title: Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years.
abstract: OBJECTIVE
  Polyarteritis nodosa (PAN) is a rare disease of childhood. The aims of this study were to describe the clinical features, treatment, and outcome of systemic childhood PAN and to identify predictors of relapse.
  
  
  METHODS
  A single-center retrospective medical records review of children with PAN fulfilling the European League Against Rheumatism (EULAR)/Paediatric Rheumatology European Society (PRES)/Paediatric Rheumatology International Trials Organisation (PRINTO) classification criteria who were seen over a 32-year period was performed. Data on demographic and clinical features, treatments, relapses (recurrence of clinical signs/symptoms or occurrence of new symptoms after initial remission requiring escalation or resumption of immunosuppressive therapy), and deaths were recorded. A disease activity score was retrospectively assigned using the Paediatric Vasculitis Activity Score (PVAS) instrument. Cox regression analysis was used to identify significant predictors of relapse.
  
  
  RESULTS
  Sixty-nine children with PAN were identified; 55% were male, and their median age was 8.5 years (range 0.9-15.8 years). Their clinical features at presentation were fever (87%), myalgia (83%), skin (88%), renal (19%), severe gastrointestinal (GI) (10%), and neurologic (10%) involvement. The PVAS at presentation was 9 of 63 (range 4-24). Histopathologic analysis of the skin showed necrotizing vasculitis in biopsy samples from 40 of 50 children. Results of selective visceral arteriography suggested the presence of PAN in 96% of patients. Treatment included cyclophosphamide and corticosteroids (83%), plasma exchange (9%), and biologic agents (after 2002; 13%). The relapse rate was 35%, and the mortality rate was 4%. Severe GI involvement was associated with increased risk of relapse (P = 0.031), while longer time to induce remission (P = 0.022) and increased cumulative dose of cyclophosphamide (P = 0.005) were associated with lower relapse risk.
  
  
  CONCLUSIONS
  Childhood PAN is a severe inflammatory disease of insidious onset and variable clinical presentation. Relapses occurred more frequently in those with severe GI involvement. A higher cumulative dose of cyclophosphamide was associated with a lower risk of relapse.
authors.affiliation: Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
authors.forename: Despina
authors.initials: D
authors.lastname: Eleftheriou
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Dillon
authors.forename: Kjell
authors.initials: K
authors.lastname: Tullus
authors.forename: Stephen D
authors.initials: SD
authors.lastname: Marks
authors.forename: Clarissa A
authors.initials: CA
authors.lastname: Pilkington
authors.forename: Derek J
authors.initials: DJ
authors.lastname: Roebuck
authors.forename: Nigel J
authors.initials: NJ
authors.lastname: Klein
authors.forename: Paul A
authors.initials: PA
authors.lastname: Brogan
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D010951:Plasma Exchange; D010488:Polyarteritis Nodosa / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001688:Biological Products; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide