El Khattabi A, Tiev K P, Ziani M, Baret M, Genereau T, Cabane J
Service de médecine interne, Horloge II, Hôpital Saint-Antoine, Paris.
Presse Med. 2004 Oct 9;33(17):1160-3. doi: 10.1016/s0755-4982(04)98883-5.
Pulmonary hypertension (PHT) represents one of the severest complications and is life-threatening for patients suffering from systemic sclerosis (SSc). In France, the modalities for screening and treating PHT related to SSc are not well codified and no consensus has been reached. We conducted a survey among physicians inscribed on the list of the French Research Group on Sclerosis (GRFS - Groupe de Recherche Francais sur la Sclerodermie) to gather information on the status of the management of PHT related to SSc.
In 2002, we sent a questionnaire to 160 physicians, members of the GRFS, to assess the epidemiology and clinical profile of SSc patients as well as the modalities of screening and management of PHT in these patients.
Eighty-eight physicians in 71 centres replied to the questionnaire. Each centre followed-up a mean of 33 SSc patients, with a global distribution of 53% limited and 47% diffused SSc. These physicians saw a mean of 5 new cases of SSc per year. The patients had been referred by town practitioners (53%) or from the hospital (47%). The mean number of SSc patients with PHT was of 5.1 per physician (1.5 new SSc + PHT patients per year). Almost all the centres (65/67) who replied systematically screened for PHT in SSc patients using Doppler echocardiography a mean of every 1.3 years. For the management of the patients exhibiting PHT, the majority (41/63) of centres collaborated with a specialized unit. Around one third of the centres treated these patients with calcium channel inhibitors (82%) and/or prostacyclin (90%). All the patients were followed-up by Doppler echocardiography. The majority of the physicians (72%) were interested in a research protocol on the subject and each could have included 4 patients, i.e., a total of 160.
Pulmonary hypertension, a severe complication of SSc is screened for by the physicians of the GRFS using echocardiography with a frequency similar to Who guidelines (1.3 versus once/year).
肺动脉高压（PHT）是系统性硬化症（SSc）最严重的并发症之一，对患者生命构成威胁。在法国，与SSc相关的PHT筛查和治疗方式尚未得到很好的规范，也未达成共识。我们对法国硬化症研究小组（GRFS - Groupe de Recherche Francais sur la Sclerodermie）名单上的医生进行了一项调查，以收集与SSc相关的PHT管理现状的信息。
2002年，我们向GRFS的160名医生发送了一份问卷，以评估SSc患者的流行病学和临床特征，以及这些患者中PHT的筛查和管理方式。
71个中心的88名医生回复了问卷。每个中心平均随访33例SSc患者，其中局限性SSc占53%，弥漫性SSc占47%。这些医生每年平均诊治5例新的SSc病例。患者由社区医生（53%）或医院（47%）转诊而来。每位医生诊治的SSc合并PHT患者平均为5.1例（每年1.5例新的SSc合并PHT患者）。几乎所有回复的中心（65/67）都使用多普勒超声心动图对SSc患者进行PHT筛查，平均每1.3年筛查一次。对于出现PHT的患者管理，大多数中心（41/63）与专科单位合作。约三分之一的中心用钙通道抑制剂（82%）和/或前列环素（90%）治疗这些患者。所有患者均通过多普勒超声心动图进行随访。大多数医生（72%）对该主题的研究方案感兴趣，每位医生可纳入4例患者，即总共160例。
GRFS的医生使用超声心动图筛查SSc的严重并发症肺动脉高压，频率与世界卫生组织指南相似（1.3年一次与每年一次）。
