Answer to CC #8

Case Challenge #8 presented a 42 year old female with fever, rash, and worsening shortness of breath.  She started to exhibit symptoms after starting a leukotriene inhibitor and tapering off steroids. Exam revealed fever, mild hypoxia, and prominent bilateral wheezing. She also had palpable purpura on both of her legs. Neurologic exam reveals foot drop on the right. Labs revealed a peripheral eosinophilia and elevated inflammatory markers. Imaging revealed bilateral peripheral infiltrates.

We asked which of the following is most likely to be helpful in the diagnosis of this case?

The results of the voting are as follows:

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The correct answer is: P-ANCA!

The patient has Eosinophilic Granulmomatosis with Polyangiitis (EGPA, formerly known as Churg-Strauss Syndrome)
  • Small vessel vasculitis associated with allergies, asthma, pulmonary disease and eosinophilia
  • Can also involve skin, heart, kidneys, GI tract and nervous system (mononeuritis multiplex)
  • Dx: granulomatous vasculitis on Bx, eosinophils; p-ANCA positivity
  • Often flares with steroid tapering or with leukotriene receptor antagonists

Thanks for playing, case challenge #9 will be posted next Tuesday!

One thought on “Answer to CC #8”

  1. Although P-Anca can be supportive of EGPA it is not necessary in this case. ACR established diagnostic criteria for EGPA with the presence of >=4 has a LR+ 283 and LR- of 0.15, which is excellent as a clinical prediction rule to rule in or rule out EGPA. She met criteria with 1) asthma; 2) > 10% eos in blood; 3) mononeuropathy; 4) transient pulmonary opacities (assuming these were transient). The use of P-ANCA in this case is of low utility since either a positive or negative P-ANCA would not change the diagnosis.

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