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EGPA Five Factor Score — SCE Rheumatology MCQ

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HardVasculitisEGPA Five Factor ScoreSCE Rheumatology

A patient newly diagnosed with eosinophilic granulomatosis with polyangiitis is being risk-stratified using the 2009 revised Five-Factor Score. Which combination comprises all five variables that score one adverse prognostic point in this patient?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: E — Age >65 years; cardiac involvement; gastrointestinal involvement; serum creatinine ≥150 micromol/L; absence of ear, nose and throat manifestations

Explanation lettering: D = shown as A · C = shown as B · E = shown as C · A = shown as D · B = shown as E

B is correct. In the 2009 revised FFS, one point is assigned for each of age >65 years, cardiac involvement, gastrointestinal involvement, peak serum creatinine ≥150 micromol/L and, in EGPA or GPA, absence of ENT manifestations. Five-year mortality in the derivation cohort was approximately 9%, 21% and 40% for scores of 0, 1 and ≥2. Option D lists the original 1996 FFS variables, including proteinuria, CNS involvement and a creatinine threshold >140 micromol/L. Option A incorrectly combines elements of the original and revised scores. ANCA status, eosinophil count, asthma severity, skin disease and arthritis are not FFS variables; option E instead resembles EGPA classification features. The FFS is prognostic and should not be used alone to determine induction therapy.

Reference: Guillevin L, Pagnoux C, Seror R, et al. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group cohort. Medicine (Baltimore). 2011;90:19-27. https://pubmed.ncbi.nlm.nih.gov/21200183/