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Imunological and clinical characteristics in children with polyarteritis nodosa: a retrospective study over the last 20 years
Pediatric Rheumatologyvolume 9, Article number: P91 (2011)
Analysis of polyarteritis nodosa (PAN) characteristics such as laboratory parameters, affected organs, treatment modalities and disease outcome.
Our study includes all children aged 1-18 with PAN diagnosed according to EULAR/PRES/PRINTO criteria at Department of Paediatrics, University Hospital Centre Zagreb, Croatia, during the period of 1991-2010.
PAN was diagnosed in 12 patients (6 girls and 6 boys). The share of PAN amongst all vasculitides was 4%. The mean age at disease onset was (±SD) 11.33±3,08 years. Systemic PAN was diagnosed in 7 children (58%), microscopic polyangiitis in 3 (25%), cutaneus PAN in 2 (17%) and classic PAN in 0 (0%). The most consistent symptoms were skin involvement (90%) and arthritis/arthralgia (60%). The CNS was affected in 40% of patients. ESR and CRP were elevated in all patients. Antineutrophil cytoplasmic antibodies were elevated in 3 patients (25%). Antistreptolysin O was elevated in 4 patients (25%). The relation between the severity of skin involvement and involvement of other organs was not found. Therapy mode for all patients was corticosteroids. Immunosuppressive drugs and Rituximab (anti-CD20) were used as additional therapy for patients with severe symptoms. Two patients with microscopic polyangiitis died due to chronic renal and pulmonary failure during the follow-up.
In comparison to available studies, we found a difference in distribution of childhood polyarteritis nodosa as well as some clinical characteristics (e.g. higher prevalence of neurological symptoms), while other researched features, laboratory and treatment, were similar.