Valeyre Dominique, Freynet Olivia, Dion Geneviève, Bouvry Diane, Annesi-Maesano Isabella, Nunes Hilario
Université Paris Nord 13, EA2363, F-93017 Bobigny Cedex, France.
Presse Med. 2010 Jan;39(1):53-9. doi: 10.1016/j.lpm.2009.09.015. Epub 2009 Dec 4.
Diffuse interstitial lung diseases (DILD) cover more than 200 conditions classified in 4 groups: secondary DILD; sarcoidosis; particular DILDs and idiopathic interstitial pneumonias. Overall, these diseases have a prevalence of 60-80/100,000 and an incidence around 30/100,000. Sarcoidosis and idiopathic pulmonary fibrosis are the 2 most frequent diseases, accounting together for more than 50% of all cases, followed by DILD related to connective tissue disease and to immunologic lung diseases. The incidence of different causes of DILD depends on epidemiologic factors: age, sex, race, smoking habits, and history. Mortality is very elevated in idiopathic pulmonary fibrosis, linked in more than 80% of cases to the fibrosis itself, with a median survival between 24 and 36 months. The epidemiologic studies on DILD have substantial limitations, and new studies must be conducted, in particular in France where we lack epidemiologic data beyond one old study of sarcoidosis and one more recent study focused on farmer's lung in the Doubs.
弥漫性间质性肺疾病(DILD)涵盖200多种疾病,分为4组:继发性DILD;结节病;特殊DILD和特发性间质性肺炎。总体而言,这些疾病的患病率为60 - 80/10万,发病率约为30/10万。结节病和特发性肺纤维化是最常见的两种疾病,共占所有病例的50%以上,其次是与结缔组织病和免疫性肺疾病相关的DILD。DILD不同病因的发病率取决于流行病学因素:年龄、性别、种族、吸烟习惯和病史。特发性肺纤维化的死亡率非常高,超过80%的病例与纤维化本身有关,中位生存期为24至36个月。关于DILD的流行病学研究有很大局限性,必须开展新的研究,尤其是在法国,除了一项关于结节病的旧研究和一项最近聚焦于杜省农民肺的研究外,我们缺乏流行病学数据。