Goodpasture syndrome vs Granulomatosis with polyangiitis
How to tell Goodpasture syndrome and Granulomatosis with polyangiitis apart when a board question makes them look alike.
Goodpasture: pulmonary-renal with anti-GBM and no ENT disease; GPA: adds sinusitis and saddle nose with c-ANCA/PR3.
Side by side
Pulmonary hemorrhage plus rapidly progressive GN with anti-GBM antibodies.
Sinus disease, lung nodules or cavitation, renal disease, and c-ANCA/PR3 positivity.
Anti-GBM antibodies target basement membrane in glomeruli and alveoli.
Necrotizing granulomatous vasculitis affects small-to-medium vessels.
Anti-GBM antibodies in serum, linear IgG deposition along glomerular and alveolar basement membranes on immunofluorescence, crescentic glomerulonephritis on biopsy, and a young smoker presenting with hemoptysis plus hematuria without ENT disease support Goodpasture syndrome.
c-ANCA (PR3) positivity in serum, pauci-immune crescentic glomerulonephritis (no immune complex deposition) on biopsy, granulomas with vasculitis on tissue sampling, and the upper-airway findings (chronic sinusitis, nasal septal perforation, saddle nose deformity) support granulomatosis with polyangiitis.
The trap
Both cause hemoptysis plus hematuria, so a pulmonary-renal label alone is not enough. The deciding bedside clue is ENT involvement: chronic sinusitis, nasal septal perforation, saddle nose, or persistent otitis media plus the pulmonary-renal syndrome is GPA, not Goodpasture. The antibody and immunofluorescence pair confirms: linear IgG on biopsy with serum anti-GBM equals Goodpasture (treat urgently with plasmapheresis plus steroids plus cyclophosphamide); pauci-immune crescentic GN with c-ANCA / PR3 positivity equals GPA (treat with rituximab plus high-dose steroids, then maintenance immunosuppression).
Watch it explained
The repair move
Add ENT disease, antibody type, and immunofluorescence pattern to the pulmonary-renal story.
Try a free question
CoreA patient has hemoptysis, rapidly progressive glomerulonephritis, anti-GBM antibodies, and linear immunofluorescence along basement membranes. Which diagnosis is most likely?
Show answer and explanation
Answer: A. Goodpasture syndrome. Pulmonary-renal syndrome with anti-GBM antibodies and linear IF points to Goodpasture syndrome.
Why the other choices are tempting
- B. GPA has granulomatous ENT/lung/kidney disease with c-ANCA/PR3.
- C. Microscopic polyangiitis lacks granulomas and is often p-ANCA/MPO.
- D. Sarcoidosis causes noncaseating granulomas but not anti-GBM linear nephritis.
- E. Membranous nephropathy causes nephrotic syndrome with subepithelial deposits.
Flashcards
Tap a card to flip it.
What is the fastest separator for Goodpasture syndrome vs Granulomatosis with polyangiitis?
Goodpasture: pulmonary-renal with anti-GBM and no ENT disease; GPA: adds sinusitis and saddle nose with c-ANCA/PR3.
What clue should make you choose Goodpasture syndrome?
Pulmonary hemorrhage plus rapidly progressive GN with anti-GBM antibodies.
Quick answers
What is the fastest way to tell Goodpasture syndrome from Granulomatosis with polyangiitis?
Goodpasture: pulmonary-renal with anti-GBM and no ENT disease; GPA: adds sinusitis and saddle nose with c-ANCA/PR3.
What is the key clue for Goodpasture syndrome?
Pulmonary hemorrhage plus rapidly progressive GN with anti-GBM antibodies.
What is the key clue for Granulomatosis with polyangiitis?
Sinus disease, lung nodules or cavitation, renal disease, and c-ANCA/PR3 positivity.
What is the common trap with Goodpasture syndrome vs Granulomatosis with polyangiitis?
Both cause hemoptysis plus hematuria, so a pulmonary-renal label alone is not enough. The deciding bedside clue is ENT involvement: chronic sinusitis, nasal septal perforation, saddle nose, or persistent otitis media plus the pulmonary-renal syndrome is GPA, not Goodpasture. The antibody and immunofluorescence pair confirms: linear IgG on biopsy with serum anti-GBM equals Goodpasture (treat urgently with plasmapheresis plus steroids plus cyclophosphamide); pauci-immune crescentic GN with c-ANCA / PR3 positivity equals GPA (treat with rituximab plus high-dose steroids, then maintenance immunosuppression).