to plasmapheresis.

Answer 5: Plasmapheresis and cyclophosphamide is appropriate management of Goodpasture syndrome; however, steroids should be added as well.

Bullet Summary:
The treatment of an acute exacerbation of Goodpasture syndrome is methylprednisolone, plasmapheresis, and cyclophosphamide.