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OBJECTIVE To investigate the prognostic significance of videofluorographic swallowing study (VFSS)-confirmed oropharyngeal dysphagia in idiopathic inflammatory myopathies (IIMs). METHOD We reviewed the medical records of patients who were diagnosed with IIM between 2009 and 2020 at Seoul St Mary's Hospital. All oropharyngeal dysphagia cases were limited to VFSS-confirmed dysphagia found during the initial diagnostic work-up for IIM. We described the findings on VFSS and the course of the dysphagic symptoms. Logistic regression and survival analyses were performed to evaluate the risk of pneumonia and mortality, respectively. RESULTS We found 88 patients with IIM who met the criteria. Among them, 17 patients (19%) had oropharyngeal dysphagia. Except for two cases lost to follow-up and one deceased case, all of the patients with dysphagia (14 of 14) had swallowing function restored within 6 months. The risk of pneumonia within 3 months from the diagnosis of IIM was significant [odds ratio = 4.49, 95% confidence interval (CI) 1.07-18.88]. The median follow-up duration was 34 and 27 months for the groups without and with dysphagia, respectively. The survival analysis failed to demonstrate that the presence of oropharyngeal dysphagia increased the risk of death (hazard ratio = 0.77, 95% CI: 0.085-7.00). CONCLUSIONS Oropharyngeal dysphagia found at the initial diagnosis of IIM improved within 3-6 months in nearly all cases. Furthermore, IIM patients who had oropharyngeal dysphagia at the initial diagnosis of IIM were not likely to have shorter survival, even if the risk of pneumonia was increased in the short term.

PURPOSE: The authors report four cases of residual intrastromal lenticule after seemingly uneventful small incision lenticule extraction (SMILE) surgery and the outcome after a secondary procedure to remove the lenticule remnant. METHODS: Case reports. RESULTS: All four cases presented with suboptimal corrected distance visual acuity (CDVA) and refractive surprise. Irregularities in the corneal curvature and elevation were detected on corneal topography and tomography. Careful review of the intraoperative video showed an incomplete removal of the SMILE lenticule. All four cases underwent a secondary surgical procedure to remove the residual lenticule remnant. All cases had improvements in the CDVA, refraction, and topography, with the CDVA returning to the preoperative value (20/20) in all cases. CONCLUSIONS: Performing an early, secondary lenticular remnant removal operation is feasible, safe, and can result in improvement in both refraction and visual acuity. During the original SMILE surgery, surgeons should routinely examine the removed lenticule for completeness. [ J Refract Surg. 2017;33(11):779–782.]

The risk factors for atypical femur fracture in patients exposed to bisphosphonates for at least 1 year were examined. Prolonged and continuous use of bisphosphonates, long-term use of glucocorticoids, and a higher body mass index were associated with increased risk of atypical femur fracture. The purpose of the present study is to determine whether rheumatoid arthritis (RA) and other clinical factors are associated with an increased risk of bisphosphonate (BP)-related atypical femur fracture (AFF). A retrospective nested case-control study of patients who had taken BPs for at least 1 year was conducted. Patients with AFF were identified by reviewing surgical and radiographic records. Three controls with no history of AFFs were randomly selected and age- and sex-matched to each patient with AFFs. Cox proportional hazard models were used to analyze the independent contribution of risk factors to BP-related AFF. Among the 35,104 patients prescribed BPs for at least 1 year, 43 females (mean age, 68 years) suffered AFFs (0.12%). Patients with AFFs were exposed to BPs for a mean of 7.3 years. Patients with AFFs were exposed to BPs for longer than those without AFFs and continued treatment without a drug holiday. More patients with AFF than controls had taken glucocorticoids and disease-modifying anti-rheumatic drugs. Multivariate Cox regression analyses estimated that long-term use of glucocorticoids, prolonged exposure to BP without cessation, and every 1 kg/m2 increase in the body mass index (BMI) increased the hazard ratio for AFFs by 3.0, 5.2, and 1.2, respectively. Prolonged and continuous use of BPs, long-term use of glucocorticoids, and a higher BMI increase the risk of AFFs. Switching long-term BP and glucocorticoid users to other bone-protective agents should be considered.

We recently reported the presence of anti-aquaporin 5 (AQP5) immunoglobulin G (IgG) in patients with primary Sjogren syndrome (SS) with a sensitivity of 0.73 and a specificity of 0.68. The aim of this study was to identify functional epitopes for the anti-AQP5 autoantibodies detected in control subjects and patients with SS. Recognition of epitopes by anti-AQP5 autoantibodies in sera ( n = 13 for control and n = 24 for SS) or purified IgG ( n = 1 for control and n = 3 for SS) was evaluated by indirect immunofluorescence (IIF) assay performed in the presence or absence of peptides corresponding to the second transmembrane helix and extracellular loops A, C, and E of AQP5. Functional epitopes were determined by measuring the effects of purified IgG and neutralizing peptides on transepithelial osmotic permeability (PfT) of MDCK cells expressing AQP5. In the IIF assay, 89% of SS samples were inhibited by at least 1 peptide, while only half of control samples were inhibited by any peptide. Overall, SS samples were inhibited by peptides corresponding to extracellular loops A, C, and E by 40% to 50%, whereas control samples were inhibited only by peptides corresponding to loop E by

Purpose To compare the demarcation line depth after 2 different protocols of simultaneous laser in situ keratomileusis and collagen crosslinking (LASIK Xtra). Methods Twenty-three patients receiving bilateral LASIK Xtra were included in this comparative interventional study. The corneal stroma was soaked with 0.22% riboflavin for 90 seconds before flap repositioning. One eye was then randomized to receive either 18 mW/cm of ultraviolet A irradiation for 120 seconds or for 180 seconds, whereas the fellow eye received the other irradiation duration. The demarcation line on anterior-segment optical coherence tomography and grading of corneal haze on slit lamp were compared at postoperative month 1. Results The average age was 29.0 ± 8.2 years. There was no statistical difference in preoperative spherical equivalent refraction (P > 0.05). At postoperative 1 month, a well-defined demarcation line was visible in 74% and 70% in the 2- and 3-minute groups, respectively. The demarcation line depth was 282 ± 51 μm and 284 ± 43 μm, respectively (P = 1), and the ratio of the demarcation line depth to the postoperative central corneal thickness was 0.69 ± 0.13 and 0.72 ± 0.10 (P = 0.61). There was no difference in the percentage of eyes with grade 1 or 2 corneal haze (P = 0.76). Conclusions The depth of the demarcation line was the same after receiving 18-mW/cm irradiation for either 2 or 3 minutes. The amount of early postoperative corneal haze was also similar. We postulate that there was lack of oxygen in the corneal tissue to fuel the photochemical reaction despite given additional time.

Background Fraxinellone is isolated from Dictamnus dasycarpus, a traditional herbal medicine that attenuates inflammatory conditions.1,2 Recent studies have suggested that fraxinellone has a potential therapeutic effect in animal models with inflammatory diseases.3–5 Objectives We aimed to evaluate the therapeutic effect of fraxinellone on inflammatory arthritis and identify the underlying mechanisms. Methods Fraxinellone (7.5 mg/kg) or a vehicle control was injected into mice with collagen-induced arthritis (CIA). The severity of arthritis was evaluated clinically and histologically. The differentiation of CD4 +T cells and CD19 +B cells was investigated in the presence of fraxinellone. Osteoclastogenesis after fraxinellone treatment was evaluated by staining with tartrate-resistant acid phosphatase (TRAP) and by measuring the mRNA levels of osteoclastogenesis-related genes. Results Fraxinellone attenuated the clinical and histologic features of inflammatory arthritis in CIA mice. Fraxinellone suppressed the expression of interleukin-17, and T helper 17 cell-related transcription factors (RORγt and phosphorylated STAT3) in CD4 +T cells. CD19 +B cells showed lower expression of activation-induced cytidine deaminase (AID) and Blimp-1 after treatment with fraxinellone. The formation of TRAP-positive cells and the expression of osteoclastogenesis-related markers were reduced in the presence of fraxinellone. Inhibition of interleukin-17 and osteoclastogenesis was also observed in experiments using human peripheral mononuclear cells. Conclusions Fraxinellone alleviated synovial inflammation and osteoclastogenesis in mice. The therapeutic effect of fraxinellone was associated with the inhibition of cellular differentiation and activation. The data suggests that fraxinellone could be a novel treatment for inflammatory arthritis, including rheumatoid arthritis. References [1] Jiang Y, Li SP, Chang HT, Wang YT, Tu PF. Pressurized liquid extraction followed by high-performance liquid chromatography for determination of seven active compounds in Cortex Dictamni. Journal of chromatography A2006;1108:268–272. [2] Sun J, Wang X, Wang P, Li L, Qu W, Liang J. Antimicrobial, antioxidant and cytotoxic properties of essential oil from Dictamnus angustifolius. Journal of ethnopharmacology2015;159:296–300. [3] Wu XF, Ouyang ZJ, Feng LL, Chen G, Guo WJ, Shen Y, Wu XD, Sun Y, Xu Q. Suppression of NF-kappaB signaling and NLRP3 inflammasome activation in macrophages is responsible for the amelioration of experimental murine colitis by the natural compound fraxinellone. Toxicology and applied pharmacology2014;281:146–156. [4] Sun Y, Qin Y, Gong FY, Wu XF, Hua ZC, Chen T, Xu Q. Selective triggering of apoptosis of concanavalin A-activated T cells by fraxinellone for the treatment of T-cell-dependent hepatitis in mice. Biochemical pharmacology2009;77:1717–1724. [5] Jiang S, Nakano Y, Rahman MA, Yatsuzuka R, Kamei C. Effects of a Dictamnus dasycarpus T. extract on allergic models in mice. Bioscience, biotechnology, and biochemistry2008;72:660–665. Acknowledgements This research was supported by the Catholic Medical Centre Research Foundation made in the program year of 2014. Disclosure of Interest None declared

Background Systemic sclerosis (SSc) is a rare autoimmune disease characterised by vasculopathy and fibrosis of various organs including skin. Although SSc has high morbidity and mortality, evidences for disease modifying treatment are still lacking due to difficulties in performing clinical trials. Patient-specific induced pluripotent stem cells (iPSCs), which can differentiate into various cell types, are used in 3D organoid formation. Objectives We generated 3D skin organoid model from SSc-derived iPSCs by differentiating them into keratinocytes and fibroblasts. SSc-mimicking 3D skin organoid can be used in studies for disease modelling and drug screening. Methods Peripheral blood mononuclear cells (PBMCs) from patients with SSc were reprogrammed to iPSCs. SSc-derived iPSCs differentiated into keratinocytes and fibroblasts in vitro. Expression of markers for iPSCs, keratinocytes, and fibroblasts were determined by reverse transcription polymerase chain reaction (RT-PCR) analysis and immunofluorescence assay (IFA). 3D skin organoid using iPSC-derived differentiation cell line was generated by 3D culture system. Histologic analysis was performed on 3D skin organoid. SSc-derived 3D skin organoid was applied to SCID skin defect mice. Histologic analysis was also performed on SCID skin graft model. Results SSc-derived iPSCs formed colonies that resemble embryonic stem cells. Alkaline phosphatase staining showed undifferentiated state of iPSCs. Expression of iPSC markers was increased on SSc-iPSCs. Differentiated keratinocytes and fibroblasts from iPSCs highly expressed their markers for keratinocytes and fibroblasts, respectively. Dermis of SSc-derived 3D skin organoid was thicker and denser than that derived from healthy control. Epidermis and dermis of SCID skin graft model were thickened in those derived from SSc compared to those derived from healthy control. Conclusions Patient-derived 3D skin organoid and animal model well represented the characteristics of SSc. These models can serve as useful research tools to understand the disease and screen new drugs for SSc. Disclosure of Interest None declared

Influence of epidemiology and ethnicity on systemic expression of primary Sjogren syndrome in 9974 patients

Background Sjogren’s syndrome (SS) is a systemic autoimmune disease that affects exocrine glands and lymphoid organs. B cell hyperactivity and imbalance between T helper 17 (Th17) cells and regulatory T (Treg) cells are involved in pathogenesis of SS. Metformin, a commonly used anti-diabetic drug, is found to have immunomodulatory effect via AMP-activated protein kinase enhanced inhibition of mTOR-STAT3 signalling. Objectives We examined the therapeutic effect of metformin on SS by using animal model of SS, non-obese diabetic (NOD) mice. Methods Metformin (50 mg/kg) or vehicle (saline) was given per oral every day from 11 weeks after birth until 20 weeks. Salivary flow rate (SFR) was addressed on every 2 or 3 weeks between 11 weeks and 20 weeks. Histologic analyses of salivary gland and spleen were performed on week 20. Expression of Inflammatory cytokine was determined by immunohistochemistry analysis and real-time PCR. Flow cytometry was performed with peripheral blood to examine Th17 and Treg cells and germinal centre (GC) B cell populations. Serum immunoglobulin level was measured by enzyme-linked immunosorbent assay. Splenic cells of NOD mice were treated with metformin or vehicle in vitro and cultured for 3 days. Results SFRs of metformin-treated mice recovered, whereas SFRs of those treated with vehicle declined. Histologic examination of salivary gland showed decreased infiltration of lymphocytes and reduced expression of IL-6 and TNF-alpha in metformin-treated mice. Relative expression of IL-6, TNF-alpha, and IL-17 mRNA in salivary gland and spleen also declined in metformin-treated mice. Flow cytometric analysis revealed decreased Th1 and Th17 cells and increased Treg cells in peripheral blood of mice treated with metformin. In addition, GC B cells and immunoglobulin levels were reduced in peripheral blood of mice treated with metformin. Decreased Tfh cells and increased Tfr cells were observed from in vitro cultures of splenic cells treated with metformin. Conclusions Metformin controls B cell differentiation and keeps balance between Th17 and Treg cells in NOD mice, in addition to reducing lymphocytic infiltration and inflammatory cytokine expression in salivary gland. Metformin has potential therapeutic effects on SS. References [1] Lee SY, Moon SJ, Kim EK, Seo HB, Yang EJ, Son HJ, et al. Metformin Suppresses Systemic Autoimmunity in Roquinsan/san Mice through Inhibiting B Cell Differentiation into Plasma Cells via Regulation of AMPK/mTOR/STAT3. J Immunol2017;198–2661–70. [2] Pontarini E, Lucchesi D, Bombardieri M. Current views on the pathogenesis of Sjgren’s syndrome. Curr Opin Rheumatol2017. Disclosure of Interest None declared

Background Spondyloarthritis (SpA) is inflammatory arthritis, and interleukin (IL) −17 is crucial on pathogenesis of SpA. Type 17 helper T cell (Th17) is one of major IL-17 secreting cells. Signal transducer and activator of transcription (STAT)−3 signalling induces Th17 cell differentiation. Present study investigated the effect of protein inhibitor of activated STAT3 (PIAS3) on SpA pathogenesis. Methods Curdlan was injected to SKG ZAP-70W163C mice for SpA induction. Then PIAS3 or Mock vector was inserted to mice for 10 weeks. Clinical score and histologic scores of paw, spine, and gut were evaluated. Expressions of IL-17, tumour necrosis factor-α (TNF-α), STAT3, bone morphogenic protein (BMP) were measured. Confocal stain and flow cytometry were used to assess helper T cell differentiation. Results PIAS3 significantly diminished the histological scores of paw and gut. PIAS3 group displayed lesser expression of IL-17, TNF-α, and STAT3 in the paw, spine, and gut. BMP-2/4 expressions were lower in spine of PIAS3 group. Helper T cell differentiation was polarised toward upregulation of regulatory T cell (Treg) and downregulation of Th17 in the PIAS3 mice. Conclusions PIAS3 showed preventive effects in mice with SpA by suppressing peripheral arthritis and gut inflammation. Proinflammatory cytokines and Th17/Treg differentiation were controlled by PIAS3. Additionally BMPs were decreased in spine of PIAS3 mice. These findings suggest that PIAS3 could be a potential therapeutic choice of SpA treatment. References [1] Smith JA, Colbert RA: Review: The interleukin-23/interleukin-17 axis in spondyloarthritis pathogenesis: Th17 and beyond. Arthritis & rheumatology2014, 66:231–41. [2] O’Shea JJ, Lahesmaa R, Vahedi G, Laurence A, Kanno Y. Genomic views of STAT function in CD4+ T helper cell differentiation. Nature reviews Immunology2011;11:239–50. [3] Miossec P, Korn T, Kuchroo VK: Interleukin-17 and type 17 helper T cells. The New England journal of medicine2009;361:888–98. [4] Ruutu M, Thomas G, Steck R, Degli-Esposti MA, Zinkernagel MS, Alexander K, Velasco J, Strutton G, Tran A, Benham H, Rehaume L, Wilson RJ, Kikly K, Davies J, Pettit AR, Brown MA, McGuckin MA, Thomas R. beta-glucan triggers spondylarthritis and Crohn’s disease-like ileitis in SKG mice. Arthritis and rheumatism2012;64:2211–22. Acknowledgements None Disclosure of Interest None declared

Objectives To take a “high-definition” picture of the main features of primary Sjogren syndrome (SjS) following a worldwide data-sharing cooperative merging of international clinical SjS databases. Methods The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014 including leading clinical centres in SjS of the 5 continents that shared a harmonised data architecture and conducted cooperative online efforts to refine collected data of primary SjS patients fulfilling the 2002 classification criteria. Results By January 2018, the participant centres had included 10 475 patients from 22 countries, including 7637 (73%) patients from Europe, 1420 (14%) from America, 1186 (11%) from Asia, 167 (1.4%) from Australia and 65 (0.6%) from Africa. The cohort included 9781 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years. The frequencies of fulfilment of the 2002 criteria were 92% for dry eye, 94% for dry mouth, 83% for abnormal ocular tests, 82% for positive minor salivary gland biopsy, 78% for abnormal oral diagnostic tests and 76% for positive anti-Ro/La antibodies. The frequency of positive immunological markers at diagnosis was 79% for ANA, 73% for anti-Ro, 49% for RF, 45% for anti-La, 13% for low C3 levels, 14% for low C4 levels and 7% for cryoglobulins. Conclusions International data sharing-based projects merging disperse clinical registries may be essential tools to increase current knowledge and to improve patient care in specific systemic autoimmune diseases. Disclosure of Interest None declared

Background Hyposecretion of tears and saliva is the main phenotype of primary Sjogren’s syndrome (PSS). However, the prevalence and degree of ocular and oral involvement in PSS is highly variable. Objectives The aims of this study were 1) to investigate oral and ocular signs and changes in primary SS patients, and 2) to explore possible predisposing factors for moderate to severe oral or ocular signs in primary SS. Methods We analysed 140 participants from the Korean Initiative of PSS cohort who completed a two-year follow-up oral and ocular sign test. The severity of keratoconjunctivitis sicca (KCS) was determined by the Schirmer I test (Moderate-to-severe [MS],≤5 mm/5 min; mild [Mi],>5 mm/5 min). Salivary gland dysfunction (SGD) was determined by unstimulated whole salivary flow rate (UWS) (MS, UWS Results Among the 140 participants enrolled in this study, 108 (61%) were placed in the MS-KCS/MS-SGD group, 17 (24%) were in the MS-KCS/Mi-SGD, 15 (16%) were in the Mi-KCS/MS-SGD at the two-year follow-up. The MS-KCS/Mi-SGD group was younger than the other two groups, had a lower xerostomia inventory, and lower level of β2 microglobulin. Participants in the Mi-KCS/MS-SGD group had less hyperimmunoglobulinemia, rheumatoid factor (RF), antinuclear antibodies, anti-Ro, and anti-La antibodies. Older patients and those with positive RF, anti-Ro, or anti-La antibodies at baseline were more likely to have moderate to severe KCS at the two-year follow-up. Conclusions Patients with PSS and positive RF, anti-Ro, or anti-La antibodies at baseline may benefit from regular ophthalmology exams, even if they do not have KCS at baseline or dry eye symptoms. References [1] Le Gall M, Cornec D, Pers J-O, Saraux A, Jousse-Joulin S, Cochener B, et al. A prospective evaluation of dental and periodontal status in patients with suspected Sjogren’s syndrome. Joint Bone Spine. doi:http://dx.doi.org/10.1016/j.jbspin.2015.02.015. [2] Lim SA, Nam S, Kwok SK, Park SH, Chung SH. Serologic Markers Are Associated With Ocular Staining Score in Primary Sjogren Syndrome. Cornea2015;34:1466–70. [3] Shiboski CH, Baer AN, Shiboski SC, Lam M, Challacombe S, Lanfranchi HE, et al. Natural History and Predictors of Progression to Sjogren’s Syndrome Among Participants of the Sjogren’s International Collaborative Clinical Alliance Registry. Arthritis Care Res (Hoboken)2017. doi:10.1002/acr.23264. Acknowledgements We wish to thank So Young Kim, the research nurse for the KISS cohort, for her excellent support. Disclosure of Interest None declared

Axial spondyloarthritis (axSpA) is associated with low bone mineral density (BMD) and fractures, although the true fracture risk is unknown. The present study examined BMD and estimated the 10-year fracture risk in axSpA patients and matched controls and identified factors associated with a high fracture risk.In total, 240 axSpA patients and 1200 healthy controls from the fifth Korean National Health and Nutrition Examination Survey (KNHANES V), matched using propensity scores, were included. Dual-energy X-ray absorptiometry (DXA) was used to measure lumbar spine and right femur BMD. Ten-year risks of major osteoporotic and hip fractures were calculated using the Fracture Risk Assessment Tool (FRAX) in subjects aged ≥ 40 years. Multivariate linear regression models were used to explore factors associated with the 10-year fracture risk in axSpA patients.Hip and lumbar spine BMDs were lower in axSpA patients than in matched controls. Osteoporosis was present in 17% of axSpA patients and 3% of controls (p 0.001). Low BMD was present in 22% of axSpA patients and 4% of controls aged 50 years (p 0.001). Ten-year major osteoporotic and hip fracture risks were significantly higher among axSpA patients. High 10-year fracture risk was observed in 10% of axSpA patients and 1.7% of controls (p = 0.003). The severity of sacroiliitis was independently associated with both major osteoporotic and hip fracture risks (p = 0.006 and 0.026, respectively).Patients with axSpA presented more frequently with low BMD and a higher calculated 10-year fracture risk than matched individuals. The severity of sacroiliitis was independently associated with a high 10-year fracture risk in axSpA patients.

This study was performed to investigate the clinical characteristics of lupus cystitis and determine the risk factors and clinical outcomes of lupus cystitis in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed 1064 patients at Seoul St. Mary’s Hospital in Seoul, Korea, from 1998 to 2013. Twenty-four patients had lupus cystitis. Lupus cystitis was defined as unexplained ureteritis and/or cystitis as detected by imaging studies, cystoscopy, or bladder histopathology without urinary microorganisms or stones. Three-fourths of patients with lupus cystitis had concurrent lupus mesenteric vasculitis (LMV). The initial symptoms were gastrointestinal in nature for most patients (79.2%). High-dose methylprednisolone was initially administered to most patients (91.7%) with lupus cystitis. Two patients (8.3%) died of urinary tract infections. Sixty-five age- and sex-matched patients with SLE who were admitted with other manifestations were included as the control group. Patients with lupus cystitis showed a lower C3 level ( p = 0.031), higher SLE Disease Activity Index score ( p = 0.006), and higher ESR ( p = 0.05) upon admission; more frequently had a history of LMV prior to admission ( p

We investigated obstetric outcomes and comorbidities during pregnancy in females with preexisting lupus nephritis (LN) and identified predictors for renal flare. In cases of renal flare during pregnancy, we assessed the long-term post-delivery renal outcome. We performed a retrospective analysis of 183 systemic lupus erythematosus (SLE) pregnancies including blood chemistry, urinalysis, urinary protein, and disease activity recorded at prepregnancy, during pregnancy, and at one month, six months, and one year post-delivery. Pregnancies with preexisting LN had a greater frequency of adverse obstetric outcomes and maternal comorbidity. Renal flares occurred in 50.7% of pregnancies with preexisting LN, 89.2% of which were reactivations. Renal flare among pregnancies with SLE was predicted based on preexisting lupus nephritis (OR 17.73; 95% CI, 5.770–54.484), an active disease prior to pregnancy (OR 2.743; 95% CI, 1.074–7.004), and prepregnancy eGFR 2 (OR 11.151; 95% CI, 3.292–37.768). Persistent LN one year after delivery was observed in 33.3% of pregnancies. The median follow-up time after delivery was 5.9 (3.1–9.7) years and chronic kidney disease (CKD) occurred in 21.4% of pregnancies with renal flare. In patients with renal flare, failing to achieve a ≥ 50% reduction in urine protein levels within six months, longer total duration of renal flare, and acute kidney injury at renal flare was associated with CKD development. Females with preexisting LN should achieve remission before pregnancy. When patients experience renal flares during pregnancy, it is important to reduce the proteinuria level by >50% within six months and to achieve early remission for excellent long-term renal outcomes.

Objectives To analyse the influence of geolocation on the clinical systemic presentation of primary Sjogren syndrome (SjS) at diagnosis. Methods The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014. Centres were classified by continent, with an additional north-south sub-classification according to latitude (>or or or Results The highest baseline ESSDAI scores were reported from Southern vs Northern countries in Europe (7.2 vs 4.6, p Conclusions This study provides the first evidence for a strong influence of geolocation on the systemic phenotype of primary SjS at diagnosis. Geographical determinants should be considered as key variables when systemic disease is scored. Disclosure of Interest None declared

Background Osteoarthritis (OA) is a chronic joint disease with complex etiologies characterized by synovial inflammation, subchondral bone remodeling, and the formation of osteophytes, which leads to cartilage deterioration. Periodontitis (PD) is also a chronic inflammatory disease characterized by loss of periodontal ligament and alveolar bone. Recently, the association between OA and metabolic diseases has been proposed, and the association between several systemic diseases such as rheumatoid arthritis, metabolic syndrome and periodontitis has been also revealed. Objectives The aim of this study was to investigate the association between OA and PD in South Korea using data from the Korea National Health and Nutrition Examination Survey (KNHANES) during 2010–2014. Methods Cross-sectional data of 7,969 adults who completed the KHANES, and participated in both a periodontal examination and a knee imaging were analyzed. OA of knee was defined when a participant had knee arthralgia and showed radiographic change of Kellgren-Lawrence (KL) grade over 1. OA patients were grouped into mild (KL grade 1–2) and severe (KL grade 3–4) OA. The periodontal status was assessed by the Community Periodontal Index. Binary logistic regression analysis was performed according to the OA and PD status, severity of OA, and subgroups (age, gender) adjusting for the socio-demographics, oral health behaviors and status, smoking, and drinking. Results Of the 7,969 participants, 1408 (17.7%) had OA and 2987 (37.5%) had PD. OA and PD showed no significant association in overall analysis. However, in subgroup analysis, female patients with severe OA were more likely to have PD (adjusted odd ratio (OR) 1.377, P=0.0316); likewise, OR for severe OA in female patient with PD was 1.367. (P=0.0.54) Conclusions Severe OA and PD were associated with each other especially in female in the Korean population. Further prospective and experimental studies are necessary to identify the impact and mechanisms of association between severe OA and PD in female. References Zhuo Q, Yang W, Chen J, Wang Y. Metabolic syndrome meets osteoarthritis. Nature reviews Rheumatology 2012; 8(12): 729–37. Kwon YE, Ha JE, Paik DI, Jin BH, Bae KH. The relationship between periodontitis and metabolic syndrome among a Korean nationally representative sample of adults. Journal of clinical periodontology 2011; 38(9): 781–6. Disclosure of Interest None declared

Background There have been several studies which demonstrated the impact of birth on the risk of certain diseases such as asthma or cardiovascular diseases. However, rheumatic diseases have not yet been thoroughly investigated in terms of association with birth month. Objectives In this study, we sought to determine whether birth month or season could affect the risk of rheumatologic diseases. Methods The birth month patterns of patients with rheumatic diseases were compared with to those of general population. We utilized the claims data of Health Insurance Review and Assessment Service (HIRA) which covers nearly 90% of total population in Korea. The associations between birth month/season and 32 diseases were investigated using logistic regression. Results Our dataset included 17,247,458 (male 8,224,670; female 9,022,788) individuals from HIRA database from January, 1997 to August, 2015. Among 27 rheumatic diseases, 8 diseases including Crohn9s disease (CD), ulcerative colitis (UC), rheumatoid arthritis (RA), systemic lupus erythematosus, polymyalgia rheumatica (PMR), ankylosing spondylitis (AS), multiple sclerosis, gout, fibromyalgia (FMS) were significantly associated with birth month (P Conclusions We found significant impacts of birth month/season on various rheumatic diseases. Seasonal variation of infective agents, sun exposure or food ingestion during gestation or early infancy may explain the association between birth month/season and certain disease development. Disclosure of Interest None declared

We investigated the clinical and laboratory characteristics of pregnancies with systemic lupus erythematosus (SLE) and identified lupus flare predictors during pregnancy. Additionally, we examined lupus activity and pregnancy outcomes in SLE patients who continued, discontinued or underwent no hydroxychloroquine (HCQ) treatment during pregnancy. We retrospectively analyzed 179 pregnancies in 128 SLE patients at Seoul St. Mary’s Hospital, Korea, between 1998 and 2012 and then assessed the clinical profiles and maternal and fetal outcomes. Overall, 90.5% of pregnancies resulted in a successful delivery and were divided into two groups: those who experienced lupus flares (80 pregnancies, 44.7%) and those who did not (99 pregnancies, 55.3%). Increased preeclampsia, preterm births, low birth weight, intrauterine growth restriction (IUGR), and low 1-minute Apgar scores occurred in pregnancies with lupus flares compared to pregnancies in quiescent disease. Lupus flares were predicted by HCQ discontinuation, a history of lupus nephritis, high pre-pregnancy serum uric acid and low C4 levels. Our study indicates that achieving pre-pregnancy remission and continuing HCQ treatment during pregnancy are important for preventing lupus flares.

Objective The objective of this paper is to identify the risk factors for development of symptomatic osteonecrosis (ON) and predictors of total hip replacement (THR) among systemic lupus erythematosus (SLE) patients in Korea. Methods The medical records of 1051 patients with SLE were reviewed, and 73 patients with symptomatic ON were identified. Among them, 64 patients were eligible for the analysis. Sixty-four age- and sex-matched SLE patients without apparent ON were included as disease controls. The risk factors for development of symptomatic ON were identified by logistic regression analyses. The predictors of THR were determined by Cox proportional hazards regression analyses. Results Among 64 patients with ON, 59 had ON of the hip and 36 underwent THR. Independent risk factors for development of symptomatic ON included Cushingoid body habitus (OR 21.792 (95% confidence interval (CI) 2.594–183.083)), use of cyclophosphamide (OR 2.779 (95% CI 1.106–6.981)) and azathioprine (OR 2.662 (95% CI 1.143–6.200)). In the Cox proportional hazards model, only advanced radiological stage of ON (Association for Research on Osseous Circulation (ARCO) stage) was a statistically significant predictor of THR. In subgroup analysis with stage I–III ON, multivariate Cox regression analysis showed neuropsychiatric SLE (NPSLE) (HR 6.295 (95% CI 2.178–18.192)) and cumulative prednisolone dose in the first six months after ON diagnosis > 0.9 g (HR 3.238 (95% CI 1.095–9.58)) to be independent predictors. Conclusions Advanced ARCO stage at the onset of ON is an independent risk factor for THR in SLE patients with ON. In ARCO stage I–III ON, patients with NPSLE and those receiving > 0.9 g prednisolone during the first six months after the ON diagnosis are likely to require THR.

Renal relapse in patients with lupus nephritis (LN) is a risk factor for poor renal function. Therefore, there is a need to identify clinical and serological risk factors for renal relapse. A total of 108 patients with LN were enrolled in this study. All the subjects had achieved complete remission or partial remission following six months of induction therapy. We retrospectively analyzed their clinical and laboratory indices, final renal function, and kidney biopsy findings. The median follow-up period after LN diagnosis was 81 months. Renal relapse had occurred in 36 patients; it occurred in 38% and 46% of patients within five and 10 years after achievement of renal remission, respectively. There was no difference between the relapsed rate in patients with complete remission and that in those with partial remission. Clinical variables at LN onset and renal biopsy findings in the patients with sustained remission and relapsed patients were also not different. The probability of renal relapse was significantly higher in patients with an earlier age of onset of systemic lupus erythematosus (SLE) (≤ 28 years versus >28 years; HR 7.308, P = 0.001), seronegativity for anti-Ro antibody (seronegativity versus seropositivity; HR 3.514, P = 0.007), and seropositivity for anti-dsDNA antibody at six months after initiation of induction therapy (HR 8.269, P = 0.001). Our study demonstrated that early onset of SLE, seronegativity for anti-Ro antibody and increased anti-dsDNA antibody following six months of induction therapy independently predict renal relapse among the LN patients.

PURPOSE: To present the 1-year outcomes of combining monocular bi-aspheric ablation profile and contralateral monofocal LASIK in hyperopic patients with presbyopia. METHODS: In this retrospective case series, 36 consecutive patients (72 eyes) who underwent simultaneous bi-aspheric ablation (PresbyMAX: SCHWIND Eye-Tech-Solutions GmbH and Co KG, Kleinostheim, Germany) in the non-dominant eye and monofocal regular LASIK in the dominant eye for correction of hyperopia and presbyopia were reviewed for 1 year. Binocular uncorrected distance (UDVA), near (UNVA), corrected distance (CDVA), and distance corrected near (DCNVA) visual acuity and manifest refraction were analyzed postoperatively. RESULTS: At 1 year, the mean binocular UDVA improved significantly from 0.26 ± 0.25 to 0.039 ± 0.088 logMAR ( P < .001). Binocular UNVA also improved from 0.73 ± 0.30 to 0.10 ± 0.22 logRAD ( P < .001). Eighty-seven percent of patients achieved UDVA of 20/25 or better and 90% had UNVA of J3 or better. Simultaneous binocular distance and near vision of 20/25 and J2 or better was achieved in 70%. Only 17% of patients had a binocular DCNVA of J2 or better. No patient suffered from a loss of 2 lines of CDVA. Refractive stability was achieved for both eyes from 1 month postoperatively. The re-treatment rate was 14% for improvement of near vision within 6 months to 1 year. CONCLUSIONS: Presbyopic correction using monocular PresbyMAX combined with monofocal regular LASIK in the fellow eye is safe and acceptable in hyperopic patients. [ J Refract Surg . 2017;33(1):37–43.]

Colitis in patients with systemic lupus erythematosus (SLE) is quite rare. It can be caused by intestinal vasculitis, mesenteric vascular thrombosis, concomitant inflammatory bowel disease or infectious colitis. It is important to make an accurate and early diagnosis as the treatments for each condition differ and a delayed diagnosis can result in life-threatening complications. However, non-specific gastrointestinal symptoms make a timely diagnosis challenging. Amoebic colitis is a rare condition in patients with SLE. Here we present a case of fulminant amoebic colitis in a patient with SLE which was initially misdiagnosed as ischemic colitis due to intestinal vasculitis. Her colitis was complicated with multiple intestinal perforations, disseminated intravascular coagulation and acute respiratory distress syndrome; but in the end, the patient was successfully treated with metronidazole and paromomycin.