ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1567

The Risk Factors and Prognosis of Interstitial Lung Disease Associated with Primary Sjogren’s Syndrome: A Multi-Center Cohort Study

Ziwei Liu1, Mengtao Li2, Qian Wang1, Yan Zhao3, Dong Xu1 and Xiaofeng Zeng4, 1Peking Union Medical College Hospital, Beijing, China, 2Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China, 3Rheumatology, Peking Union Medical College Hospital, Beijing, China, 4Rheumatology, Peking Union Medical College and Chinese Academy of Medical Sciences, Peking Union Medical College Hospital, Beijing, China

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords:

Session Information

Date: Monday, October 22, 2018

Title: Sjögren's Syndrome – Basic and Clinical Science Poster

Session Type: ACR Poster Session B

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

Background/Purpose:

The present study aimed to investigate the risk factors and prognosis of primary Sjogren’s syndrome-associated interstitial lung disease (pSS-ILD).

Methods:

Data were retrospectively collected from 28 hospitals in China during August 2008 and May 2018. ILD was confirmed by chest high resolution CT (HRCT). Baseline demographic data, clinical manifestations, laboratory tests, pulmonary function, radiology patterns, and treatment regimens were analyzed. Patients were followed up every 6 to 12 months. The primary end point was all-cause death.

Results:

Of the 184 patients enrolled, 90.2% were female, with a mean age of 59.8 ± 10.7 years at baseline. The median disease duration of pSS was 39 (0-324) months, while the median disease duration of ILD was 13 (0-236) months. In 50.5% patients, ILD was the initial clinical manifestation of pSS. Presenting ILD manifestations were: dry cough (19%), productive cough (23.4%), dyspnea on exertion (41.8%), and asymptomatic patients exhibiting abnormalities consistent with ILD on HRCT and/or pulmonary function test (47.8%). NSIP was the most common HTCT pattern, including f-NSIP (23.9%) and c-NSIP (15.9%). Pulmonary function presented restrictive ventilation impairment as well as reduced diffuse function, with FVC 68.8 ± 33.5 % of predicted and DLCO 48.8 ± 28.3% of predicted. Steroid was administrated in 123 (66.8%) patients. Intensive immunosuppressive treatment included cyclophosphamide (32.6%), mycophenolate mofetil (9.2%), and azathioprine (3.3%). Nine patients died in this cohort. Predictive risk factors of ILD in pSS was older age (OR 1.222, 95%CI 1.076-1.169, p <0.001), late onset of pSS(OR 1.041, 95%CI 1.006-1.080, p = 0.024), elevated ESR (OR 2.011, 95%CI 1.107-3.653, p = 0.022), and positive anti-Ro52 antibody (OR 3.658, 95%CI 1.780-7.514, p <0.001). Predictive factors of death in pSS-ILD were older age (p <0.001), history of smoking (p = 0.002), and honeycomb lung pattern on HRCT (p = 0.026).

Conclusion:

ILD can be the initial manifestation of pSS. The results provide strong evidence that patients with older age, late onset of pSS, and positive anti-Ro52 antibody were more likely to complicate ILD. We also suggest that patients with older age, history of smoking, and honeycomb pattern in HRCT may need a closer follow-up.

Table 1. Comparing the demographic and clinical data in pSS patients with and without ILD

pSS-ILD

pSS-non-ILD

p-value

N

184

1000

Female, n(%)

166 (90.2)

953 (95.3)

0.005*

Age, year

59.8 ± 10.7

51.0 ± 13.1

<0.001*

Age of pSS onset, year

52.8 ± 13.0

46.3 ± 13.1

<0.001*

Age of pSS diagnosis, year

56.6 ± 11.1

48.9 ± 13.0

<0.001*

Enlargement of parotid gland

16 (8.7)

193 (19.3)

0.001*

Schimer test +, n(%)

102 (55.4)

685 (68.5)

0.001*

Ocular staining + c, n (%)

40 (21.7)

302 (30.2)

0.020*

Labial salivary biopsy +, n(%)

69 (37.5)

459 (45.9)

0.035*

Purpura, n (%)

4 (2.2)

40 (4.0)

0.229

Leukopenia, n (%)

7 (3.8)

105 (10.5)

0.004*

Elevated ESR, n (%)

81/123 (65.9)

181/352 (51.4)

0.006*

Elevated IgG, n (%)

76/173 (43.9)

485/889 (54.6)

0.010*

Hypocomplementemia, n (%)

30/132 (22.7)

61/303 (20.1)

0.541

ANA+, n (%)

160/179 (89.4)

835/891 (93.7)

0.038*

Anti-SSA+, n(%)

144/182 (79.1)

780/899 (86.8)

0.008*

Anti-SSB+, n(%)

64/179 (35.8)

455/887 (51.3)

<0.001*

Anti-Ro52+，n(%)

132/166 (79.5)

553/849 (65.1)

<0.001*

Table 2. Prognostic factors of pSS-ILD

Survivors

Non Survivors

P-value

N

175

9

Age, year

59.1 ± 10.3

74.3 ± 5.5

<0.001*

Duration of pSS, month

72.6 ± 94.3

65 ± 45.7

0.812

Time between onsets of pSS and ILD, month

49.0 ± 81.2

21.8 ± 48.2

0.446

ILD as the initial manifestation, n (%)

86 (49.1)

7 (77.8)

0.094

History of smoking, n (%)

12 (6.9)

3 (33.3%)

0.002

Dry cough, n (%)

32 (18.3)

3 (33.3)

0.262

Productive cough, n (%)

39 (22.3)

4 (44.4)

0.126

Dyspnea on exertion, n (%)

73 (41.7)

4 (44.4)

0.871

Ground glass occupation, n (%)

88 (50.3)

0

0.022

Honeycomb pattern, n (%)

17 (9.7)

3 (33.3)

0.026*

FVC, %pred

72.7 ± 30.1

69.2 ± 32.9

0.734

TLC, %pred

61.7 ± 34.2

50.2 ± 31.0

0.355

DLCO, %pred

54.4 ± 25.1

27.0 ± 17.0

0.003*

FEV1, %pred

72.3 ±27.3

58.6 ±46.0

0.168

Disclosure: Z. Liu, None; M. Li, None; Q. Wang, None; Y. Zhao, None; D. Xu, None; X. Zeng, None.

To cite this abstract in AMA style:

Liu Z, Li M, Wang Q, Zhao Y, Xu D, Zeng X. The Risk Factors and Prognosis of Interstitial Lung Disease Associated with Primary Sjogren’s Syndrome: A Multi-Center Cohort Study [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/the-risk-factors-and-prognosis-of-interstitial-lung-disease-associated-with-primary-sjogrens-syndrome-a-multi-center-cohort-study/. Accessed .

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