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Objectives The primary objective was to define the incidence of JSLE in children <16 years of age in the UK and Republic of Ireland (ROI). The secondary objective was to describe presenting features, classification criteria, initial management and disease damage in newly presenting JSLE patients. Methods A prospective JSLE epidemiological study was undertaken between September 2017 and September 2019 with support of the British Paediatric Surveillance Unit and other professional groups involved in diagnosis and management of JSLE patients. Treating consultants reported all cases of JSLE seen. A follow-up study at 1 year examined management and progression of disease and treatment. Results There were 124 incident cases included in the final analysis. Incidence was estimated using ACR-1997 classification criteria (0.36/100 000), SLICC-2012 classification criteria (0.41/100 000) and clinician expert opinion (0.46/100 000). A high disease burden was seen, with 71.0% of patients requiring ongoing systemic CS treatment at 1 year; 98.2% receiving immunomodulatory treatment; and 20.4% accruing damage in the year following diagnosis (predominantly neuropsychiatric-related), with substantial involvement from multiple speciality teams. Conclusions The minimum UK and ROI incidence of JSLE is between 0.36 and 0.46/100 000, depending on the case definition used. Challenges in classification of patients with JSLE are highlighted, but overall this study supports the use of SLICC-2012 classification criteria. The high levels of disease damage and ongoing CS use 1 year after diagnosis is concerning, highlighting the need for further interventions to improve outcomes in JSLE. [ABSTRACT FROM AUTHOR]

Objectives. The initiating cause of Behçet's disease (BD) is unknown, but an aberrant response to infection has been suggested. In this study, single nucleotide polymorphisms in Toll-like receptors (TLRs) and associated molecules that have a sentinel function at mucosal surfaces were analysed in patients with BD.Methods. TLR expression was determined by immunohistochemistry in buccal mucosal tissue from patients with BD, in tissue from patients with lichen planus (LP) or pyogenic granuloma (PG) as disease controls, or from healthy individuals. Using SSP-PCR we analysed SNP in CD14, TLR2, TLR4 and TIRAP (TIR domain-containing adaptor protein) in patients with BD from different geographical regions.Results. TLR expression was increased in buccal lesions from patients with BD compared with healthy controls; however, a similar increase was seen in lesion tissue from patients with LP or PG, suggesting that this was a generalized inflammatory response as opposed to a BD-specific response. SNP analysis showed no association between CD14, TLR2 or TLR4 polymorphisms. However, TIRAP 180Leu was significantly associated with BD in UK, but not Middle Eastern, patients.Conclusion. TLR expression showed no difference in tissue from patients with BD compared with either disease or healthy controls. Likewise, SNPs in TLR genes were no different from healthy controls. The association with the increased function variant of TIRAP suggests that encounter with a pathogen at mucosal sites will lead to increased cytokine production and tissue damage with persistence of mucosal lesions. [ABSTRACT FROM PUBLISHER]

The article presents abstracts of studies on connective tissue diseases which include the benefit of vitamin D in improving endothelial function in systemic lupus erythematosus (SLE), the response of SLE patients to rituximab and the safety of subcutaneous tocolizumab.

Poster presentation Tuesday 8 October Background Sjögren's syndrome, a chronic multisystem autoimmune disease is characterised by inflammation of the exocrine glands, principally the salivary and lacrimal glands resulting in xerostomia and xerophthalmia. It can also present with more extensive exocrinopathy as well as extra-glandular, systemic features. Defined as primary SS (pSS) when there is no association with other autoimmune disease, reported incidence and prevalence rates vary. Juvenile-onset pSS is believed to be rare; however it is likely that it is under-recognised and therefore under-diagnosed. To date there have been no studies reporting accurate incidence or prevalence of paediatric pSS (PpSS). Diagnosing pSS can be challenging. Many of the cardinal symptoms are non-specific and no gold standard biomarker of disease exists. Between 1965–2002 eleven diagnostic criteria sets were developed, none of which have gained universal acceptance or been validated for use in a paediatric population. Until recently, the most widely used criteria were those developed by the American-European Consensus Group. It remains well-recognised that international consensus on classification is important for standardisation, particularly in relation to research and monitoring treatment outcomes. With this in mind, the 2016 ACR/EULAR criteria were developed. However, there still remains a paucity of validated classification criteria for diagnosis of juvenile-onset pSS. Paediatric-focused criteria are required as features of pSS in children differ from those observed in adults. Children experience less dryness and more frequently experience systemic symptoms and parotid enlargement. Hence, simply applying adult criteria to a paediatric population may lead to mis- and/or under-diagnosis. The overarching aim of this study is to identify epidemiological, clinical and laboratory characteristics of PpSS in a multi-centre cohort of patients. Using this data our goal is to develop universally accepted classification criteria validated for use in a paediatric population. Going forward, this would enable standardisation of PpSS classification allowing for robust studies into disease pathogenesis, management and prognosis. Methods Inclusion criterion for entry into the UK/Ireland PpSS cohort study and repository is diagnosis of pSS before 18 years. A data collection pack will be sent to authors willing to participate. Information collected will include demographic, clinical and laboratory/histological data at diagnosis and subsequent follow-up appointments. Biological samples including blood, tears, saliva, urine, and glandular and extra-glandular (e.g. renal) tissue will be collected prospectively if available. Outcome measures related to disease activity and damage, as well as patient reported outcomes will also be collected at set time-points. Results No results to report. Conclusion The UK/Ireland PpSS cohort study and repository will capture data on a significant cohort of children with pSS providing a powerful resource to help improve our understanding of this rare disease. Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy, damage accrual and variable outcome of PpSS. Conflicts of Interest The authors declare no conflicts of interest. [ABSTRACT FROM AUTHOR]

The article cites studies on rheumatic disease and the kidney which include the management of lupus nephritis in the era of biologic therapies, the management of renal vasculitis and the prescription of rheumatic drugs.