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首页> 外文期刊>Sarcoidosis, vasculitis, and diffuse lung diseases: official journal of WASOG >The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract.
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The importance of complete screening for amyloid fibril type and systemic disease in patients with amyloidosis in the respiratory tract.

机译：在呼吸道淀粉样变性患者中完整筛查淀粉样蛋白原纤维类型和系统性疾病的重要性。

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BACKGROUND: Patients with symptomatic thoracic involvement by amyloidosis are virtually all of AL-type, and have historically been divided into systemic and localised disease, a subdivision that helps predict outcome and aids management. Assessment and classification of amyloid has evolved in recent years to include a variety of tests, including radiolabelling of the serum amyloid P component (SAP scan) to assess anatomical distribution and immunohistochemical studies to assess fibril subtypes. Furthermore, CT scanning is now a frequent investigation for the diagnosis of thoracic disease. We wished to determine the value of these investigations on the management of such patients with amyloidosis. METHODS: Clinicopathological data, including immunohistochemical analysis, CT scans and SAP scan results, were retrospectively reviewed from patients presenting with amyloidosis in the respiratory tract. These were then analysed to determine their impact on classification and prognosis. RESULTS: Seventeen patients over ten years were identified, one case being related to metastatic medullary carcinoma of the thyroid. Of the remaining 16 cases, one was shown to have hereditary amyloid of transthyretin-type (TTR) on immunohistochemistry, altering management. The remaining 15 cases were AL-type, with 6 cases being classified as localised and 9 cases as systemic, after evaluation for serum and/or urine monoclonal products, cardiac involvement via echocardiography, plasma cell abnormalities on bone marrow examination, CT scan and SAP scan. 3/3 patients with localised AL-type disease had a negative SAP scan, whilst 3/5 patients with systemic AL-type disease had a positive scan. SAP scan of the patient with TTR-type disease provided information on extent of disease and supported the diagnosis by the pattern of distribution. Using CT scans to discriminate between localised and systemic disease showed a significant association with mortality at 2 years (p = 0.03). CONCLUSION: Although the majority of symptomatic patients with pulmonary amyloidosis have AL-type disease, immunohistochemical confirmation is necessary in order not to miss rarer subtypes with completely different treatment regimes. Furthermore, a comprehensive evaluation, including SAP scan and CT scan of the thorax, in conjunction with echocardiography, bone marrow, serum and urine studies, needs to be undertaken in order to achieve maximum accuracy with regard to localised and systemic disease.
机译：背景：淀粉样变性病伴有症状性胸廓受累的患者实际上都是AL型，历史上已分为全身性疾病和局部性疾病，这是一个有助于预测结局和辅助治疗的细分。淀粉样蛋白的评估和分类在最近几年中已发展到包括多种测试，包括对血清淀粉样蛋白P成分进行放射性标记（SAP扫描）以评估解剖分布，以及进行免疫组化研究以评估原纤维亚型。此外，CT扫描现在是诊断胸腔疾病的常见检查方法。我们希望确定这些研究对此类淀粉样变性患者的管理价值。方法：回顾性分析呼吸道淀粉样变性患者的临床病理资料，包括免疫组织化学分析，CT扫描和SAP扫描结果。然后对这些进行分析，以确定它们对分类和预后的影响。结果：确定了17名10年以上的患者，其中1例与甲状腺转移性髓样癌有关。在其余的16例患者中，有1例在免疫组织化学上显示具有甲状腺素转运蛋白型（TTR）遗传性淀粉样蛋白，改变了治疗方法。其余15例属于AL型，在评估血清和/或尿液单克隆产物，超声心动图检查对心脏的累及，骨髓检查，CT扫描和SAP的浆细胞异常后，分为局部性和局部性6例，全身性9例。扫描。 3/3例局部AL型疾病患者的SAP扫描阴性，而3/5例全身性AL型疾病的患者阳性扫描。 TTR型疾病患者的SAP扫描可提供有关疾病范围的信息，并通过分布方式支持诊断。使用CT扫描来区分局部疾病和全身性疾病显示与2岁时的死亡率有显着相关性（p = 0.03）。结论：尽管大多数有症状的肺淀粉样变性病患者都患有AL型疾病，但为了不漏诊具有完全不同治疗方案的罕见亚型，必须进行免疫组化确认。此外，需要进行综合评估，包括对胸部的SAP扫描和CT扫描，以及超声心动图，骨髓，血清和尿液研究，以在局部和全身性疾病方面获得最大的准确性。

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