title: Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival.
abstract: OBJECTIVE
  Pulmonary arterial hypertension (PAH) is a severe, life-limiting complication of systemic sclerosis (SSc). Guidelines recommend early detection and management of SSc-PAH. However, little is known about the impact of detection programs on patients with SSc-PAH. This study was undertaken to assess the clinical characteristics of patients with SSc-PAH at diagnosis and their long-term outcomes.
  
  
  METHODS
  Two incident cohorts of patients with SSc-PAH from the same management era (2002/2003) were studied. The first cohort (designated the routine practice cohort) included consecutive adult patients with symptomatic SSc in whom a diagnosis of PAH was made by right-sided heart catheterization (RHC) at the time of recruitment into the French PAH Registry. The second cohort (designated the detection cohort) comprised consecutive patients with SSc who entered a systematic PAH detection program and were subsequently found to have PAH on RHC. Clinical characteristics at diagnosis of PAH and subsequent 8-year mortality were compared between the cohorts.
  
  
  RESULTS
  There were 16 patients in each cohort. At the time of PAH diagnosis, patients in the detection cohort had less advanced pulmonary vascular disease compared with patients in the routine practice cohort, as evidenced by more patients being in New York Heart Association class I and class II, a lower mean pulmonary artery pressure and pulmonary vascular resistance index, and a higher cardiac output. Patients in the detection cohort were less likely to receive diuretics and warfarin, but there was no difference in exposure to PAH-specific therapies between the cohorts. The 1-, 3-, 5-, and 8-year survival rates were 75%, 31%, 25%, and 17%, respectively, in the routine practice cohort compared with 100%, 81%, 73%, and 64%, respectively, in the detection cohort (P = 0.0037).
  
  
  CONCLUSIONS
  Compared with patients in routine clinical practice, PAH detection programs in SSc are able to identify patients with milder forms of the disease, allowing earlier management.
authors.affiliation: Université Paris-Sud, Le Kremlin-Bicêtre, France; AP-HP Hôpital Antoine Béclère, Clamart, France. marc.humbert@abc.aphp.fr
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.forename: Azzedine
authors.initials: A
authors.lastname: Yaici
authors.forename: Pascal
authors.initials: P
authors.lastname: de Groote
authors.forename: David
authors.initials: D
authors.lastname: Montani
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.forename: Virginie
authors.initials: V
authors.lastname: Gressin
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Pierre
authors.initials: P
authors.lastname: Clerson
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
mesh_terms: D000368:Aged; D015331:Cohort Studies; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D012042:Registries; D012595:Scleroderma, Systemic / Q000150:complications / Q000401:mortality*; D015996:Survival Rate