系统性淀粉样变的呼吸系统表现

目的提高临床对系统性淀粉样变患呼吸系统受累的认识。方法回顾性分析1985年-2003年收治的46例系统性淀粉样变患的呼吸系统表现。结果46例系统性淀粉样变患中，37例具有呼吸系统临床表现，分别累及上呼吸道、气管支气管、肺实质、胸膜和纵隔、肺门淋巴结，12例经活检证实。胸膜病变和肺实质病变包括局限性和弥漫性病变分别占呼吸系统受累的系统性淀粉样变患的62．12％、51．35％和40．54％。结论系统性淀粉样变患呼吸系统受累远高于既往献报道，尤其是胸膜病变，故应提高系统性淀粉样变对呼吸系统受累的认识，早期诊断,早期治疗。

The clinic features of respiratory tract in systemic amyloidosis

ObjectiveTo comprehend the clinical presentation s of systemic amyloidosis in respiratory tract MethodsThe rec ords of all patients with biopsy-confirmed systemic amyloidosis admitted to our hospital between 1985 and 2003 were retrospectively reviewed All relevant info rmations of respiratory tract,such as clinical,image,and demographic,were an alyzed ResultsAmong 46 cases of systemic amyloidosis,respira tory amyloidosis was diagnosed in 37 patients (27 men and 10 men) clinically,in vovling upper respiratory tract,tracheobronchial,lung parenchyma,pleura,medi asteinal and hilar,with a mean age of 51 49 years 12 cases were confirmed by the characteristic Congo-red staining of respiratory biopsies The most common sites at presentation were lung parenchyma and pleura ConclusionsThe respiratory tract was involved in about 80 4% of patients with systemic amylodosis meaning a higher prevalence than previously reported It is necessar y to pay more attention to respiratory amyloidosis to diagnose and treat early