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Granulomatosis with polyangiitis: Study of 795 patients from the French Vasculitis Study Group registry
Michele Iudici1, Christian Pagnoux2, Delphine S Courvoisier3
1National Referral Center for Rare Systemic Autoimmune Diseases, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), 27, rue du faubourg Saint-Jacques, Paris, Cedex 14 75679, France; Division of Rheumatology, Department of Internal Medicine Specialties, Geneva University Hospitals, Switzerland.
Insights
This study on granulomatosis with polyangiitis (GPA) found that while survival has improved, relapse rates remain high. PR3-ANCA positivity is linked to a greater chance of relapse and better survival outcomes in GPA patients.
Area of Science:
- Rheumatology
- Internal Medicine
- Immunology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease affecting small to medium-sized blood vessels.
- Understanding long-term outcomes and predictors of survival is crucial for managing GPA patients.
Purpose of the Study:
- To describe the characteristics and long-term outcomes of GPA patients.
- To identify predictors of overall survival (OS) and relapse-free survival (RFS) in GPA.
Main Methods:
- Analysis of clinical and laboratory data from 795 newly diagnosed GPA patients in the French Vasculitis Study Group database.
- Assessment of disease activity using the Birmingham Vasculitis Activity Score (BVAS).
- Cox regression analysis to determine predictors of OS and RFS.
Main Results:
- The study followed 795 patients for a median of 3.5 years, with common initial manifestations in the ear, nose, throat (ENT), lungs, and kidneys.
- Relapses occurred in 50% of patients, and 5- and 10-year RFS rates were 37% and 17%, respectively.
- PR3-ANCA positivity independently predicted relapse and prolonged survival. Ten-year OS reached 88.2% for patients diagnosed after 2000, with infections being the primary cause of death.
Conclusions:
- Survival in GPA patients has significantly improved over time, particularly for those diagnosed recently.
- High relapse rates remain a significant concern, necessitating optimization of therapeutic strategies.
- PR3-ANCA positivity is associated with an increased probability of relapse and improved survival in GPA.
Objective:
To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database.
Methods:
Patients' clinical and laboratory characteristics, Birmingham Vasculitis Activity Score (BVAS)-assessed disease activity, malignancies, opportunistic infections, and vital status were collected at diagnosis and each visit. Estimated probabilities and predictors of overall (OS) and relapse-free survival (RFS) were analyzed by Cox regression.
Results:
We enrolled 795 newly diagnosed patients, followed for a median of 3.5 years. Initial clinical manifestations involved ear, nose & throat (ENT; 80%), lungs (68%) and kidneys (56%). Among the 728 available ELISA results, 75.0% were PR3-ANCA-positive, 16.5% MPO-ANCA-positive and 62 (8.5%) ANCA-negative. Relapses occurred in 394 (50%) patients, involving ≥1 organ(s) affected at onset in 179 (46%), mainly ENT, lungs and kidneys, with mean BVAS 10.2 points below that at diagnosis (p<0.001). Five- and 10-year RFS rates were 37% and 17%, respectively. PR3-ANCA-positivity independently predicted relapse (p = 0.05) and prolonged survival (p = 0.038). OS-but not RFS-improved significantly over time (p<0.001); 10-year OS reached 88.2% (95% CI 83.9 to 92.7) for the 660 patients diagnosed after 2000. Infections were the main causes of death. Malignancy or opportunistic infection each occurred in ≤5% of the patients.
Conclusion:
Survival has improved dramatically over the last decades but the high relapse rate remains a major concern for GPA patients, once again stressing the need for therapeutic strategy optimization to lower it. PR3-ANCA-positivity was associated with increased probability of relapse and survival.