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Sinüzit, Miyokardit, Akciğerde Kitle ve Hızla İlerleyen Glomerülonefrit: Wegener Granülomatozu

An Insidious Disaster Composed of Sinusitis, Myocarditis, Lung Mass And Rapidly Progressive Glomerulonephritis: Wegener Granulomatosis

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DOI: 
10.5262/tndt.2010.1001.13
Abstract (2. Language): 
Wegener’s Granulomatosis is a multisystemic granulomatous inflammation of small vessels that typically effects the kidney, skin, joints and lower and upper airway tracts. Because of the multisystemic involvement, the signs and symptoms of disease may arise in heterogeneous fashion. Under the appearance of a nonspecific sytemic complaints, the suspicion of clinician may be a final clue for diagnosis. Here we present a patient with Wegener’s granolomatosis sufferring from generalized malaise, fever, heart failure, lung mass and acute renal failure. Multidisciplinary and close colloborative management is important to establish early diagnosis. We belive that plasmapheresis at initial acute onset of vasculitis syndromes especially in severe extrarenal involvements may be a lifesaving management.
Abstract (Original Language): 
Wegener granulomatozu klasik olarak alt ve üst hava yolları, böbrekler, deri ve eklemleri etkileyen nekrotizan granülomatoz yangılı vaskülitik sendromdur. Bir çok organ etkilenebileceğinden tutulan organa bağlı heterojen klinik bulgu verme özelliği vardir. Özgün olmayan sistemik klinik bulgular eşliğinde bazen, sadece klinisyenin şüphesi ile tanı konulabilir. Burada, genel durum bozukluğu, ateş, klinik kalp yetmezliği ve akciğerde kitle tanısı ile izlenen ve takipte akut böbrek yetmezliği gelişen Wegener Granulomatozlu bir hasta sunulmuştur. Multidisipliner yaklaşım tanı ve tedavide önemlidir. Akut vaskülit sendromların özellikle şiddetli böbrek dışı tutuluşlarında sitotoksik tedavi ve/veya plazma değişimi hayat kurtarıcı olabilir.
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