Pathophysiology: Wegener's granulomatosis (VEG-eh-nerz gran-u-loe-muh-TOE-sis) is an uncommon disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs. Wegener's granulomatosis, which is also called granulomatosis with polyangiitis (GPA), often affects your kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. Wegener's can affect other organs, but this isn't as common, and generally isn't as serious.
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.
Medications such as prednisone help suppress the immune system and treat the early signs and symptoms of Wegener's granulomatosis.
Most people require another immunosuppressive drug, such as cyclophosphamide, azathioprine or methotrexate, to counteract the body's immune reaction.
Statistics: The incidence, prevalence, and clinical presentation of Wegener's granulomatosis (WG) have occurred in the stable population of northern Norway during a 15-year period. The prevalence of WG has tripled in northern Norway over the last 15 years. While more efficacious therapy may explain part of this increase, we also found a significant trend toward increased incidence over that period. The incidence rate over the last 5 years is the highest reported so far, while the clinical presentation has remained unchanged.