Respiratory manifestations in amyloidosis

Background Amyloidosis is a collection of diseases in which different proteins are deposited·Amyloiddeposits occur in systemic and organ-limited forms·In both systemic and localized forms of the disease, lung canbe involved·The aim of this study was to explore the different respiratory manifestations of amyloidosis·Methods Chest radiology, clinical presentations, bronchoscopic/laryngoscopic findings and lung function dataof 59 patients with amyloidosis involving respiratory tract collected during January 1986 to March 2005, wereanalysed·Results Of the 16 cases with localized respiratory tract amyloidosis, 8 had the lesions in the trachea and thebronchi, 2 in the larynx and the trachea, 5 in the larynx and/or the pharynx, and 1 in the lung parenchyma·Of43 systemic amyloidosis with respiratory tract involvement, 3 had the lesions in bronchi, 13 in lung parenchyma,33 in pleura, 8 in mediastina, 1 in nose and 1 in pharynx·Chest X-rays were normal in most cases oftracheobronchial amyloidosis·CT, unlike chest X-rays, showed irregular luminal narrowing, airway wallthickening with calcifications and soft tissue shadows in airway lumen·Localized lung parenchymal amyloidosispresented as multiple nodules·Multiple nodular opacities, patch shadows and reticular opacities were the mainradiological findings in systemic amyloidosis with lung parenchymal involvement·In pleural amyloidosis, pleuraleffusions and pleural thickening were detected·Mediastinal and/or hilar adenopathy were also a form of lunginvolvement in systemic amyloidosis·The major bronchoscopic findings of tracheobronchial amyloidosis werenarrowing of airway lumen, while nodular,‘tumour like’or‘bubble like’masses, with missing or vaguecartilaginous rings, were detected in about half of the patients·Conclusions Localized respiratory tract amyloidosis mostly affects the trachea and the bronchi·Chest X-raysare not sensitive to detect these lesions·Systemic amyloidosis often involves lung parenchyma and the pleura·Open lung biopsy or pleural biopsy should be performed for the diagnosis·