Article Text

Download PDFPDF
Extended report
Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry
  1. Sangmee Bae1,
  2. Rajeev Saggar2,
  3. Marcy B Bolster3,
  4. Lorinda Chung4,
  5. Mary Ellen Csuka5,
  6. Chris Derk6,
  7. Robyn Domsic7,
  8. Aryeh Fischer8,
  9. Tracy Frech9,
  10. Avram Goldberg10,
  11. Monique Hinchcliff11,
  12. Vivien Hsu12,
  13. Laura Hummers13,
  14. Elena Schiopu14,
  15. Maureen D Mayes15,
  16. Vallerie McLaughlin14,
  17. Jerry Molitor16,
  18. Nausheen Naz17,
  19. Daniel E Furst1,
  20. Paul Maranian1,
  21. Virginia Steen18,
  22. Dinesh Khanna14
  1. 1Department of Internal Medicine, University of California, Los Angeles, California, USA
  2. 2Department of Internal Medicine, St Joseph Hospital and Medical Center, Phoenix, Arizona, USA
  3. 3Department of Internal Medicine, Medical University of South Carolina, Charleston, South Carolina, USA
  4. 4Department of Internal Medicine, Stanford University, Palo Alto, California, USA
  5. 5Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
  6. 6Department of Internal Medicine, Thomas Jefferson University, Philadelphia, Pennsylvania, USA
  7. 7Department of Internal Medicine, University of Pittsburgh, Pittsburgh, Pennsylvania, USA
  8. 8Department of Internal Medicine, National Jewish Health, Denver, Colorado, USA
  9. 9Department of Internal Medicine, University of Utah, Salt Lake City, Utah, USA
  10. 10Department of Internal Medicine, North Shore–Long Island Jewish Medical Center, New Hyde Park, New York, USA
  11. 11Department of Internal Medicine, Northwestern University, Chicago, Illinois, USA
  12. 12Department of Internal Medicine, University of Medicine and Dentistry, Rutgers, New Jersey, USA
  13. 13Medicine/Rheumatology, Johns Hopkins University, Baltimore, Maryland, USA
  14. 14Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA
  15. 15Department of Rheumatology and Clinical Immunogenetics, University of Texas Medical School at Houston, Houston, Texas, USA
  16. 16Department of Internal Medicine, University of Minnesota, Minneapolis, Minnesota, USA
  17. 17Department of Internal Medicine, University of Massachusetts, Worcester, Massachusetts, USA
  18. 18Georgetown University Medical Center, Washington, DC, USA
  1. Correspondence to Dinesh Khanna, University of Michigan Scleroderma Programme, Division of Rheumatology/Department of Internal Medicine, 24 Frank Lloyd Wright Drive, Lobby M, Suite 2500, SPC 5753, Ann Arbor, Michigan 48106, USA; khannad{at}


Background Patients with normal (mean pulmonary arterial pressure (mPAP) ≤20 mm Hg) and borderline mean pulmonary pressures (21–24 mm Hg) are “at risk” of developing pulmonary hypertension (PH). The objectives of this analysis were to examine the baseline characteristics in systemic sclerosis (SSc) with normal and borderline mPAP and to explore long-term outcomes in SSc patients with borderline mPAP versus normal haemodynamics.

Methods PHAROS is a multicentre prospective longitudinal cohort of patients with SSc “at risk” or recently diagnosed with resting PH on right heart catheterisation (RHC). Baseline clinical characteristics, pulmonary function tests, high-resolution CT, 2-dimensional echocardiogram and RHC results were analysed in normal and borderline mPAP groups.

Results 206 patients underwent RHC (results showed 35 normal, 28 borderline mPAP, 143 resting PH). There were no differences in the baseline demographics. Patients in the borderline mPAP group were more likely to have restrictive lung disease (67% vs 30%), fibrosis on high-resolution CT and a higher estimated right ventricular systolic pressure on echocardiogram (46.3 vs 36.2 mm Hg; p<0.05) than patients with normal haemodynamics. RHC revealed higher pulmonary vascular resistance and more elevated mPAP on exercise (≥30; 88% vs 56%) in the borderline mPAP group (p<0.05 for both). Patients were followed for a mean of 25.7 months and 24 patients had a repeat RHC during this period. During follow-up, 55% of the borderline mPAP group and 32% of the normal group developed resting PH (p=NS).

Conclusions Patients with borderline mPAP have a greater prevalence of abnormal lung physiology, pulmonary fibrosis and the presence of exercise mPAP ≥30 mm Hg.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Funding The PHAROS registry is funded by unrestricted grant from Actelion Inc and Gilead Inc and the Scleroderma Foundation. DK was supported by a National Institutes of Health award (NIAMS K23 AR053858-05).

  • Ethics approval The study received ethics approval from the review board at each institution.

  • Patient consent Obtained.

  • Competing interests SB, RS, MBB, RD, AG, MH, VH, JM, NN and PM have no conflicts of interest to declare; LC reports being involved in clinical trials with Gilead, United Therapeutics and Pfizer; MEC reports serving on the advisory board meeting on PAH and rheumatology by Gilead; CD has received research grants from Aspreva and Gilead; AF reports being a speaker at Actelion and Gilead, serving as a consultant, advisory board member at Actelion; LH reports research support from Actelion and Medimmune; MDM reports consulting, speakers bureaux and/or received grant/research support from Actelion, Gilead, United Therapeutics and Novartis; VMcL reports receiving research grants from Actelion, Novartis, United Therapeutics, acting as a consultant for Actelion, BMS, Mondo Biotech, Gilead, United Therapeutics and as a speaker's bureau for Actelion, Gilead and United Therapeutics; DEF reports honoraria, speaking, receiving research grants, and serving on the advisory board of Actelion and Gilead, grants and advisory board of Pfizer; VS reports research grants from United Therapeutics, Pfizer, Gilead, Actelion, Bristol Myers Squibb and Sibley foundation, acting as a consultant to United Therapeutics and Gilead, and as a speaker at Gilead and Actelion; DK reports serving on the advisory board of Actelion and Pfizer and received grants from Actelion and Gilead.

  • Provenance and peer review Not commissioned; externally peer reviewed.