Ep 30. GUIDELINES - BSR management recommendations for ANCA-associated vasculitis 2025

BSR has published a new set of management recommendations for ANCA-associated vasculitis (AAV), serving as an update to our AAV guideline last published in 2017.

Find out more via our blog. 

Join guideline working group Chair, Lorraine Harper, and group members Rosemary Hollick, Harold Wilson-Morkeh, Georgina Ducker and expert by experience member, Zoi Anastasa, in a roundtable discussion hosted by Prof Ernest Choy, Editor in Chief of Rheumatology to find out what is included in the publication and what has changed since the 2017 guideline was published. 

Sign up for the BSR educational webinars to accompany the recommendations here: https://www.rheumatology.org.uk/events-learning/webinars/updated-recommendations-for-managing-anca-associated-vasculitis 

Read the full guideline and download the summary pdf here. 

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BSR is the UK's leading specialist medical society for rheumatology and MSK health professionals. To discover how we can support you in delivering the best care for your patients, visit our website.

Transcript

Ernest   0:17
 Hello and welcome. I'm Ernest Choy, Editor in Chief of Rheumatology, Oxford. Welcome to this episode of the BSR Rheumatology Roundtable. Joining me today are members of the BSR working group, who drafted the new management recommendations for ANCA-associated vasculitis. And we'll be talking about these recommendations during this webinar.
 So I want to ask each of those who are present at this webcast to introduce themselves, perhaps starting with Rosemary.

 Rosemary Hollick   1:05
 Hello my name's Rosemary Hollick and I'm a consultant rheumatologist working in Aberdeen.
Ernest   1:11
 And Zoi.
Zoi   1:13
 Hi, my name is Zoi Anastasa. I'm a patient with GPA-vasculitis and I'm the Director of Operations of Vasculitis UK.
Ernest   1:23
 Lorraine.
Lorraine Harper  1:27
 Hi, my name is Lorraine Harper. I'm Professor of Renal Medicine at Birmingham and I was Chair of the working group.
Ernest   1:37
 Georgina.
Georgina Ducker  1:39
 I'm Georgina. I'm the vasculitis specialist nurse based in Norwich.
Ernest   1:44
 And last but not least, Harold.
Harry   1:48
 I'm Harry Wilson-Morkeh, I'm a rheumatology registrar and clinical research fellow at Imperial, London, and I've been part of the EGPA, ENT and subglottic stenosis subgroups and privileged to be named Co first author on these recommendations alongside Kathryn and Judith, the other rheumatology registrars, part of the working group.
Ernest   2:05
 Wonderful. Welcome, everyone. Perhaps I can turn to Lorraine, first, maybe you can tell us a little bit about how these recommendations came about and why they're needed.
Lorraine Harper  2:23
 So the 2014 guidelines for the management of ANCA-vasculitis really set the scene and were a real advance for the management of disease, but they're outdated, they don't reflect what's best practise now. There's been a huge amount of research - clinical trials delivered since 2014 - and we manage things differently. 

But we didn't want to completely reinvent the wheel and do a complete literature review based on advances since 2014, because there have been other guidelines produced, the EULAR guidelines on ANCA-management, the American College of Rheumatology and the KDIGO renal equivalent. And so what we wanted to do was use those guidelines, as they've done the literature search already, compare those guidelines, in the setting of what we do in the UK, update the literature from 2021 when EULAR had completed their recommendations and then add any additional areas of interest such as the management of subglottic stenosis, patient information and really thinking about service specification - making it fit for purpose for the UK. But it also has wider validity for the more global management of disease.

 Ernest   4:00
 Thank you, Lorraine. I mean, that makes it slightly different from the standard guidelines that BSR has produced. So perhaps, Harry, you can explain a little bit about the structure of the publication and how it was developed.
Harry   4:15
 So I'll be delighted - the working group, gosh, we were set up back in 2023 and tasked at reviewing sort of the latest evidence since the EULAR recommendations have been published in 2021.
 
 

The working group was comprised of 32 members from across the breadth of ANCA-vasculitis expertise in the UK. So these were experts in both adult and paediatric rheumatology, nephrology, respiratory and ear, nose and throat surgery, as well as some registrars in both rheumatology and nephrology, and we also had allied healthcare professionals in the shape of specialist nurses, an expert through experience and Vasculitis UK charity representatives.

 And as Lorraine’s kind of detailed, we had a scope that was agreed in our first virtual meeting and created five key working subgroups or domains from that to best cover the various nuanced aspects of ANCA-vasculitis care. So those included subgroup one, which was tasked at looking at GPA and MPA treatment, two, management of subglottic stenosis and ENT disease associated with GPA, three, EGPA management,
 four, service specification and, five, patient education and support.

 And, so, alongside the previous literature review that had been performed by the guidelines that Lorraine referenced, each subgroup then was tasked at performing separate systematic literature searches since the convening time, looking at English language publications. And we performed this in MedLine, using key search terms agreed among members of each sub domain. And from those we were able to draft the initial recommendations and then subsequently these were refined over a series of virtual meetings with the full working group present, to a total of 26 final recommendations that you see in the manuscript that was published recently. And these were done in accordance with BSR Protocol, so the quality of evidence was graded according to the grading of recommendations, assessment, development and evaluation (AGREE) approach. And the evidence graded strong, moderate or weak, depending on confidence in estimates of benefit or harm. And strength of the agreement for each recommendation was voted anonymously, and these were scored on a scale of one reflecting total disagreement to 100, reflecting full agreement, with the mean agreement of each recommendation expressed as a percentage of at least 80% for that recommendation to be included in the final manuscript.

 We then submitted these recommendations to peer review and underwent public consultation prior to the publication, so hopefully you'll agree that these recommendations were held up to rigour and inform best practise management to improve the lives of people living with ANCA-vasculitis.

 Ernest   7:11
 Great. Well, Lorraine you mentioned that the last BSR guideline was done in 2014, so there's a need to update this and a lot has changed. Perhaps you can highlight for us.
 What are the main changes in these new management recommendations?
Lorraine Harper  7:32
 So, we are recommending that everybody be considered for rituximab or cyclophosphamide as induction therapy, and that's treatment for all, and everybody is considered as having life- or organ-threatening disease.
 
 

We are recommending that plasma exchange be considered for patients who've got a creatinine greater than 350. But that that is discussed fully and patients are fully considered for their infection risk because, although there is a benefit, plasma exchange is defined by the meta-analysis, there is also a price to pay which is an increased risk of serious infections. We are not recommending plasma exchange for pulmonary haemorrhage because the evidence from PEXIVAS doesn't support this.
 
 

We have changed our recommendations for maintenance therapy to now suggest that patients should all receive, as maintenance therapy, rituximab over azathioprine, and that treatment duration should be between 24 and 48 months.
 
 

And those really are the main take home recommendations for GPA and MPA, and perhaps I could turn to Harry, who was intimately involved with the EGPA and the ENT groups for him to get some highlights of the recommendations around about those areas.

 Harry   9:22
 Yeah. Thanks, Lorraine. So I think maybe the first thing for me to do would be to point people towards the graphical abstract which accompanies the publication and this, we hope, gives a helpful snapshot of the key updates and take-home messages from these latest recommendations. I think, specifically from an EGPA perspective, we've tried to highlight, number one, who should be suspected as potentially having EGPA, namely anyone with asthma, sinus disease and is eosinophilia with more than 1x109 per litre.
 
 

Secondly, how to thoroughly investigate a potential new case of EGPA? We include investigative an algorithm within the guidelines. 

And then, three, what treatments to use in confirmed disease, with a particular emphasis on biologics that inhibit interleukin-5 signalling, so, mepolizumab and benralizumab, which have EMA and FDA approval for use in EGPA and that we can currently access in the UK through severe asthma centres.

 And then moving on to subglottic stenosis and ENT disease associated with GPA, we've aimed to highlight that these really are challenging manifestations that really do require sort of dynamic imaging direct visualisation when possible and definitely expert management including systemic therapy with cyclophosphamide or rituximab to achieve early disease control at disease onset or in the context of relapsing or refractory disease.
 
 

And we've also included within this section some useful tables covering potential sino-nasal disease mimics that should be actively excluded in the diagnostic workup and also suggested additional topical therapies that might be used in certain instances.

 Lorraine Harper  11:01
 Perhaps, Ernest, I could just add that both across all three subtypes of ANCA-vasculitis, we've got a real focus on reducing steroid use and so advising us for those with severe disease that we should be considering avacopan or, if not using avacopan, then using the PEXIVAS regime for lower dose steroids. And for those who have not got organ- or life-threatening disease, if you're not using avacopan then consideration should be used to even lower doses of steroids. So with a starting dose of 0.5 milligrammes per kilogramme and that's a focus across all three subtypes.
Ernest   11:53
 Thank you, Lorraine, for that nice summary. So, Harry, you mentioned that one of the subgroups is about design and delivery of the service which is a bit unique among recommendations for management of ANCA-associated vasculitis. So perhaps I can turn to Rosemary, to ask her to tell us about what is the recommended design and delivery of an ANCA-associated vasculitis clinical service.
Rosemary Hollick   12:22
 Thanks very much, Ernest. Yeah. So you know to deliver the right care to the right person at the right time, we need services that enable us to do that. And, prior to now, the service recommendations and guidelines have been based on expert opinion. But, for the first time, we do have now robust evidence of key components of services which are associated with improved health outcomes for people with ANCA vasculitis, and these are reflected in in the latest guidelines and they're focused around timely access to care - integrated care - access to expertise and also, recognising the geographical variation in size and shapes of services, recommendations for specialist centres to support smaller centres, because we felt that that was really important in terms of supporting local, regional and national planning. 

So, Harry's already referred to the graphical abstract, which summarises the, sort of, the key service elements. So, being able to see a new patient with a high index of suspicion of ANCA vasculitis within seven days is associated with reduced serious infections, emergency hospital admissions and mortality. We also found that nurse-led components of care. So, for example, advice lines and nurse led clinics were also associated with fewer serious infections and emergency hospital admissions.
 As was access to a specialist vasculitis MDT, and, for the first time, this provides evidence-based standards which are associated with improved outcomes. But the other thing we already know is that there's really a lack of accurate and timely data to inform service planning. And so the guidelines also include audit recommendations around the key service specifications. And I'm delighted to say that that's been integrated into the BSR Early inflammatory arthritis audit (NEIAA). So, therefore, we were able to look, certainly within rheumatology services, at whether, where, these, whether these service specifications are present and use this as a lever for change and to measure improvement.

 Ernest   14:49
 Fantastic. So, Rosemary, you mentioned that, within the delivery of the ideal service, a nurse-led service has got a very important role. So, perhaps I can talk turn to Georgina and ask her, having been working within the recommendations and development, maybe she can explain in a little bit more detail about the exact role of nurses.
Georgina Ducker  15:17
 Thank you. So I've been a vasculitis nurse for just over five years, and when I started my role, I did do a quick search to see what other vasculitis nurses were around in the UK and there aren't many, but we have a long history of specialist nurses in rheumatology looking after other conditions. And it's actually really nice to see the guidelines that we've produced now specifying the need for a specialist nurse for patients with ANCA vasculitis. We know that specialist nurses are really good at providing patient education on their disease - what to expect, and what the outcomes might be, what the treatment is going to be like for them, whether they're starting cyclophosphamide or rituximab. Or the steroids and managing the side effects. So it's about giving that additional education and support to help manage patient expectations and then we can offer those ongoing clinics that patients can access for additional support. So we can monitor the side effects of the treatment. So we can be offsetting things like diabetes, osteoporosis management, considering adrenal insufficiency further down the line as we wean patients off steroids.
 
 

There's lots of things that we can do with patients to support them through their diagnosis and through their treatment programme and having nurse advice lines for our patients. So, they've got that point of contact in between their scheduled appointments is a really useful resource for them to be able to reach out if they're having difficulties in between. And we can also perform part of their multidisciplinary team. So, there's often multi specialities involved with looking after these patients. I know from our caseload we have a heavy reliance on our respiratory and renal physicians as well as rheumatology. It can be that sort of person that just helps draw that together. So, if the patient calls us, we know who to reach out to, depending on what problems the patient might be experiencing.

 Ernest   17:00
 Fantastic. So, I want to turn to Zoi, having been working with the team in developing these recommendations and coming up with these recommendations, how do you think that these recommendations will benefit patients with vasculitis?
Zoi   17:17
 Thank you, Ernest. So the updated guidelines are really great news for anyone living with ANCA-associated vasculitis. They represent the latest advances in medical understanding and treatment of the condition, which means that patients can expect more up-to-date and effective care. What is especially important is that this guideline helps patients become more informed and empowered - knowing what the current recommendations are.
 
 

It allows people to take a more active role in their care, asking the right questions, being part of shared decision making, and even advocating for the best possible treatment. I've been a patient of ANCA-associated vasculitis for more than 10 years and I believe that it is very valuable to be aware of the recommendations. It helps ensure your treatment is based on the latest evidence, which can improve your chances of a better outcome.

 Ernest   18:20
 Thank you. Rosemary, in your talk, you mentioned that, in the ideal service a patient needs to suspected of ANCA-associated vasculitis should get a diagnosis within seven days. So there clearly is an important role for primary care to look at potential patients that require urgent referral to specialists. So, I welcome any members of the team to chime in about how primary care physicians, should, their roles in helping implementing this guideline.
Rosemary Hollick   19:06
 I mean, I see the service recommendations. and that one in particular about being able to see people quickly within seven days, the guidelines very much emphasise and the evidence suggests that that should be part of our of our broader pathway, which absolutely starts within primary care with being able to recognise symptoms, knowing who to refer in to quickly and being able to get appropriate tests done and who gets the results and being able to appropriately triage and manage. So we very much see the service recommendations as a suite, as a complement. And part of that broader pathway.
Ernest   19:48
 OK, great. Georgina, you want to chime in?
Georgina Ducker  19:51
 Yeah, I think it's also about the ongoing management. Once patients have their diagnosis, we're not expecting the GPs to change their treatment or make sort of complex decisions, but it's just about supporting the patient once they've got their diagnosis, making sure they've got access to their vaccinations to prevent infection. You know, we know the incidence of vasculitis tends to occur in the latter ages, but we do get younger patients that might need help with family planning and referral to maternal medicine services.

 So it's just looking at the bigger picture and just being aware of these sort of complex patients and their needs.

 Ernest   20:23
 Fantastic. Maybe then I can turn to Lorraine and ask, I mean, as you highlighted, I mean the management requires multidisciplinary, special input. And so how can we support listeners and readers of the guideline to ensure the recommendations are implemented into clinical practice, particularly how they will work with their colleagues?
Lorraine Harper  20:47
 So I think one thing that is very different from the process that we've gone through is that most guidelines are produced in isolation with their own specialty. And as you can see from the KDIGO guidelines and the EULAR guidance, there are differences.
 But with this guideline we have endorsement from all major professional societies that are associated with the care of patients living with ANCA vasculitis. So I think you know we are now all singing from the same hymn sheet to suggest that if you as a patient were presenting to ENT to renal medicine to rheumatology, you would be getting hopefully the same advice from each different professional. So I think that is really important, but we also need to spread the word about these guidelines so the BSR is they kindly hosting some webinars, in three parts, with the first happening on the 19th of June at 4:00pm, with further parts in July and September.
 
 

Harry's already talked about the graphical abstract that's been produced to help people when thinking about ANCA vasculitis, and also Rosemary's talked about the audit tool to help us follow up and ensure that we're delivering the right care. And just to remind everybody to enter patients into the NEIA audit so, working together, I think we can really deliver the expert great care, the patients living with vasculitis deserve.

 Ernest   22:48
 Thank you very much and I want to thank all of you for your input. I hope the listeners find this webcast helpful and please do access the BSR website who have got many information regarding these recommendations. Thank you very much and I hope you have a good evening.