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Remission and Low Disease Activity in Granulomatosis With Polyangiitis and Microscopic Polyangiitis: Prevalence and
Paolo Delvino1, Federica Sardanelli2, Sara Monti1
1Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy.
Objective:
To assess the prevalence and impact on damage accrual of different levels of disease activity in patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Methods:
Patients with GPA and MPA followed for ≥5 years in 2 different centers were included. Disease activity and damage were assessed using the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI), respectively. Three levels of remission were defined: complete remission (BVAS = 0, negative for antineutrophil cytoplasmic antibody [ANCA], off treatment), clinical remission off therapy (CROffT; BVAS = 0, positive for ANCA), and clinical remission on therapy (CROnT; BVAS = 0, negative or positive for ANCA, glucocorticoids ≤5 mg/day and/or immunosuppressant). A low disease activity state (LDAS) was defined as 0 < BVAS ≤3, low-dose glucocorticoids (≤7.5 mg/day), and/or immunosuppressant. Remission or LDAS were defined as prolonged when lasting ≥2 consecutive years.
Results:
A total of 167 patients were included: 128 (76.6%) with GPA, 39 (23.4%) with MPA, mean ± SD age 51.0 ± 16.7 years. During a 5-year follow-up, 10 patients (6.0%) achieved prolonged complete remission, 6 (3.6%) prolonged CROffT, 89 (53.3%) prolonged CROnT, 42 (25.1%) prolonged LDAS, and 20 (12.0%) never achieved LDAS. The VDI score at 5 years progressively worsened according to increasing levels of disease activity targets (complete remission, CROffT, CROnT, and LDAS). The mean ± SD 5-year VDI score was higher in patients not achieving prolonged remission compared to those who did (3.7 ± 2.0 versus 2.2 ± 1.9; P < 0.0001). By multivariate analysis, baseline ear, nose, and throat (P = 0.006), and lung involvement (P = 0.047) were negative predictors of prolonged remission.
Conclusion:
More than 60% of patients with GPA/MPA achieved prolonged remission, which was associated with better long-term outcomes. In contrast, prolonged LDAS correlated with increased damage accrual and was not a sufficient treatment target.
Insights
Over 60% of patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) achieved prolonged remission, leading to better long-term outcomes. However, prolonged low disease activity state (LDAS) increased damage accrual and is not an adequate treatment target.
Area of Science:
- Rheumatology and Immunology
- Vasculitis Research
- Autoimmune Disease Management
Background:
- Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are systemic autoimmune diseases characterized by inflammation of blood vessels.
- Disease activity and damage accrual are key factors influencing long-term prognosis in patients with GPA and MPA.
- Defining optimal disease activity targets is crucial for improving patient outcomes in these conditions.
Purpose of the Study:
- To evaluate the prevalence of different disease activity levels in patients with GPA and MPA.
- To assess the impact of varying disease activity states on long-term damage accrual.
- To determine the association between achieving prolonged remission and patient outcomes.
Main Methods:
- A cohort of 167 patients with GPA (76.6%) and MPA (23.4%) were followed for at least 5 years.
- Disease activity was measured using the Birmingham Vasculitis Activity Score (BVAS), and damage was assessed with the Vasculitis Damage Index (VDI).
- Defined remission states included complete remission, clinical remission off therapy (CROffT), clinical remission on therapy (CROnT), and low disease activity state (LDAS), with prolonged status defined as lasting ≥2 years.
Main Results:
- Over 60% of patients achieved prolonged remission (complete, CROffT, or CROnT), associated with significantly lower VDI scores at 5 years compared to those not achieving remission (2.2 vs 3.7, P < 0.0001).
- Prolonged LDAS was achieved by 25.1% of patients and was associated with higher VDI scores, indicating increased damage accrual.
- Baseline ear, nose, and throat (ENT) and lung involvement were identified as negative predictors for achieving prolonged remission.
Conclusions:
- Achieving prolonged remission in GPA and MPA is associated with better long-term outcomes and reduced damage.
- A prolonged low disease activity state (LDAS) is insufficient to prevent damage accrual and should not be the sole treatment target.
- Early identification of factors like ENT and lung involvement may help in optimizing treatment strategies to achieve deeper remission.
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