Wegener's is another rare disease that causes granulomas to form throughout the body, including the nose and sinuses. Here, however, the granulomas damage the walls of small- and medium-sized blood vessels. This damage interferes with normal blood supply, which can lead to injury and destruction of nearby tissues. The cause of Wegener's is also unknown, but research suggests it's an immune disorder in which the body's defenses are mistakenly aimed at the blood vessels.
Wegener's most commonly affects three areas: the lungs, the kidneys, and the nose and sinuses. Unlike most people with sinus-
itis, whose nasal membranes are swollen and secrete too much mucus, those with Wegener's have excessively thin and dry nasal tissues. The blood vessels in these membranes become fragile, leading to frequent nosebleeds. What mucus there is in the nose tends to dry out, leading to the formation of crusts. More advanced cases of Wegener's cause erosion of cartilage in the nose, resulting in a hole in the septum (also known as a septal perforation).
Sinus infections are common among people with Wegener's, and the disease is often diagnosed during treatment for sinusitis. An ENT doctor who suspects a patient has Wegener's will order a series of blood tests, including one that detects the presence of antineutrophil cytoplasmic antibody, a marker for Wegener's. The diagnosis can also be confirmed with a biopsy of tissue from a turbinate.
Wegener's is treated with oral steroids and medications known as immunosuppressive agents, which blunt the response of the body's immune system. With treatment, more than 90 percent of people with Wegener's enter remission, although as many as half will relapse when medication is reduced or stopped. Those who do not relapse may remain in remission for many years, often without needing more medication. Like sarcoidosis, sinus surgery is reserved for those cases in which the sinuses become blocked by diseased tissue.
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