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Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis
Kimberly A Morishita1, Lakshmi N Moorthy2, Joanna M Lubieniecka3
1Kimberly A. Morishita, MD, MHSc, David A. Cabral, MBBS: British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
Insights
This study on childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) found most pediatric patients improved with treatment. However, over half experienced organ damage early in their disease course.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Vasculitis Research
Background:
- Childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) requires characterization of its early disease course and outcomes.
- Understanding treatment response and long-term sequelae in pediatric AAV is crucial for effective management.
Purpose of the Study:
- To characterize the early disease course of pediatric AAV.
- To evaluate 12-month outcomes, including remission, inactive disease, and organ damage, in children with AAV.
Main Methods:
- A cohort of 105 children diagnosed with AAV before age 18 was analyzed.
- Outcomes included remission (PVAS=0, low-dose corticosteroids), inactive disease, improvement, organ damage (PVDI), and relapse rates at 12 months.
Main Results:
- 42% of patients achieved remission, and 61% had inactive disease at 12 months.
- 92% showed at least a 50% improvement in PVAS score by postinduction.
- The median PVDI damage score was 1 at 12 months, with 63% experiencing at least one form of organ damage.
Conclusions:
- While the majority of pediatric AAV patients respond to treatment, a significant proportion do not achieve remission.
- Early organ damage is a common complication in children with AAV, highlighting the need for vigilant monitoring and management strategies.
Objective:
To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV.
Methods:
Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4-6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months.
Results:
In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9-15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4-6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0-6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months.
Conclusion:
This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.
