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Score to assess the probability of relapse in granulomatosis with polyangiitis and microscopic polyangiitis
Maxime Samson1, Hervé Devilliers2, Sara Thietart3
1Department of Internal Medicine and Clinical Immunology, University Hospital Centre Dijon Bourgogne, Dijon, France maxime.samson@u-bourgogne.fr.
Objective:
To develop a score assessing the probability of relapse in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Methods:
Long-term follow-up data from GPA and MPA patients included in five consecutive randomised controlled trials were pooled. Patient characteristics at diagnosis were entered into a competing-risks model, with relapse as the event of interest and death the competing event. Univariate and multivariate analyses were computed to identify variables associated with relapse and build a score, which was then validated in an independent cohort of GPA or MPA patients.
Results:
Data collected from 427 patients (203 GPA, 224 MPA) at diagnosis were included. Mean±SD follow-up was 80.6±51.3 months; 207 (48.5%) patients experienced ≥1 relapse. Relapse risk was associated with proteinase 3 (PR3) positivity (HR=1.81 (95% CI 1.28 to 2.57); p<0.001), age ≤75 years (HR=1.89 (95% CI 1.15 to 3.13); p=0.012) and estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.73 m² (HR=1.67 (95% CI 1.18 to 2.33); p=0.004) at diagnosis. A score, the French Vasculitis Study Group Relapse Score (FRS), from 0 to 3 points was modelised: 1 point each for PR3-antineutrophil cytoplasmic antibody positivity, eGFR ≥30 mL/min/1.73 m² and age ≤75 years. In the validation cohort of 209 patients, the 5-year relapse risk was 8% for a FRS of 0, 30% for 1, 48% for 2 and 76% for 3.
Conclusion:
The FRS can be used at diagnosis to assess the relapse risk in patients with GPA or MPA. Its value for tailoring the duration of maintenance therapy should be evaluated in future prospective trials.
Insights
A new score, the French Vasculitis Study Group Relapse Score (FRS), helps predict relapse risk in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) patients at diagnosis. Higher FRS scores correlate with increased likelihood of experiencing disease relapse over time.
Area of Science:
- Rheumatology
- Clinical Immunology
- Nephrology
Background:
- Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are severe autoimmune diseases characterized by relapsing disease courses.
- Predicting relapse risk is crucial for optimizing patient management and treatment strategies in these conditions.
Purpose of the Study:
- To develop and validate a predictive score for assessing the probability of relapse in patients diagnosed with GPA or MPA.
- Identify key clinical and laboratory parameters at diagnosis that are associated with an increased risk of relapse.
Main Methods:
- Pooled long-term follow-up data from 427 GPA and MPA patients across five randomized controlled trials.
- Utilized a competing-risks model with relapse as the event of interest and death as the competing event.
- Developed and validated the French Vasculitis Study Group Relapse Score (FRS) based on univariate and multivariate analyses.
Main Results:
- Over 48% of patients experienced at least one relapse during a mean follow-up of 80.6 months.
- Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA) positivity, estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.73 m², and age ≤75 years at diagnosis were significantly associated with relapse risk.
- The 5-year relapse risk stratified by FRS (0-3 points) ranged from 8% to 76% in the validation cohort.
Conclusions:
- The French Vasculitis Study Group Relapse Score (FRS) provides a valuable tool for assessing relapse probability in GPA and MPA patients at the time of diagnosis.
- The FRS can aid clinicians in risk stratification and potentially inform treatment decisions.
- Future prospective trials are warranted to evaluate the FRS's utility in guiding the duration of maintenance therapy.
