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 Furst; Mary Ellen Csuka; Jerry A Molitor; Lesley Ann Saketkoo; Christian R Salazar; Virginia D Steen

doi:10.3899/jrheum.180018

Risk Factors for Mortality and Cardiopulmonary Hospitalization in Systemic Sclerosis Patients At Risk for Pulmonary Hypertension, in the PHAROS Registry

J Rheumatol. 2019 Feb;46(2):176-183. doi: 10.3899/jrheum.180018. Epub 2018 Oct 1.

Authors

Vivien M Hsu^{1

2}, Lorinda Chung^{3

4}, Laura K Hummers^{3

4}, Ami Shah^{3

4}, Robert Simms^{3

4}, Marcy Bolster^{3

4}, Faye N Hant^{3

4}, Richard M Silver^{3

4}, Aryeh Fischer^{3

4}, Monique E Hinchcliff^{3

4}, John Varga^{3

4}, Avram Z Goldberg^{3

4}, Chris T Derk^{3

4}, Elena Schiopu^{3

4}, Dinesh Khanna^{3

4}, Lee S Shapiro^{3

4}, Robyn T Domsic^{3

4}, Thomas Medsger^{3

4}, Maureen D Mayes^{3

4}, Daniel Furst^{3

4}, Mary Ellen Csuka^{3

4}, Jerry A Molitor^{3

4}, Lesley Ann Saketkoo^{3

4}, Christian R Salazar^{3

4}, Virginia D Steen^{3

4}

Affiliations

¹ From the Department of Medicine, Division of Rheumatology, Rutgers-Robert Wood Johnson Medical School, New Brunswick, New Jersey; Department of Medicine, Division of Immunology and Rheumatology, Stanford University, Stanford, California; Department of Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; Department of Medicine, Division of Rheumatology, Boston University, Boston; Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, Massachusetts; Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; Department of Medicine, Division of Rheumatology, and Pulmonary Sciences and Critical Care Medicine, University of Colorado, Denver, Colorado; Department of Medicine, Division of Rheumatology, Northwestern University, Chicago, Illinois; Department of Medicine, Division of Rheumatology, New York University Medical Center, New York, New York; Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, Michigan; Department of Medicine, Division of Rheumatology, Albany Medical College, Albany, New York; Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, Pennsylvania; Department of Medicine, Division of Rheumatology, University of Texas, Houston, Texas; Department of Medicine, Division of Rheumatology, University of California at Los Angeles, Los Angeles, California; Department of Medicine, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, Wisconsin; Department of Medicine, Division of Rheumatology, University of Minnesota, Minneapolis, Minnesota; Department of Medicine, Divisions of Pulmonary and Rheumatology, Tulane University, New Orleans, Louisiana; Department of Epidemiology and Health Promotion, New York University, New York, New York; Department of Medicine, Division of Rheumatology, Immunology and Allergy, Georgetown University, Washington, D.C., USA. [email protected].
² V.M. Hsu, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Rutgers-Robert Wood Johnson Medical School; L. Chung, MD, MS, Associate Professor of Medicine and Dermatology, Department of Medicine, Division of Immunology and Rheumatology, Stanford University; L.K. Hummers, MD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, Johns Hopkins University; A. Shah, MD, Assistant Professor of Medicine, Department of Medicine, Division of Rheumatology, Johns Hopkins University; R. Simms, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Boston University; M. Bolster, MD, Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital; F.N. Hant, DO, MSCR, Associate Professor, Department of Medicine, Division of Rheumatology, Medical University of South Carolina; R.M. Silver, MD, Department of Medicine, Division of Rheumatology, Medical University of South Carolina; A. Fischer, MD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, and Pulmonary Sciences and Critical Care Medicine, University of Colorado; M.E. Hinchcliff, MD, MS, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, Northwestern University; J. Varga, MD, Professor of Medicine, Dermatology and Pharmacology, Department of Medicine, Division of Rheumatology, Northwestern University; A.Z. Goldberg, MD, Clinical Assistant Professor of Medicine, Department of Medicine, Division of Rheumatology, New York University Medical Center; C.T. Derk, MD, MS, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pennsylvania; E. Schiopu, MD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Michigan; D. Khanna, MD, MBBS, MSc, Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Michigan; L.S. Shapiro, MD, Clinical Professor of Medicine, Department of Medicine, Division of Rheumatology, Albany Medical College; R.T. Domsic, MD, MPH, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pittsburgh; T. Medsger, MD, Gerald P. Rodnan Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pittsburgh; M.D. Mayes, MD, MPH, Professor of Medicine, Rheumatology and Immunogenetics, Department of Medicine, Division of Rheumatology, University of Texas; D. Furst, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, University of California at Los Angeles; M.E. Csuka, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Medical College of Wisconsin; J.A. Molitor, MD, PhD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Minnesota; L.A. Saketkoo, MD, MPH, Associate Professor of Medicine, Department of Medicine, Divisions of Pulmonary and Rheumatology, Tulane University; C.R. Salazar, PhD, MPH, Adjunct Assistant Professor, Department of Epidemiology and Health Promotion, New York University; V.D. Steen, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Immunology and Allergy, Georgetown University. [email protected].
³ From the Department of Medicine, Division of Rheumatology, Rutgers-Robert Wood Johnson Medical School, New Brunswick, New Jersey; Department of Medicine, Division of Immunology and Rheumatology, Stanford University, Stanford, California; Department of Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; Department of Medicine, Division of Rheumatology, Boston University, Boston; Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital, Boston, Massachusetts; Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; Department of Medicine, Division of Rheumatology, and Pulmonary Sciences and Critical Care Medicine, University of Colorado, Denver, Colorado; Department of Medicine, Division of Rheumatology, Northwestern University, Chicago, Illinois; Department of Medicine, Division of Rheumatology, New York University Medical Center, New York, New York; Department of Medicine, Division of Rheumatology, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Medicine, Division of Rheumatology, University of Michigan, Ann Arbor, Michigan; Department of Medicine, Division of Rheumatology, Albany Medical College, Albany, New York; Department of Medicine, Division of Rheumatology, University of Pittsburgh, Pittsburgh, Pennsylvania; Department of Medicine, Division of Rheumatology, University of Texas, Houston, Texas; Department of Medicine, Division of Rheumatology, University of California at Los Angeles, Los Angeles, California; Department of Medicine, Division of Rheumatology, Medical College of Wisconsin, Milwaukee, Wisconsin; Department of Medicine, Division of Rheumatology, University of Minnesota, Minneapolis, Minnesota; Department of Medicine, Divisions of Pulmonary and Rheumatology, Tulane University, New Orleans, Louisiana; Department of Epidemiology and Health Promotion, New York University, New York, New York; Department of Medicine, Division of Rheumatology, Immunology and Allergy, Georgetown University, Washington, D.C., USA.
⁴ V.M. Hsu, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Rutgers-Robert Wood Johnson Medical School; L. Chung, MD, MS, Associate Professor of Medicine and Dermatology, Department of Medicine, Division of Immunology and Rheumatology, Stanford University; L.K. Hummers, MD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, Johns Hopkins University; A. Shah, MD, Assistant Professor of Medicine, Department of Medicine, Division of Rheumatology, Johns Hopkins University; R. Simms, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Boston University; M. Bolster, MD, Department of Medicine, Division of Rheumatology, Allergy and Immunology, Massachusetts General Hospital; F.N. Hant, DO, MSCR, Associate Professor, Department of Medicine, Division of Rheumatology, Medical University of South Carolina; R.M. Silver, MD, Department of Medicine, Division of Rheumatology, Medical University of South Carolina; A. Fischer, MD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, and Pulmonary Sciences and Critical Care Medicine, University of Colorado; M.E. Hinchcliff, MD, MS, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, Northwestern University; J. Varga, MD, Professor of Medicine, Dermatology and Pharmacology, Department of Medicine, Division of Rheumatology, Northwestern University; A.Z. Goldberg, MD, Clinical Assistant Professor of Medicine, Department of Medicine, Division of Rheumatology, New York University Medical Center; C.T. Derk, MD, MS, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pennsylvania; E. Schiopu, MD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Michigan; D. Khanna, MD, MBBS, MSc, Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Michigan; L.S. Shapiro, MD, Clinical Professor of Medicine, Department of Medicine, Division of Rheumatology, Albany Medical College; R.T. Domsic, MD, MPH, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pittsburgh; T. Medsger, MD, Gerald P. Rodnan Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Pittsburgh; M.D. Mayes, MD, MPH, Professor of Medicine, Rheumatology and Immunogenetics, Department of Medicine, Division of Rheumatology, University of Texas; D. Furst, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, University of California at Los Angeles; M.E. Csuka, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Medical College of Wisconsin; J.A. Molitor, MD, PhD, Associate Professor of Medicine, Department of Medicine, Division of Rheumatology, University of Minnesota; L.A. Saketkoo, MD, MPH, Associate Professor of Medicine, Department of Medicine, Divisions of Pulmonary and Rheumatology, Tulane University; C.R. Salazar, PhD, MPH, Adjunct Assistant Professor, Department of Epidemiology and Health Promotion, New York University; V.D. Steen, MD, Professor of Medicine, Department of Medicine, Division of Rheumatology, Immunology and Allergy, Georgetown University.

PMID: 30275260
DOI: 10.3899/jrheum.180018

Abstract

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.

Keywords: OUTCOMES; PULMONARY HYPERTENSION; RISK FACTORS; SYSTEMIC SCLEROSIS.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Aged
Arterial Pressure
Echocardiography
Female
Follow-Up Studies
Hospitalization*
Humans
Hypertension, Pulmonary / diagnostic imaging
Hypertension, Pulmonary / etiology*
Hypertension, Pulmonary / mortality*
Male
Middle Aged
Proportional Hazards Models
Prospective Studies
Registries*
Risk Factors
Scleroderma, Systemic / complications*
Sex Factors
Survival Rate
Vital Capacity