Wegener's Granulomatosis

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What is Wegener's Granulomatosis?

Wegener's Granulomatosis is a disease where the blood vessels are inflamed. This is called vasculitis. This inflammation causes damage to the organs of the body by decreasing their blood flow, which destroys their normal tissue. This is called necrosis.

The inflammation damages important organs of the body by limiting blood flow to those organs and destroying normal tissue. The organs afffected most commonly are the lungs and the kidneys.

Wegener's Granulomatosis is a disease that can involve any organ system, but specifically affects the upper and lower respiratory tract (nose, sinuses, windpipe and lungs), kidneys, and small blood vessels (capillaries, venules, arterioles and arteries) of the body.

Who gets Wegener's Granulomatosis?

The disease is uncommon occuring in 0.4 per 100 000 people. Peak incidence is in the fifth decade of life, with an average age at diagnosis of 45 years however, the disease can occur in all age groups.

The male:female ratio is 2:1. The disease occurs mostly in caucasion people.

Predisposing Factors

The cause of the disease is unknown, however, there is strong evidence that the disease is of an immunologic mechanism.

Progression

The complete syndrome usually quickly progresses to renal failure once the vascular phase begins. Nasal and pulmonary lesions are common in those with limited disease, these individuals may or may not have systemic involvement. These pulmonary lesions can improve or worsen spontaneously.

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