Capizzi S A, Betancourt E, Prakash U B
Division of Pulmonary and Critical Care Medicine and Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
Mayo Clin Proc. 2000 Nov;75(11):1148-52. doi: 10.4065/75.11.1148.
To evaluate the presentation and clinical course of patients with tracheobronchial amyloidosis.
We retrospectively reviewed the records of all patients with biopsy-proven tracheobronchial amyloidosis who were evaluated at the Mayo Clinic in Rochester, Minn, between 1973 and 1999. All relevant information, such as clinical, historical, demographic, laboratory, and histological data, was examined.
Tracheobronchial amyloidosis was diagnosed in 17 patients (9 women and 8 men), with a mean age of 56.6 years. The most common symptoms at presentation were dyspnea, cough, hemoptysis, and hoarseness. None of the 14 patients who underwent investigation for systemic amyloidosis had any evidence of disease outside the tracheobronchial system except for a man in whom multiple myeloma had been diagnosed 3 years before the development of respiratory disease. Treatment of tracheobronchial amyloidosis consisted of laser or forceps resection, external radiation, systemic therapy, or observation. Two patients died of complications directly related to their disease.
Patients presenting with tracheobronchial amyloidosis have symptoms similar to those caused by various airway disorders. Tracheobronchial amyloidosis is not typically associated with systemic amyloidosis or pulmonary parenchymal involvement. It often recurs and despite repeated attempts with bronchoscopic techniques, airway compromise remains a major problem.
评估气管支气管淀粉样变患者的临床表现及临床病程。
我们回顾性分析了1973年至1999年间在明尼苏达州罗切斯特市梅奥诊所接受评估的所有经活检证实为气管支气管淀粉样变患者的病历。检查了所有相关信息，如临床、病史、人口统计学、实验室及组织学数据。
确诊17例气管支气管淀粉样变患者（9例女性，8例男性），平均年龄56.6岁。最常见的临床表现为呼吸困难、咳嗽、咯血及声音嘶哑。在接受系统性淀粉样变检查的14例患者中，除1例在呼吸系统疾病发生前3年已诊断为多发性骨髓瘤的男性患者外，其余患者均无气管支气管系统以外的疾病证据。气管支气管淀粉样变的治疗包括激光或钳夹切除、外照射、全身治疗或观察。2例患者死于与疾病直接相关的并发症。
气管支气管淀粉样变患者的症状与各种气道疾病引起的症状相似。气管支气管淀粉样变通常与系统性淀粉样变或肺实质受累无关。它常复发，尽管多次尝试支气管镜技术，但气道狭窄仍是主要问题。
