Incidence and survival in non-hereditary amyloidosis in Sweden
Incidence and survival in non-hereditary amyloidosis in Sweden
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DOI：
10.1186/1471-2458-12-974
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发表时间：
2012-11-13
影响因子：
4.5
通讯作者：
Sundquist, Kristina
中科院分区：
文献类型：
--
作者：
Hemminki, Kari;Li, Xinjun;Sundquist, Kristina
背景资料：淀粉样变性是一种异质性疾病，由淀粉样纤维在器官中沉积引起，从而干扰生理功能。几乎没有任何发病率的数据是可用的，大多数生存数据仅限于专科clinics.Methods：淀粉样变性患者被确定从瑞典医院出院和门诊登记，从2001年至2008年。结果：非遗传性淀粉样变性的发病率为8.29每百万人-年，诊断年龄的最高发病率为65岁以上。继发性系统性淀粉样变性的发病率为1/100万，女性发病率高，随后类风湿性关节炎死亡人数最多;中位生存期为4年。然而，由于类风湿性关节炎死亡也发生在其他诊断亚型中，继发性系统性淀粉样变性的发病率可能约为2.0/100万。器官局限性淀粉样变性患者的中位生存期为6年。大多数骨髓瘤死亡发生在诊断为不明或“其他”淀粉样变性的患者中。这些亚型可能占大多数的免疫球蛋白轻链（AL）淀粉样变性的情况下，中位生存时间为3 years.Conclusions：目前的诊断分类不能挑出AL淀粉样变性在瑞典的出院数据，但外推骨髓瘤病例，发病率为3.2每百万可归因于AL淀粉样变性。同样，基于类风湿性关节炎死亡率，2.0的发病率可归因于继发性系统性淀粉样变性。
Background: Amyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics.Methods: Amyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008.Results: The incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years. However, as rheumatoid arthritis deaths also occurred in other diagnostic subtypes, the incidence of secondary systemic amyloidosis was likely to be about 2.0 per million. The median survival of patients with organ-limited amyloidosis was 6 years. Most myeloma deaths occurred in patients diagnosed with unspecified or 'other' amyloidosis. These subtypes probably accounted for most of immunoglobulin light chain (AL) amyloidosis cases; the median survival time was 3 years.Conclusions: The present diagnostic categorization cannot single out AL amyloidosis in the Swedish discharge data but, by extrapolation from myeloma cases, an incidence of 3.2 per million could be ascribed to AL amyloidosis. Similarly, based on rheumatoid arthritis death rates, an incidence of 2.0 could be ascribed to secondary systemic amyloidosis.