Clinical Edge

Summaries of Must-Read Clinical Literature, Guidelines, and FDA Actions

Patients with SSc and Pulmonary Hypertension Risk

J Rheumatol; ePub 2018 Oct 1; Hsu, Chung, et al

Risk factors for poor outcome in patients with systemic sclerosis (SSc) who are at risk for pulmonary hypertension (PH) were similar to others with SSc-PH and SSc-pulmonary arterial hypertension, a recent study found. Other risk factors were male sex, diffusing capacity of the lungs for carbon monoxide (DLCO)< 50%, exercise oxygen desaturation, and pericardial effusions. This according to researchers who sought to identify predictors of mortality and cardiopulmonary hospitalizations in patients at risk for PH and enrolled in PHAROS, a recent prospective cohort study to investigate the natural history of PH in SSc. They found:

  • Of the 236 at-risk subjects who were followed for a median of 4 years, 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.


Hsu VM, Chung L, Hummers LK, et al. Risk factors for mortality and cardiopulmonary hospitalization in systemic sclerosis patients at risk for pulmonary hypertension, in the PHAROS Registry. [Published online ahead of print October 1, 2018]. J Rheumatol. doi:10.3899/jrheum.180018.

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