Imunological and clinical characteristics in children with polyarteritis nodosa: a retrospective study over the last 20 years

Aim

Analysis of polyarteritis nodosa (PAN) characteristics such as laboratory parameters, affected organs, treatment modalities and disease outcome.

Methods

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.

Results

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.

Conclusion

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.