ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1702

Disease Progression in Systemic Sclerosis Patients with Concomittant or Isolated Interstitial Lung Disease and Pulmonary Arterial Hypertension in the Scleroderma Cohort Singapore

Maria Noviani1, Seyed Ehsan Saffari2, Sandra Mei Yu Kua1, Grace Yin Lai Chan3, Gim Gee Teng4, Weng Giap Law5, Amelia Santosa4, Anita Yee Nah Lim4, Swee Cheng Ng1 and Andrea Hsiu Ling Low1, 1Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore, Singapore, 2Center for Quantitative Medicine, Duke-NUS Medical School, Singapore, Singapore, Singapore, 3Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, SIngapore, Singapore, 4Division of Rheumatology, University Medicine Cluster, National University Health System, Singapore, Singapore, Singapore, 5Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore, Singapore

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: , , ,

Session Information

Date: Monday, November 6, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are leading causes of mortality in patients with systemic sclerosis (SSc).  We aimed to determine factors associated with disease progression in SSc patients with concomitant ILD-PAH, PAH alone or ILD alone in the Scleroderma Cohort Singapore. 

Methods:

In this multi-centre study involving 3 tertiary Rheumatology institutions (January 2008 to June 2016), factors associated with progression in full vital capacity (predicted FVC ≥10% decrease from baseline), NT-proBNP (≥4x baseline) and New York Heart Association (NYHA ≥1 class progression) were identified among SSc patients with ILD-PAH, PAH alone and ILD alone. Stepwise multivariable logistic regression analyses were performed to determine independent factors associated with worse outcomes. Inclusion criteria were fulfillment of the ACR 2013 classification criteria for SSc and significant pulmonary involvement. ILD was based on high resolution computed tomography scan and predicted FVC <70%. Patients with suspected PAH based on echocardiographic systolic pulmonary arterial pressure (PAP) >50mmHg, and definite PAH based on right heart catheterization findings of mean PAP >25mmHg and pulmonary capillary wedge pressure (PCWP) <15 mmHg were included. In patients with PCWP >15mmHg, those with mixed pre- and post-capillary PH based on transpulmonary gradient >12mmHg were also included in the PAH group analyses.

Results:

Among 541 subjects, 88 (16%) had ILD, 49 (9%) PAH and 44 (8%) concomitant ILD-PAH (Table 1). Of 93 patients classified to have PAH or ILD-PAH, 56 (60%) were based on echocardiography and 37 (40%) on right heart catheterization. Patients with ILD-PAH had increased risk of NT-proBNP progression; additionally, telangiectasia and baseline NYHA >II increased the risk of NT-proBNP progression. SSc disease duration and malabsorption increased the risk of NYHA progression. Reflux/ dysphagia and malabsorption increased the risk of FVC progression (Table 2).

Conclusion:

SSc patients with concomitant ILD-PAH had worse clinical outcomes based on surrogate biomarker NT-proBNP. We also identified other risk factors associated with increased risk of disease progression in SSc patients with pulmonary involvement.

Table 1. Demographics and Clinical Characteristics

ILD (n=88)

PAH (n=49)

ILD-PAH (n=44)

Female, n (%)

74 (84.1%)

43 (87.8%)

39 (88.6%)

Ethnicity, n (%)

   Chinese

55 (62.5%)

36 (73.5%)

29 (65.9%)

   Malay

13 (14.8%)

8 (16.3%)

9 (20.5%)

   Indian

11 (12.5%)

4 (8.2%)

3 (6.8%)

   Others

9 (10.2%)

1 (2%)

3 (6.8%)

Age at cohort entry, years (mean ± SD)

53.4 ± 11.8

57.3 ± 13.5

59.5 ± 13.2

Follow up duration from entry, years (mean ± SD)

4.6 ± 1.9

4.5 ± 2.1

4.7 ± 2.2

Age at SSc diagnosis, years (mean ± SD)

46.5 ± 12.1

51.1 ± 16.4

53.6 ± 15.1

SSc Subtype, n (%)

   DcSSc

43 (48.9%)

13 (27.1%)

13 (30.2%)

   LcSSc

28 (31.8%)

22 (45.8%)

19 (44.2%)

   MCTD

17 (19.3%)

13 (27.1%)

11 (25.6%)

Antibodies, n (%)*

   Anti-centromere

3 (4.9%)

11 (32.4%)

3 (10.3%)

   Anti-Scl-70

49 (60.5%)

10 (20.4%)

17 (40.5%)

   Anti-Ro

21 (31.3%)

15 (34.1%)

9 (23.7%)

Treatment, n (%)

   Peripheral vasculopathy: CCB, ACE-I, ARB

61 (69.3%)

31 (63.3%)

29 (65.9%)

   Peripheral vasculopathy: PC, PDE5i, ETA

10 (11.4%)

9 (18.4%)

10 (22.7%)

   Parenteral nutrition

2 (2.3%)

1 (2.0%)

0 (0%)

   Immunosuppression: MTX, CYC, MMF

63 (71.6%)

25 (51.02%)

28 (63.6%)

   PAH: PC, PDE5i, ETA

N/A

27 (55.1%)

26 (59.1%)

NOTE: DcSSc: diffuse cutaneous systemic sclerosis; LcSSc: limited cutaneous systemic sclerosis; MCTD: mixed connective tissue disease; CCB: calcium channel blocker; ACE-I: angiotensin converting enzyme inhibitor; ARB: angiotensin receptor blocker; PC: prostacyclin; PDE-5i: phosphodiesterase type 5 inhibitor; ETA: endothelin receptor antagonist; MTX: methotrexate; CYC: cyclophosphamide; MMF: mycophenolate mofetil; PAH: pulmonary arterial hypertension. *The denominator for (%) calculation was based on number of subjects tested for the respective antibodies.

Table 2. Multivariable Logistic Regression

Adjusted Odds Ratio (95% CI)

P value

NT-proBNP progression

   Age at censor date

0.916 (0.847, 0.990)

0.0269

   Cumulative telangiectasia

36.84 (2.933, 462.8)

0.0052

   Baseline NYHA Class II,III, or IV (vs. I)

35.98 (1.858, 696.6)

0.0178

   ILD-PAH subgroup (vs. PAH only)

8.386 (1.295, 54.3)

0.0256

NYHA progression

   Cumulative malabsorption

3.332 (1.144, 9.704)

0.0273

   SSc duration at cohort entry

1.099 (1.027, 1.177)

0.0064

   SSc subgroup

0.1193

      ILD-PAH subgroup (vs. ILD only)

3.475 (1.124, 10.74)

0.0720

      PAH subgroup (vs. ILD only)

1.876 (0.545, 6.455)

0.9912

FVC progression

   Baseline reflux/ dysphagia

3.106 (1.095, 8.811)

0.0331

   Cumulative malabsorption

7.405 (1.767, 31.03)

0.0062

   ILD-PAH subgroup (vs. ILD only)

1.833 (0.589, 5.706)

0.2954

NOTE: NYHA: New York Heart Association; FVC: forced vital capacity.
Disclosure: M. Noviani, None; S. E. Saffari, None; S. M. Y. Kua, None; G. Y. L. Chan, None; G. G. Teng, None; W. G. Law, None; A. Santosa, None; A. Y. N. Lim, None; S. C. Ng, None; A. H. L. Low, None.

To cite this abstract in AMA style:

Noviani M, Saffari SE, Kua SMY, Chan GYL, Teng GG, Law WG, Santosa A, Lim AYN, Ng SC, Low AHL. Disease Progression in Systemic Sclerosis Patients with Concomittant or Isolated Interstitial Lung Disease and Pulmonary Arterial Hypertension in the Scleroderma Cohort Singapore [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/disease-progression-in-systemic-sclerosis-patients-with-concomittant-or-isolated-interstitial-lung-disease-and-pulmonary-arterial-hypertension-in-the-scleroderma-cohort-singapore/. Accessed .

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