Max Yates

EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis

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 life-threatening 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.

Oncological Emergency Admissions to the Norfolk and Norwich University Hospital: An Audit of Current Arrangements and Patient Satisfaction

AIMS To assess current emergency admission pathways for patients with cancer, reasons for admission, subsequent management and patient perceptions of and satisfaction with the care they received. MATERIALS AND METHODS A prospective audit was carried out of emergency admissions of patients with cancer over a 3-month period from the beginning of October to the end of December 2007, collecting data at two time points a week, giving a total of 60 patients. The date, time and place of admission, patient demographics (age, gender) and reasons for admission, diagnosis and care pathway were analysed. Questionnaires were completed by a subgroup of 12 patients to assess satisfaction with care. RESULTS The mean age was 63.62 years with a range of 29-85 years. Thirty patients were 65 years or older. Lung, bowel, breast, prostate and oesophageal cancers were the most prevalent in terms of emergency admissions. Admissions were grouped into admission due to cancer progression (30/60), treatment-related reasons (20) or for other medical conditions (10). Specialist investigation or management was required by all. Twenty-nine patients were admitted to the Emergency Assessment Unit, 27 were admitted directly to the oncology ward and four patients were admitted through the Accident and Emergency Department. Three patients might have been more appropriately admitted to palliative care services. There was correlation with patient satisfaction and admission to the oncology ward rather than through the Emergency Assessment Unit first. CONCLUSION The cancer burden is increasing, with more patients with cancer being admitted as emergencies. Patients seem to favour continuity of care and admission to an oncology ward directly. Further research needs to be carried out in this area as to how best care may be achieved, taking into account local resources and the changes in practice that have come from continuing treatment much longer into the course of the disease. The organisation of healthcare services has to take account of patient preference to balance efficiency and patient satisfaction. Training in palliative oncology may need to be extended to other groups than oncologists.

ANCA-associated vasculitis

ABSTRACT 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 antineutrophil cytoplasmic antibody (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, focusing on developments in treatment following the introduction of rituximab, in particular.

Is Etanercept 25 mg Once Weekly as Effective as 50 mg at Maintaining Response in Patients with Ankylosing Spondylitis? A Randomized Control Trial.

Objective. To investigate, in a pilot randomized controlled trial, whether etanercept (ETN) 25 mg once weekly is effective at maintaining a clinical response in patients with ankylosing spondylitis (AS) who have responded to the standard 50 mg dose. Methods. Adults with AS not responding to conventional therapies were prescribed ETN 50 mg once weekly for 6 months. Responders as defined by the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) were randomly assigned to taper to 25 mg once weekly or continue on 50 mg and followed for a further 6 months. The primary outcome measure was maintenance of a 50% reduction in the BASDAI or fall in BASDAI by ≥ 2 units and a ≥ 2-unit reduction in BASDAI spinal pain as measured on a 10-point visual analog scale at 6 months postrandomization. Results. Of 89 patients assessed for eligibility, 59 were enrolled; 47 (80%) had sufficient clinical response and were eligible for randomization, 24 were assigned to continue receiving ETN 50 mg, and 23 to taper to 25 mg. After 6 months, 20 (83%) of the 50 mg arm maintained clinical response compared with 12 (52%) of the 25 mg arm (a difference of −31%, 95% CI −58% – −5%). Conclusion. 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.

Validation of the EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis by disease content experts

The European League Against Rheumatism recommendations for the management of antineutrophil cytoplasmic antibody-associated vasculitis have been recently published. Unique to recommendation development, they were also voted on by members of a learned society. This paper explores the wider validity of the recommendations among people who self-identify as clinicians caring for patients with vasculitis. In addition to the task force, a learned society (European Vasculitis Society—EUVAS) was invited, through online survey, to rate independently the strength of evidence of each recommendation to obtain an indication of the agreement among the final target audience and ultimate end-users of the recommendations. The survey took place in June 2015. Of the 158 EUVAS members surveyed, there were 88 responses (55.7%). There was a large degree of agreement in the voting patterns between EUVAS survey participants and task force members. Notable exceptions were lower grades for the recommendation of the use of rituximab for remission induction in patients with eosinophilic granulomatosis with polyangiitis and for methotrexate and mycophenolate mofetil as remission maintenance agents in patients with granulomatosis with polyangiitis/microscopic polyangiitis by EUVAS members. These results are encouraging and suggest that the voting patterns of the task force are representative of the wider vasculitis community. We recommend future recommendations adopt this approach for data/expert-based treatment guidelines, especially for multisystem diseases.

The association of vascular risk factors with visual loss in giant cell arteritis

Objective. Blindness is a recognized complication of GCA; however, the frequency of and risk factors for this complication have not been firmly established. The aim of this study was to examine the incidence and determinants of blindness in patients with GCA, using a large international cohort. Methods. The analysis was conducted among subjects recruited into the Diagnosis and Classification Criteria in Vasculitis Study. The study captures consecutive patients presenting to clinic‐based physicians. New‐onset blindness was assessed 6 months after diagnosis by completion of the Vasculitis Damage Index. Logistic regression analysis was used to assess the association between blindness and clinical variables. Results. Of 433 patients with GCA from 26 countries, 7.9% presented with blindness in at least one eye at 6 months. Risk factors identified at baseline for blindness at 6 months were identified and included prevalent stroke [odds ratio (OR) = 4.47, 95% CI: 1.30, 15.41] and peripheral vascular disease (OR = 10.44, 95% CI: 2.94, 37.03). Conclusion. This is the largest study to date of subjects with incident GCA and confirms that blindness remains a common complication of disease and is associated with established vascular disease. Trial registration: ClinicalTrials.gov, http://clinicaltrials.gov, NCT01066208.

Dietary Fat Intake in the Aetiology of Barrett's Oesophagus and Oesophageal Adenocarcinoma – Data From a Prospective Cohort Study (EPIC-Norfolk) Using 7-Day Food Diary Data

Introduction Dietary fat may be involved in the aetiology of both Barrett9s oesophagus (BO) and oesophageal adenocarcinoma (OAC) through its direct and indirect effects on increasing oesophageal reflux and second the release of adipose derived hormones. The aim of this investigation was to conduct the first prospective cohort study of dietary fat in the aetiology of these diseases, using nutritional data derived from 7-day food diaries (7-DFD). Methods A total of 23 658 healthy men and women were recruited into EPIC-Norfolk (European Prospective Investigation In to Cancer and Nutrition) between the years 1993 and 1997. Participants completed 7-DFDs at recruitment which recorded detailed information on food types consumed, brands, quantities and frequency of intake and cooking methods. The diaries were coded by nutritionists using a computer programme containing information on 11 000 food items and 55 000 portion sizes. Participants were followed up to identify those who had a new diagnosis of either BO or OAC and the diagnoses confirmed by reviewing the medical records. Hazard ratios were estimated using Cox regression for quintiles of total fat, saturated fat and total polyunsaturated fat (PUFA) intakes, for BO and OAC respectively, adjusted for age, gender, smoking, body mass index and total energy intake. Results In the cohort, 80 participants had endoscopy showing a new diagnosis of BO (80% men, median age=69.4 years range 41.3–84.4 years) and a further 58 were diagnosed with OAC (84% men, median age=73 years, range 52–86 years). The risk of OAC was positively associated with a higher fat intake (highest vs lowest quintiles HR=3.77, 95% CI=0.83 to 17.03, p=0.085, trend HR=1.54, 95% CI=1.08 to 2.19) and saturated fat intake (trend HR=1.35, 95%, 95% CI=1.01 to 1.79), but not with PUFAs (trend HR=1.11, 95% CI=0.87 to 1.42). For BO, there were no associations with either: total fat, saturated fat or PUFAs. Conclusion The data, together with plausible biological mechanisms, support a role for total fat and saturated fatty acids in the aetiology of OAC. Their role in BO needs further clarification as this sub-group were participants diagnosed as a result of developing symptoms and undergoing gastroscopy. Future epidemiological work should measure dietary fat intake when investigating the aetiology of this aggressive cancer. This has implications for potential public health interventions lowering dietary fat to reduce the incidence of OAC.

Does the co-existence of acute anterior uveitis and chronic back pain prompt primary care physicians to consider a diagnosis of axial spondyloarthritis?

We read with interest the article by Haroon et al 1 indicating that approximately 40% of patients with acute anterior uveitis (AAU) have undiagnosed spondyloarthritis. Unfortunately, delay to diagnosis remains a huge challenge in axial spondyloarthritis (axSpA) despite the publication of the Assessment of SpondyloArthritis international Society (ASAS) classification criteria, which formally recognises the role of MRI in diagnosis.2 We recently reported that the mean delay in diagnosis among 1193 patients attending two large secondary care centres in the UK was 8.5 years.3 Despite a 51% increase in new diagnoses since 2009, the delay in diagnosis has not improved and the presence of AAU was associated …

Incidence of inflammatory polyarthritis in polymyalgia rheumatica: a population-based cohort study

The relationship between polymyalgia rheumatica (PMR) and inflammatory polyarthritis (IP) remains a source of debate in rheumatology: although both conditions have been classified separately as distinct entities, they share many clinical features.1–4 It remains unclear whether synovitis in IP is part of a spectrum of PMR, or if the symptoms of PMR are early manifestations of a distinct diagnosis of IP. Alternatively, the arthritis that develops in PMR might represent a phenotypic transformation in susceptible individuals. We examined the risk of IP following the diagnosis of PMR in the data from the European Prospective Investigation into Cancer and Nutrition (EPIC)-Norfolk study, a prospective population-based cohort.5 Incident cases of PMR were identified retrospectively among 24 068 volunteers enrolled after 2002 by (1) free-text questionnaire responses at baseline, 18 months, and at 3, 10 and 13 years; (2) linkage to hospital electronic discharge summaries containing International Classification of Diseases codes; and (3) linkage to keyword searches ( polymyalgia or rheumatica ) of outpatient clinic letters. To be identified as PMR, participants were required to …

Response to: ‘Renal biopsies should be performed whenever treatment strategies depend on renal involvement’ by Chemouny et al

We thank Chemouny et al for their letter and concur with their conclusions.1 As we state2: “A positive biopsy for ANCA associated vasculitis (AAV) is helpful when considering an initial diagnosis or recurrent disease”. In our view, renal biopsy is important to establish diagnosis …