A Single-Center Study on Childhood Rare Vasculitides: Clinical and Outcome Analysis

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Türkmen Ş., Coşkuner T., SÖZERİ B.

Turkish Archives of Pediatrics, cilt.61, sa.5, ss.388-398, 2026 (ESCI, Scopus)

Özet

Objective: To evaluate rare childhood vasculitides using standardized clinical, laboratory, imaging, and outcome data. Methods: A retrospective cohort of 74 children with 8 rare vasculitides was assessed at a single center. Demographics, imaging, and disease activity (Pediatric Vasculitis Activity Score (PVAS)) and damage (Pediatric Vasculitis Damage Index) scores were recorded at diagnosis, 12 months, and last visit. Results: Among 74 patients, 39 (52.7%) were girls. Median diagnosis age was 13.5 years. Subtype distribution was vascular Behçet syndrome 22 (29.7%), Takayasu arteritis (TA) 16 (21.6%), deficiency of adenosine deaminase 2 (DADA2) 14 (18.9%), polyarteritis nodosa 11 (14.9%), granulomatosis with polyangiitis (GPA) 6 (8.1%), primary angiitis of the central nervous system (PACNS) 3 (4.1%), eosinophilic GPA 1 (1.4%), and Cogan syndrome 1 (1.4%). The DADA2 had the longest diagnostic delay (median [IQR]: 20 [6-99.75] months), while PACNS had the youngest median age at onset (4.6 [3.07-7.95] years). Overall, 13.5% (n = 10) were diagnosed before age 5, showing recurrent fever (60%, n = 6) and anemia (50%, n = 5), less skin/mucosal (40%, n = 4), musculoskeletal (30%, n = 3), cardiovascular (20%, n = 2), and pulmonary involvement (20%, n = 2), and higher PVAS (median 2.0, IQR 1.25-2.75). At 12 months, all had low disease activity. The TA had the longest corticosteroid use, highest damage, and slower remission. Overall remission was 91.9% (n = 68/74), while GPA patients had more flares in the first year (median 1.0, IQR 0.25-1.75). Conclusion: Prognosis was favorable, but TA and monogenic vasculitides showed greater damage. In children <5 years, higher activity but good early response emphasize timely, individualized management.