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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 arthritis1,2. 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 (FVCResults: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 (FVCReferences:[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 diseaseTotal(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)0.88Female sex, n (%)15 (68%)4 (80%)12 (70%)1.00Smoking, n (%)4 (22%)0 (0%)4 (21%)0.54Baseline FVC, % of predicted value, median (IQR)63.6% (53.8 - 68.2)63.9% (63.7 - 67.5)61.3% (50.2 - 68.2)0.43Baseline DLCO, % of predicted value, median (IQR)54.4% (39.4 - 66.4)69.1% (57.8 - 75.5)52.1% (38.5 - 58.1)0.12CT 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%)1.00CYC, cyclophosphamide; oDMARDs, oral DMARDs; RTX, rituximabFigure 1.FVC change before and after the immunosuppressive treatmentDisclosure of Interests:None declared

Background:Systemic lupus erythematosus (SLE) could affect multiple parts of the eye. (1,2) Also, there is risk of ocular toxicity associated with treatment, such as hydroxychloroquine.(3) Early diagnosis is essential to avoid major ocular complications. However, complaints of eye discomfort may be vague, and various presentations may pose challenges on rheumatologists. Knowing the real-world prevalence of ocular problems in patients with SLE is important and helpful, yet this has been less common to be reported.Objectives:To describe the prevalence of various ophthalmologic diagnoses in patients with SLE.Methods:This is a retrospective observational study conducted in a tertiary hospital in Taipei, Taiwan. Patients diagnosed with SLE in the period between 1st Jan, 2002 and 31th Dec, 2015 and evaluated by ophthalmologists in 2 years before 29th Jan, 2021 were included. Demographic and clinical data, and ophthalmologic diagnoses were recorded by chart review.Results:A total of 121 patients were included, and 118 (97.5%) were female. Average age was 46.2 years [standard deviation (SD) 11.4 years] upon ophthalmologic evaluation, and average duration suffering from lupus was 11.6 years (SD 3.3 years). Keratoconjunctivitis sicca (n = 48, 39.7%), myopia (n = 39, 32.2%), and cataract (n = 21, 17.4%) were the most common findings. It was also noted that suspicious finding of hydroxychloroquine retinal toxicity was found in 12 patients (9.9% of total patients). Also, two patients had lupus retinopathy, and another two were diagnosed with cytomegalovirus retinitis. In addition, glaucoma was diagnosed in 9 patients (7.4%), which seemed to be higher than the general population.Conclusion:Higher prevalence of different ocular problems is noted in this cohort of SLE patients. Keratoconjunctivitis sicca was the most common ocular diagnosis, which is consistent with literature. However, high rates of myopia, cataract, glaucoma, and suspicious hydroxychloroquine retinal toxicity were also found. Above result warrants more aggressive ophthalmologic evaluation in SLE patients. More research on the association of lupus and different ocular diseases is needed in the future.References:[1]Silpa-archa S, Lee JJ, Foster CS. Ocular manifestations in systemic lupus erythematosus. Br J Ophthalmol. 2016 Jan;100(1):135–41.[2]Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford). 2007 Dec;46(12):1757–62.[3]Jorge A, Ung C, Young LH, Melles RB, Choi HK. Hydroxychloroquine retinopathy - implications of research advances for rheumatology care. Nat Rev Rheumatol. 2018;14(12):693–703.Table 1.Demographic data and ophthalmologic diagnosis of patients with systemic lupus erythematosus (N = 121)Variablen (% of N)Age (years) average (SD)46.2 (11.4)Disease duration average (SD)11.6 (3.3)Female sex118 (97.5)Chronic medical disease Hypertension28 (23.1) Diabetes mellitus4 (3.3) Dyslipidemia7 (5.8) Cancer3 (2.5) End-stage renal disease1 (0.8)Ophthalmologic diagnosis Keratoconjunctivitis sicca48 (39.7) Myopia39 (32.2) Cataract21 (17.4) Glaucoma9 (7.4) Suspicious retinopathy related to HCQ12 (9.9) Unspecified retinopathy5 (4.1) Lupus retinopathy2 (1.7) CMV retinitis2 (1.7) Central retinal vein thrombosis1 (0.8) Retinal break1 (0.8) Optic neuritis1 (0.8) Vitreal hemorrhage1 (0.8) Myodesopia4 (3.3) Scleritis1 (0.8)SD, standard deviation; HCQ, hydroxychloroquine; CMV, cytomegalovirus.Disclosure of Interests:None declared