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Abstract Number: 847

Forced Vital Capacity Predicts Lung Fibrosis Progression and Mortality in Systemic Sclerosis

Anna Hoffmann-Vold1, Elizabeth R. Volkmann2, Oyvind Midtvedt3, Torhild Garen3, Anders Heiervang Tennøe3, Trond Mogens Aalokken4, May Brit Lund5 and Øyvind Molberg3, 1Division of Rheumatology, Oslo University Hospital, Oslo, Norway, 2University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 3Rheumatology, Oslo University Hospital, Oslo, Norway, 4Radiology, Oslo University Hospital, Oslo, Norway, 5Respiratory Medicine, Oslo University Hospital, Oslo, Norway

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: interstitial lung disease, morbidity and mortality, pulmonary fibrosis and scleroderma

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Session Information

Date: Sunday, November 13, 2016

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

Session Type: ACR Poster Session A

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

Background/Purpose:  Systemic sclerosis (SSc) carries high risk for progressive interstitial lung disease (ILD), but there are no valid methods for early detection of SSc-ILD or algorithms for ILD progression available. The aim of this study was to assess the impact of baseline FVC on lung fibrosis, fibrosis progression and mortality.

Methods: Paired pulmonary function tests (PFT) and high resolution computed tomography (HRCT) images were obtained at baseline and follow-up in consecutive SSc patients (n=305) from the prospective Oslo University Hospital (OUH) cohort. All patients met the 2013 ACR/EULAR classification criteria. Extent of fibrosis was scored on 10 sections from every HRCT and expressed as percentage of total lung volumes. The annual fibrosis progression was defined as the difference in extent of fibrosis between the baseline and follow-up HRCT divided by the actual follow-up period in years.

Results: At baseline, 186/305 patients (61%) had a baseline FVC <100% (Table 1). Lower baseline FVC (<90, 80 and 70%) was associated with male gender and higher extent of fibrosis at baseline and follow up (Table 1). In multivariate cox regression analyses, all baseline FVC thresholds (<100, 90, 80 and 70%) were significantly associated with mortality (Table 2). Other parameters significantly associated with mortality were age at onset (HR 1.1, 95% CI 1.1-1.2, p-value<0.001) and diffuse cutaneous SSc (HR 2.2, 95% CI 1.2-3.6, p-value 0.002). All baseline FVC thresholds were also associated with annual fibrosis progression in univariate cox regression analyses (Table 2). In multivariate analyses, only baseline FVC <80% was associated with annual fibrosis progression (HR 1.8, 95% CI 1.0-3.0, p-value 0.042), as well as male gender (HR 1.9, 95% CI 1.1-3.4, p-value 0.023) and anti-Topoisomerase I (HR 2.2, 95% CI 1.3-3.9, p-value 0.006). Of the 119 patients with baseline FVC>100%, 56 had no lung fibrosis. These 56 patients were predominantly female (90.4%); most of them had limited cutaneous SSc (91%) and anti-centromere antibodies (76%) and 18% died during the observation period.

Conclusion: These prospective cohort data suggest that baseline FVC%, including normal range values, predict mortality and fibrosis progression in SSc. Based on these data, we suggest that all SSc patients should be assessed with PFT and HRCT at baseline, and followed with PFTs on a regular basis.   Table 1: Demographics, extent of fibrosis at baseline and follow-up, annual fibrosis progression and pulmonary function segregated by baseline FVC% in 305 SSc patients

Baseline FVC

>100%

119 (39)

90-100%

60 (20)

80-90%

48 (16)

70-80%

32 (11)

<70%

46 (15)

p-value¹

Age at onset, yrs

51 (12.9)

46¹(16.3)

49(16.5)

47(17.9)

45¹ (14)

n.s.

Gender, no (%)

15 (13)

11 (18)

9 (19)

8 (25)

20 (44)

<0.001

Follow-up duration, yr

3.9 (2.8)

4.1 (3.6)

3.4 (2.3)

3.8 (2.7)

3.9 (3.2)

n.s.

Disease duration, yr

10.2 (6.6)

11.9(9.1)

10.6(6.7)

10.3(9.1)

11.8 (8.6)

n.s.

Deceased, no (%)

19 (16)

14 (23)

16 (33)

10 (31)

22 (48)

<0.001

lcSSc, no (%)

102 (86)

40 (68)

30 (64)

21 (68)

21 (47)

<0.001

ACA, no (%)

82 (69)

22 (37)

17 (35)

12 (37)

6 (13)

<0.001

Any fibrosis, n (%)

63 (53)

36 (60)

31 (65)

22 (69)

45 (98)

<0.001

Baseline fibrosis %

1.6 (3.8)

4.7 (9.3)

5.3 (7.8)

5.3(10.9)

25.7(14.9)

<0.05

Follow up fibrosis %

2.1 (4.9)

6.5(11.7)

6.7 (9.2)

8.3(15.4)

28.8(20.9)

<0.05

Annual fibrosis progression%

0.1 (1.1)

0.7 (2.5)

0.6 (1.8)

1.0 (3.1)

1.0 (3.5)

n.s.

Annual FVC decline

1.8 (5.7)

1.9 (6.1)

2.2 (7.8)

2.8 (9.5)

2.6 (5.4)

n.s.

Baseline DLCO %

77.2(20.1)

65.8(15.9)

65.5(15.7)

62 (14.9)

43.6(14.9)

<0.05

Annual DLCO decline

2.9 (4.3)

0.7¹(15.5)

1.8 (6.4)

4.7 (9.1)

2.5 (6.8)

n.s.

¹:p-value < 0,05 compared to baseline FVC>100%, n.s. not significant except for values marked: x¹ (p-value<0.05), (SD) if not stated differently Table 2: Univariate cox regression analyses with mortality and annual fibrosis progression as outcome measures

N (%)

Mortality

HR (95% CI)

p-value

Annual fibrosis progression

HR (95% CI)

p-value

Baseline FVC<100%

186

(61)

2.1 (1.3-3.6)

0.005

1.8 (1.0-3.2)

0.045

Baseline FVC <90%

126

(41)

2.0 (1.2-3.2)

0.004

2.2 (1.3-3.7)

0.003

Baseline FVC <80

98

(32)

2.0 (1.2-3.2)

0.006

2.3 (1.4-3.9)

0.001

Baseline FVC <70%

46

(15)

2.5 (1.4-4.5)

0.003

2.1 (1.2-3.6)

0.012


Disclosure: A. Hoffmann-Vold, None; E. R. Volkmann, None; O. Midtvedt, None; T. Garen, None; A. Heiervang Tennøe, None; T. M. Aalokken, None; M. B. Lund, None; Ø. Molberg, None.

To cite this abstract in AMA style:

Hoffmann-Vold A, Volkmann ER, Midtvedt O, Garen T, Heiervang Tennøe A, Aalokken TM, Lund MB, Molberg Ø. Forced Vital Capacity Predicts Lung Fibrosis Progression and Mortality in Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/forced-vital-capacity-predicts-lung-fibrosis-progression-and-mortality-in-systemic-sclerosis/. Accessed July 5, 2022.
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