Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment

被引:62
作者
Almouhawis, Hanan A. [1 ]
Leao, Jair C. [1 ,2 ]
Fedele, Stefano [1 ]
Porter, Stephen R. [1 ]
机构
[1] UCL Eastman Dent Inst, Oral Med unit, Dept Maxillofacial Med & Surg, London WC1X 8LD, England
[2] Univ Fed Pernambuco, Dept Clin & Odontol Prevent, Recife, PE, Brazil
关键词
Wegener's granulomatosis; granulomatous disease; oral disease; OF-THE-LITERATURE; ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES; MICROSCOPIC POLYANGIITIS; INITIAL MANIFESTATION; PULMONARY VASCULITIS; RARE PRESENTATION; PRESENTING SIGN; DISEASE; INVOLVEMENT; GLAND;
D O I
10.1111/jop.12030
中图分类号
R78 [口腔科学];
学科分类号
1003 ;
摘要
Wegener's granulomatosis (WG) is an idiopathic, systemic inflammatory disease characterized by necrotizing granulomatous inflammation and pauci-immune small-vessel vasculitis of upper and lower respiratory tract and kidneys. The condition affects both genders equally, although some inconsistent gender differences have been observed. The aetiology of WG remains unknown although a number of exogenous factors have been suggested to be of aetiological relevance. Most clinical characteristics of this disease are non-specific, making clinical diagnosis challenging. Histopathological examination of lesional and peritoneal tissue is not pathognomonic, but is an essential investigation to confirm the presence of disease and exclude other disorders. At present, despite the increasingly wide range of potential therapies, cyclophosphamide plus corticosteroids remain the most recognized and effective means of inducing and sustaining remission of WG.
引用
收藏
页码:507 / 516
页数:10
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