POS0700 TREATMENT OF INTERSTITIAL LUNG DISEASE IN PATIENTS WITH PRIMARY SJOGREN SYNDROME: A SINGLE-CENTER RETROSPECTIVE ANALYSIS
Authors: T. Y. Lan, H. T. Huang, J. H. Kao, C. F. Cheng, C. H. Lu, C. Y. Shen, K. J. Li, S. C. Hsieh
Journal: Annals of the Rheumatic Diseases (2021)

Background: Interstitial lung disease (ILD) is one of the main contributor to morbidity and mortality in patients with primary Sjogren syndrome (pSS), and whether immunosuppressive treatments are effective for pSS-ILD is uncertain. Cyclophosphamide (CYC) and rituximab (RTX) are effective in stabilizing pulmonary function in patients with ILD caused by systemic sclerosis, inflammatory myositis, and rheumatoid arthritis 1,2 . Reports regarding the treatment response for cyclophosphamide or rituximab in pSS-ILD patients are lacking. Objectives: To investigate whether add-on CYC or RTX in addition to steroid and oral DMARDs leads to greater improvement in pulmonary function test than conventional treatment in patients with pSS-ILD with impaired baseline pulmonary function. Methods: pSS-ILD patients with ILD confirmed by a chest computed tomography were screened. Those with imparied baseline pulmonary function test (FVC&lt;80% and DLCO&lt;80%) and available results of pulmonary function tests at 6th-18th month post-treatment were retrospectively enrolled. The patients were clustered into two groups: steroid and oral DMARDs alone (steroid/oDMARD group), and add-on CyC and/or RTX (Add-on CyC/RTX group). Results: This study enrolled 22 patients. The median age of these patients was 65. Among them, 68% were female and 18% were smokers. The median baseline FVC was 63.5% and the median DLCO was 54.4%. The predominant CT pattern was NSIP (54%), followed by UIP (22%), LIP (13.6%) and OP (9%) (table 1). Five patients received steroid and oral DMARDs alone (hydroxychloroquine +/- azathioprine), while 17 patients received add-on CYC and/or RTX (8 patients received CYC, 3 received RTX, and 6 received CYC and RTX). There was no significant difference in baseline characteristic between these two groups (table 1). With a median follow-up period of 9.8 months (interquartile range 7.6 - 13.4 months) after treatment, the FVC improved significantly from 61.3% to 65.5% ( p =0.013) in CYC/RTX group, while the FVC improvement was not significant in steroid/oDMARD group (figure 1A). In CYC/RTX group, patients with non-UIP pattern (NSIP, LIP and OP) on chest CT had significant FVC improvement ( p =0.021), contrasting to the patients with UIP pattern on chest CT (figure 1B). Conclusion: In pSS patients with ILD and impairment of pulmonary function at baseline (FVC&lt;80%, DLCO&lt;80%), FVC improved significantly in patients received add-on cyclophosphamide and/or rituximab therapy. References: [1]Wells AU, Denton CP. Interstitial lung disease in connective tissue disease--mechanisms and management. Nat Rev Rheumatol. 2014 Dec;10(12):728-39. [2]Gao Y, Moua T. Treatment of the Connective Tissue Disease-Related Interstitial Lung Diseases: A Narrative Review. Mayo Clin Proc. 2020 Mar;95(3):554-573. Table 1. Baseline characteristics of patients with Sjögren’s syndrome and interstitial lung disease Total (n = 22) Steroid/oDMARDs (n = 5) Add-on CYC/RTX (n = 17) Comparison between two groups ( p -value) Age, year, median (IQR) 65.1 (61.7 - 69.1) 64 (64.6 - 67.4) 65.6 (61.6 - 69.2) 0.88 Female sex, n (%) 15 (68%) 4 (80%) 12 (70%) 1.00 Smoking, n (%) 4 (22%) 0 (0%) 4 (21%) 0.54 Baseline FVC, % of predicted value, median (IQR) 63.6% (53.8 - 68.2) 63.9% (63.7 - 67.5) 61.3% (50.2 - 68.2) 0.43 Baseline DLCO, % of predicted value, median (IQR) 54.4% (39.4 - 66.4) 69.1% (57.8 - 75.5) 52.1% (38.5 - 58.1) 0.12 CT pattern, n (%) NSIP - 12 (54%) NSIP 1 (20%) NSIP - 11 (65%) - UIP - 5 (22%) UIP 1 (20%) UIP - 4 (24%) LIP - 3 (13.6%) LIP 2 (40%) LIP -1 (6%) OP - 2 (9%) OP 1 (20%) OP -1 (6) UIP pattern, n (%) 5 (22%) 1 (20%) 4 (24%) 1.00 CYC, cyclophosphamide; oDMARDs, oral DMARDs; RTX, rituximab Figure 1. FVC change before and after the immunosuppressive treatment Disclosure of Interests: None declared