Cottin V, Cadranel J, Crestani B, Dalphin J C, Delaval P, Israel-Biet D, Kessler R, Reynaud-Gaubert M, Valeyre D, Wallaert B, Bouquillon B, Cordier J F
National Reference Centre for Rare Pulmonary Diseases, Louis Pradel University Hospital, Lyon, France.
Competence Centre for Rare Pulmonary Diseases, AP-HP, Tenon Hospital, Pierre & Marie Curie University, Paris, France.
Respir Med. 2014 Jan;108(1):195-202. doi: 10.1016/j.rmed.2013.11.017. Epub 2013 Dec 3.
The present survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' current diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF).
From December 7, 2011 to February 18, 2012, all French pulmonologists (n = 2608) were contacted. Those who reported following up at least one IPF patient (n = 509) were administered a 26-item questionnaire by phone or e-mail.
509 pulmonologists (41% of responders, 20% of French pulmonologists) were involved in the management of IPF patients. Of those, 36% discussed the cases with radiologists and pathologists. Out of 406 community pulmonologists practicing outside of reference or competence (e.g. expert) centres, 141 (35%) indicated referring patients to those centres. The 2011 international guidelines for IPF were known by 67% of pulmonologists involved in IPF, 84% of whom considered them appropriate for practice. About 58% of patients were diagnosed with mild to moderate IPF as defined by percentage predicted forced vital capacity ≥50% and percentage predicted diffusing capacity of the lung for carbon monoxide ≥35%. Management resulted from multidisciplinary discussion in 36% of the cases. By the end of December 2011, 49% of patients with mild to moderately severe IPF were treated with oral corticosteroids, and 27% received no treatment.
Despite correct awareness of international IPF guidelines, modalities of multidisciplinary discussion and of early diagnosis and management need to be improved through the network of expert centres.
本次由法国罕见肺病专家中心协调开展的调查,探究了法国肺科医生目前针对特发性肺纤维化(IPF)的诊断和治疗实践。
在2011年12月7日至2012年2月18日期间,联系了所有法国肺科医生(共2608人)。那些报告称至少随访过1例IPF患者的医生(共509人),通过电话或电子邮件接受了一份包含26个条目的问卷。
509名肺科医生(占回复者的41%,法国肺科医生的20%)参与了IPF患者的管理。其中,36%的医生会与放射科医生和病理科医生讨论病例。在406名在参考或专业(如专家)中心以外执业的社区肺科医生中,141人(35%)表示会将患者转诊至这些中心。参与IPF诊治的肺科医生中,67%知晓2011年IPF国际指南,其中84%认为这些指南适用于临床实践。约58%的患者被诊断为轻度至中度IPF,定义为预测用力肺活量百分比≥50%且预测肺一氧化碳弥散量百分比≥35%。36%的病例通过多学科讨论进行管理。到2011年12月底,49%的轻度至中度重度IPF患者接受了口服糖皮质激素治疗,27%的患者未接受治疗。
尽管对IPF国际指南有正确认识,但仍需通过专家中心网络改善多学科讨论以及早期诊断和管理的方式。