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Pathophysiology: 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.
Findings: 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.
Research: Research work on Wegener’s Granulomatosis was conducted in Istituto Giannina Gaslini Pediatria II - Reumatologia, PRINTO, Genova, Italy. The study was approved by the ethics committee of the Gaslini Hospital (Genoa, Italy) and by the ethics committees of all remaining participating centres and informed consent was obtained from parent(s) as required by the national law in each participating country.
Treatment
Corticosteroids: Medications such as prednisone help suppress the immune system and treat the early signs and symptoms of Wegener's granulomatosis.
Immune suppressant medications: Most people require another immunosuppressive drug, such as cyclophosphamide, azathioprine or methotrexate, to counteract the body's immune reaction.