Amiloidoz, amiloid olarak adlandýrýlan özel bir proteinin vücuttaki deðiþik dokularda anormal biçimde depolanmasý sonucunda ortaya çýkan bir grup hastalýktýr. Organize pnömoni, akciðer hastalýklarý içinde ender görülen ama oldukça karakteristik kliniko-patolojik özellikleri olan bir tablodur. Olgumuz nefes darlýðý, öksürük, balgam, yan aðrýsý yakýnmasý ile hastanemize baþvurdu. Postero-anterior akciðer grafisinde sol parakardiak sýnýrda heterojenite, sol sinüs kapalý olarak izlendi. Yüksek çözünürlüklü bilgi-sayarlý tomografide sol akciðer üst lob apikoposterior segmentte ve sol akciðer alt lobda yaygýn buzlu cam dansitesi zemininde retikülonodüler deðiþiklikler, yer yer konsolide alanlar, bronþektazik deðiþiklikler ve hacim kaybý saptandý. Olgunun proteinürisi olmasý üzerine renal amiloidoz açýsýndan böbrek biyopsisi yapýldý. Patoloji sonucu renal amiloidozis ile uyumlu geldi. Fiberoptik bronkoskopide sol alt lob mukozasýnda beyaz plak alanýndan biyopsi yapýldý. Biopsi örneði “organize pnömoni” (interstisyel pnömoni ve interstisyel fibrozis) bulgularý ile uyumlu geldi. Bu yazýda, kistik bronþektaziye baðlý geliþen renal tutulumu olan sekonder amiloidoz ve bronþiolitis obliterans organize pnömoni olgusu sunulmuþtur.
Anahtar Kelimeler: Kistik Bronþiektazi, Sekonder Amiloidozis, Bronþiolitis Obliterans Organize Pnömoni.Amyloidosis is group of conditions occurring as a result of abnormal storage of a specific protein, amyloid, in various tissues in the body. Organizing pneumonia, a rarely seen within lung diseases, but rather possessing a characteristic clinicopathologic features is a table. A case applied to our hospital with shortness of breath, cough, sputum, and chest pain. Heterogeneity at the left paracardiac borderline, by closing the left sinus, was observed in the posteroanterior chest x-ray. Reticulonodular changes on the basis of extensive ground-glass density, locally consolidated areas, changes in bronchiectasis, and a loss of volume in the apical-posterior segment of the upper and lower lobes of the left lung were determined by High-resolution computed tomography. Because of proteinuria, renal biopsy was performed on the basis of renal amyloidosis. The pathologic examination was consistent with renal amyloidosis. A biopsy was carried out by fiberoptic bronchoscopy in the lower left lobe mucosa from the area of white plaque. The biopsy specimen was compatible with "organized pneumonia” (interstitial pneumonia and interstitial fibrosis). Secondary amyloidosis and bronchiolitis obliterans organizing pneumonia with renal involvement, progressing due to infected cystic bronchiectasis, are presented in this study.
Keywords: Cystic Bronchiectasis, Secondary Amyloidosis, Bronchiolitis Obliterans Organizing Pneu-monia.