WEGENER'S GRANULOMATOSIS

uncommon disorder marked by inflammation and necrosis (death of tissue) of small blood vessels. The disease commonly occurs in mid-adult life. Almost any organ may be affected, but most often the diseased vessels are in the respiratory tract, kidneys, and spleen. The lesions closely resemble those in polyarteritis nodosa (q.v.). The disease is of unknown cause but is believed to involve hypersensitivity. A runny nose, nosebleeds, and symptoms suggestive of chronic inflammation of the sinuses are among the first indications of the disease. Subsequently, inflammation of blood vessels becomes widespread. Death occurs in a matter of months, most often from kidney damage. There is evidence that survival can be prolonged by administration of cytotoxic drugs and possibly also adrenal steroids-hormones secreted by the outer substance of the adrenal glands or synthetic substitutes for these hormones.