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居住在德国或土耳其的土耳其裔成年家族性地中海热患者的疾病严重程度。居住国是否会影响疾病的病程?

Disease severity in adult patients of Turkish ancestry with familial mediterranean fever living in Germany or Turkey. Does the country of residence affect the course of the disease?

机构信息

Central Patient Admission Unit/Emergency Department, Marienhospital Herne, Ruhr-University Bochum, Herne, Germany.

出版信息

J Clin Rheumatol. 2013 Aug;19(5):246-51. doi: 10.1097/RHU.0b013e31829ce005.

Abstract

BACKGROUND

The environment may affect the course of familial Mediterranean fever (FMF).

OBJECTIVE

The objective of this study was to compare disease severity between adult FMF patients in Turkey (TR) and Germany (G).

METHODS

Adult FMF patients of Turkish ancestry on colchicine living in Turkey (n = 40) or G (n = 35) were compared. Disease severity, C-reactive protein (CRP), and erythrocyte sedimentation rate were assessed.

RESULTS

Groups differed significantly in the following aspects: age at onset of disease (TR: 15.6, G: 10.8 years; P = 0.02), delay between onset and initiation of colchicine treatment (TR: 6.8 years, G: 14.9 years; P < 0.001), female gender (TR: 80%, G: 57.1%; P = 0.04), and duration of disease (TR: 14.4 years, G: 23.4 years; P < 0.001). There was no significant difference in colchicine treatment concerning average dosing and duration of therapy. No significant difference could be found between the 2 groups in CRP and disease severity as assessed by the score of Pras et al. (Am J Med Genet. 1998;75:216-219) even after adjusting for potential confounding variables. Mean erythrocyte sedimentation rate was significantly higher among patients living in G (TR: 13.2 mm/first hour, G: 26.3 mm/first hour; P < 0.001). Among patients living in Germany, there was a significant difference in age at FMF onset depending on their country of birth (born in TR: 14.9 years, born in G: 6.9 years; P = 0.0001).

CONCLUSIONS

In adult FMF patients living in Turkey or Germany, no difference in disease activity or CRP could be found. German patients were younger at onset of disease and had a longer delay between onset and initiation of colchicine treatment.

摘要

背景

环境可能会影响家族性地中海热(FMF)的病程。

目的

本研究旨在比较土耳其(TR)和德国(G)成年 FMF 患者的疾病严重程度。

方法

比较居住在土耳其(n=40)或德国(n=35)的土耳其裔成年 FMF 患者。评估疾病严重程度、C 反应蛋白(CRP)和红细胞沉降率。

结果

两组在以下方面存在显著差异:疾病发病年龄(TR:15.6 岁,G:10.8 岁;P=0.02)、发病至开始秋水仙碱治疗的时间延迟(TR:6.8 年,G:14.9 年;P<0.001)、女性比例(TR:80%,G:57.1%;P=0.04)和疾病持续时间(TR:14.4 年,G:23.4 年;P<0.001)。秋水仙碱治疗的平均剂量和治疗时间在两组之间无显著差异。调整潜在混杂因素后,两组间 CRP 和 Pras 等人评估的疾病严重程度评分无显著差异(Am J Med Genet. 1998;75:216-219)。居住在德国的患者的平均红细胞沉降率明显较高(TR:13.2 毫米/第一小时,G:26.3 毫米/第一小时;P<0.001)。居住在德国的患者中,根据出生地,FMF 发病年龄存在显著差异(出生于 TR:14.9 岁,出生于 G:6.9 岁;P=0.0001)。

结论

在居住在土耳其或德国的成年 FMF 患者中,未发现疾病活动或 CRP 存在差异。德国患者发病年龄较小,且发病至开始秋水仙碱治疗的时间间隔较长。

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