title: Clinical overview and outcome in a cohort of children with polyarteritis nodosa.
abstract: OBJECTIVE
  Polyarteritis nodosa (PAN) is a rare vasculitis in childhood and poor information is known about its long-term outcome. Our aim was to describe the clinical features, at onset and during the disease course, of childhood-onset PAN and identify a potential correlation with persistent organ damage and worse outcome in a cohort of paediatric patients with a confirmed diagnosis of PAN.
  
  
  METHODS
  A retrospective collection of demographic and clinical data of 52 Caucasian children diagnosed with PAN, fulfilling the EULAR/PRES diagnostic criteria, recruited from eight paediatric rheumatologic centres and one transition unit, was performed. A statistical correlation was made between clinical involvement at onset or during the overall disease course and patients' final outcome.
  
  
  RESULTS
  Data from 52 patients (31 males, 21 females) were collected: their mean age at onset was 7.9 years (median 6.3) and mean follow-up period was 6.2 years (median 5.4). At the last follow-up visit, 27 patients (51.9%) were off therapy in clinical remission, 17 (32.7%) were in clinical remission while on medication, and 6 (11.6%) had a persistent or relapsing disease course. Two patients (3.8%) deceased because of severe cerebral involvement. Cranial nerve palsy during the disease course was significantly correlated with a worse prognosis (p=0.011). The presence of nephrogenic hypertension at onset and seizures during the disease course were significantly associated with the development of irreversible organ damage (p= 0.040 and 0.011, respectively).
  
  
  CONCLUSIONS
  Childhood PAN is a severe disease with substantial risk of long-term morbidities. In our cohort of patients the worst outcome was significantly correlated with renal and neurological involvement.
authors: Department of Internal Medicine, Rheumatology Section, Transition clinic, University of Florence, Florence, Italy. falcini@unifi.it.
authors: Fernanda
authors: F
authors: Falcini
authors: Francesco
authors: F
authors: La Torre
authors: Fabio
authors: F
authors: Vittadello
authors: Donato
authors: D
authors: Rigante
authors: Giorgia
authors: G
authors: Martini
authors: Fabrizia
authors: F
authors: Corona
authors: Antonella
authors: A
authors: Buoncompagni
authors: Maria
authors: M
authors: Alessio
authors: Elisabetta
authors: E
authors: Cortis
authors: Antonella
authors: A
authors: Insalaco
authors: Silvia
authors: S
authors: Magni-Manzoni
authors: Luciana
authors: L
authors: Breda
authors: Marco
authors: M
authors: Matucci-Cerinic
authors: Francesco
authors: F
authors: Zulian
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D002648:Child; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007558:Italy / Q000453:epidemiology; D008297:Male; D063305:Organ Dysfunction Scores; D062072:Patient Acuity; D010488:Polyarteritis Nodosa / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D018570:Risk Assessment; D055502:Secondary Prevention; D013995:Time; D020293:Vasculitis, Central Nervous System / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology
chemical_list: D007166:Immunosuppressive Agents