All patients diagnosed with PAN who were treated in our department from March 2003 to February 2020, including those living outside our local catchment area, were eligible for this study. All patients were diagnosed prior to 18 years of age and satisfied both the American College of Rheumatology criteria2 and the European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) criteria11. In accordance with these criteria, patients were diagnosed with PAN when they presented with a systemic illness characterised by either a biopsy showing necrotising vasculitis (i.e. granulocyte or mixed leukocyte infiltrates in the arterial wall) in small- or medium-sized arteries or confirmed angiographic abnormalities (e.g., vascular aneurysms and occlusions). Additionally, patients were required to exhibit at least one of the following five clinical items: skin signs, myalgia/muscle tenderness, peripheral neuropathy, hypertension, or renal vascular disease. Cutaneous PAN was classified as follows: necrotising vasculitis of medium-sized arteries within the skin, without involvement of the internal organs, accompanied by fever, painful subcutaneous nodules, livedo reticularis, arthralgia, or myalgia19,31. The present study was approved by the Institutional Review Board of Seoul National University Hospital in South Korea (H-2004227–1119), who waived the requirement for informed consent because of the retrospective nature of the study.

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