title: Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.
abstract: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, which affects small- and medium-sized blood vessels and is often associated with cytoplasmic ANCA. GPA occurs in patients between 45 and 60 years old of both genders, and is rarely observed in blacks. The prevalence of GPA increases along a south-north gradient in Europe (20 to 150/million). The main clinical characteristics involve the upper and/or lower respiratory tract and kidneys. Ear, nose and throat manifestations with recurrent sinusitis and crusting rhinorrhea are usually severe. Lung nodules are frequently seen, sometimes excavated. Renal involvement is characterized by rapidly progressive necrotizing glomerulonephritis with extracapillary crescents. Limited forms of GPA predominantly affect the upper respiratory tract, whereas generalized forms of GPA include renal manifestations and/or alveolar hemorrhage and/or vital organ involvement with an altered general condition. The combination of immunosuppressant drugs and corticosteroids has converted this typically fatal illness into one in which 80% of patients achieve remission. However, despite considerable therapeutic progress over the last decades, relapses remain frequent (50% at 5 years), and maintenance treatment is now the main therapeutic challenge.
authors.affiliation: Département de Médecine Interne et Immunologie Clinique, Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié-Salpêtrière, F-75013 Paris, France; Département Hospitalo-Unviversitaire I2B, UPMC Univ Paris 06, F-75013 Paris, France; INSERM UMR 7211, F-75005 Paris, France; CNRS, UMR 7211, F-75005 Paris, France; INSERM, UMR_S 959, F-75013 Paris, France.
authors.forename: Cloé
authors.initials: C
authors.lastname: Comarmond
authors.affiliation: Département de Médecine Interne et Immunologie Clinique, Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié-Salpêtrière, F-75013 Paris, France; Département Hospitalo-Unviversitaire I2B, UPMC Univ Paris 06, F-75013 Paris, France; INSERM UMR 7211, F-75005 Paris, France; CNRS, UMR 7211, F-75005 Paris, France; INSERM, UMR_S 959, F-75013 Paris, France. Electronic address: patrice.cacoub@psl.aphp.fr.
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003937:Diagnosis, Differential; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010641:Phenotype; D011379:Prognosis
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
keywords: ANCA; Characteristics; Granulomatosis with polyangiitis; Treatment