Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study

Vivien M. Hsu, Lorinda Chung, Laura Hummers, Fredrick Wigley, Robert Simms, Marcy Bolster, Rick 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 Furst, Mary E. Csuka & 3 others Jerry A. Molitor, Firas Alkassab, Virginia D. Steen

Research output: Contribution to journalArticle

Abstract

Objectives: PHAROS registry is a prospective longitudinal cohort study to understand the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). Methods: "At-risk" pulmonary arterial hypertension (PAH) is defined by these entry criteria: echocardiogram (echo) systolic pulmonary arterial pressure (sPAP) >40. mmHg, diffusion lung capacity of carbon monoxide (DLco) 1.6, as measured by pulmonary function testing (PFT). Patients were followed up annually and right heart catheterization (RHC) performed if PH was suspected. We used descriptive statistics and Kaplan-Meier estimate of time to PH diagnosis. Results: A total of 251 "at-risk" subjects were enrolled between 2005 and 2012 and followed up for mean of 2.5 ± 1.2 years. The mean age at entry was 56.7 ± 11.0 and disease duration was 9.9 ± 8.7 years.Overall, 82 patients had RHC, and 35 were confirmed to have new PH. There were no differences in age, gender, SSc subtypes, antibodies, and disease duration between the "at-risk" and new PH groups. Using Kaplan-Meier survival, the time to PH was 10% at 2 years, 13% at 3 years, and 25% at 5 years. Most new PH patients at entry met the PFT criteria (76%), had significantly higher sPAP (p = 0.013), had shorter 6-min walk distance, and had exercise-induced hypoxia (p = 0.003) than "at-risk" PAH group. Conclusions: A low DLco, high FVC/DLco, exercise-induced hypoxia and entry echo sPAP > 40 were strongly associated with future PH, though RHC was necessary to confirm PH. This ongoing prospective study confirms that these high-risk factors do predict future PH.

Original languageEnglish (US)
Pages (from-to)55-62
Number of pages8
JournalSeminars in Arthritis and Rheumatism
Volume44
Issue number1
DOIs
StatePublished - 2014

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Systemic Scleroderma
Pulmonary Hypertension
Cohort Studies
Outcome Assessment (Health Care)
Population
Lung
Cardiac Catheterization
Arterial Pressure
Exercise
Lung Volume Measurements
Kaplan-Meier Estimate
Patient Rights
Carbon Monoxide
Longitudinal Studies
Registries

Keywords

  • Pulmonary arterial hypertension
  • Registry
  • Scleroderma
  • Systemic

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine
  • Medicine(all)

Cite this

Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. / Hsu, Vivien M.; Chung, Lorinda; Hummers, Laura; Wigley, Fredrick; Simms, Robert; Bolster, Marcy; Silver, Rick; Fischer, Aryeh; Hinchcliff, Monique E.; Varga, John; Goldberg, Avram Z.; Derk, Chris T.; Schiopu, Elena; Khanna, Dinesh; Shapiro, Lee S.; Domsic, Robyn T.; Medsger, Thomas; Mayes, Maureen D.; Furst, Daniel; Csuka, Mary E.; Molitor, Jerry A.; Alkassab, Firas; Steen, Virginia D.

In: Seminars in Arthritis and Rheumatism, Vol. 44, No. 1, 2014, p. 55-62.

Research output: Contribution to journalArticle

Hsu, VM, Chung, L, Hummers, L, Wigley, F, Simms, R, Bolster, M, Silver, R, Fischer, A, Hinchcliff, ME, Varga, J, Goldberg, AZ, Derk, CT, Schiopu, E, Khanna, D, Shapiro, LS, Domsic, RT, Medsger, T, Mayes, MD, Furst, D, Csuka, ME, Molitor, JA, Alkassab, F & Steen, VD 2014, 'Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study', Seminars in Arthritis and Rheumatism, vol. 44, no. 1, pp. 55-62. https://doi.org/10.1016/j.semarthrit.2014.03.002
Hsu, Vivien M. ; Chung, Lorinda ; Hummers, Laura ; Wigley, Fredrick ; Simms, Robert ; Bolster, Marcy ; Silver, Rick ; Fischer, Aryeh ; Hinchcliff, Monique E. ; Varga, John ; Goldberg, Avram Z. ; Derk, Chris T. ; Schiopu, Elena ; Khanna, Dinesh ; Shapiro, Lee S. ; Domsic, Robyn T. ; Medsger, Thomas ; Mayes, Maureen D. ; Furst, Daniel ; Csuka, Mary E. ; Molitor, Jerry A. ; Alkassab, Firas ; Steen, Virginia D. / Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study. In: Seminars in Arthritis and Rheumatism. 2014 ; Vol. 44, No. 1. pp. 55-62.
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abstract = "Objectives: PHAROS registry is a prospective longitudinal cohort study to understand the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). Methods: {"}At-risk{"} pulmonary arterial hypertension (PAH) is defined by these entry criteria: echocardiogram (echo) systolic pulmonary arterial pressure (sPAP) >40. mmHg, diffusion lung capacity of carbon monoxide (DLco) 1.6, as measured by pulmonary function testing (PFT). Patients were followed up annually and right heart catheterization (RHC) performed if PH was suspected. We used descriptive statistics and Kaplan-Meier estimate of time to PH diagnosis. Results: A total of 251 {"}at-risk{"} subjects were enrolled between 2005 and 2012 and followed up for mean of 2.5 ± 1.2 years. The mean age at entry was 56.7 ± 11.0 and disease duration was 9.9 ± 8.7 years.Overall, 82 patients had RHC, and 35 were confirmed to have new PH. There were no differences in age, gender, SSc subtypes, antibodies, and disease duration between the {"}at-risk{"} and new PH groups. Using Kaplan-Meier survival, the time to PH was 10{\%} at 2 years, 13{\%} at 3 years, and 25{\%} at 5 years. Most new PH patients at entry met the PFT criteria (76{\%}), had significantly higher sPAP (p = 0.013), had shorter 6-min walk distance, and had exercise-induced hypoxia (p = 0.003) than {"}at-risk{"} PAH group. Conclusions: A low DLco, high FVC/DLco, exercise-induced hypoxia and entry echo sPAP > 40 were strongly associated with future PH, though RHC was necessary to confirm PH. This ongoing prospective study confirms that these high-risk factors do predict future PH.",
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author = "Hsu, {Vivien M.} and Lorinda Chung and Laura Hummers and Fredrick Wigley and Robert Simms and Marcy Bolster and Rick Silver and Aryeh Fischer and Hinchcliff, {Monique E.} and John Varga and Goldberg, {Avram Z.} and Derk, {Chris T.} and Elena Schiopu and Dinesh Khanna and Shapiro, {Lee S.} and Domsic, {Robyn T.} and Thomas Medsger and Mayes, {Maureen D.} and Daniel Furst and Csuka, {Mary E.} and Molitor, {Jerry A.} and Firas Alkassab and Steen, {Virginia D.}",
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T1 - Development of pulmonary hypertension in a high-risk population with systemic sclerosis in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) cohort study

AU - Hsu, Vivien M.

AU - Chung, Lorinda

AU - Hummers, Laura

AU - Wigley, Fredrick

AU - Simms, Robert

AU - Bolster, Marcy

AU - Silver, Rick

AU - Fischer, Aryeh

AU - Hinchcliff, Monique E.

AU - Varga, John

AU - Goldberg, Avram Z.

AU - Derk, Chris T.

AU - Schiopu, Elena

AU - Khanna, Dinesh

AU - Shapiro, Lee S.

AU - Domsic, Robyn T.

AU - Medsger, Thomas

AU - Mayes, Maureen D.

AU - Furst, Daniel

AU - Csuka, Mary E.

AU - Molitor, Jerry A.

AU - Alkassab, Firas

AU - Steen, Virginia D.

PY - 2014

Y1 - 2014

N2 - Objectives: PHAROS registry is a prospective longitudinal cohort study to understand the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). Methods: "At-risk" pulmonary arterial hypertension (PAH) is defined by these entry criteria: echocardiogram (echo) systolic pulmonary arterial pressure (sPAP) >40. mmHg, diffusion lung capacity of carbon monoxide (DLco) 1.6, as measured by pulmonary function testing (PFT). Patients were followed up annually and right heart catheterization (RHC) performed if PH was suspected. We used descriptive statistics and Kaplan-Meier estimate of time to PH diagnosis. Results: A total of 251 "at-risk" subjects were enrolled between 2005 and 2012 and followed up for mean of 2.5 ± 1.2 years. The mean age at entry was 56.7 ± 11.0 and disease duration was 9.9 ± 8.7 years.Overall, 82 patients had RHC, and 35 were confirmed to have new PH. There were no differences in age, gender, SSc subtypes, antibodies, and disease duration between the "at-risk" and new PH groups. Using Kaplan-Meier survival, the time to PH was 10% at 2 years, 13% at 3 years, and 25% at 5 years. Most new PH patients at entry met the PFT criteria (76%), had significantly higher sPAP (p = 0.013), had shorter 6-min walk distance, and had exercise-induced hypoxia (p = 0.003) than "at-risk" PAH group. Conclusions: A low DLco, high FVC/DLco, exercise-induced hypoxia and entry echo sPAP > 40 were strongly associated with future PH, though RHC was necessary to confirm PH. This ongoing prospective study confirms that these high-risk factors do predict future PH.

AB - Objectives: PHAROS registry is a prospective longitudinal cohort study to understand the natural history of pulmonary hypertension (PH) in systemic sclerosis (SSc). Methods: "At-risk" pulmonary arterial hypertension (PAH) is defined by these entry criteria: echocardiogram (echo) systolic pulmonary arterial pressure (sPAP) >40. mmHg, diffusion lung capacity of carbon monoxide (DLco) 1.6, as measured by pulmonary function testing (PFT). Patients were followed up annually and right heart catheterization (RHC) performed if PH was suspected. We used descriptive statistics and Kaplan-Meier estimate of time to PH diagnosis. Results: A total of 251 "at-risk" subjects were enrolled between 2005 and 2012 and followed up for mean of 2.5 ± 1.2 years. The mean age at entry was 56.7 ± 11.0 and disease duration was 9.9 ± 8.7 years.Overall, 82 patients had RHC, and 35 were confirmed to have new PH. There were no differences in age, gender, SSc subtypes, antibodies, and disease duration between the "at-risk" and new PH groups. Using Kaplan-Meier survival, the time to PH was 10% at 2 years, 13% at 3 years, and 25% at 5 years. Most new PH patients at entry met the PFT criteria (76%), had significantly higher sPAP (p = 0.013), had shorter 6-min walk distance, and had exercise-induced hypoxia (p = 0.003) than "at-risk" PAH group. Conclusions: A low DLco, high FVC/DLco, exercise-induced hypoxia and entry echo sPAP > 40 were strongly associated with future PH, though RHC was necessary to confirm PH. This ongoing prospective study confirms that these high-risk factors do predict future PH.

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