Session Information
Date: Monday, October 22, 2018
Session 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 |
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 March 21, 2023.« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-risk-factors-and-prognosis-of-interstitial-lung-disease-associated-with-primary-sjogrens-syndrome-a-multi-center-cohort-study/