ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2678

Comparison of Scleroderma Associated Isolated Pulmonary Arterial Hypertension and Pulmonary Hypertension with Concomitant Interstitial Lung Disease

Alexander Hannan1, Raed Dweik2, Kristin B. Highland3, Gustavo Heresi4, Adriano Tonelli5, William Messner6 and Soumya Chatterjee1,7, 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, 7Rheumatic and Immunologic Ds, 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: Relatively little data exist in the literature to characterize the differences between patients with scleroderma-associated isolated pulmonary arterial hypertension (SSc-PAH, WHO Group 1) and pulmonary hypertension that coexists with interstitial lung disease (SSc-ILD-PH, WHO Group 3). Our aim was to compare the characteristics of SSc-PAH and SSc-ILD-PH patients from the Cleveland Clinic Pulmonary Hypertension Database to help define differences that may exist between these two groups.

Methods: We performed a retrospective chart-review comparing demographic, laboratory, and hemodynamic data from a total of 176 patients (155 with SSc-PAH and 21 with SSc-ILD-PH) enrolled in the Cleveland Clinic Pulmonary Hypertension Database. The diagnosis of PAH was confirmed by right-heart cardiac catheterization (RHC). The diagnosis of SSc was confirmed by a rheumatologist and that of ILD was confirmed by a pulmonologist. Thirty-three variables were ultimately chosen for evaluation; the decision to include a given variable was based either on previous evidence in the literature that said variables were relevant in predicting outcomes in SSc-PAH or SSc-ILD-PH patients. Multivariate and univariate evaluations were performed using ANOVA, the Kruskal-Wallis test, Pearson’s chi-square test, and Fisher’s Exact test. A p-value of <0.05 was considered significant.

Results: Statistically significant differences between the SSc-PAH and SSc-ILD-PH groups were found for age, forced vital capacity (% estimated), pulmonary vascular resistance, cardiac index by thermodilution, and NT-proBNP level. Additionally, we observed that in our cohort SSc-ILD-PH patients tended to live longer than those with SSc-PAH. Among SSc-ILD-PH, the estimated median time-to-death was 13.19 years (95% CI: 3.79 – 20.72 years), whereas in SSc-PAH patients, the estimated median time-to-death was 4.89 years (95% CI: 4.01 – 6.98 years). However, this difference in survival was not statistically significant (P = 0.1743).

Factor

Total
(N=176)

SSc-PAH
(N=155)

SSc-ILD-PH
(N=21)

p-value

BMI*

28.2±7.6

28.1±7.2

28.9±10.3

0.64a

Patient Age

63.8±11.9

64.5±11.3

58.0±14.7

0.019a

Forced Vital Capacity Percent*

69.8±20.5

71.2±21.0

60.6±14.2

0.027a

Forced Expiratory Vol Percent*

69.7±19.9

70.8±20.4

61.6±14.4

0.047a

Total Lung Capacity Percent*

76.7±17.3

77.2±17.0

72.5±19.4

0.29a

Ejection Fraction, TTE*

57.9±6.6

57.8±6.4

58.7±8.3

0.55a

Right Atrial Area, TTE*

24.1±8.2

24.4±8.3

20.0±5.8

0.21a

Systolic Blood Pressure*

131.4±21.6

130.7±21.4

137.4±22.7

0.21a

Diastolic Blood Pressure*

77.9±13.4

77.7±13.9

79.6±8.7

0.57a

Pulmonary Cap. Wedge Pressure*

12.1±7.0

12.1±7.2

12.0±5.9

0.96a

Pulmonary Vascular Resistance*

8.0±5.2

8.4±5.3

5.2±3.8

0.013a

Systemic Vascular Resistance*

1644.7±678.3

1650.8±699.3

1590.7±465.5

0.74a

Uric Acid Level*

7.1±3.4

7.5±3.3

3.7±3.7

0.13a

Walking Test Distance*

272.9±105.3

269.4±108.7

295.1±79.8

0.32a

FEV1/FVC Percent Ratio*

78.1±9.6

77.8±9.6

80.2±9.9

0.28a

Right Vent. Pressure, TTE*

71.1±23.2

72.3±22.9

61.7±24.1

0.062a

Cardiac Output (THERMO)*

4.6±1.6

4.6±1.7

5.1±1.6

0.23a

Cardiac Index (THERMO)*

2.5±0.79

2.5±0.76

2.9±0.90

0.036a

Cardiac Output (FICK)*

4.7±1.6

4.6±1.6

4.8±1.2

0.72a

Cardiac Index (FICK)*

2.6±0.83

2.5±0.84

2.7±0.71

0.49a

DLCO on PFT*

29.0[0.48,45.0]

29.0[0.49,46.0]

32.0[0.43,42.0]

0.59b

C-Reactive Protein Level*

1.00[0.40,2.8]

1.2[0.45,2.9]

0.60[0.30,1.7]

0.24b

NT-proBNP Level*

1079.5[381.0,4099.0]

1485.5[512.0,4248.5]

187.0[110.0,597.0]

0.002b

Serum Creatinine Level*

0.92[0.79,1.2]

0.97[0.80,1.2]

0.83[0.68,1.05]

0.10b

Serum Ferritin Level*

96.8[35.0,227.0]

95.8[35.0,227.0]

105.2[85.0,178.6]

0.84b

Patient Gender

0.68c

. Female

148(84.1)

131(84.5)

17(81.0)

. Male

28(15.9)

24(15.5)

4(19.0)

Patient Race*

0.006c

. Caucasian

149(86.1)

135(88.8)

14(66.7)

. Other

24(13.9)

17(11.2)

7(33.3)

Smoking Status

0.28c

. No

89(50.6)

81(52.3)

8(38.1)

. Unknown

30(17.0)

27(17.4)

3(14.3)

. Yes

57(32.4)

47(30.3)

10(47.6)

IPF Status

0.29c

. No

120(68.2)

107(69.0)

13(61.9)

. Unknown

33(18.8)

30(19.4)

3(14.3)

. Yes

23(13.1)

18(11.6)

5(23.8)

ANA Status

0.74c

. Negative

15(8.5)

13(8.4)

2(9.5)

. Unknown

55(31.3)

50(32.3)

5(23.8)

. Positive

106(60.2)

92(59.4)

14(66.7)

*Data not available for all subjects. Missing values: BMI = 1, Forced Vital Capacity Percent = 9, Forced Expiratory Vol Percent = 9, Total Lung Capacity Percent = 36, Ejection Fraction, TTE = 10, Right Atrial Area, TTE = 99, Systolic Blood Pressure = 9, Diastolic Blood Pressure = 9, Pulmonary Cap. Wedge Pressure = 2, Pulmonary Vascular Resistance = 9, Systemic Vascular Resistance = 19, Uric Acid Level = 155, Walking Test Distance = 37, FEV1/FVC Percent Ratio = 10, Right Vent. Syst. Pressure, TTE = 8, Cardiac Output (THERMO) = 22, Cardiac Index (THERMO) = 22, Cardiac Output (FICK) = 21, Cardiac Index (FICK) = 21, DLCO on PFT = 30, C-Reactive Protein Level = 116, Brain Natriuretic Peptide = 80, NT-proBNP Level = 106, Serum Creatinine Level = 5, Serum Ferritin Level = 118, Patient Race = 3, NYHA class (at diagnosis) = 51, NYHA class (at 3 months) = 105, NYHA class (at 6 months) = 119.
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: In our study, statistically significant differences were found between SSc-PAH and SSc-ILD-PH patients. The suggestion of longer survival in SSc-ILD-PH patients may be explained by some lurking variables that were not available for analysis, such as autoantibody subsets. SSc-PAH patients were older and possibly were more likely to have limited SSc with positive anti-centromere antibody, representing a subpopulation that is distinct from the SSc-ILD-PH patients. More research is necessary to elucidate these differences.

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. Comparison of Scleroderma Associated Isolated Pulmonary Arterial Hypertension and Pulmonary Hypertension with Concomitant Interstitial Lung Disease [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/comparison-of-scleroderma-associated-isolated-pulmonary-arterial-hypertension-and-pulmonary-hypertension-with-concomitant-interstitial-lung-disease/. Accessed .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/comparison-of-scleroderma-associated-isolated-pulmonary-arterial-hypertension-and-pulmonary-hypertension-with-concomitant-interstitial-lung-disease/