It is the result of inflammation within the tissues called granulomatous inflammation and blood vessel inflammation "vasculitis" , which can damage organ systems. The areas most commonly affected by GPA include the sinuses, lungs, and kidneys, but any site can be affected. Vasculitis is a general term that refers to inflammation of the blood vessels. When inflamed, the blood vessel may become weakened and stretch forming an aneurysm , or become so thin that it ruptures resulting in bleeding into the tissue.

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This article discusses GPA in general. For organ specific radiographic features please refer to individual articles:. There is a slight male predilection and onset is typically at approximately 50 years of age 8. Presentation depends on which organ systems are involved, however upper respiratory tract symptoms are most common 8. Cough and hemoptysis , proteinuria and hematuria as well as systemic symptoms such as anorexia, malaise and fever are also common 9.

Interstitial fibrosis at the bases is usually the first finding, but is usually asymptomatic. Pleural effusions and mediastinal nodal enlargement are also encountered. Focal lesions can give proteinuria and hematuria while diffuse lesions can give acute renal failure.

Causes mucosal ulceration and granulomatous masses within the nasal cavities with adjacent bony and cartilaginous destruction. Can result in splenic infarction More recently agents such as Rituximab are also used. Appropriate medical therapy has dramatically increased long term survival 7.

Wegener was a member of the Nazi party and it is suspected that he took part in experiments on concentration camp inmates.

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Please use another browser until we can get it fixed. Robbins and Cotran pathologic basis of disease. W B Saunders Co. Read it at Google Books - Find it at Amazon. Promoted articles advertising. Edit article Share article View revision history Report problem with Article. URL of Article. Article information. Tags: pr3 , lungs. Support Radiopaedia and see fewer ads. Cases and figures. Figure 1: gross pathology Figure 1: gross pathology. Case 1 Case 1. Figure 2: histology - non-caseating granuloma Figure 2: histology - non-caseating granuloma.

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Wegener's Granulomatosis

The detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 PR3-ANCA is highly specific for Wegener's granulomatosis. Vasculitis is an independent risk factor for diffuse endothelial dysfunction and may be a consequence of TNF-alpha action on endothelial cells. Recently, another study has shown intima-media thickening of the wall of the common carotid artery and bulb, as well as a significantly increased incidence of stroke, myocardial infarction and occlusive artery disease in Wegener's granulomatosis. This study suggests that systemic inflammation and vasculitis contribute to accelerated arteriosclerosis in Wegener's granulomatosis. This site needs JavaScript to work properly. Please enable it to take advantage of the complete set of features!


Translation of "granulomatoza Wegener" in English

Granulomatosis with polyangiitis GPA , previously known as Wegener's granulomatosis WG , [1] [2] [3] [4] [5] is an extremely rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels vasculitis. It is a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. Typical signs and symptoms include nosebleeds , stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. The cause of GPA is unknown. GPA treatment depends on the severity of the disease.

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