Clinical manifestations and treatment of Wegener's granulomatosis.
Wegener's granulomatosis (WG) is characterized by granulomatous lesions and vasculitic disease manifestations. Granulomatous lesions are found in the upper and lower respiratory tract (eg, granulomatous sinusitis, orbital masses, and pulmonary granuloma), whereas vasculitic manifestations occur frequently in lung (alveolar hemorrhage) and kidney (glomerulonephritis). Vasculitis is typically associated with antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3. WG has been traditionally associated with a poor outcome and increased mortality as documented by numerous studies; however, recent cohort studies report an improved outcome, probably a consequence of increased awareness leading to an earlier diagnosis, and to improved treatment strategies derived from evidence from controlled trials. Treatment regimens for WG, adapted to disease stage and activity, are reviewed and discussed in this article.
Holle
Julia U
JU
Department of Rheumatology and Clinical Immunology, Vasculitis Center, University Hospital Schleswig-Holstein, Bad Bramstedt, 24576 Bad Bramstedt, Germany. Holle@klinikumbb.de
Laudien
Martin
M
Gross
Wolfgang L
WL
D019268:Antibodies, Antineutrophil Cytoplasmic; D014890:Granulomatosis with Polyangiitis / Q000000981:diagnostic imaging* / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000000981:diagnostic imaging / Q000469:parasitology; D008168:Lung / Q000000981:diagnostic imaging; D053146:Myeloblastin / Q000276:immunology; D011859:Radiography; D012074:Remission Induction
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents; D053146:Myeloblastin