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NBME 17 Answers

nbme17/Block 0/Question#0 (reveal difficulty score)
A 40-year-old woman with a 6-month history of ...
Wegener granulomatosis ๐Ÿ” / ๐Ÿ“บ / ๐ŸŒณ / ๐Ÿ“–
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submitted by โˆ—cassdawg(1780)
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Wegener granulomatosis, also called granulomatosis with polyangiitis, is a vasculitide that commonly presents with the triad of focal necrotizing vasculitis, necrotizing granulomas in the lung and upper airway, and necrotizing crescentic glomerulonephritis (FA2020 p 314 and 596)

Wegener is associated with chronic sinusitis, hemoptysis, and PR3-ANCA (antineutrophil cytoplasmic antibody, formerly called c-ANCA).

None of the other answers are associated with ANCA except Churg-strauss syndrome. Churg-strauss syndrome (also called eosinophilic guanulomatosis with polyangiitis) is associated with MPO-ANCA (formerly called p-ANCA). However, Churg-strauss does not have lower respiratory involvement, hemoptysis, or patchy lung opacities on CXR.

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j44n  All ANCA's are against proteins in the cytoplasm, they were intentionally vague and wanted you to ddx this off of clinical pres +1
jurrutia  Churg-Strauss can affect the lungs, but not the upper airway. +2
shieldmaiden  Actually Churg-Strauss does affect the upper airway (sinusitis); you are confusing it with microscopic polyangiitis which is also associated with MPO-ANCA like CS and with lower airway involvement like Wegener. A way to tell these apart is that (1) CS includes asthma, granulomas, eosinophils, while (2) MPA has no granulomas and does not involve nasopharynx, and (3) W has granulomas in both upper and lower airways and involves PR3-ANCA instead. +1



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