| Subject | Contents |
| Definition | A rare disorder which causes inflammation of blood vessels ( vasculitis ) in the upper respiratory tract (nose, sinuses, ears), lungs, and kidneys. Many other areas of the body may also be affected, with arthritis (joint inflammation) occurring in almost half of all cases. The eyes and skin may also be affected. |
| Alternative Names | Midline granulomatosis |
| Causes, incidence, and risk factors | The cause is unknown, but Wegener's Granulomatosis is thought to be an autoimmune disorder and is often classified as one of the rheumatic diseases. Destructive lesions develop in the upper and lower respiratory tract and the kidney. In the kidney, these lesions cause glomerulonephritis that may result in hematuria (blood in the urine) and kidney failure . It occurs most often between the ages of 30 to 50, and 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.
The kidney disease can progress rapidly, with kidney failure occurring within months of the initial diagnosis. If untreated, kidney failure and death occur in more than 90% of all patients with Wegener's granulomatosis.
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| Symptoms | Early symptoms are often systemic and may include fatigue , malaise (an ill feeling), fever , and a sense of discomfort around the nose and sinuses. Upper respiratory infections such as sinusitis or ear infections frequently precede the diagnosis of Wegener's Granulomatosis. Other upper respiratory symptoms include nose bleeds, pain, and ulcers '>ulcers or sores around the opening of the nose. Persistent fever without an obvious cause (fever of undetermined origin -- FUO) may be an initial symptom. Night sweats may accompany the fever. Anorexia and weight loss '>weight loss are common. Skin lesions are common, but there is no one characteristic lesion associated with the disease. Kidney disease is necessary to make the definitive diagnosis of Wegener's Granulomatosis. The urine may be bloody, which often first appears as red or smoky urine. It may have no symptoms, however, it is readily diagnosed with laboratory studies. Eye problems develop in a significant number of patients and may range from a mild conjunctivitis to severe inflammation of the eyeball and the tissues around the eyeball. Additional symptoms include:weaknessloss of appetiteweight lossbloody discharge from the nose pain over the sinuses sinusitis lesions ( ulcers '>ulcers , sores, and crusting) in and around opening of the nose coughcoughing up bloodbloody sputumshortness of breathwheezingchest painblood in the urinerashesjoint pains |
| Signs and tests | biopsy of abnormal tissue, which may include: open lung biopsyupper airway biopsynasal mucosal biopsybronchoscopy with transtracheal biopsyurinalysis , protein and blood in the urine ( proteinuria and hematuria ) chest X-ray (cavity formation, pulmonary infiltrates) bone marrow aspiration (may be done) blood tests to look for the presence of autoantibodies (antibodies that the body makes against its own tissue) |
| Treatment | Treatment with corticosteroids, cyclophosphamide, methotrexate, or azathioprine produces long-term remission in over 90% of affected people. |
| Support Groups | Support groups with others who suffer from similar diseases may help patients and their families learn about their diseases and adjust to the changes associated with the treatment. |
| Expectations (prognosis) | With treatment, most people recover within months, although chronic renal failure may develop. Without treatment, the disease is usually fatal within a few months of diagnosis. |
| Complications | chronic renal failure hemoptysis (coughing up blood) respiratory failure inflammation of the eyes nasal septum perforation (hole) rash side effects of medications used to treat the disease Note: Complications usually result from lack of treatment. |
| Calling your health care provider | Call your health care provider if chest pain , coughing up blood , blood in the urine , or other symptoms of this disorder are present. |
| Prevention | No preventive measures are known. |
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