Wegeners Granulomatosis

Pathophysiology: Wegener’s granulomatosis (WG) is a rare multisystemic autoimmune disease of unknown aetiology, characterized by necrotizing granulomatous inflammatory and pauci-immune vasculitis in small- and medium-sized blood vessels (capillaries, venules, arterioles and arteries) associated with antineutrophil cytoplasmic antibodies directed against proteinase 3 (PR3), a neutrophil serine protease, presented in primary azurophil granules of polymorphonuclear neutrophils and lysosomes of monocytes.

Diagnosis: Whenever possible, it is important to confirm the diagnosis of Wegener’s by biopsying an involved organ and finding the pathologic features of this disease under the microscope. Because many diseases may mimic Wegener’s (and vice versa), before starting a treatment regimen it is essential to be as certain of the diagnosis as possible.

Treatment: With early diagnosis and appropriate treatment, you may recover from Wegener's granulomatosis within a few months. Maintenance therapy often needs to be continued for 18 to 24 months. In some cases, longer treatment may be necessary. Because the disease can recur, your doctor will continue monitoring your condition closely after treatment. 

Statistics: A retrospective cohort study using hospital discharge registers with a review of hospital case records. A total of 492 patients received a verified diagnosis of WG between 1981 and 2000. Of these, 203 died before the end of June 2005. The overall 1-year survival rate was 83.3% and the 5-year survival rate was 74.2%. The standardized mortality ratio (SMR) for all WG patients was 3.43 [95% confidence interval (CI) 2.98-3.94], for women 4.38 (95% CI 3.59-5.61) and for men 2.80 (95% CI 2.28-3.41).