Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry

The PHAROS Investigators

Research output: Contribution to journalArticle

Abstract

Objective: To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Methods: Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration. Results: In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37%) had AC, 39 (24%) NUC, 11 (7%) Scl-70, 28 (17%) had other, 9 (6%) RNA pol, 8 (5%) U1RNP autoantibodies, and 7 (4%) had negative antibodies; 32 (20%) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94% and 78% for AC, 94% and 72% for NUC, 89% and 63% for Scl-70, 92% and 79% for the other group, and 100% and 93% for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies. Conclusion: Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.

Original languageEnglish (US)
Pages (from-to)309-314
Number of pages6
JournalSeminars in Arthritis and Rheumatism
Volume45
Issue number3
DOIs
StatePublished - Dec 1 2015

Fingerprint

Systemic Scleroderma
Pulmonary Hypertension
Autoantibodies
Registries
Outcome Assessment (Health Care)
Survival
Serum
RNA Polymerase III
Social Security
Electronic Health Records
Kaplan-Meier Estimate
Cardiac Catheterization
Proportional Hazards Models
RNA

Keywords

  • Mortality
  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Risk factors
  • Scleroderma
  • Serum autoantibody
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine

Cite this

@article{2ab360a6934448f782dc4216534c161c,
title = "Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry",
abstract = "Objective: To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Methods: Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration. Results: In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37{\%}) had AC, 39 (24{\%}) NUC, 11 (7{\%}) Scl-70, 28 (17{\%}) had other, 9 (6{\%}) RNA pol, 8 (5{\%}) U1RNP autoantibodies, and 7 (4{\%}) had negative antibodies; 32 (20{\%}) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94{\%} and 78{\%} for AC, 94{\%} and 72{\%} for NUC, 89{\%} and 63{\%} for Scl-70, 92{\%} and 79{\%} for the other group, and 100{\%} and 93{\%} for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies. Conclusion: Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.",
keywords = "Mortality, Pulmonary arterial hypertension, Pulmonary hypertension, Risk factors, Scleroderma, Serum autoantibody, Systemic sclerosis",
author = "{The PHAROS Investigators} and Monique Hinchcliff and Saira Khanna and Hsu, {Vivien M.} and Jungwha Lee and Orit Almagor and Chang, {Rowland W.} and Virginia Steen and Lorinda Chung and Bolster, {Marcy B.} and Csuka, {Mary E.} and Derk, {Chris T.} and Domsic, {Robyn T.} and Aryeh Fischer and Tracy Frech and Daniel Furst and Goldberg, {Avram Z.} and Mardi Gomberg-Maitland and Gordon, {Jessica K.} and Laura Hummers and Firas Kassab and Molitor, {Jerry A.} and Ioana Preston and {Ann Saketkoo}, Lesley and Elena Schiopu and Rick Silver and Robert Simms and John Varga",
year = "2015",
month = "12",
day = "1",
doi = "10.1016/j.semarthrit.2015.06.011",
language = "English (US)",
volume = "45",
pages = "309--314",
journal = "Seminars in Arthritis and Rheumatism",
issn = "0049-0172",
publisher = "W.B. Saunders Ltd",
number = "3",

}

TY - JOUR

T1 - Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry

AU - The PHAROS Investigators

AU - Hinchcliff, Monique

AU - Khanna, Saira

AU - Hsu, Vivien M.

AU - Lee, Jungwha

AU - Almagor, Orit

AU - Chang, Rowland W.

AU - Steen, Virginia

AU - Chung, Lorinda

AU - Bolster, Marcy B.

AU - Csuka, Mary E.

AU - Derk, Chris T.

AU - Domsic, Robyn T.

AU - Fischer, Aryeh

AU - Frech, Tracy

AU - Furst, Daniel

AU - Goldberg, Avram Z.

AU - Gomberg-Maitland, Mardi

AU - Gordon, Jessica K.

AU - Hummers, Laura

AU - Kassab, Firas

AU - Molitor, Jerry A.

AU - Preston, Ioana

AU - Ann Saketkoo, Lesley

AU - Schiopu, Elena

AU - Silver, Rick

AU - Simms, Robert

AU - Varga, John

PY - 2015/12/1

Y1 - 2015/12/1

N2 - Objective: To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Methods: Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration. Results: In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37%) had AC, 39 (24%) NUC, 11 (7%) Scl-70, 28 (17%) had other, 9 (6%) RNA pol, 8 (5%) U1RNP autoantibodies, and 7 (4%) had negative antibodies; 32 (20%) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94% and 78% for AC, 94% and 72% for NUC, 89% and 63% for Scl-70, 92% and 79% for the other group, and 100% and 93% for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies. Conclusion: Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.

AB - Objective: To determine the association between serum autoantibodies and survival in patients with incident systemic sclerosis (SSc)-pulmonary arterial hypertension (PAH) enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Methods: Patients with definite PAH diagnosed by right heart catheterization within 6 months of registry enrollment were studied. Serum autoantibodies were assayed at each participating institution's clinical laboratory. Mortality data were collected from electronic medical records and/or the Social Security Death Index. Kaplan-Meier survival estimates were reported for five autoantibody groups (anticentromere/AC, nucleolar ANA/NUC, anti-topoisomerase/Scl-70, overlapping or non-specific autoantibodies/other, and a combined group with similar survival consisting of RNA polymerase III, U1RNP, and autoantibody-negative patients). Cox proportional hazards models permitted examination of the association between autoantibody groups and overall survival, controlling for age, sex, race, and SSc disease duration. Results: In all, 162 subjects had PAH, and serum autoantibody and survival information; 60 (37%) had AC, 39 (24%) NUC, 11 (7%) Scl-70, 28 (17%) had other, 9 (6%) RNA pol, 8 (5%) U1RNP autoantibodies, and 7 (4%) had negative antibodies; 32 (20%) subjects died over a median follow-up time of 2.1 years (range: 0.01-6.8); 1- and 3-year survival estimates were, respectively, 94% and 78% for AC, 94% and 72% for NUC, 89% and 63% for Scl-70, 92% and 79% for the other group, and 100% and 93% for the combined group. Unadjusted and adjusted hazard ratios revealed no statistically significant association between risk of death and autoantibodies. Conclusion: Anticentromere and NUC autoantibodies are prevalent in SSc-PAH patients. An association between serum autoantibodies and survival in patients with SSc-PAH was not identified in the PHAROS cohort.

KW - Mortality

KW - Pulmonary arterial hypertension

KW - Pulmonary hypertension

KW - Risk factors

KW - Scleroderma

KW - Serum autoantibody

KW - Systemic sclerosis

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U2 - 10.1016/j.semarthrit.2015.06.011

DO - 10.1016/j.semarthrit.2015.06.011

M3 - Article

C2 - 26210782

AN - SCOPUS:84955693022

VL - 45

SP - 309

EP - 314

JO - Seminars in Arthritis and Rheumatism

JF - Seminars in Arthritis and Rheumatism

SN - 0049-0172

IS - 3

ER -