In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in lifethreatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.

The vasculitides are a heterogeneous group of conditions typified by their ability to cause vessel inflammation with or without necrosis. They present with a wide variety of signs and symptoms and, if left untreated, carry a significant burden of mortality and morbidity. The ANCA-associated vasculitides (AAV) are three separate conditions - granulomatosis with polyangiitis (GPA - formerly known as Wegener’s granulomatosis); microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA - previously known as Churg-Strauss Syndrome). This review examines recent developments in the pathogenesis and treatment of AAV

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Although this pilot study demonstrates that treatment with ETN 25 mg was less effective at maintaining treatment response in the stepdown phase, 52% of participants maintained treatment response. Future research should address which patients are suitable for tapering.

BackgroundThe timing of the risk factors cigarette smoking, alcohol and obesity in the development of Barrett’s esophagus (BE) and esophageal adenocarcinoma (EAC) is unclear.AimsTo investigate these exposures in the aetiology of BE and EAC in the same population.MethodsThe cohort included 24,068 men and women, aged 39–79 years, recruited between 1993 and 1997 into the prospective EPIC-Norfolk Study who provided information on anthropometry, smoking and alcohol intake. The cohort was monitored until December 2008 and incident cases identified.ResultsOne hundred and four participants were diagnosed with BE and 66 with EAC. A body mass index (BMI) above 23 kg/m2 was associated with a greater risk of BE [BMI ≥23 vs. 18.5 to <23, hazard ratio (HR) 3.73, 95 % CI 1.37–10.16], and within a normal BMI, the risk was greater in the higher category (HR 3.76, 95 % CI 1.30–10.85, BMI 23–25 vs. 18.5 to >23 kg/m2). Neither smoking nor alcohol intake were associated with risk for BE. For EAC, all BMI categories were associated with risk, although statistically significant for only the highest (BMI >35 vs. BMI 18.5 to <23, HR 4.95, 95 % CI 1.11–22.17). The risk was greater in the higher category of a normal BMI (HR 2.73, 95 % CI 0.93–8.00, p = 0.07, BMI 23–25 vs. 18.5 to >23 kg/m2). There was an inverse association with ≥7 units alcohol/week (HR 0.51, 95 % CI 0.29–0.88) and with wine (HR 0.49, 95 % CI 0.23–1.04, p = 0.06, drinkers vs. non-drinkers).ConclusionsObesity may be involved early in carcinogenesis and the association with EAC and wine should be explored. The data have implications for aetiological investigations and prevention strategies.

ObjectivesTo determine the impact of COVID-19 pandemic social restriction measures on people with rheumatic and musculoskeletal diseases (RMDs) and to explore how people adapted to these measures over time.DesignMixed-methods investigation comprising a national online longitudinal survey and embedded qualitative study.SettingUK online survey and interviews with community-dwelling individuals in the East of England.ParticipantsPeople in the UK with RMDs were invited to participate in an online survey. A subsection of respondents were invited to participate in the embedded qualitative study.Primary and secondary outcome measuresThe online survey, completed fortnightly over 10 weeks from April 2020 to August 2020, investigated changes in symptoms, social isolation and loneliness, resilience and optimism. Qualitative interviews were undertaken assessing participant’s perspectives on changes in symptoms, exercising, managing instrumental tasks such a shopping, medication and treatment regimens and how they experienced changes in their social networks.Results703 people with RMDs completed the online survey. These people frequently reported a deterioration in symptoms as a result of COVID-19 pandemic social restrictions (52% reported increase vs 6% reported a decrease). This was significantly worse for those aged 18–60 years compared with older participants (p=0.017). The qualitative findings from 26 individuals with RMDs suggest that the greatest change in daily life was experienced by those in employment. Although some retired people reported reduced opportunity for exercise outside their homes, they did not face the many competing demands experienced by employed people and people with children at home.ConclusionsPeople with RMDs reported a deterioration in symptoms when COVID-19 pandemic social restriction measures were enforced. This was worse for working-aged people. Consideration of this at-risk group, specifically for the promotion of physical activity, changing home-working practices and awareness of healthcare provision is important, as social restrictions continue in the UK.

To conduct a meta-analysis of published data of the effectiveness of drug treatment in giant cell arteritis (GCA) to provide evidence to support the optimal use of glucocorticoids (GCs) and adjunct therapy. MEDLINE, CENTRAL and EMBASE searches were used to identify randomised control trials on the treatment of GCA. Studies included were trials in which: (1) the participants were classified as having GCA by the 1990 ACR criteria or biopsy proven disease; (2) parallel-group randomised control of at least 16 weeks duration had been conducted with at least 20 participants; (3) the design included either alternative adjunct immunosuppressant regimens, alternative GCs dosing or routes of administration; and (4) outcome data was included on either relapse rates or rates of infection. One thousand eight hundred thirty-six articles were retrieved, of which only 37 met the primary inclusion criteria. Sixteen of these studies reported some information about the GCs or adjuvant regimen used. Only ten studies were of sufficient quality to be included in the meta-analysis. Together these comprised 638 participants of which 72 % were female. Three studies compared various GCs regimens, with two comparing IV GCs, the latter showing a marginal benefit with respect to relapse (risk ratio (RR) = 0.78, 95 % CI = 0.54 to 1.12) but a greater risk of infection (RR = 1.58, 95 % CI = 0.90 to 2.78). Another three used methotrexate as an adjunctive agent and showed marginal benefit with respect to relapse (RR = 0.85, 95 % CI = 0.66 to 1.11). The remaining four trials compared prednisolone to dapsone, infliximab, adalimumab and hydroxychloroquine, respectively. There are various clinical trials of varying quality. The results from this meta-analysis show that the use of adjunct agents is not associated with improved outcome.

BackgroundTo update community-based prevalence values for Polymyalgia Rheumatic (PMR) and Giant Cell Arteritis (GCA) using case record review supplemented by population survey and subsequent clinical review.MethodsClinical data were obtained from case records of a large primary care practice in Norfolk, UK and reviewed for diagnoses of GCA and PMR. In addition postal survey was carried out to capture potentially undiagnosed cases within the practice population. Those screening positive for potential diagnoses of GCA and PMR were invited for clinical review. A cumulative prevalence estimate was subsequently calculated on those diagnosed within the GP practice and subsequently on those fulfilling the various published classification criteria sets. The date of the database lock and mail merge was March 2013.ResultsThrough detailed systematic review of 5,159 GP case records, 21 patients had a recorded diagnosis of GCA and 117 had PMR.No new cases were identified among 2,227 completed questionnaires returned from the population survey of a sample of 4,728. The resulting cumulative prevalence estimate in those aged ≥ 55 years meeting the ACR classification criteria set for GCA was 0.25 % (95 % CI 0.11 to 0.39 %) and for five published criteria sets for PMR ranged from 0.91 to 1.53 % (95 % CI ranges 0.65 %, 1.87 %). The prevalence of both conditions was higher in women than in men and in older age groups.ConclusionThis study provides the first UK prevalence estimate of GCA and PMR in over 30 years and is the first to apply classification criteria sets.