title: Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease.
abstract: BACKGROUND
  Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) is usually associated with a poor prognosis. However, data are either lacking or scarce on prognostic factors in this condition. The objectives of this study were to compare the survival of patients with ILD-associated PH (PH-ILD) or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD.
  
  
  METHODS
  Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right-sided heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mean pulmonary arterial pressure [mPAP] ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg).
  
  
  RESULTS
  As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently men with a history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies, and a lower FVC/diffusing capacity of lung for carbon monoxide (DLCO) ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively; P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio [HR], 2.44; P = .04) and lower DLCO (HR, 0.96; P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group.
  
  
  CONCLUSIONS
  Patients with SSc with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower DLCO and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance.
authors.affiliation: Service de Médecine Interne, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille; Centre de référence de la sclérodermie, Hôpital Claude-Huriez, CHRU Lille, Faculté de Médecine, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille. Electronic address: david.launay@chru-lille.fr.
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.affiliation: Faculté de Médecine, Université Paris-Sud, Kremlin-Bicêtre; AP-HP, Centre national de référence de l'hypertension pulmonaire sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart; INSERM U999, Hypertension Artérielle Pulmonaire: Physiopathologie et Innovation Thérapeutique, Centre Chirurgical Marie-Lannelongue, Le Plessis-Robinson.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Université Paris Descartes, Pôle de Médecine Interne, Centre de référence des vascularites et de la sclérodermie, Hôpital Cochin, AP-HP, Paris.
authors.forename: Alice
authors.initials: A
authors.lastname: Berezne
authors.affiliation: Hospices Civils de Lyon, Service de pneumologie, Centre de référence des maladies pulmonaires rares, Lyon; Université Claude-Bernard Lyon I, UMR 754, Lyon.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.affiliation: Université Paris Descartes, Service de Rhumatologie A, Hôpital Cochin, Paris.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
authors.affiliation: Service de Pneumologie, Hôpital Foch, Suresnes.
authors.forename: Louis-Jean
authors.initials: LJ
authors.lastname: Couderc
authors.affiliation: Service de Médecine Interne, Hôpital Foch, Suresnes.
authors.forename: Olivier
authors.initials: O
authors.lastname: Bletry
authors.affiliation: Faculté de Médecine, Université Paris-Sud, Kremlin-Bicêtre; AP-HP, Centre national de référence de l'hypertension pulmonaire sévère, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Clamart.
authors.forename: Azzedine
authors.initials: A
authors.lastname: Yaici
authors.affiliation: Service de Médecine Interne, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille.
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
authors.affiliation: Université Paris Descartes, Pôle de Médecine Interne, Centre de référence des vascularites et de la sclérodermie, Hôpital Cochin, AP-HP, Paris.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Centre de référence de la sclérodermie, Hôpital Claude-Huriez, CHRU Lille, Faculté de Médecine, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Le Pavec
authors.affiliation: Orgamétrie, Roubaix, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Clerson
authors.affiliation: Service de Médecine Interne, d'Immunologie EA2686, IMPRT IFR 114, Université Lille 2, Lille.
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D001794:Blood Pressure / Q000502:physiology; D002248:Carbon Monoxide / Q000378:metabolism; D015897:Comorbidity; D003430:Cross-Sectional Studies; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000401:mortality* / Q000503:physiopathology*; D008168:Lung / Q000378:metabolism; D017563:Lung Diseases, Interstitial / Q000453:epidemiology / Q000401:mortality* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000453:epidemiology / Q000401:mortality* / Q000503:physiopathology*; D012720:Severity of Illness Index; D015996:Survival Rate
chemical_list: D000974:Antibodies, Antinuclear; C538950:anticentromere antibody; D002248:Carbon Monoxide