ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2680

Analysis of Prognostic Determinants of Scleroderma-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension

Alexander Hannan1, Raed Dweik2, Kristin B. Highland3, Gustavo Heresi4, Adriano Tonelli5, William Messner6 and Soumya Chatterjee1, 1Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 2Respiratory Institute, Cleveland Clinic, Cleveland, OH, 3Rheumatology.org, Cleveland Clinic, Cleveland, OH, 4Respiratory Institute - Pulmonary Medicine, Cleveland Clinic, Cleveland, OH, 5Pulmonary Medicine - Respiratory Institute, Cleveland Clinic, Cleveland, OH, 6Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords:

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: Systemic sclerosis (SSc) associated pulmonary arterial hypertension (SSc-PAH) is a serious and often life-threatening complication of SSc. Perhaps the most important enigma in the context of SSc-PAH care is the fact that the prognosis of SSc-PAH patients is substantially worse than that of demographically similar patients with idiopathic PAH (iPAH). Moreover, it has been shown that these differences in survival are not related to a higher prevalence of left-heart disease in SSc-PAH patients. Previous studies have not extensively compared demographic, laboratory, and hemodynamic data from cohorts of iPAH and SSc-PAH patients to determine which specific characteristics of these groups may explain the worsened clinical course and prognosis of SSc-PAH sufferers. The aims of our study were to identify specific characteristics that distinguish these two groups in a large cohort of SSc-PAH and iPAH patients.

Methods: A retrospective chart-review was conducted comparing demographic, laboratory, and hemodynamic [echocardiographic and right heart catheterization (RHC)] 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. Thirty variables were chosen for evaluation; the decision to include a given variable was based either on inclusion of the variables in previous studies on SSc-PAH or on theoretical suspicion that specific variables may be relevant in distinguishing the two conditions. Multivariate and univariate analyses were completed utilizing ANOVA, the Kruskal-Wallis test, Pearson’s chi-square test, and Fisher’s Exact test.

Results: Statistically significant differences between the SSc-PAH and iPAH groups were found for the variables of age, gender, forced vital capacity, total lung capacity, 6-minute walk distance, FEV1/FVC ratio, transfer factor (DLCO), right ventricular systolic pressure (on transthoracic echocardiogram), mean pulmonary artery pressure and pulmonary vascular resistance (on RHC), systolic blood pressure, BNP and NT-proBNP level, and mortality status.

Factor

Total
(N=862)

Idiopathic PAH
(N=686)

Scleroderma Associated
(N=176)

p-value

BMI*

29.0±7.8

29.2±7.9

28.2±7.6

0.11a

Patient Age

54.7±16.3

52.4±16.5

63.8±11.9

<0.001a

Forced Vital Capacity Percent*

74.1±20.4

75.3±20.2

69.8±20.5

0.002a

Forced Expiratory Vol Percent*

70.1±19.9

70.2±19.9

69.7±19.9

0.75a

Total Lung Capacity Percent*

83.4±17.9

85.4±17.6

76.7±17.3

<0.001a

Ejection Fraction, TTE*

57.1±7.8

56.9±8.1

57.9±6.6

0.16a

Right Atrial Area, TTE*

25.5±8.8

25.9±8.9

24.1±8.2

0.098a

Systolic Blood Pressure*

127.5±24.2

126.5±24.7

131.4±21.6

0.018a

Diastolic Blood Pressure*

76.7±14.7

76.4±15.0

77.9±13.4

0.24a

Pulmonary Cap. Wedge Pressure*

12.9±7.1

13.1±7.2

12.1±7.0

0.086a

Mean Pulmonary Artery Pressure*

49.1±14.7

50.9±14.8

42.3±11.8

<0.001a

Pulmonary Vascular Resistance*

9.3±6.1

9.7±6.3

8.0±5.2

0.002a

Systemic Vein Resistance*

1606.0±665.8

1595.4±662.5

1644.7±678.3

0.41a

Uric Acid Level*

7.3±3.8

7.3±3.9

7.1±3.4

0.86a

Walking Test Distance*

305.5±115.4

313.8±116.5

272.9±105.3

<0.001a

FEV1/FVC Percent Ratio*

76.0±9.5

75.3±9.3

78.1±9.6

<0.001a

Right Vent. Pressure, TTE*

75.3±24.6

76.5±24.9

71.1±23.2

0.012a

Cardiac Output (THERMO)*

4.8±2.0

4.9±2.1

4.6±1.6

0.12a

Cardiac Index (THERMO)*

2.6±0.96

2.6±1.00

2.5±0.79

0.45a

Cardiac Output (FICK)*

4.8±1.9

4.8±2.0

4.7±1.6

0.27a

Cardiac Index (FICK)*

2.6±1.02

2.6±1.07

2.6±0.83

0.74a

DLCO on PFT*

36.0[0.76,60.0]

41.0[0.81,65.0]

29.0[0.48,45.0]

<0.001b

C-Reactive Protein Level*

1.00[0.40,2.9]

1.1[0.40,2.9]

1.00[0.40,2.8]

0.78b

Brain Natriuretic Peptide*

167.0[45.0,557.0]

134.0[41.0,494.0]

339.5[124.5,975.5]

<0.001b

NT-proBNP Level*

680.0[232.0,2621.0]

619.0[202.0,2513.0]

1079.5[381.0,4099.0]

0.013b

Serum Creatinine Level*

0.90[0.74,1.2]

0.90[0.73,1.1]

0.92[0.79,1.2]

0.22b

Serum Ferritin Level*

85.0[35.8,210.4]

82.7[36.0,210.0]

96.8[35.0,227.0]

0.83b

Patient Gender

<0.001c

. Female

616(71.5)

468(68.2)

148(84.1)

. Male

246(28.5)

218(31.8)

28(15.9)

Patient Race*

0.088c

. Caucasian

690(81.7)

541(80.5)

149(86.1)

. Other

155(18.3)

131(19.5)

24(13.9)

Smoking Status

0.75c

. No

414(48.0)

325(47.4)

89(50.6)

. Unknown

157(18.2)

127(18.5)

30(17.0)

. Yes

291(33.8)

234(34.1)

57(32.4)

IPF Status

<0.001c

. No

640(74.2)

520(75.8)

120(68.2)

. Unknown

179(20.8)

146(21.3)

33(18.8)

. Yes

43(5.0)

20(2.9)

23(13.1)

ANA Status

<0.001c

. Negative

300(34.8)

285(41.5)

15(8.5)

. Unknown

334(38.7)

279(40.7)

55(31.3)

. Positive

228(26.5)

122(17.8)

106(60.2)

NYHA class (at diagnosis)*

0.11c

. Class 1 or 2

186(30.9)

157(33.0)

29(23.2)

. Class 3

314(52.2)

242(50.8)

72(57.6)

. Class 4

101(16.8)

77(16.2)

24(19.2)

NYHA class (at 3 months)*

0.77c

. Class 1 or 2

137(42.4)

109(43.3)

28(39.4)

. Class 3

147(45.5)

114(45.2)

33(46.5)

. Class 4

39(12.1)

29(11.5)

10(14.1)

NYHA class (at 6 months)*

0.040c

. Class 1 or 2

142(48.0)

116(48.5)

26(45.6)

. Class 3

131(44.3)

109(45.6)

22(38.6)

. Class 4

23(7.8)

14(5.9)

9(15.8)

Mortality Status

0.016c

. Alive

368(42.7)

307(44.8)

61(34.7)

. Dead

494(57.3)

379(55.2)

115(65.3)

*Data not available for all subjects. Missing values: BMI = 5, Forced Vital Capacity Percent = 113, Forced Expiratory Vol Percent = 112, Total Lung Capacity Percent = 269, Ejection Fraction, TTE = 97, Right Atrial Area, TTE = 505, Systolic Blood Pressure = 57, Diastolic Blood Pressure = 61, Pulmonary Cap. Wedge Pressure = 42, Mean Pulmonary Artery Pressure = 9, Pulmonary Vascular Resistance = 89, Systemic Vascular Resistance = 136, Uric Acid Level = 771, Walking Test Distance = 177, FEV1/FVC Percent Ratio = 116, Right Vent. Pressure, TTE = 93, Cardiac Output (THERMO) = 194, Cardiac Index (THERMO) = 194, Cardiac Output (FICK) = 169, Cardiac Index (FICK) = 171, DLCO on PFT = 215, C-Reactive Protein Level = 650, Brain Natriuretic Peptide = 401, NT-proBNP Level = 557, Serum Creatinine Level = 19, Serum Ferritin Level = 615, Patient Race = 17, NYHA class (at diagnosis) = 261, NYHA class (at 3 months) = 539, NYHA class (at 6 months) = 566.
Statistics presented as Mean ± SD, Median [P25, P75], Median (min, max) or N (column %).
p-values: a=ANOVA, b=Kruskal-Wallis test, c=Pearson’s chi-square test, d=Fisher’s Exact test.

Conclusion: Our analysis confirms findings from previous studies that have shown that SSc-PAH patients demonstrate important differences in hemodynamic and pulmonary function parameters compared to iPAH patients (table). Additionally, patients with SSc-PAH demonstrated much higher BNP and NT-proBNP levels compared to iPAH patients with similar hemodynamic parameters. Further research will be necessary to help delineate other relevant factors that may aid in caring for these patients.

Disclosure: A. Hannan, None; R. Dweik, None; K. B. Highland, None; G. Heresi, None; A. Tonelli, None; W. Messner, None; S. Chatterjee, None.

To cite this abstract in AMA style:

Hannan A, Dweik R, Highland KB, Heresi G, Tonelli A, Messner W, Chatterjee S. Analysis of Prognostic Determinants of Scleroderma-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/analysis-of-prognostic-determinants-of-scleroderma-associated-pulmonary-arterial-hypertension-and-idiopathic-pulmonary-arterial-hypertension/. Accessed .

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