Session Information
Session Type: Abstract Submissions (ACR)
Background/Purpose
Lower socioeconomic status (SES) and male gender have been associated with worse survival in idiopathic pulmonary arterial hypertension (PAH). Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) is a multicenter, prospective registry of systemic sclerosis (SSc) patients with pulmonary hypertension (PH) or at high risk for the development of PH. In this study we examined whether survival in patients with SSc-PH varied with gender, SES, or race.
Methods
276 patients with SSc and newly diagnosed PH were enrolled in the PHAROS registry between 2006 and 2014. We used level of education and employment status as surrogates for SES, although more than half of our patients were retired or medically disabled at the time of PH diagnosis. For employment status we analyzed working versus all other. For level of education, we analyzed 12th grade or less versus some college or more. Statistical analysis was performed using chi-square, univariate and multivariate Cox proportional hazard models.
Results
Baseline characteristics of the patients are described in Table 1. The following variables were examined as prognostic factors for survival: age, scleroderma subtype, disease duration, World Health Organization (WHO) PH Group classification, baseline mean pulmonary artery pressure (mPAP), gender, level of education, and employment status. The univariate and multivariate Cox Proportional Hazard Models are reported in Table 2. Male gender, diffuse subtype, and unemployment were associated with an increased risk of death. WHO group was not a significant predictor. Race was not found to be a significant prognostic factor. Lower level of education was a significant prognostic factor when evaluating the entire group; however, if the analysis is limited to WHO Group 1 PH subtype only, then lower level of education is associated with an increased risk of death with HR 2.1 (95% CI 1.2, 3.6), p<0.01 when corrected for age, SSc subtype and mPAP.
Conclusion
Male gender, lower SES, and diffuse SSc are associated with a higher risk of death in the PHAROS cohort. Lower level of education was a risk factor for death only in WHO Group 1 (PAH). Worse survival in males is seen also in idiopathic PAH despite the fact that PAH is more prevalent in females, and the explanation for this is a topic for additional study. Addressing health disparities associated with lower SES may improve the outcomes of patients with SSc-PH.
|
Table 1. Patient Characteristics |
|
|
Age – mean ± S.D. |
58.4 ± 11.0 |
|
Gender – (n = 275) Male – n (%) Female – n (%) |
52 (18.9) 223 (81.1) |
|
Scleroderma Subtype – (n = 276) Diffuse – n (%) Limited – n (%) |
90 (32.6) 171 (61.9) |
|
Duration of Disease from first non RP symptom– mean ± S.D. |
7.6 ± 8.2 |
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Race (n = 274) Caucasian – n (%) African-American – n (%) Other – n (%) |
201 (73.4) 45 (16.4) 28 (10.2) |
|
Education Status – (n = 239) 8th grade or less – n (%) 12th grade – n (%) Associate, technical or some college – n (%) College degree – n (%) Post-graduate degree – n (%) |
13 (5.4) 78 (32.6) 63 (26.3) 50 (20.9) 35 (14.6) |
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Employment Status – (n=218) Working – n (%) Homemaker – n (%) Student – n (%) Medically disabled – n (%) Retired – n (%) Unemployed – n (%) |
69 (31.7) 13 (6.0) 1 (0.5) 55 (25.2) 72 (33.0) 8 (3.7) |
|
WHO Group – (n = 276) 1 – PAH – n (%) 2 – PVH – n (%) 3 – PH-ILD – n (%) |
181 (65.6) 49 (17.8) 46 (16.7) |
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Mean Pulmonary Artery Pressure at Baseline – mean ± S.D. |
36.2 ± 10.2 |
Table 2
|
Factors |
HR |
95% CI |
p-value |
|
Univariate Analyses
|
|||
|
Male Gender |
1.948 |
1.152, 3.292 |
0.0128 |
|
Not working |
3.895 |
1.92, 7.902 |
<0.001 |
|
Education |
0.65 |
0.4, 1.063 |
0.0863 |
|
Education (WHO1 only) |
2.09 |
1.199, 3.641 |
<0.01 |
|
Diffuse scleroderma |
1.648 |
1.017, 2.671 |
0.043 |
|
African American Race |
0.925 |
0.456, 1.877 |
0.83 |
|
Mean PAP |
1.044 |
1.026, 1.062 |
<0.0001 |
|
Multivariate Analyses
|
|||
|
Age |
1.004 |
0.982, 1.027 |
.07275 |
|
Mean PAP |
1.044 |
1.023, 1.064 |
<0.0001 |
|
Male Gender |
2.348 |
1.368, 4.029 |
.002 |
|
Diffuse SSc |
2.034 |
1.202, 3.442 |
0.0016 |
|
Not working |
2.835 |
1.484, 5.415 |
0.0016 |
Disclosure:
J. K. Gordon,
None;
W. Zhang,
None;
L. Chung,
Gilead Science,
9;
Y. Ma,
None;
V. D. Steen,
Actelion Pharmaceuticals US,
8,
United Therapeutics,
5,
Gilead Science,
8,
Roche Pharmaceuticals,
2,
Sanofi-Aventis Pharmaceutical,
2,
CSL Berhing,
2,
Intermune,
2,
Bayer,
5.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/lower-socioeconomic-status-male-gender-and-diffuse-scleroderma-are-associated-with-worse-survival-in-the-pulmonary-hypertension-assessment-and-recognition-of-outcomes-in-scleroderma-cohort/