Clinical Manifestations and Treatment of Wegener's Granulomatosis

Julia U. Holle*, Martin Laudien, Wolfgang L. Gross

*Corresponding author for this work
38 Citations (Scopus)


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.

Original languageEnglish
JournalRheumatic Disease Clinics of North America
Issue number3
Pages (from-to)507-526
Number of pages20
Publication statusPublished - 01.08.2010


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