title: Wegener's granulomatosis: a review of clinical features and an update in diagnosis and treatment.
abstract: 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.
authors.affiliation: Oral Medicine unit, Department of Maxillofacial Medicine and Surgery, UCL Eastman Dental Institute, London, UK.
authors.forename: Hanan A
authors.initials: HA
authors.lastname: Almouhawis
authors.forename: Jair C
authors.initials: JC
authors.lastname: Leao
authors.forename: Stefano
authors.initials: S
authors.lastname: Fedele
authors.forename: Stephen R
authors.initials: SR
authors.lastname: Porter
mesh_terms: D003937:Diagnosis, Differential; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008264:Macrophages / Q000473:pathology; D009059:Mouth Diseases / Q000175:diagnosis; D009336:Necrosis; D014657:Vasculitis / Q000473:pathology
chemical_list: D007166:Immunosuppressive Agents
keywords: Wegener's granulomatosis; granulomatous disease; oral disease