Demographic and clinical profile of systemic and cutaneous Polyarteritis nodosa in a group of pediatric patients in Medellin, 2010-2019

Objective: To describe the main demographic and clinical characteristics of patients who were diagnosed with childhood polyarteritis nodosa (PAN). Methods: A descriptive study was conducted using retrospective data Results: The clinical registries from patients under 18th years with vasculitis or PAN diagnosis attended between 2010 and 2019 in two Medical Centers from Medellin were obtained. Nineteen patients were included: eleven classified as cutaneous PAN (PANC) according to the definition of the European League Against Rheumatism and the European Society of Pediatric Rheumatology and eight as systemic PAN according to Ankara 2008 Criteria. 57.9% were male. The median age at diagnosis was ten years (3-17), and the median follow-up period was 15 months (0-105). The most common clinical characteristics were subcutaneous nodules (84.2%), fever (57.9%), arthralgias (57.9%), and calf pain (52.6%). SPAN patients had more frequent weight loss and lower hemoglobin. Five patients had ulcers (26.3%), four of them with CPAN; two patients had lingual necrosis (10.5%), and two digital necrosis (10.5%), all of them also with CPAN. CRP mean was 10.2 mg/dl (0.33-45.3) and mean erythrocyte sedimentation rate (ESR) was 74 mm/h (15-127 mm/h). Meningeal enhancing, intrahepatic, and mesenteric aneurism, among others, were reported in diagnostic images. All patients required treatment with systemic glucocorticoid. None of the patients died during the follow-up period. Conclusions: In this pediatric series from MedellinColombia, PAN was more common in boys of school age. CPAN was more frequent than SPAN. The clinical characteristics most reported were subcutaneous nodules, myalgias, arthralgias, and calf pain. Although CPAN has been considered a benign disease, these patients may require systemic glucocorticoid and immunosuppressive therapy.