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Wegener’s granulomatosis
Definition:
Wegener's granulomatosis is a rare disorder that causes blood vessels in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys to become swollen and inflammed. The eyes, skin, and joints may also be affected. Arthritis occurs in about half of all cases. Alternative Names:
Midline granulomatosis Text Continues Below
Causes, incidence, and risk factors:
The cause is unknown. Wegener's granulomatosis is thought to be an autoimmune disorder. Patients with this disease develop sores (lesions) in the respiratory tract and the kidneys. Kidney lesions cause glomerulonephritis, which may result in blood in the urine and kidney failure. Kidney disease can quickly get worse. If untreated, kidney failure and death occur in more than 90% of patients. Wegener's granulomatosis is most common in middle-aged adults. Men are affected twice as often as women. It is rare in children, but has been seen in infants as young as 3 months old. References:
Murray J, Nadel J. Textbook of Respiratory Medicine. 3rd ed. Philadelphia, Pa: W.B. Saunders Company; 2000: 1462-1463.
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