- Poster presentation
- Open Access
Imunological and clinical characteristics in children with polyarteritis nodosa: a retrospective study over the last 20 years
© Topic et al; licensee BioMed Central Ltd. 2011
- Published: 14 September 2011
- Severe Symptom
- Neurological Symptom
- Disease Onset
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.
This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.