title: Polyarteritis nodosa in Croatian children: a retrospective study over the last 20 years.
abstract: To analyze the disease characteristics, treatment modalities and outcome of polyarteritis nodosa (PAN) in Croatian children. 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 (±SD) 11.33 ± 3.08 years. The share of PAN among all vasculitides was 3.8 %. Systemic PAN was diagnosed in 7 children (58 %), microscopic polyangiitis in 3 (25 %), cutaneous PAN in 2 (17 %). The most consistent symptoms were skin involvement (90 %) and arthritis/arthralgia (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 cytoplasmatic 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 with 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 some clinical characteristics (e.g., higher prevalence of neurological and pulmonary symptoms), while other researched features, laboratory and treatment were similar.
authors.affiliation: Division of Paediatric Immunology and Rheumatology, Department of Paediatrics, University Hospital Centre Zagreb, Zagreb University School of Medicine, Kispaticeva 12, 10000, Zagreb, Croatia, marija.jelusic.drazic@gmail.com.
authors.forename: Marija
authors.initials: M
authors.lastname: Jelusic
authors.forename: Masa
authors.initials: M
authors.lastname: Vikic-Topic
authors.forename: Danica
authors.initials: D
authors.lastname: Batinic
authors.forename: Danko
authors.initials: D
authors.lastname: Milosevic
authors.forename: Branko
authors.initials: B
authors.lastname: Malenica
authors.forename: Ivan
authors.initials: I
authors.lastname: Malcic
mesh_terms: D000293:Adolescent; D001706:Biopsy; D001799:Blood Sedimentation; D002097:C-Reactive Protein / Q000378:metabolism; D002648:Child; D017523:Croatia / Q000453:epidemiology; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D010488:Polyarteritis Nodosa / Q000097:blood / Q000453:epidemiology* / Q000208:ethnology*; D015995:Prevalence; D012189:Retrospective Studies; D012867:Skin / Q000473:pathology
chemical_list: D002097:C-Reactive Protein