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Il Giornale Italiano di Radiologia Medica 2018 Novembre-Dicembre;5(6):783-8

DOI: 10.23736/S2283-8376.18.00127-4

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: Italian

Wegener’s granulomatosis in childhood: the role of diagnostic imaging in a rare case of exclusive gastrointestinal involvement

Alessandra MIRABILE , Antonella MARZOCCA, Federica AMMIRATI, Dino MANGIALARDI, Mariantonietta MONTEDURO, Amato A. STABILE IANORA

Sezione di Radiologia, Dipartimento Interdisciplinare di Medicina, Università degli Studi di Bari/Policlinico, Bari, Italia


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Granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis (WG), is a necrotizing granulomatous vasculitis belonging to the group of antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) with microscopic polyangiitis and Churg-Strauss Syndrome. WG is very rare in childhood and it frequently has multisystemic presentation, targeting mainly the upper and lower respiratory tracts and kidneys, but gastrointestinal involvement is uncommon. Crohn’s disease and ulcerative colitis are inflammatory bowel diseases (IBD) with many extraintestinal manifestations. WG presenting primarily with gastro-intestinal symptoms without extraintestinal involvement is very rare in adults and even rarer in pediatric patients. Clinically, symptoms of WG and IBD can mimic each other: the patient could have abdominal pain, weight loss, bloody diarrhea with mucus. This case illustrates the difficulties in establishing the diagnosis when symptoms of the diseases mimic each other and the importance of integrating clinical, laboratory and imaging data in the diagnosis. In addition, since 2008 the EULAR/PRINTO/PRES criteria improved diagnostic sensitivity and specificity for WG among children with AAVs. About treatment, with the use of pediatric therapeutic protocols (corticosteroids, azathioprine, methotrexate, cyclophosphamide) AAVs are no longer rapidly fatal in the majority of affected children.


KEY WORDS: Granulomatosis with polyangiitis - Anti-neutrophil cytoplasmic antibody-associated vasculitis - Inflammatory bowel diseases

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