engleski

Polyarteritis nodosa in Croatian children : a retrospective study over the last 20 years

To analyze the disease characteristic, treatment modalities and outcome of polyarteritis nodosa (PAN) in Croatian chilčdren. Cross-sectional study included all children with PAN diagnosed according to EULAR/PRES/PRINTO criteria during the last two decades. PAN was diagnosed in 12 patients (6 girls and 6 boys) mean age (+/- SI)11.33+/-3.08 years. The share of PAN among all vasculitides was 3.8%. Systemic PAN was diagnosed in 7 children (58%), macroscopic poliangiitis in 3 (2.5%), cutaneous PAN in 2 (17%). The most consistent symptoms were skin involement (90%) and arthritis/athralgia (60%). The CNS was affected in 33% of patients. Inflammatory markers (C-reactive protein and erythrocyte sedimentation rate (ESR) were elevated in all patients, and anti-neutrophil cytoplasmic antibodies were positive in all patients with microscopic polyangiitis. Therapy mode for all patients was corticosteroids. Immunosuppressive drugs were used as additional therapy for patients severe symptoms. Two patients (17%), both suffering from microscopic polyangiitis, died due to renal failure during the follow-up. In comparison with available studies, we found a difference in distribution of childhood polyarteritis nodosa as well as clinical characteristics (e.g. higher prevalence of neurological and pulmonary symptoms), while other researched features, laboratory and treatment were similar.

polyarteritis nodosa; chilhood; treatment outcome

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