title: Clinical manifestation of pediatric granulomatosis with polyangiitis - the experience of two regions in Poland.
abstract: BACKGROUND
  The purpose of this study was to describe clinical manifestations, laboratory findings and outcome of granulomatosis with polyangiitis (GPA) in pediatric patients living in two regions (Southern and Central) of Poland.
  
  
  METHODS
  Retrospective analysis of patient hospital records from four large hospitals during a period from 1995 to 2013. Patients with confirmed diagnosis of GPA according to American College of Rheumatology (ACR) and EULAR/PRINTO/PRES criteria for GPA were analyzed. All patients were subjected to clinical, laboratory, radiological and immunological assessment.
  
  
  RESULTS
  During this 18-year period only 9 children with confirmed diagnosis of GPA (6 girls, 3 boys) were identified. The average age of the disease onset was 12 years (range: 8-16 years). Average delay between first symptoms and diagnosis was approx. 20 months (range: 0-84 months). Organ system involvement at presentation included: kidneys 88.8% (8/9), lungs 77.7% (7/9), ear/nose/ throat 55.5% (5/9), gastrointestinal tract 55.5% (5/9), skin 44.4% (4/9), joints 22.2% (2/9), eyes 11.1% (1/9) and nervous system 11.1% (1/9). In 5 children disease course was progressive (constant progression of sinusitis in one case, end-stage renal disease in two, chronic kidney disease stage IV in one and one child died due to alveolar hemorrhage).
  
  
  CONCLUSIONS
  The majority of our patients were females. Clinical features of pediatric GPA were similar to those described in adults. None of our patients developed subglottic stenosis and in only 2 children saddle-nose deformity was observed. Although GPA was treated according to contemporary standards care, disease progression was observed in more than a half of children.
authors.affiliation: Second Department of Internal Medicine, Unit of Allergy and Clinical Immunology, Jagiellonian University Medical College, Krakow, Poland. joanna.kosalka@gmail.com.
authors.forename: Joanna
authors.initials: J
authors.lastname: Kosalka
authors.forename: Stanislawa
authors.initials: S
authors.lastname: Bazan-Socha
authors.forename: Maria
authors.initials: M
authors.lastname: Ignacak
authors.forename: Anna
authors.initials: A
authors.lastname: Zugaj
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Zachwieja
authors.forename: Izabela
authors.initials: I
authors.lastname: Glodzik
authors.forename: Grzegorz
authors.initials: G
authors.lastname: Lis
authors.forename: Marcin
authors.initials: M
authors.lastname: Tkaczyk
authors.forename: Zbigniew
authors.initials: Z
authors.lastname: Zuber
authors.forename: Jacek
authors.initials: J
authors.lastname: Musial
mesh_terms: D000293:Adolescent; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D044963:Biopsy, Fine-Needle; D002648:Child; D018450:Disease Progression; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D006304:Health Status; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000175:diagnosis; D010038:Otorhinolaryngologic Diseases / Q000175:diagnosis* / Q000209:etiology*; D011044:Poland; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Wegener’s granulomatosis; childhood; children; granulomatosis with polyangiitis; vasculitis