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

Springer Science and Business Media LLC - Tập 12 - Trang 1-5 - 2014
Marek Bohm1,2, 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 Martini1,25, Nicolino Ruperto1
1Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy
2Charles University in Prague, 1st Medical Faculty and General University Hospital in Prague, Prague, Czech Republic
3Department of Pediatric Rheumatology and Nephrology, Hacettepe University Children’s Hospital, Ankara, Turkey
4Istituto Giannina Gaslini, Servizio di Epidemiologia e Biostatistica, Genova, Italy
5Department of Rheumatology, Great Ormond St Hospital, NHS Foundation Trust, London, UK
6Istituto Giannina Gaslini, Nefrologia, Genova, Italy
7Department of Pediatrics, Division of Pneumology and Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany
8Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, USA
9Université 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
10Department of Pediatrics, The Queen Silvia Children’s Hospital, University of Gothenburg, Göteborg, Sweden
11Department of Pediatrics, Skejby Sygehus, Aarhus University Hospital, Pediatric Rheumatology Clinic, Aarhus, Denmark
12Universidade Federal de São Paulo, Pediatrics, São Paulo, Brazil
13Juliane Marie Centret, Rigshospitalet, Pediatrisk klinik II, Copenhagen, Denmark
14Department of Pediatrics, Emma Children Hospital AMC, Amsterdam, Netherlands
15Department of Allergology, Rheumatology and Clinical Immunology, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
16Hospital Dr Felipe Glasman, Rheumatolgy Section, Bahía Blanca, Argentina
17Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany
18Department of Pediatric Rheumatology and Immunology, University Children’s Hospital, Muenster, Germany
19Pediatric Department, University Hospital Centre, Tirana, Albania
20CHU Le Kremlin Bicêtre, APHP, University of Paris Sud, CEREMAI (Centre de référence national des maladies auto-inflammatoires, rhumatologie pédiatrique), Paris, France
21Asklepios Klinik Altona, Hamburg, Germany
22Kinderklinik Garmisch-Partenkirchen gGmbH, Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany
23Department of Pediatric Immunology and Rheumatology, Wilhelmina Kinderziekenhuis, Utrecht, Netherlands
24Benghazi Children Hospital – Benghazi, MUB - Rheumatology Clinic, Benghazi, Lybia
25Dipartimento di Pediatria, Università degli Studi di Genova, Genova, Italy

Tóm tắt

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.

Tài liệu tham khảo

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