Exposure to ultraviolet (UV) radiation is among the environmental factors that have been proposed and studied in association with systemic lupus erythematosus (SLE). While it is known that UV radiation exposure may exacerbate pre-existing lupus, it remains unclear whether UV exposure is a risk factor for the development of SLE. Experimental studies show a significant immunomodulatory role for UV radiation, but strong epidemiologic data regarding its role in triggering SLE onset are lacking. Further studies are needed to assess the role of UV radiation in relation to development of incident SLE, yet they are challenging to design due to difficulties in accurate exposure assessment, the heterogeneous nature of SLE, and the challenge of assessing photosensitivity, a feature of SLE, which often precedes its diagnosis.

BackgroundThis 4 phase project1 jointly supported by EULAR and ACR has led to draft criteria.2 ObjectivesTo simplify and validate the new criteria in a large international cohort.Methods23 expert centres each contributed up to 100 patients with SLE and with non-SLE diagnoses. Diagnoses were verified by 3 independent reviewers for 1,193 SLE and 1059 non-SLE patients. 500 randomly selected SLE and non-SLE patients formed the derivation cohort and the remainder the validation cohort.ResultsThe criteria were fine-tuned and simplified, using ANA of ≥1:80 as entry criterion and a classification threshold of 10.RenalClass III/IV nephritis10Class II/V nephritis8Proteinuria≥0.5 g/day4 Specific antibodiesAnti-Sm orAnti-dsDNA6Muco-cutaneousACLE6SCLE orDLE4Alopecia or oral ulcers2SerosaAcute pericarditis6Effusion5Musculo-skeletalArthritis6CNSSeizures5Psychosis3Delirium2BloodAutoimmune hemolysis or thrombocytopenia4Leukopenia3ComplementLow C3 and C44Low C3 or C43Anti-phospholipidAnti-Cardiolipin or anti-β2-GPI or lupus anticoagulant2ConstitutionalFever2Sensitivity was close to the SLICC 2012 criteria, specificity maintained at the level of the ACR 1997 criteria. This performance was independently confirmed in the validation cohort.ACR 1997 criteriaSLICC criteriaNew criteria DerivationSensitivity84.6396.8198.00Specificity95.2090.0096.40ValidationSensitivity82.7696.7096.12Specificity93.3883.6293.38ConclusionsThe new criteria developed with EULAR/ACR support achieved sensitivity close to the SLICC criteria, while maintaining the specificity of the ACR criteria.References[1] Aringer, et al. Ann Rheum Dis2017;76(S2):4.[2] Tedeschi, et al. Ann Rheum Dis2017;76(S2):50.Disclosure of InterestNone declared

Objective The chronicity and severity of childhood onset Systemic Lupus Erythematosus (cSLE) necessitate effective transition from pediatric to adult providers. We studied transition outcomes in a cSLE cohort. Methods We identified patients at an adult Lupus clinic diagnosed with SLE ≤ 18 years who had been followed by a pediatric rheumatologist. Data extracted from the first 3 years in adult care (“post-transition period”) included: sociodemographics, depression, anxiety, SLE manifestations, SLE Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborating Clinics/ACR Damage Index for SLE (SLICC) scores, non-adherence, and gaps in care (no appointments in the recommended time frame). Multivariable logistic regression analyses for predictors of: (1) time between pediatric and adult providers, (2) gaps in care, (3) unscheduled utilization (Emergency Department visits and admissions) (4) depression and/or anxiety were performed, as was a multivariable Poisson regression analysis for number of missed appointments. Results In 50 patients, SLEDAI scores were stable (mean 5.7 ± 5.0 at start vs. 4.7 ± 4.8 at year 3, p=0.2), but SLICC scores increased (0.46 ± 0.84, vs. 0.78 ± 1.25, p=0.01). Depression and anxiety increased significantly (10% vs. 26%, p=0.02). Mean time from last pediatric to first adult provider visit was almost 9 months (253 ± 392 days). Nearly 75% of patients had ≥1 gap in care. White race, low education level and non-adherence were significantly associated with missed appointments. Conclusion Despite moderate disease activity in this cSLE transition cohort, prolonged time between pediatric and adult providers and gaps in care in the post-transition period occurred. Anxiety and depression were frequently reported. Future work should identify methods to improve transition.

Systemic lupus erythematosus (SLE) is a complex multisystem autoimmune disease whose pathogenesis is thought to involve both genetic and environmental factors. It is possible that common environmental exposures, such as cigarette smoking and alcohol consumption, might modify risk of disease development in certain individuals. Here we aim to review the epidemiologic evidence related to the association of cigarette smoking, alcohol consumption and the risk of developing SLE. A growing body of evidence suggests that cigarette smoking confers a short-term increased risk of SLE in genetically susceptible individuals. On the other hand, alcohol consumption in moderate doses may have a protective effect against the development of SLE, although this is still debated. We also have reviewed proposed mechanistic explanations underlying the role of cigarette smoking and alcohol consumption in SLE pathogenesis.