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Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis)

Marek Bohm12, Maria Isabel Gonzalez Fernandez1, Seza Ozen3, Angela Pistorio4, Pavla Dolezalova2, Paul Brogan5, Giancarlo Barbano6, Claudia Sengler7, Marisa Klein-Gitelman8, Pierre Quartier9, Anders Fasth10, Troels Herlin11, Maria Teresa R A Terreri12, Susan Nielsen13, Marion A J van Rossum14, Tadej Avcin15, Esteban Rodolfo Castell16, Ivan Foeldvari17, Dirk Foell18, Anuela Kondi19, Isabelle Koné-Paut20, Rolf-Michael Kuester21, Hartmut Michels22, Nico Wulffraat23, Halima Ben Amer24, Clara Malattia25, Alberto Martini125, Nicolino Ruperto1* and the Paediatric Rheumatology International Trials Organisation (PRINTO)

Author Affiliations

1 Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy

2 Charles University in Prague, 1st Medical Faculty and General University Hospital in Prague, Prague, Czech Republic

3 Department of Pediatric Rheumatology and Nephrology, Hacettepe University Children’s Hospital, Ankara, Turkey

4 Istituto Giannina Gaslini, Servizio di Epidemiologia e Biostatistica, Genova, Italy

5 Department of Rheumatology, Great Ormond St Hospital, NHS Foundation Trust, London, UK

6 Istituto Giannina Gaslini, Nefrologia, Genova, Italy

7 Department of Pediatrics, Division of Pneumology and Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany

8 Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

9 Université Paris-Descartes, Institut IMAGINE, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d’Immunologie, Hématologie et Rhumatologie Pediatrique, Paris, France

10 Department of Pediatrics, The Queen Silvia Children’s Hospital, University of Gothenburg, Göteborg, Sweden

11 Department of Pediatrics, Skejby Sygehus, Aarhus University Hospital, Pediatric Rheumatology Clinic, Aarhus, Denmark

12 Universidade Federal de São Paulo, Pediatrics, São Paulo, Brazil

13 Juliane Marie Centret, Rigshospitalet, Pediatrisk klinik II, Copenhagen, Denmark

14 Department of Pediatrics, Emma Children Hospital AMC, Amsterdam, Netherlands

15 Department of Allergology, Rheumatology and Clinical Immunology, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia

16 Hospital Dr Felipe Glasman, Rheumatolgy Section, Bahía Blanca, Buenos Aires, Argentina

17 Hamburger Zentrum für Kinder- und Jugendrheumatologie, Klinikum Eilbek Hs.6, Hamburg, Germany

18 Department of Pediatric Rheumatology and Immunology, University Children’s Hospital, Muenster, Germany

19 Pediatric Department, University Hospital Centre, Tirana, Albania

20 CHU Le Kremlin Bicêtre, APHP, University of Paris Sud, CEREMAI (Centre de référence national des maladies auto-inflammatoires, rhumatologie pédiatrique), Le Kremlin Bicêtre, Paris, France

21 Asklepios Klinik Altona, Hamburg, Germany

22 Kinderklinik Garmisch-Partenkirchen gGmbH, Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany

23 Department of Pediatric Immunology and Rheumatology, Wilhelmina Kinderziekenhuis, Utrecht, Netherlands

24 Benghazi Children Hospital – Benghazi, MUB - Rheumatology Clinic, Benghazi, Lybia

25 Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy

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Pediatric Rheumatology 2014, 12:18  doi:10.1186/1546-0096-12-18

Published: 26 May 2014



Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients.

Children with age at diagnosis ≤ 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. The clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature.


The 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. The most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. In adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series.


Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.

Wegener’s granulomatosis; Granulomatosis with polyangiitis; Clinical study; Clinical picture of disease; Comparison with literature