Wegener's Granulomatosis |
Overview Wegener's granulomatosis is a rare disease in which the blood vessels become inflamed. This inflammation damages important organs of the body by limiting blood flow and destroying normal tissue. Although the disease can involve any organ Wegener's granulomatosis mainly affects the respiratory tract (sinuses nose windpipe and lungs) and kidneys. This disorder can affect people at any age and strikes men and women equally. Symptoms The first symptoms of Wegener's granulomatosis are often vague and frequently include upper respiratory tract symptoms joint pains weakness and tiredness. Upper respiratory tract The most common sign of Wegener's granulomatosis is involvement of the upper respiratory tract which occurs in nearly all patients. Symptoms include sinus pain discolored or bloody fluid from the nose and occasionally nasal ulcers. A common sign of the disease is an almost constant runny nose or other cold symptoms that do not respond to usual treatment or that become increasingly worse. Nasal inflammation or sinus drainage can cause a runny nose. Worse a hole may develop in the cartilage of the nose. This can cause it to collapse a condition known as saddle-nose deformity. The eustachian tubes which connect the middle ear to the back of the nose could become blocked. This can cause chronic ear problems. You could even lose your hearing. Bacterial infection can cause Wegener's-related sinusitis (inflammation of the sinuses) with symptoms of congestion and chronic sinus pain. Lungs Most people with Wegener's granulomatosis will have some problem with their lungs. In some cases there may be no symptoms. In others the person will cough; perhaps even cough up blood. He or she may also experience chest discomfort and shortness of breath. Kidneys Three-quarters of people with this disorder will have kidney problems. Most will not have any symptoms and the problem can only be detected with blood and urine tests. It is important to have these tests as proper treatment can prevent long-term damage to the kidneys. Musculoskeletal system Most people with Wegener's granulomatosis will complain of aching muscles and joints. This can be very uncomfortable. However it does not result in permanent joint damage or deformities. Eyes Wegener's granulomatosis can affect the eyes in several ways. People may develop:
Symptoms in the eye include redness burning or pain. Double vision or reduced vision are serious symptoms requiring immediate medical attention. Skin lesions Nearly half of all those with Wegener's granulomatosis develop skin lesions. These may appear as small red or purple raised areas or blister-like lesions ulcers or nodules. They may or may not hurt. Other symptoms Some people experience narrowing of the windpipe. The symptoms can include voice change hoarseness shortness of breath or cough. The nervous system and heart occasionally may be affected. Fever and night sweats may occur. Fever also may signal an infection often of the upper respiratory tract. Causes The cause of Wegener's granulomatosis is unknown. It is thought to be an autoimmune disorder in which the immune system destroys normal body tissue. Diagnosis To treat people with Wegener's granulomatosis most effectively doctors must diagnose the disease early. There are no blood tests available to diagnose Wegener's granulomatosis but blood tests are important to rule out other causes of illness and to determine which organ may be affected. Most blood tests can only suggest that a person has inflammation somewhere in the body. Anemia (low red blood cell count) elevated white blood cell count and platelet count and an elevated sedimentation rate are commonly found in people with Wegener's granulomatosis. If the kidneys are involved a health care provider can see red blood cells and structures called red blood cell casts in the urine when viewed under a microscope and the blood tests measuring kidney function may show abnormalities. X-ray results can be very helpful in diagnosing Wegener's granulomatosis. People with lung problems will have abnormal chest x-rays. CT (computed tomography) scans in people with sinus problems may show thickening of the sinus lining. Many people with active Wegener's granulomatosis have a blood test that shows the presence of a specific type of antibody called antineutrophil cytoplasmic antibodies (ANCA) (an antibody is a disease-fighting protein). Although a positive ANCA test is useful to support a suspected diagnosis of Wegener's granulomatosis in most instances health care providers do not use it by itself to diagnose this disorder. The ANCA test may be negative in some people with active Wegener's granulomatosis. Currently the only clear-cut way to diagnose Wegener's granulomatosis is by performing a biopsy (removing a tiny piece of tissue) of an involved organ (usually the sinuses lung or kidney). A health care provider will examine tissue from the organ under the microscope to confirm the presence of vasculitis and granulomas (a specific type of inflammation) which together are features of Wegener's granulomatosis. A biopsy is very important both to confirm the presence of the disease and also to make sure other disorders that may have similar signs and symptoms are not present. Treatment With appropriate treatment the outlook is good for people with Wegener's granulomatosis. In most cases treatment consists of a combination of a glucocorticoid (a steroid) and a cytotoxic medicine. Although these medicines are helpful in treating Wegener's granulomatosis they potentially have serious side effects. In many instances these can be minimized or prevented by careful monitoring by both the doctor and patient. Approximately half of people with Wegener's granulomatosis may experience a return of their disease. This occurs most frequently within 2 years of stopping medicine but can occur at any point both during treatment and after stopping treatment. |