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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: During the past 15 years, the epidemiology of Wegener's granulomatosis (WG) has become better understood. Descriptive epidemiological studies carried out primarily in European countries estimate a prevalence of WG ranging from 24 to 157 per million and annual incidence rates from 3 to 14 per million. These studies suggest a North-South declining gradient in disease risk in the Northern Hemisphere and an increase in incidence overtime, although latter it is due to improved diagnostic ascertainment.