Janice Mooney

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.

The epidemiology of Takayasu arteritis in the UK

OBJECTIVES Takayasu arteritis (TAK) is a large-vessel vasculitis of unknown aetiology. The annual incidence in hospital-based studies is 1-2/million. The UK General Practice Research Database (UKGPRD) contains complete primary care records on 3.6 million people. There are no data on the incidence of TAK in the UK or from primary care anywhere in the world. The aim of this study was to determine the annual incidence of TAK in the UK using the UKGPRD and in a well-defined hospital population [Norfolk Vasculitis Register (NORVASC)]. METHODS We identified all patients in the UKGPRD with a first diagnosis of TAK during 2000-05, using the Read code (G757); and in the NORVASC population. The annual incidence was calculated as the number of incident cases divided by total person-years. RESULTS A total of 14 (13 females) subjects were identified with a first diagnosis of TAK during 2000-05 in the UKGPRD. The median age was 51.0 years (interquartile range 28-66). The overall annual incidence of TAK was 0.8/million (95% CI 0.4, 1.3). The incidence was stable throughout the study period. The mean prevalence of TAK was 4.7/million. There were six patients (five females) aged <40 years presenting in 2000-05 with TAK. The annual incidence in those aged <40 years was 0.3/million. In the NORVASC population, one case was identified (0.4/million/year) with three prevalent cases (7.1/million). CONCLUSION This is the first population-based study of the epidemiology of TAK. The annual incidence and prevalence are consistent with previous studies.

The contrasting epidemiology of granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis

OBJECTIVES Granulomatosis with polyangiitis (Wegeners) (GPA) and microscopic polyangiitis (MPA) are uncommon and have unknown aetiology. The aim of the study was to investigate the epidemiology of GPA and MPA in a stable, well-defined population looking for differences in the pattern of occurrence, which might suggest a different aetiology. METHODS Since 1988, we have maintained a prospective register of all patients with systemic vasculitis attending the Norfolk and Norwich University Hospital. Patients presenting with new-onset GPA and MPA as defined by the European Medicines Agency algorithm and registered with general practitioners in the former Norwich Health Authority area between 1988 and 2010 were identified. The population in 2008 was estimated to be 459 000 (221 000 males). RESULTS One hundred and eleven GPA and 58 MPA incident cases were identified during 1988-2010. The overall annual incidence of GPA and MPA was 11.3/million and 5.9/million, respectively. There was evidence of a cyclical pattern of occurrence with a periodicity of 7.6 years for GPA with a peak incidence of 28.3/million in 2005 and the lowest in 2002 (2.2/million). Other lesser peaks occurred in 1990 and 1996. While the peak incidence of MPA was in 2008 (15.2/million), there was no convincing evidence of periodicity. The incidence of cANCA/PR3- or pANCA/MPO-positive vasculitis showed a similar pattern to GPA and MPA, respectively. CONCLUSION This study lends support to the notion that the aetiology of GPA and MPA may be distinct conditions with different aetiologies. The cyclical incidence of GPA is possibly an indication for the influence of infection.

Systemic rheumatoid vasculitis in the era of modern immunosuppressive therapy

OBJECTIVES Systemic rheumatoid vasculitis (SRV) is a rare but potentially serious systemic disease manifestation of rheumatoid arthritis (RA) characterized by the development of necrotizing vasculitis. The incidence of SRV appears to be decreasing possibly reflecting progress in RA treatment. The aims of this study were to review the clinical manifestations of SRV in a stable well-defined population during 2001-10 and to compare with our previous cohort (1988-2000) and also a cohort from 1975 to 1981. METHODS Using Norfolk Vasculitis Register, a prospective register of patients with systemic vasculitis since 1988, all patients with a diagnosis of SRV from 1 January 2001 until 31 December 2010 were identified. SRV was defined according to the Scott and Bacon criteria (1984). Clinical features were obtained by retrospective case note review. RESULTS Eighteen patients with SRV were identified (10 male), median age at diagnosis was 72 years and average disease duration 15.6 years. The average annual incidence for 2001-10 was 3.9 per million. One-year mortality was 12% and 5-year mortality 60%. The clinical manifestations were similar apart from systemic and cutaneous features which were more common in the earlier cohorts. CONCLUSION The incidence of SRV has declined significantly in the last 40 years; but the clinical manifestations remain similar. Systemic symptoms, and cutaneous manifestations such as infarcts and nodules, are slightly less common in the recent cohort. Despite modern immunosuppressive therapy the prognosis remains poor.

The outcome and cost-effectiveness of nurse-led care in the community for people with rheumatoid arthritis: a non-randomised pragmatic study

Objective To determine the outcome and cost-effectiveness of nurse-led care in the community for people with rheumatoid arthritis (RA). Design Non-randomised pragmatic study. Setting Primary (7 primary care practices) and secondary care (single centre) in the UK. Methods In a single area, pragmatic non-randomised study, we assessed the outcome, cost-effectiveness of community-based nurse-led care (NLC) compared with rheumatologist-led outpatient care (RLC). Participants were 349 adults (70% female) with stable RA assessed at baseline, 6 and 12 months. In the community NLC arm there were 192 participants. Outcome was assessed using Stanford Health Assessment Questionnaire (HAQ). The economic evaluation (healthcare perspective) estimated cost relative to change in HAQ and quality-adjusted life years (QALY) derived from EQ-5D-3L. We report complete case and multiple imputation results from regression analyses. Results The demographics and baseline characteristics of patients in the community group were comparable to those under hospital care apart from use of biological disease-modifying antirheumatic drugs (DMARDS), which were adjusted for in the analysis. The mean incremental cost was estimated to be £224 less for RLC compared to the community NLC, with wide CIs (CI –£213 to £701, p=0.296). Levels of functional disability were not clinically significantly higher in the community NLC group: HAQ 0.096 (95% CI −0.026 to 0.206; p=0.169) and QALY 0.023 (95% CI −0.059 to 0.012; p=0.194). Conclusions The results suggest that community care may be associated with non-significant higher costs with no significant differences in clinical outcomes, and this suggests a low probability that it is cost-effective.

The informational needs of patients with ANCA-associated vasculitis—development of an informational needs questionnaire

OBJECTIVE The aim of the study was to compare the informational needs of patients with ANCA-associated vasculitis (AAV). METHODS We developed a Vasculitis Informational Needs Questionnaire that was distributed to members of Vasculitis UK (VUK) by mail and registrants of the Vasculitis Clinical Research Consortium (VCRC) online registry with self-reported AAV. Patients were asked to use a 5-point scale (1 = not important, 5 = extremely important) to rank aspects of information in the following domains: disease, investigations, medication, disease management and psychosocial care. The source and preferred method of educational delivery were recorded. RESULTS There were 314 VUK and 273 VCRC respondents. Respondents rated information on diagnosis, prognosis, investigations, treatment and side effects as extremely important. Information on patient support groups and psychosocial care was less important. There was no difference in the ratings of needs based on group, sex, age, disease duration, disease or method of questionnaire delivery. The most-preferred methods of providing information for both groups were by a doctor (with or without written material) or web based; educational courses and compact disc/digital video disc (CD/DVD) were the least-preferred methods. CONCLUSION This study demonstrates that people with AAV seek specific information concerning their disease, treatment regimes and side effects and the results of investigations. Individuals preferred to receive this information from a doctor. Patients with AAV should be treated in a similar manner to patients with other chronic illnesses in which patient education is a fundamental part of care.

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.

An evaluation of rheumatology practitioner outreach clinics: a qualitative study.

BackgroundServices for Rheumatoid Arthritis (RA) have evolved with the development of independently led outreach Rheumatology Practitioner (RP) clinics in Primary Care (PC). Their clinical and cost effectiveness, compared with Secondary Care (SC) services, has not been assessed. The RECIPROCATE study aims to evaluate their clinical and cost effectiveness. This part of the study aimed to explore health professionals’ opinions of rheumatology outreach service.MethodsUsing a qualitative design, semi-structured interviews were conducted with GPs, practice nurses, hospital doctors and RPs, from one hospital and seven PC practices in Norfolk, to elicit their opinions of the service. The interviews were analysed using thematic analysis.ResultsAll participants agreed the service was supportive and valuable providing high quality personalised care, disease management, social, and educational support. Advantages identified included convenience, continuity of care and proximity of services to home. RPs helped bridge the communication gap between PC and SC. Some participants suggested having a doctor alongside RPs. The service was considered to be cost effective for patients but there was uncertainty about cost effectiveness for service providers. Few disadvantages were identified the most recurring being the lack of other onsite services when needed. It was noted that more services could be provided by RPs such as prescribing and joint injections as well as playing a more active role in knowledge transfer to PC.ConclusionsProfessionals involved in the care of RA patients recognised the valuable role of the RP outreach clinics. This service can be further developed in rheumatology and the example can be replicated for other chronic conditions.

A literature review regarding the management of varicella-zoster virus

Chickenpox and shingles (varicella-zoster virus [VZV]) pose a threat to individuals who are immunosuppressed and/or have rheumatoid arthritis or another inflammatory musculoskeletal condition. In this paper we present a literature review of the condition, management and prevention.

HEAL: a strategy for advanced practitioner assessment of reduced urine output in hospital inpatients

AIMS AND OBJECTIVES To produce a model for advanced nurse practitioners (ANPs) to follow in the assessment and management of patients with a reduced or low urine output. BACKGROUND Reduced urine output can be caused by a multitude of factors, some of which signify serious disease or may lead to serious morbidity. Assessment of reduced urine output is essential to ensure that patient safety is maintained at all times. Advanced nurse practitioners can add to patient safety by undertaking assessments of these patients at appropriate times and managing appropriately. DESIGN This paper presents a new model for immediate triage, assessment and treatment for reduced urine output, using history, early warning score, accuracy and laboratory results, or HEAL, with clear points for referral where appropriate. CONCLUSION This model can be used by advanced nurse practitioners to guide assessments and ensure that patients are appropriately managed in response, with minimal additional training. RELEVANCE TO CLINICAL PRACTICE This can aid advanced nurse practitioners in assessment, increasing patient safety and expatiating management. This shows how ANPs can be used to safely blur traditional professional boundaries with the aid of protocols.