Session Information
Date: Tuesday, November 7, 2017
Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster III
Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Survival in scleroderma associated pulmonary arterial hypertension (SSc-PAH) is known to be significantly worse compared to that in idiopathic pulmonary arterial hypertension (iPAH). However, specific factors contributing to the worse survival in SSc-PAH is currently unknown. Our objective was to investigate how the age at diagnosis of PAH and systolic blood pressure may influence mortality in these patients.
Methods: A retrospective chart-review was conducted comparing demographic, clinical and hemodynamic data from a total of 862 patients (686 with iPAH and 176 with SSc-PAH) enrolled in the Cleveland Clinic Pulmonary Hypertension Database. The diagnosis of PAH was confirmed by RHC, and the diagnosis of SSc was confirmed by a rheumatologist. We focused on the characteristics of age, systolic blood pressure (SBP) and cardiac output/cardiac index (on right heart catheterization) with regards to their effect on mortality in both the SSc-PAH and the iPAH groups.
Results: Among younger patients (age < 63) those with SSc-PAH had significantly worse survival compared to those with iPAH. Interestingly, a similar difference in mortality between the 2 groups was not seen in patients of age ≥ 63 years.
|
PAH Status Group |
Age |
P-value* |
N |
Est. Median Time-to-Death (years) |
95% CI |
|
Idiopathic PAH |
Any non-missing |
0.0002 |
598 |
10.26 |
(8.45, 12.00) |
|
Scleroderma Associated |
Any non-missing |
159 |
5.33 |
(4.22, 7.59) |
|
|
Idiopathic PAH |
< 63 |
0.0087 |
439 |
14.41 |
(10.81, 16.49) |
|
Scleroderma Associated |
< 63 |
76 |
6.74 |
(4.22, 20.72) |
|
|
Idiopathic PAH |
≥ 63 |
0.9752 |
159 |
5.11 |
(4.58, 6.62) |
|
Scleroderma Associated |
≥ 63 |
83 |
4.75 |
(3.78, 7.59) |
|
|
Scleroderma Associated |
< 63 |
0.1061 |
76 |
6.74 |
(4.22, 20.72) |
|
Scleroderma Associated |
≥ 63 |
83 |
4.75 |
(3.78, 7.59) |
|
|
Idiopathic PAH |
< 63 |
<0.0001 |
439 |
14.41 |
(10.81, 16.49) |
|
Idiopathic PAH |
≥ 63 |
159 |
5.11 |
(4.58, 6.62) |
A statistically significant difference in survival was also noted when comparing SSc-PAH patients with SBP >125 mmHg with those with SBP < 125 mmHg; the group with lower SBP had worse survival. A similar mortality difference was not noted in the iPAH patients.
|
PAH Status Group |
Systolic Blood Pressure |
P-value* |
N |
Est. Median Time-to-Death (years) |
95% CI |
|
Idiopathic PAH |
Any non-missing |
0.0007 |
559 |
10.02 |
(8.24, 11.84) |
|
Scleroderma Associated |
Any non-missing |
151 |
5.33 |
(4.24, 7.68) |
|
|
Idiopathic PAH |
< 125 |
< 0.0001 |
277 |
9.48 |
(6.98, 12.31) |
|
Scleroderma Associated |
< 125 |
63 |
4.01 |
(2.51, 6.03) |
|
|
Idiopathic PAH |
≥ 125 |
0.2313 |
282 |
10.26 |
(8.23, 12.75) |
|
Scleroderma Associated |
≥ 125 |
88 |
7.68 |
(4.70, 18.60) |
|
|
Scleroderma Associated |
< 125 |
0.0099 |
63 |
4.01 |
(2.51, 6.03) |
|
Scleroderma Associated |
≥ 125 |
88 |
7.68 |
(4.70, 18.59) |
|
|
Idiopathic PAH |
< 125 |
0.8082 |
277 |
9.48 |
(6.98, 12.31) |
|
Idiopathic PAH |
≥ 125 |
282 |
10.26 |
(8.23, 12.75) |
To determine if SBP served as a surrogate for cardiac output, we assessed Kendall tau-b correlations of SBP versus cardiac output and cardiac index (CI) (both by thermodilution and by the Fick equation); no strong correlation was noted. However, a correlation between CI < 2.5 L/min/m2 and mortality was noted. A similar mortality difference was not noted in the iPAH patients.
|
PAH Status Group |
Cardiac Index (FICK) [L/min/m2] |
P-value* |
N |
Est. Median Time-to-Death (years) |
95% CI |
|
Idiopathic PAH |
Any non-missing |
0.0126 |
470 |
9.38 |
(7.72, 11.50) |
|
Scleroderma Associated |
Any non-missing |
142 |
5.59 |
(4.24, 8.19) |
|
|
Idiopathic PAH |
< 2.5 |
0.0003 |
261 |
9.38 |
(7.51, 14.41) |
|
Scleroderma Associated |
< 2.5 |
73 |
4.10 |
(3.38, 6.03) |
|
|
Idiopathic PAH |
≥ 2.5 |
0.9208 |
209 |
9.02 |
(6.81, 11.84) |
|
Scleroderma Associated |
≥ 2.5 |
69 |
10.74 |
(5.37, 18.59) |
|
|
Scleroderma Associated |
< 2.5 |
0.0192 |
73 |
4.10 |
(3.38, 6.03) |
|
Scleroderma Associated |
≥ 2.5 |
69 |
10.74 |
(5.37, 18.59) |
|
|
Idiopathic PAH |
< 2.5 |
0.5030 |
261 |
9.38 |
(7.51, 14.41) |
|
Idiopathic PAH |
≥ 2.5 |
209 |
9.02 |
(6.81, 11.84) |
Conclusion: Younger age, lower SBP, and lower CI influences survival in SSc-PAH patients, but not in iPAH patients. Younger SSc-PAH patients have markedly lower survival times than similarly aged iPAH patients for unclear reasons. The relationship between lower SBP, decreased CI, and increased mortality has been previously noted in some smaller studies. These data may have important implications for treatment of SSc-PAH especially since some PAH therapies may decrease SBP.
To cite this abstract in AMA style:
Hannan A, Dweik R, Highland KB, Heresi G, Tonelli A, Messner W, Chatterjee S. Mortality Is Increased in Scleroderma Associated Pulmonary Arterial Hypertension Patients with Younger Age, Lower Systolic Blood Pressure, and Lower Cardiac Index, but Not in Idiopathic Pulmonary Arterial Hypertension [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/mortality-is-increased-in-scleroderma-associated-pulmonary-arterial-hypertension-patients-with-younger-age-lower-systolic-blood-pressure-and-lower-cardiac-index-but-not-in-idiopathic-pulmonary-arte/. Accessed .« Back to ACR/ARHP Annual Meeting 2017
ACR Meeting Abstracts - https://acrabstracts.org/abstract/mortality-is-increased-in-scleroderma-associated-pulmonary-arterial-hypertension-patients-with-younger-age-lower-systolic-blood-pressure-and-lower-cardiac-index-but-not-in-idiopathic-pulmonary-arte/