Context and objectives: 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.
Methods: 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.
Results: 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.
Conclusion: 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).