Correct!
3. Granulomatosis with polyangiitis (Wegner's granulomatosis)

There are three ANCA-associated vasculitides (AAV):

• Microscopic polyangiitis (MPA)
• Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis)
• Eosinophilic granulomatosis with polyangiitis (EGPA, Churg– Strauss syndrome)

These are pauci-immune, necrotizing, small-vessel vasculitis. They often affect the kidneys, lungs, and peripheral nervous system. GPA has +ANCA in a cytoplasmic immunofluorescence pattern (cANCA) with a positive auto-antibodies to proteinase 3 (PR3). Our patient was PR3+ at >8 U (normal <0.4). MPA and EGPA have a perinuclear immunofluorescence pattern (pANCA) and a positive antibody to myeloperoxidase (MPO). The MPO was negative in our patient.

Which of the following are recommended therapy(ies) for GPA? (Click on the correct answer to proceed to the fifth and final panel)

1. Corticosteroids + cyclophosphamide
2. Corticosteroids + methotrexate
3. Corticosteroids + rituximab
4. 1 and 3
5. All of the above

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