Navegando por Palavras-chave "Granulomatosis with polyangiitis"
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- ItemAcesso aberto (Open Access)Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)(Biomed Central Ltd, 2014-05-26) Bohm, Marek; Fernandez, Maria Isabel Gonzalez; Ozen, Seza; Pistorio, Angela; Dolezalova, Pavla; Brogan, Paul; Barbano, Giancarlo; Sengler, Claudia; Klein-Gitelman, Marisa; Quartier, Pierre; Fasth, Anders; Herlin, Troels; Terreri, Maria Teresa R. A. [UNIFESP]; Nielsen, Susan; van Rossum, Marion A. J.; Avcin, Tadej; Rodolfo Castell, Esteban; Foeldvari, Ivan; Foell, Dirk; Kondi, Anuela; Kone-Paut, Isabelle; Kuester, Rolf-Michael; Michels, Hartmut; Wulffraat, Nico; Ben Amer, Halima; Malattia, Clara; Martini, Alberto; Ruperto, Nicolino; Paediat Rheumatology Int; PRINTO; Charles Univ Prague; Gen Univ Hosp Prague; Hacettepe Univ; Ist Giannina Gaslini; NHS Fdn Trust; Charite; Ann & Robert H Lurie Childrens Hosp Chicago; Univ Paris 05; Univ Gothenburg; Aarhus Univ Hosp; Universidade Federal de São Paulo (UNIFESP); Rigshosp; Emma Children Hosp AMC; Univ Childrens Hosp; Hosp Dr Felipe Glasman; Klinikum Eilbek Hs 6; Univ Hosp Ctr; Univ Paris 11; Asklepios Klin Altona; Kinderklin Garmisch Partenkirchen gGmbH; Wilhelmina Childrens Hosp; Benghazi Children Hosp Benghazi; Univ GenoaBackground: 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.Findings: 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.Conclusions: Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.
- ItemSomente MetadadadosM2 macrophage is the predominant phenotype in airways inflammatory lesions in patients with granulomatosis with polyangiitis(Biomed Central Ltd, 2017) Silva de Souza, Alexandre Wagner [UNIFESP]; van Timmeren, Mirjan; Sanders, Jan-Stephan; Stegeman, Coen; Heeringa, Peter; Kallenberg, Cees G. M.; Westra, JohannaBackground: Macrophages may present two distinct phenotypes indicated as M1 and M2 under different stimuli. M1 and M2 macrophages have divergent functions that range from enhancement of inflammation for M1 to tissue repair and remodeling for M2 macrophages. The objective of this study was to evaluate the distribution of M1 and M2 macrophage phenotypes in biopsies from the airways of patients with active granulomatosis with polyangiitis (GPA) and to analyze their associations with T and B cells in those biopsies, and with nasal carriage of Staphylococcus aureus, disease parameters and therapy. Methods: Consecutive GPA patients (n = 35) with active airway disease, who underwent respiratory tract biopsy were included. Immunohistochemical evaluation was performed to assess the distribution of macrophages and T and B cells using the markers CD68, CD3 and CD20, respectively. CD86 was used as the M1 marker and CD163 as the M2 marker while Tbet and GATA-3 were used as Th1 and Th2 markers, respectively. At the time of the biopsy patients were assessed for nasal carriage of Staphylococcus aureus and treatment. Results: Percentages of macrophages and T cells were significantly higher than those of B cells in lesional tissue from the respiratory tract in GPA. M2 macrophages and Th2 cells were more frequent than M1 macrophages (p = 0.0007) and Th1 cells (p < 0.0001), respectively. Percentages of T cells were higher in nose biopsies than in biopsies from other sites (p = 0.021)
- ItemSomente MetadadadosRecommendations of the Brazilian Society of Rheumatology for the induction therapy of ANCA-associated vasculitis(Elsevier Science Inc, 2017) Silva de Souza, Alexandre Wagner [UNIFESP]; Calich, Ana Luisa [UNIFESP]; Mariz, Henrique de Ataide; Gomes Ochtrop, Manuella Lima; Santos Bacchiega, Ana Beatriz; Ferreira, Gilda Aparecida; Rego, Jozelia; Perez, Mariana Ortega; Rodrigues Pereira, Rosa Maria; Bernardo, Wanderley Marques; Levy, Roger AbraminoThe purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease. (c) 2017 Published by Elsevier Editora Ltda.