Childhood-Onset ANCA- Associated Vasculitis: single center experience from Central California
Authors' conclusion
Microscopic polyangiitis was the most frequent AAV subtype with female preponderance, shorter duration of symptoms at onset and higher proportion of racial/ ethnic minority patients. Hispanic children demonstrated frequent MPO positivity. Trends towards higher rates of ICU requirement and need for dialysis upon initial presentation was noted in MPA. Patients with MPA received rituximab more frequently.
Study context
- Population
- Patients ≤18 years with childhood-onset ANCA-associated vasculitides (AAV) diagnosed 2010–2021, Central California
- Participants
- 21 patients
- Exposure
- Microscopic polyangiitis (MPA) vs granulomatosis with polyangiitis (GPA)
- MPAn = 12
- GPAn = 9
- Outcome
- Characteristics at initial presentation
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
- Median age at diagnosis, MPA
- 13.7 years
- Median age at diagnosis, GPA
- 14 years
- Female, MPA vs GPA
- 92% versus 44%
- Racial/ethnic minority
- 57%
- White
- 43%
- Hispanic, MPA
- 67%
- White, GPA
- 78%
- Median symptom duration before diagnosis, MPA
- 14 days
- Median symptom duration before diagnosis, GPA
- 21 days
- Renal involvement, MPA
- 100%
- Renal involvement, GPA
- 78%
- Ear, nose and throat (ENT) involvement, GPA
- 89%
- PR3 positive, white patients
- 89%
- ICU admission, MPA
- 67%
- Dialysis, MPA
- 50%
Observational data: associations, not effects.
Further findings
- Deaths · related to Aspergillus pneumonia and pulmonary hemorrhage — MPA
- 2 events
Treatment
- MPA: CYC plus steroids
- 42%
- MPA: RTX plus steroids
- 42%
- GPA: CYC with steroids alone
- 78%
- GPA: CYC with steroids and RTX
- 22%
Original publication
Childhood-Onset ANCA- Associated Vasculitis: single center experience from Central California. Pediatr Rheumatol Online J. Published 3 July 2023. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →