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FRI0399 Improved survival in patients with systemic sclerosis (SSC)-related pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) treated with aggressive use of pah therapy.
  1. E. Volkmann1,
  2. R. Saggar1,
  3. N. Borazan1,
  4. B. Torres1,
  5. H. Agrawal1,
  6. M. Flora1,
  7. R. Saggar2,
  8. P. Maranian1,
  9. D. E. Furst1
  1. 1Department of Medicine, University of California, Los Angeles, Los Angeles
  2. 2Heart and Lung Institute, St. Joseph Hospital and Medical Center, Phoenix, United States


Background The two leading causes of death in SSc are PAH and ILD. Historically, survival is worse in patients with combined PAH-ILD compared with isolated PAH (1), and there may not be any survival benefit with using PAH-medications in patients with PAH-ILD (2).

Objectives To investigate the effects of aggressive PAH therapy on survival in SSc-PAH patients with and without ILD, and to determine which factors predict mortality.

Methods All UCLA SSc patients who had a right heart catheterization diagnostic for PAH (mean pulmonary artery pressure (mPAP) ≥25 mm Hg, pulmonary capillary wedge pressure ≤15 mm Hg, pulmonary vascular resistance ≥240 dynesxsecond/cm5), between 2001-2012 were enrolled. ILD was defined as a forced vital capacity <70%. Kaplan-Meier and Cox proportional hazards models were used to analyze survival and to identify prognostic variables. Mortality was defined as death (due to any cause) or lung transplantation.

Results At baseline, patients with isolated PAH (n=41) and combined PAH-ILD (n=59) were similar for ethnicity, comorbidities, SSc type and duration, mean mPAP (PAH: 42; PAH-ILD: 39; p=NS). Patients with isolated PAH were older (mean 60 vs. 54 yrs in PAH-ILD, p=0.03), and had a greater percentage of women (93% vs. 68% in PAH-ILD, p=0.003). Mean follow up time was 30 and 27 months for the PAH and PAH-ILD groups, respectively, p=NS. There were no significant group differences in use of a systemic prostacyclin (PAH: 29%, PAH-ILD: 32%), use of a second PAH agent (61% in each group), or use of third PAH agent (PAH: 35%, PAH-ILD: 34%) during the study period. The 1-, 2-, 3-year survival estimates were 80%, 64%, 54%, and 74%, 64%, 56%, for the PAH and PAH-ILD groups, respectively, p=NS. In a multivariate model of the entire cohort (with the following covariates: mPAP, ILD, age, gender, ethnicity, body mass index, SSc type and duration, use of immunosuppression, hemoglobin T≤11 gm/dL), age was the only significant risk factor for mortality.

Conclusions Compared with historical series (1, 2), long-term survival in SSc patients with both isolated PAH and PAH-ILD has improved, perhaps partly due to aggressive use of PAH therapy.


  1. Condliffe R, et al. Am J Respir Crit Care Med 2009;179:151.

  2. Le Pavec J, et al. Arthritis Rheum 2011;63:2456.

Disclosure of Interest None Declared

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