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Background Behçet’s disease (BD), a chronic systemic vasculitis, has distinct geographical and ethnic variation. Data regarding the epidemiology of patients with BD in the U.S. are limited; therefore, we sought to describe BD patient characteristics and medication use in the U.S., and compared them with data from patients from endemic regions. Methods We conducted a cross-sectional study using data from the RISE registry (2014–2018). Patients aged ≥ 18 years with BD were included. Sociodemographic and treatment information was extracted. We compared patients from the RISE registry to data from other published studies of patients with BD from endemic areas. Results One thousand three hundred twenty-three subjects with BD from the RISE registry were included. Mean age was 48.7 ± 16.3 years, female to male ratio was 3.8:1, and 66.7% were White. The most frequently used medications included glucocorticoids (67.6%) and colchicine (55.0%). Infliximab and adalimumab were the most used biologics (14.5% and 14.1%, respectively); 3.2% of patients used apremilast. The RISE registry had more women (79.3%), and patients were older compared to previously published BD studies from endemic areas. Methotrexate and TNFi were more commonly reported in RISE (21.8% and 29.4%) compared to studies from Egypt and Turkey. Colchicine, cyclosporine, and cyclophosphamide were more commonly used in cohorts from Egypt, Turkey, and Iran. Conclusions Findings from the largest BD dataset in the U.S. suggest that BD patients are predominantly female. Further research is needed to explore the reasons for the higher prevalence of BD among women in the U.S. and its possible impact on disease severity and management.

Objective Immunosuppressant drugs can increase the risk of hepatitis B virus (HBV) and hepatitis C virus (HCV) and tuberculosis (TB) reactivation. Using the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) registry, we examined pre-treatment screening among new users of biologic or targeted synthetic disease modifying drugs (DMARDs). Methods Data, derived from RISE, included patients ≥ 18 years old who were new users of biologic or targeted synthetic DMARDs. We developed quality measures related to pre-treatment screening for HBV, HCV, and TB in addition to a “composite” measure for all applicable tests. We assessed patient-level screening rates, practice-level variation among practices reporting on ≥ 20 patients, and the frequency of positive results. Results We included 26,802 patients across 213 rheumatology practices nationwide. Patients were 58 (14) years old, 75.9% female; 59.6% had rheumatoid arthritis, and TNFi were the most common index DMARDs (64.9%). Overall, 44.8% and 40.5% patients had any documented HBV or HCV screening, respectively, prior to the index date; 29.7% had TB screening in the year prior to drug start. Only 15.5% had documentation of screening for all appropriate infections prior to drug start. Practice-level performance on the composite measure was low (range 0 to 48.3%). 2.4% of screening tests were positive. Conclusion We found gaps in documentation of key safety measures among practices participating in RISE. Given the small but significant number of patients with active or latent infections that pose safety risks, developing standardized and reliable strategies to capture safety screening measures is paramount.

Background Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations. Case presentation Case 1 presented with mononeuritis multiplex and characteristic skin findings. Case 2 presented with thunderclap headache and myelopathy due to spinal artery aneurysm rupture. Both patients experienced disease remission upon treatment. Case 3 presented with headache and bulbar symptoms due to partially thrombosed intracranial aneurysms, followed by systemic manifestations related to visceral aneurysms. She demonstrated clinical improvement with treatment, was lost to follow-up, then clinically deteriorated and entered hospice care. Conclusions Although the peripheral manifestations of PAN are well-known, PAN association with CNS neurovascular disease is relatively underappreciated. Clinician awareness of the spectrum of neurologic disease is required to reduce diagnostic delay and promote prompt diagnosis and treatment with immunosuppressants.

Although multiple national quality measures focus on the management and safety of rheumatoid arthritis, few measures address the care of patients with systemic lupus erythematosus (SLE). Our objective was to apply a group of quality measures relevant to the care of patients with SLE, and we used the American College of Rheumatology's Rheumatology Informatics System for Effectiveness (RISE) registry to assess nationwide variations in care.The data derived from RISE and included patients who had ≥2 visits with SLE codes ≥30 days apart in 2017-2018. We calculated performance on 5 quality measures: renal disease screening, blood pressure assessment and management, hydroxychloroquine (HCQ) prescribing, safe dosing for HCQ, and prolonged glucocorticoid use at doses of7.5 mg/day. We reported performance on these measures at the practice level. We used logistic regression to assess independent predictors of performance after adjusting for sociodemographic and utilization factors.We included 27,567 unique patients from 186 practices; 91.7% were female and 48% White, with a mean age of 53.5 ± 15.2 years. Few patients had adequate screening for the development of renal manifestations (39.5%). Although blood pressure assessment was common (94.4%), a meaningful fraction of patients had untreated hypertension (17.7%). Many received HCQ (71.5%), but only 62% at doses of ≤5.0 mg/kg/day. Some received at least moderate-dose steroids for ≥90 days (18.5%). We observed significant practice variation on every measure.We found potential gaps in care for patients with SLE across the US. Although some performance variation may be explained by differences in disease severity, dramatic differences suggest that developing quality measures to address important health care processes in SLE may improve care.

OBJECTIVE To evaluate time trends in mortality for hospitalized adults with systemic lupus erythematosus (SLE) compared to the general hospitalized population (GHP), and to identify factors associated with increased risk of death among hospitalized SLE patients. METHODS We used the National (Nationwide) Inpatient Sample to estimate all-cause mortality for adults discharged from community hospitals in the US between 2006 and 2016. Poisson regression models were used to estimate the risk of in-hospital death among all patients, including demographic characteristics, socioeconomic factors, comorbidity score, hospital region, SLE diagnosis, and race/ethnicity as covariates. RESULTS Among 340,467,049 hospitalizations analyzed, 1,903,279 had a discharge diagnosis of SLE. In adjusted analysis, the risk of inpatient death decreased among hospitalizations for patients with SLE from 2.2% to 1.5% (P < 0.001) between 2006 and 2016. All of the decrease in SLE mortality occurred between 2006 and 2008; after 2008, mortality stabilized at a rate statistically similar to the GHP. Hospitalizations for Black, Hispanic, and Asian/Pacific Islander patients with SLE were more likely to end in death compared to hospitalizations for either White patients with SLE or individuals of the same non-White race/ethnicity without SLE. CONCLUSION In the largest study of in-hospital SLE mortality published to date, we found significant improvements in mortality for hospitalized patients with SLE in the US from 2006 until 2008, after which mortality stabilized at a level similar to that of the GHP. Our results also demonstrate a persistently high mortality burden among Black and Hispanic patients with SLE in the US and contribute new data revealing high mortality among Asian/Pacific Islander patients with SLE.

Background An estimated 22% to 28% of US SLE patients are hospitalised each year.1 SLE and other connective tissue diseases have been ranked the sixth most likely conditions to result in 30 day readmission to the hospital.2 Objectives To determine if the number of SLE patient hospital admissions, primary reasons for hospitalisation, and mortality have changed over time. We also sought to evaluate changes in immunosuppressant use in these patients. Methods A retrospective chart review was conducted on all adult patients with SLE admitted to the University of California San Diego Medical Centre in 2005 and 2013. Patients were identified by SLE billing code 710.0 of the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM). Results The number of yearly SLE patient hospitalizations decreased from 178 to 86 between the two times of observation. Infection was the most common reason for hospitalisation accounting for 39.9% of hospitalizations in 2005 versus 31.4% of hospitalizations in 2013 (p=0.29). Lupus flare accounted for 9.6% of admissions in 2005 versus 8.1% of admissions in 2013 (p=0.722). SLE patient mean length of hospital stay decreased from 7.6 days to 6.4 days compared to all patient length of stay which decreased from 6 days to 5.75 days. Seven patients (3.9%) died during their hospitalisation in 2005 as opposed no inpatient deaths in 2013. Of the 261 admissions between 2010 and 2013, six admissions resulted in death (2.3%). Corticosteroid use decreased (79.8% to 61.6%, p=0.109), while hydroxychloroquine (27.0% to 59.3%, p Conclusions Hydroxychloroquine and leflunomide use in hospitalised patients more than doubled from 2005 to 2013 with statistical significance. Rise in mycophenolate mofetil use and drop in corticosteroid use trended toward statistical significance. Number of hospitalizations, length of stay, and mortality decreased. Infection was the primary reason for inpatient hospitalisation and there was a decrease in admission for both infections and SLE flares between the time points although this did not reach statistical significance. We hypothesise that changing medication prescribing patterns may have altered the rate of damage accrual and mortality in SLE patients, and plan to perform a national study to further evaluate SLE hospitalisation data on a larger scale. References [1] Chakravarty EF, Bush TM, Manzi S, et al. Prevalence of adult systemic lupus erythematosus in California and Pennsylvania in 2000: estimates obtained using hospitalization data. Arthritis Rheum2007;56(6):2092–2094. [2] Elixhauser A, Steiner C. Readmissions to U.S.Hospitals by Diagnosis,2010: Statistical Brief #153. Healthcare Cost and Utilization Project (HCUP) Statistical Briefs. Agency for Healthcare Policy and Research. URL: http://www.ncbi.nlm.nih.gov/books/NBK52651/ [Accessed May 2016]. Disclosure of Interest None declared

Objectives To describe how immunosuppressant use and hospitalisation patterns for SLE have evolved by comparing admission statistics at one academic centre between 2005 and 2013. Methods We identified admissions for SLE and for all hospitalised patients by using the hospital electronic database. For adult patients with SLE, a comprehensive chart review was conducted to identify primary indications for hospitalisation, in-hospital mortality, mean length of stay and immunosuppressant use. Results The number of yearly SLE patient hospitalisations decreased from 178 to 86 between the two times of observation. Infection was the most common reason for hospitalisation accounting for 39.9% of hospitalisations in 2005 versus 31.4% of hospitalisations in 2013 (p=0.29). Lupus flare accounted for 9.6% of admissions in 2005 versus 8.1% of admissions in 2013 (p=0.72). Seven patients died during their hospitalisation (3.9% of admissions) in 2005 as opposed to no inpatient deaths in 2013. Of the 261 admissions between 2010 and 2013, six admissions resulted in death (2.3% of admissions). SLE patient mean length of hospital stay decreased from 7.6 days to 6.4 days (p=0.36) compared with all patient length of stay, which decreased from 6 days to 5.8 days. Corticosteroid use decreased (79.8% to 61.6%, p=0.11) while hydroxychloroquine (27.0% to 59.3%, p

Figure 7. Knocking down NFkB leads to diminished IMP-3 expression. HNSCCs are notably aggressive cancers which cause death primarily due to metastases2. Metastasis involves a complex change in epithelial cell behavior wherein the cell locally invades through the epithelial basement membrane, enters blood or lymph vessels, travels through the circulation, exits the vessel, and replicates at a distant organ. Growing evidence suggests such enhanced mobility requires the cell undergo an epithelial-mesenchymal transition. EMT is a normal part of embryonic development. Therefore, it has been hypothesized that in adults the reactivation of genes involved in embryonic EMT underlies the mechanism of cancer metastasis3.