title: Long-term outcome of children with pediatric-onset cutaneous and visceral polyarteritis nodosa.
abstract: OBJECTIVE
  To assess the prognostic impact of clinical presentation in children with polyarteritis nodosa (PAN).
  
  
  METHODS
  Children diagnosed between 1986 and 2006 in a tertiary care pediatric rheumatology center were classified as "cutaneous PAN" (group 1), "cutaneous PAN with significant extra-cutaneous features" (group 2) or "visceral childhood PAN" (group 3).
  
  
  METHODS
  (1) clinical remission off-therapy at last follow-up, (2) requirement and length of glucocorticoid therapy, (3) presence of disease-related sequelae.
  
  
  RESULTS
  Twenty-nine children were included. Sixteen met the Ankara criteria for PAN. Nine patients were qualified as group 1, 11 as group 2, and 9 as group 3. At last follow-up, 15 children were in clinical remission off-therapy: 4 from group 1 (44%), 4 from group 2 (36%) and 7 from group 3 (78%). Glucocorticoid therapy was required for 8 (89%), 7 (64%) and 7 (78%) patients from groups 1, 2 and 3, respectively. Seven children did not require any glucocorticoid therapy. Time-dependent probability of achieving glucocorticoid-free clinical remission was similar between the three groups. Three patients (one from each group) had digital ischemia leading to amputation. There were no significant between-group differences in outcome based on the three outcome measures addressed.
  
  
  CONCLUSIONS
  Outcome was not strikingly predictable from initial presentation in children with PAN. The organ distribution-based distinction between cutaneous and visceral PAN had little prognostic power in this series.
authors.affiliation: Service de Pédiatrie, CHU Clermont-Ferrand, Clermont-Ferrand, France; Inserm CIC 1405, Centre de Recherche Chez l'Enfant, Clermont-Ferrand, France.
authors.forename: Etienne
authors.initials: E
authors.lastname: Merlin
authors.affiliation: Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: Richard
authors.initials: R
authors.lastname: Mouy
authors.affiliation: Service de Pédiatrie, CHU Clermont-Ferrand, Clermont-Ferrand, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Pereira
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, AP-HP, Paris, France; Université Paris-Descartes, Paris, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Département de Santé Publique, Institut Cancérologique de la Loire, Saint-Étienne, France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Bourmaud
authors.affiliation: Service de Médecine Interne, Université Pierre-et-Marie-Curie, Paris-6, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France.
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.affiliation: Hôpital Trousseau, AP-HP, Paris, France.
authors.forename: Judith
authors.initials: J
authors.lastname: Landman-Parker
authors.affiliation: Service de Pédiatrie Générale, CHU Niort, France.
authors.forename: Patricia
authors.initials: P
authors.lastname: Chellun
authors.affiliation: Service de Pédiatrie, hôpital Mère-Enfant, CHU de Limoges, Limoges, France.
authors.forename: Mustapha
authors.initials: M
authors.lastname: Layadi
authors.affiliation: Service d'Hématologie-Oncologie, Hôpital Mère-Enfants, Nantes, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Thomas
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularites Nécrosantes et la Sclérodermie Systémique, AP-HP, Paris, France; Université Paris-Descartes, Paris, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.affiliation: Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Prieur
authors.affiliation: Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Necker-Enfants Malades, Paris, France; Université Paris-Descartes, Paris, France; Institut Hospitalo-Universitaire IMAGINE, Paris, France. Electronic address: pierre.quartier@nck.ap-hop-paris.fr.
authors.forename: Pierre
authors.initials: P
authors.lastname: Quartier
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000188:drug therapy; D011379:Prognosis; D012189:Retrospective Studies; D012871:Skin Diseases / Q000175:diagnosis / Q000209:etiology*; D013997:Time Factors
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: Classification; Criteria; Necrotizing vasculitis; Prognosis; Relapse