Some features of the current course of ANCA-associated systemic vasculitides in children
DOI:
https://doi.org/10.15574/SP.2025.7(151).2232Keywords:
ANCA-associated vasculitis, systemic vasculitis, children, pediatrics, granulomatosis with polyangiitis, microscopic polyangiitis, antineutrophil cytoplasmic antibody, clinical course, immunosuppressive therapy, vasculitis diagnosisAbstract
Aim - to analyze the pathomorphism of the clinical course of ANCA-associated systemic vasculitides in children in Ukraine, considering clinical manifestations, serological profile, disease activity, and treatment outcomes.
Materials and methods. A single-center retrospective non-comparative study was conducted. A total of 4,173 medical records of children with rheumatic diseases treated between 2005 and 2025 were reviewed. Fifteen patients (0.36%) were diagnosed with ANCA-associated systemic vasculitis. Clinical features, laboratory and serological markers (PR3-ANCA, MPO-ANCA), imaging findings, and biopsy results were analyzed. Disease activity was assessed using BVAS and PVAS scores, and treatment effectiveness was evaluated 6 months after therapy initiation.
Results. Adolescent girls predominated among the patients. In most cases, the time to diagnosis exceeded 12 months. Constitutional symptoms and involvement of the respiratory tract, kidneys, and musculoskeletal system were most common. PR3-ANCA positivity was detected in 66.7% of patients. After 6 months of treatment, a significant reduction in disease activity was observed, and medical remission was achieved in 60% of cases. Two fatal outcomes were recorded.
Conclusions. ANCA-associated systemic vasculitides in children in Ukraine are characterized by delayed diagnosis and frequent multisystem involvement. Despite treatment in accordance with international guidelines, the risks of disability and mortality remain high, emphasizing the need for earlier diagnosis and a multidisciplinary management approach.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the institution mentioned in the paper. Informed consent was obtained from the children's guardians for the study.
The authors declare no conflict of interest.
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