Systemic VasculitisPrognostic Score

Five Factor Score
1996 Original and 2011 Revised Versions

A prognostic score for systemic necrotizing vasculitis. It counts five adverse factors present at diagnosis. It measures prognosis, not disease activity. Choose the version that matches the diagnosis, then check each factor present.

Original Development
Guillevin L, et al.
Medicine (Baltimore), 1996; revised 2011
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Choose a Version
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Poor-Prognosis Factors at Diagnosis
📊 Interpreting the Score
Factors PresentCategoryClinical Meaning
0No FactorsNo poor-prognosis factor present at diagnosis. Lowest risk stratum in the derivation cohort.
1One FactorIntermediate stratum. Mortality was higher than in the zero-factor group. Identify the factor present and address it directly.
2 or moreTwo or MoreHighest-risk stratum. Mortality rose with each additional factor in the derivation cohorts. Warrants closer review of disease management and monitoring. The score does not specify a treatment regimen.

The score is a prognostic guide. It does not replace assessment of organ involvement, disease activity (BVAS), or cumulative damage (VDI). Treatment decisions depend on the full clinical picture.

💡 Pearls and Pitfalls
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Use the version that matches the diagnosis. The 1996 version was derived in polyarteritis nodosa and EGPA and validated in MPA. The 2011 revision extended the cohort to include GPA, MPA, PAN, and EGPA.
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Creatinine thresholds differ between versions. 140 µmol/L (1.58 mg/dL) in 1996 and 150 µmol/L (1.70 mg/dL) in 2011. Enter the value that corresponds to the version you selected.
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The ENT item is not universal. Scoring "no ENT involvement" for MPA or PAN inflates the score and mislabels risk. This calculator counts the item in the 2011 version only when you check it.
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Score at diagnosis. The factors were defined as baseline prognostic variables. Scoring later in the course answers a different question.
🔬 Evidence

The original Five Factor Score was derived from a prospective French cohort of 342 patients with polyarteritis nodosa or Churg-Strauss syndrome (now EGPA). The 1996 version identified five adverse factors at diagnosis: proteinuria above 1 g/24 hours, creatinine above 140 µmol/L, cardiomyopathy, severe gastrointestinal involvement, and central nervous system involvement. Mortality increased with the number of factors present.

The 2011 revision by the French Vasculitis Study Group extended the analysis to GPA, MPA, PAN, and EGPA. It replaced proteinuria and CNS involvement with age over 65 and absence of ENT involvement (scored for GPA and EGPA only), and raised the creatinine threshold to 150 µmol/L.

View References▾
1
Guillevin L, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome: a prospective study in 342 patients. Medicine (Baltimore). 1996;75(1):17-28.
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Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P; French Vasculitis Study Group. The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011;90(1):19-27.
For clinical decision support only. The Five Factor Score is a prognostic tool derived from observational cohorts. Factor definitions follow the original publications and should be applied as written. Results require interpretation in clinical context by a qualified clinician.
Five Factor Score
0
/ 5
012345
No Poor-Prognosis Factors
Version
1996 original. Creatinine threshold 140 µmol/L (1.58 mg/dL).
Clinical Interpretation
Check each poor-prognosis factor present at diagnosis. The score is the number of factors present.
FFS = number of factors present (0 to 5)
Reviewed by Mahiar Rabie, MS, MD · AutoimmuneCalc