Risk factors for mortality and cardiopulmonary hospitalization in systemic sclerosis patients at risk for pulmonary hypertension, in the pharos registry

Vivien M. Hsu, Lorinda Chung, Laura K. Hummers, Ami Shah, Robert Simms, Marcy Bolster, Faye N. Hant, Richard M. Silver, Aryeh Fischer, Monique E. Hinchcliff, John Varga, Avram Z. Goldberg, Chris T. Derk, Elena Schiopu, Dinesh Khanna, Lee S. Shapiro, Robyn T. Domsic, Thomas Medsger, Maureen D. Mayes, Daniel FurstMary Ellen Csuka, Jerry A. Molitor, Lesley Ann Saketkoo, Christian R. Salazar, Virginia D. Steen

Research output: Contribution to journalArticlepeer-review

24 Scopus citations


OBJECTIVE: We sought to identify predictors of mortality and cardiopulmonary hospitalizations in patients at risk for pulmonary hypertension (PH) and enrolled in PHAROS, a prospective cohort study to investigate the natural history of PH in systemic sclerosis (SSc).

METHODS: The at-risk population for PH was defined by the following entry criteria: echocardiogram systolic pulmonary arterial pressure > 40 mmHg, or DLCO < 55% predicted or ratio of % forced vital capacity/%DLCO > 1.6, measured by pulmonary function testing. Baseline clinical measures were evaluated as predictors of hospitalization and death between 2005 and 2014. Cox proportional hazards models were censored at date of PH onset or latest study visit and adjusted for age, sex, race, and disease duration.

RESULTS: Of the 236 at-risk subjects who were followed for a median of 4 years (range 0.4-8.5 yrs), 35 developed PH after entering PHAROS (reclassified as PH group). In the at-risk group, higher mortality was strongly associated with male sex, low %DLCO, exercise oxygen desaturation, anemia, abnormal dyspnea scores, and baseline pericardial effusion. Risks for cardiopulmonary hospitalization were associated with increased dyspnea and pericardial effusions, although PH patients with DLCO < 50% had the highest risk of cardiopulmonary hospitalizations.

CONCLUSION: Risk factors for poor outcome in patients with SSc who are at risk for PH were similar to others with SSc-PH and SSc-pulmonary arterial hypertension, including male sex, DLCO < 50%, exercise oxygen desaturation, and pericardial effusions. This group should undergo right heart catheterization and receive appropriate intervention if PH is confirmed.

Original languageEnglish (US)
Pages (from-to)176-183
Number of pages8
JournalJournal of Rheumatology
Issue number2
StatePublished - Feb 1 2019

Bibliographical note

Publisher Copyright:
© 2019 The Journal of Rheumatology.


  • Systemic sclerosis
  • outcomes
  • pulmonary hypertension
  • risk factors

PubMed: MeSH publication types

  • Research Support, Non-U.S. Gov't
  • Journal Article


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