title: Lung manifestations in MPO-ANCA associated vasculitides in children.
abstract: OBJECTIVE
  To describe lung manifestations in MPO-ANCA associated vasculitides in children.
  
  
  METHODS
  We retrospectively reviewed the medical records of patients with MPO-ANCA associated vasculitis, who were followed in two pediatric nephrology departments from January 2000 to December 2010.
  
  
  RESULTS
  Twelve patients were identified with MPO-ANCA over the study period. Their median age (IQR) at diagnosis was 10.5 (6.3-12.0) years, and their median duration of follow-up was 4.8 (1.2-7.5) years. Only five of them had pulmonary involvement with diffuse alveolar hemorrhage. Lung involvement was inaugural for four of five children. One child with severe chronic respiratory disease and renal failure died after 6 years of disease progression. Pulmonary function tests were available for 10 children. They were within normal ranges in four of five patients without clinical lung manifestations, and no significant impairment was observed in children with pulmonary complications.
  
  
  CONCLUSIONS
  Diffuse alveolar hemorrhage complicates 40% of cases of MPO-ANCA associated vasculitides in children with renal involvement. After an acute potentially severe phase, a complete recovery without significant functional impairment was observed in four of five affected children.
authors.affiliation: Pneumologie Pédiatrique, Necker, AP-HP, Paris, France.
authors.forename: Salma
authors.initials: S
authors.lastname: Ben Ameur
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Véronique
authors.initials: V
authors.lastname: Baudouin
authors.forename: Muriel
authors.initials: M
authors.lastname: Le Bourgeois
authors.forename: Véronique
authors.initials: V
authors.lastname: Houdouin
authors.forename: Christophe
authors.initials: C
authors.lastname: Delacourt
authors.forename: Alice
authors.initials: A
authors.lastname: Hadchouel
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000276:immunology / Q000503:physiopathology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D007674:Kidney Diseases / Q000150:complications; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology*; D008297:Male; D009195:Peroxidase / Q000276:immunology; D011859:Radiography; D012189:Retrospective Studies
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase
keywords: Wegener granulomatosis; alveolar hemorrhage; lung restriction; microscopic polyangiitis