Wegener's granulomatosis

Wegener's granulomatosis with pulmonary eosinophilia group, even though there were some features of this group is divided into diseases.

There are three pathological features of the disease: 1) associated with aseptic necrosis of the upper respiratory tract and lung granulomatosis, 2) widespread arteritis, and 3) glomerulonephritis. Cause of the disease is unknown. Developing mechanism is thought to be related to an abnormal immune.

The disease is more in men aged 40-50 and most of the body may develop localized in any organ. The three main symptoms are characteristic: 1) persistent and sinüsitis rinitis, 2) nodular pulmonary lesions, and 3) the terminal uremia. The first symptoms usually occur in the respiratory tract: common cold, sinusitis, cough, hemoptysis, pleurisy or pneumonia. Otitis can in some cases, deafness. Hoarseness and swallowing difficulties may develop. Gums can be painful and ulcerations. Eye lesions are common.

Typical lesions in the lungs so many times in the form of a circular density granülomatosis'dir necrosis. There are in the middle of Intracavernous. The lesions are usually bilateral. Kavemlerin thick walls. Furthermore, scattered bronchopneumonitises infiltrates or nodular lesions are seen peribronişiyal. Secondary bacterial or fungal infections are common.

Skin, joint and muscle lesions are too.

Progressive nature of kidney disease. During the terminal uremia and other symptoms of the disease seen in general arteritis. High fever, arteritis, hemorrhagic lesions on the skin and mucous membranes, anemia, and leukocytosis are.

Wegener's granulomatosis disease prognosis is poor. Untreated cases, death is 6-8 months. Worse prognosis in patients with kidney disease. Effective treatment is provided by cyclophosphamide. Oral or intravenous drug use cases, showing very rapid progress. 1-3 weeks of treatment, improvement in clinical symptoms observed. WBC count should be carefully monitored and adjusted dose of cyclophosphamide to be kept up from 3.000/mm3. 1.000-1.500/mm3 should not fall down from the number of lymphocytes, so that the patient would be protected from infections. The other is an immunosuppressive drug azathioprine Siklofosfamid'e patients without tolerance. This is the same dose of the drug siklofos-famidin. If the disease is a severe at the beginning of cyclophosphamide together with corticosteroids, the dose of 1 mg / kg / day in reducing the clinical symptoms-lınca or disappeared (about 2 weeks) corticosteroid is reduced gradually, the day is over and then cut off. A full recovery after treatment with cyclophosphamide husule should continue for at least another year. Cyclophosphamide treatment improved the prognosis of Wegener's granulomatosis disease, most patients live for many years after discontinuation of medication.

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