TY - JOUR T1 -肺外结节病的临床表型:法国的分析,多民族、多中心队列JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.01160 -2020欧元六世- 57 - 4 SP - 2001160盟利霍,拉斐尔AU - Annesi-Maesano,伊莎贝拉AU - Nunes,希拉里奥盟——Launay大卫盟——Borie拉斐尔盟——减少,卡里姆盟——Schleinitz尼古拉斯盟——HamidouMohamed AU - Mahevas, Matthieu AU - Devilliers, Hervé AU - Bonniaud, Philippe AU - Lhote, François AU - Haroche, Julien AU - Rufat, Pierre AU - Amoura, Zahir AU - Valeyre, Dominique AU - Cohen Aubart，N2 -结节病是一种病因不明的罕见疾病，在临床特征和预后方面存在广泛的异质性。我们的目的是探讨结节病的表型及其与肺外亚群的临床相关性。结节病流行病学研究(EpiSarc)是一项法国多中心回顾性研究。根据一项标准化方案，在2013年至2016年期间，使用11家医院中心的适当代码，通过国家住院记录识别结节病患者。回顾了医疗图表。利用层次聚类分析确定结节病的表型。共纳入1237例患者(男性562例，女性675例)。诊断结节病的平均年龄为43.5±13岁。根据器官受累、疾病类型和症状，聚类分析发现5种不同的表型:1)结节性红斑、关节受累和肺门淋巴结(n=180); 2) eye, neurological, digestive and kidney involvement (n=137); 3) pulmonary involvement with fibrosis and heart involvement (n=630); 4) lupus pernio and a high percentage of severe involvement (n=41); and 5) hepatosplenic, peripheral lymph node and bone involvement (n=249). Phenotype 1 was associated with being European/Caucasian and female and with non-manual work, phenotype 2 with being European/Caucasian, and phenotypes 3 and 5 with being non-European/Caucasian. The labour worker proportion was significantly lower in phenotype 5 than in the other phenotypes.This multicentre study confirms the existence of distinct phenotypes of sarcoidosis, with a non-random distribution of organ involvement. These phenotypes differ according to sex, geographical origin and socioprofessional category.There are five distinct phenotypes of sarcoidosis, with a non-random distribution of organ involvement. These five phenotypes differ according to sex, geographical origin and socioprofessional category. https://bit.ly/3iCurZK ER -