Vasculitis & polymyalgia rheumatica
Clinical manifestations, treatment and prognosis analysis of childhood antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis
Authors' conclusion
In this cohort, childhood-onset AAV occurred predominantly in females, with MPA as the major subtype and the kidneys being the most frequently involved organ. Glucocorticoids formed the mainstay of treatment. Approximately half of the children achieved remission following treatment. Patients with elevated serum creatinine levels are at higher risk of progressing to ESRD. Most children achieved long-term survival.
Study context
- Population
- Patients aged <18 years diagnosed with AAV at the First Affiliated Hospital of Zhengzhou University22 female
- Participants
- 29 patients
- Exposure
- Microscopic polyangiitis (MPA) vs granulomatosis with polyangiitis (GPA)
- MPAn = 23
- Outcome
- Progression to ESRD (risk factors)
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
Patients progressed to end-stage renal disease (ESRD)
15
ESRD at initial diagnosis
12
Observational data: associations, not effects.
Further findings
Diagnosis
- Median age at diagnosis (IQR 12.3-16.5 years)
- 13.9 years
- Median diagnostic interval (IQR 0.6-1.5 months)
- 1 month
Clinical manifestations
- Most common: renal, respiratory and musculoskeletal involvement; presentations similar between MPA and GPA
Outcomes after treatment · 12 months post-treatment
- Remission
- 12
- Renal remission
- 3
- Remained on dialysis
- 8
- Renal transplantation
- 6
- GC the mainstay of treatment
Long-term survival
- Deaths
- 1
- Long-term survival achieved in most patients
Original publication
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