Utz J P, Swensen S J, Gertz M A
Mayo Medical School, Rochester, Minnesota, USA.
Ann Intern Med. 1996 Feb 15;124(4):407-13. doi: 10.7326/0003-4819-124-4-199602150-00004.
To define the prognosis for and radiographic presentation of patients with pulmonary amyloidosis.
Retrospective review of the Mayo Clinic experience with biopsy-proven pulmonary amyloidosis from 1980 to 1993.
Tertiary care center.
Patients with pulmonary biopsy specimens showing amyloid deposition.
Medical records were reviewed, and pertinent information was recorded, including demographic data, type of pulmonary biopsy, results of biopsies of nonpulmonary sites and of immunoelectrophoresis, and other clinical, radiographic, and laboratory information necessary for distinguishing localized pulmonary amyloidosis, primary systemic amyloidosis, secondary amyloidosis, and familial amyloidosis.
35 of 55 patients with pulmonary amyloidosis had primary systemic amyloidosis that presented radiographically as an interstitial or reticulonodular pattern with or without pleural effusion. The median survival after diagnosis was 16 months. Nodular pulmonary "amyloidomas" (nodular amyloid lesions) were not associated with systemic disease and were associated with a benign prognosis. Three of 4 patients with localized tracheobronchial amyloidosis required Nd:YAG (neodymium:yttrium-aluminum-garnet) laser therapy for obstructive symptoms. "Senile" amyloid deposition was an incidental finding in some patients at autopsy.
Localized amyloidomas are characterized by a benign course and are not associated with systemic amyloidosis. Despite its localized nature, tracheobronchial amyloid deposition may be asymptomatic or may result in significant morbidity due to obstructive phenomena. Pulmonary amyloidosis associated with primary systemic amyloidosis generally presents as a diffuse interstitial pattern with or without pleural effusion. Complete survival data indicate that long-term outcome is poor after diagnosis. We describe the largest series of patients diagnosed by bronchoscopic lung biopsy. Despite reports to the contrary, we have found bronchoscopic lung biopsy to be a safe and effective diagnostic technique.
明确肺淀粉样变性患者的预后及影像学表现。
对梅奥诊所1980年至1993年经活检证实的肺淀粉样变性病例进行回顾性研究。
三级医疗中心。
肺活检标本显示淀粉样蛋白沉积的患者。
查阅病历并记录相关信息，包括人口统计学数据、肺活检类型、非肺部部位活检及免疫电泳结果，以及区分局限性肺淀粉样变性、原发性系统性淀粉样变性、继发性淀粉样变性和家族性淀粉样变性所需的其他临床、影像学和实验室信息。
55例肺淀粉样变性患者中，35例患有原发性系统性淀粉样变性，其影像学表现为间质或网状结节状，伴或不伴有胸腔积液。诊断后的中位生存期为16个月。结节性肺“淀粉样瘤”（结节性淀粉样病变）与全身性疾病无关，预后良好。4例局限性气管支气管淀粉样变性患者中有3例因阻塞性症状需要接受钕：钇铝石榴石（Nd:YAG）激光治疗。“老年性”淀粉样蛋白沉积在部分尸检患者中为偶然发现。
局限性淀粉样瘤病程良性，与系统性淀粉样变性无关。尽管气管支气管淀粉样蛋白沉积具有局限性，但可能无症状，也可能因阻塞现象导致严重发病。与原发性系统性淀粉样变性相关的肺淀粉样变性通常表现为弥漫性间质模式，伴或不伴有胸腔积液。完整的生存数据表明，诊断后的长期预后较差。我们描述了经支气管镜肺活检诊断的最大系列患者。尽管有相反的报道，但我们发现支气管镜肺活检是一种安全有效的诊断技术。
