2002-2006 年法国米迪-庇里牛斯地区自身免疫性天疱疮的发病率。

Incidence of auto-immune pemphigus in the Midi-Pyrénées region in 2002-2006.

机构信息

Dermatology Department, CHU de Toulouse, Paul Sabatier University, Toulouse, France.

出版信息

Dermatology. 2010;220(2):97-102. doi: 10.1159/000261503. Epub 2009 Nov 21.

DOI:10.1159/000261503

PMID:19940441

Abstract

INTRODUCTION

Auto-immune pemphigus is an organ-specific immune disorder due to pathogenic auto-antibodies. Both genetic and environmental factors have been associated with the occurrence of auto-immune pemphigus. Little is known about the epidemiology of auto-immune pemphigus in western Europe.

OBJECTIVE

To evaluate the incidence of auto-immune pemphigus in south-western France (namely the Midi-Pyrénées region) in a 5-year period between 2002 and 2006.

MATERIALS AND METHODS

We performed a retrospective study of the annual incidence of auto-immune pemphigus diagnosed in the Midi-Pyrénées region, between January 1, 2002, and December 31, 2006. Possible cases of pemphigus were initially selected using skin direct immunofluorescence (DIF) databases from all laboratories in the Midi-Pyrénées region. Systematic validation of all cases was performed by two of the authors (N.M., M.T.). To qualify as a case of auto-immune pemphigus, patients had to fulfil the following criteria: history of clinical signs of pemphigus as assessed by a dermatologist and a positive DIF. To be included in the study, validated cases of auto-immune pemphigus had to fulfil the following criteria: the date of first positive skin DIF between January 1, 2002, and December 31, 2006, and patient living in the Midi-Pyrénées region at the time of the first positive skin DIF.

RESULTS

Between 2002 and 2006, 91 patients with positive DIF were identified. Fifty-four patients with positive DIF did not meet the validation criteria for the study. Thirty-seven cases were included in the analysis. The crude annual mean incidence of auto-immune pemphigus between 2002 and 2006 was 2.7 cases/10(6) inhabitants/year (95% CI: 1.87-3.69). The world-population-standardized annual mean incidence of auto-immune pemphigus was 1.55 (95% CI: 0.99-2.11).

CONCLUSION

We report a 1.55/10(6) inhabitants/year world-population-standardized incidence of auto-immune pemphigus in the south-west of France. A nationwide epidemiological study of pemphigus should be performed in France.

摘要

简介

自身免疫性天疱疮是一种由致病性自身抗体引起的器官特异性免疫紊乱。遗传和环境因素都与自身免疫性天疱疮的发生有关。关于西欧自身免疫性天疱疮的流行病学知之甚少。

目的

评估 2002 年至 2006 年期间法国西南部（即米迪-庇里牛斯地区）的自身免疫性天疱疮发病率。

材料和方法

我们对 2002 年 1 月 1 日至 2006 年 12 月 31 日期间在米迪-庇里牛斯地区诊断出的自身免疫性天疱疮的年发病率进行了回顾性研究。使用来自米迪-庇里牛斯地区所有实验室的皮肤直接免疫荧光 (DIF) 数据库初步选择天疱疮可能病例。两名作者（N.M.，M.T.）对所有病例进行了系统验证。要符合自身免疫性天疱疮的病例标准，患者必须满足以下标准：皮肤科医生评估的天疱疮临床体征史和 DIF 阳性。要纳入研究，验证的自身免疫性天疱疮病例必须满足以下标准：2002 年 1 月 1 日至 2006 年 12 月 31 日期间首次 DIF 阳性皮肤的日期，以及首次 DIF 阳性皮肤时居住在米迪-庇里牛斯地区的患者。

结果

在 2002 年至 2006 年间，共发现 91 例 DIF 阳性患者。54 例 DIF 阳性患者不符合研究的验证标准。37 例病例被纳入分析。2002 年至 2006 年期间自身免疫性天疱疮的粗年平均发病率为 2.7 例/10(6)居民/年（95%CI：1.87-3.69）。自身免疫性天疱疮的世界人口标准化年平均发病率为 1.55（95%CI：0.99-2.11）。