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更多与炎症性风湿性疾病相关的淀粉样变性发病率下降的证据。

More evidence of declining incidence of amyloidosis associated with inflammatory rheumatic diseases.

机构信息

Department of Medicine, Kainuuu Central Hospital, Kajaani, Finland.

出版信息

Scand J Rheumatol. 2010 Nov;39(6):461-5. doi: 10.3109/03009741003747481. Epub 2010 Jun 21.

Abstract

OBJECTIVE

To assess the incidence, prevalence, and outcome of amyloidosis associated with inflammatory rheumatic diseases.

METHODS

An observational study was performed in the outpatient department of Kainuu Central Hospital from 1993 to 2007. The following criteria were used for the performance of abdominal subcutaneous fat aspiration (ASFA) and/or rectal biopsies: erythrocyte sedimentation rate (ESR) > 40 mm/h at two consecutive visits; and proteinuria (> 0.5 g/day) or serum creatinine > 150 μmol/L. Renal biopsy was performed when there was a high suspicion of amyloidosis in cases with negative findings in the above-mentioned biopsies. In addition, amyloid staining was used routinely for mucosal specimens taken in gastroscopy and colonoscopy. The patients were followed until death or to the end of 2007.

RESULTS

New diagnoses of amyloidosis in the consecutive 5-year periods from 1993 onwards numbered 11, 3, and 5, respectively. During the study period, there was a mean annual incidence of amyloidosis of 1.8 [95% confidence interval (CI) 1.1-2.8)/100,000]. At the end of 2007 there were eight subjects with amyloidosis, giving a point prevalence of 12.0/100,000 (95% CI 5.2-23.6). Five patients out of the 19 underwent haemodialysis because of terminal uraemia and three of them also had renal transplantation. Overall, 12 (63%) patients died after a median survival time of 6 (95% CI 4-8) years, one-third from amyloidosis. The 5-year survival rate of the series was 67% (95% CI 41-86).

CONCLUSION

Amyloidosis is rarely encountered today. ASFA or rectal biopsy facilitates its early diagnosis.

摘要

目的

评估与炎症性风湿性疾病相关的淀粉样变性的发病率、患病率和结局。

方法

1993 年至 2007 年在凯努区中心医院的门诊进行了一项观察性研究。进行腹部皮下脂肪抽吸术(ASFA)和/或直肠活检的标准如下:连续两次就诊时红细胞沉降率(ESR)>40mm/h;蛋白尿(>0.5g/天)或血清肌酐>150μmol/L。如果上述活检无阳性发现且高度怀疑淀粉样变性,则进行肾活检。此外,常规对胃镜和结肠镜检查获得的粘膜标本进行淀粉样染色。对患者进行随访,直至死亡或 2007 年底。

结果

1993 年以后的连续 5 年期间,新诊断的淀粉样变性分别为 11、3 和 5 例。在研究期间,淀粉样变性的年平均发病率为 1.8 [95%置信区间(CI)1.1-2.8)/100,000]。2007 年底,有 8 例淀粉样变性患者,点患病率为 12.0/100,000(95%CI 5.2-23.6)。19 例患者中有 5 例因终末期尿毒症接受血液透析,其中 3 例还进行了肾移植。总的来说,12 例(63%)患者在中位生存时间 6 (95%CI 4-8)年后死亡,其中三分之一死于淀粉样变性。该系列的 5 年生存率为 67%(95%CI 41-86)。

结论

如今淀粉样变性很少见。ASFA 或直肠活检有助于早期诊断。

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