Wegener’s granulomatosis

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Adenocarcinoma - chest X-ray

Respiratory system

Wegener's granulomatosis on the leg

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 inflamed. The eyes, skin, and joints may also be affected. Arthritis occurs in about half of all cases.

Midline granulomatosis

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.

Review Date: 08/10/2005
Reviewed By: David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.