Etienne Merlin1, Richard Mouy2, Bruno Pereira3, Luc Mouthon4, Aurélie Bourmaud5, Jean-Charles Piette6, Judith Landman-Parker7, Patricia Chellun8, Mustapha Layadi9, Caroline Thomas10, Loïc Guillevin4, Anne-Marie Prieur2, Pierre Quartier11. 1. Service de Pédiatrie, CHU Clermont-Ferrand, Clermont-Ferrand, France; Inserm CIC 1405, Centre de Recherche Chez l'Enfant, Clermont-Ferrand, France. 2. Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Necker-Enfants Malades, Paris, France. 3. Service de Pédiatrie, CHU Clermont-Ferrand, Clermont-Ferrand, France. 4. 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. 5. Département de Santé Publique, Institut Cancérologique de la Loire, Saint-Étienne, France. 6. Service de Médecine Interne, Université Pierre-et-Marie-Curie, Paris-6, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France. 7. Hôpital Trousseau, AP-HP, Paris, France. 8. Service de Pédiatrie Générale, CHU Niort, France. 9. Service de Pédiatrie, hôpital Mère-Enfant, CHU de Limoges, Limoges, France. 10. Service d'Hématologie-Oncologie, Hôpital Mère-Enfants, Nantes, France. 11. 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.
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). OUTCOME MEASURES: (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. CONCLUSION: 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.
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). OUTCOME MEASURES: (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. CONCLUSION: 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.