Ep 21. GUIDELINES - BSR-BAD Behçets living guideline 2024

BSR has co-published a living guideline with the British Association of Dermatologists for managing people of all ages with Behçets.
 
Join guideline working group Co-Chairs, Ruth Murphy and Robert Moots, and members Clare Pain, Harry Petrushkin and Steve Higgins in a roundtable discussion hosted by Prof Ernest Choy.

The living guideline is presented as a detailed review with highlighted recommendations for practical use in all appropriate community and hospital settings. The guideline development group (GDG) set out to provide an up-to-date, holistic and evidence-based approach to optimise the management of people with Behçets, factoring in patient values and preferences. 

Read the full guideline and view all the accompanying resources 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

Rheumatology Roundtable 2024

Behçets guideline update

Transcript

 Ernest Choy   0:07
 Welcome to this episode of the BSR Rheumatology Roundtable, where we will be delving into the brand new living guideline for managing people with Behçets disease, which has been coproduced by the British Association of Dermatologists and the British Society for Rheumatology. I’m Ernest Choy. I'm Editor-in-Chief of Rheumatology, Oxford, and it's my pleasure to be asking the panel to summarise the new guidelines. So, in this discussion we'll provide a very brief and succinct summary of what the key take-home messages are from the guideline, to help clinicians to benefit from the guidelines. So, without further ado, I want to go round and ask each of our esteemed panel members to introduce themselves to you. Steve, will you start?

Steve Higgins   1:10
 I'm Steve Higgins. I'm the clinical psychologist at the London Behçets Centre of Excellence.

Robert Moots   1:22
 Hi, I’m Robert Moots. I'm a rheumatologist and I'm director of the Liverpool Centre of Excellence for Behçets.

Ernest Choy   1:28
 Ruth.

Ruth Murphy   1:30
 Hi, I'm Ruth Murphy. I'm a dermatologist. I work at Sheffield in an MDT for Behçets disease.

Ernest Choy   1:40
 Harry.

Harry Petrushkin   1:42
 My name's Harry Petrushkin. I'm a consultant ophthalmologist, and I work across Moorfields Eye Hospital and Great Ormond Street Hospital for children. I work quite closely with all the Behçets teams in London.

Ernest Choy   1:56
 Clare.

Clare Pain   1:57
 Hi everyone. I'm Clare Pain. I'm a paediatric rheumatologist at Alder Hey, in Liverpool, and I’m the paediatric lead of the Liverpool National Centre of Excellence for Behçets.

Ernest Choy   2:06
 Thank you and welcome all of you and I appreciate your time sharing your expertise with us and you can see that we have a very diverse and broad panel, which represent the diversity of the guidelines and the multisystem nature of the disease. Perhaps I can start with Ruth. Ruth, could you start by giving us an overview of the guideline, why is it important and what does it include?

Ruth Murphy   2:35
 The reason that we wanted to do these guidelines was because Behçets is a very rare disorder, and it presents in many ways because of all the organs which can be affected. One of the problems with it is that people who live with the disorder, and those caring for them, their issues are such that it is very, very difficult sometimes to meet all their needs throughout their life. About a fifth of patients with Behçets start in childhood, many ladies affected will want to go on to have children and we wanted a list of recommendations from evidence-based guidelines which allowed us to give the most up-to-date and safest recommendations for the care of these patients. So that was where we sort of started from. Over a period of a couple of years, these guidelines were therefore published. 

The shape that they take is a series of recommendations, some of which are general, stressing the need often for multidisciplinary team input to make a diagnosis because of the very many ways the disorder can present. Explaining that, because the disease is chronic but will have flares, it's very important when healthcare professionals see these patients that they assess for disease activity in all the relevant organs when people are assessed. We were very, very aware of living with such a rare and complicated disorder meant that the psychological impact for this group was particularly important and therefore we gave emphasis to these patient needs.

 At the end of the whole process, it was evident that there were many more questions to answer and therefore there were future research recommendations which were published.

 I think as well the whole point of this is that it is a very exciting area because there are many new drugs that are coming to market, some repurposed, some designed for Behçets. And so we very much wanted to make this a living guideline and other people will talk about that more as this interview progresses.

Ernest Choy   5:05
 With that, perhaps I can move on to Robert and ask him, how was the guideline developed and, in particular, what makes this a living guideline?

Robert Moots   5:15
 Well, thanks, Ernest. It's taken many years, actually, to develop the guideline from start to publication. And the crucial thing, as in any guideline, it's got to be thorough. It's got to be robust. We wanted to make sure it's holistic and patient focused. So the first thing was to get together a multidisciplinary multi-professional working group of experts and this is the first joint guideline that has been set up in collaboration between the British Association of Dermatologists and the British Society of Rheumatology. So, just getting all of that together, both of the groups are accredited for making guidelines, and, to actually get harmonisation of the two procedures took a little bit of time, but actually increases the robustness of the actual end product. So we're trying to make it holistic, patient focused, robust, it's accredited by NICE. Then the final thing is, in contrast to many of the other guidelines that people will be familiar with, this is a living guideline, and that basically means that individual recommendations can be updated as soon as new relevant evidence is available. So what we're not doing is waiting for some artificial arbitrary time when a new committee is being convened. We're constantly going to be reviewing what's going on, and, if something is published that means we should be reconsidering things and, potentially, revising a recommendation, we should be able to do it much faster. So the idea is this is something that's living. It's going to be updated constantly. All of that's great apart from those of us that are doing the regular updating. It makes our life a bit harder, but it makes your job much easier when you're actually looking for up-to-date relevant guidelines.

Ernest Choy   7:12
 Great. Clare, this guideline is unique in many ways, but one aspect is that it covers people of all ages. How does Behçets affect young people and how is the guideline relevant to paediatric healthcare professionals?

Clare Pain   7:29
 Thanks, Ernest. So we run a study a couple of years ago in the UK that showed that Behçets was extremely rare in children and young people under 16. Most had mucocutaneous disease, very similar to adults in the UK, but actually the brain, the neurological, ocular and vascular disease was less common in children and young people. But GI involvement was more common. We also found that over 80% of children and young people are being looked after by three or more specialties and some of those up to six.

So it's fantastic to have this guideline that talks about developmentally appropriate multidisciplinary care and also, for the first time, best practise around transition from paediatric to adult care for children and young people with Behçets. Because Behçets is so rare in children and young people and those life threatening and organ threatening involvement, many people, especially paediatric rheumatologists, will not have seen that. So having recommendations that you can go to, to look for how to treat vascular involvement or neurological involvement, that are evidence-based, are really key to improve outcomes for children and young people.

 Also, as Ruth said, there are also recommendations for future research and I'm really pleased to see that that the unmet need for more research in paediatric Behçets has been highlighted, particularly the experiences of those living with Behçets and also randomised control trials for paediatric Behçets.

Ernest Choy   8:56
 Fantastic. So Ruth, Robert and Clare have all mentioned the importance of representation across multiple specialties in developing this guideline, because Behçets is a multi-system disorder. So I want to move on to Harry to ask him how can the guidelines support ophthalmologists to work with patients, to manage eye complications associated with Behçets disease.

Harry Petrushkin   9:23
 Thank you, Ernest, so Behçets, as everyone knows, affects the eyes as well as everywhere else and the eye disease, much like neuro-Behçets and large vessel involvement Behçets can lead to irreversible damage. So unlike the part of Behçets. say with mucocutaneous disease that relapses and remits, and you have a chance to intervene, with Behçets if you have a nasty eye flare, then you can irreversibly lose vision. And the study from the 80s that people cite when they talk about how bad it can be, was that 90% of patients lost vision in both eyes within four years with no treatment. Now, fortunately, in my working lifetime, that's not our reality and I reassure patients that that's not something we see, ever, really, anymore. But it really can be quite devastating. And so, the importance of knowing when the ophthalmologist needs to treat straight away and doesn't have time to involve other specialties for a holistic management plan and times when actually you can say, well, this is severe, but we can start treatment, but it's really important to get an underlying diagnosis. One of the concerns of the past was when you treated too aggressively when patients first presented. The rest of the Behçets then went away and effectively you've had this silent trail of all of the previous manifestations. So a really good multidisciplinary clinic is completely essential. But you also need to be looked after by ophthalmologists that know Behçets and understand when you need to urgently step in. And so this guideline is really clear on that. It tells ophthalmologists: if you have a site-threatening manifestation, you need to treat in “this” way and you therefore don't wait for an MDT response, whereas if, you have, say, some milder ophthalmic manifestations, then you've got the time for that.

Ernest Choy   11:16
 Wonderful. So, Ruth, right at the beginning, mentioned that Behçets disease has a significant impact on the individuals’ mental well-being. So, I want to turn to Steve to ask him what sort of impact did yourself and other clinical psychologists in the working group have on the guideline development?

Steve Higgins   11:38
 I mean first of all, I think the biggest impact we have is actually to be in the document. All right, it’s slightly obvious, I know. But the history of that is being embedded within the Centres of Excellence. So psychology was always a planned part of the service, rather than an afterthought.

 When we did the review, I guess two areas kind of jumped out at us for the lack of evidence. One was service models, and I think that's really important in terms of impact, and the other, for us, was the specific psychological therapies that we were offering, or could potentially offer people.

 Because there are so few of us in such a small service, we have to use what's already there, what people are already using. So it's tapping into the existing mental health and psychology services that are around the country, rather than trying to reinvent a comprehensive service ourselves. So we built in the guideline a service model kind of idea that really shifts the idea of mental well-being psychological concerns from a box that says psychology to actually much more refined multi-level needs for support and multi-level rules for everybody in the team. And I think that's fits with the MDT ethos.

 So we adopted a model that mimics quite similarly to mental health models, a kind of step-care approach. That's really helpful for us because we think that helps communication liaison with these types of services, which can often be problematic.
 We could also cut across the services because the mental health services and psychological therapies are sub-divided into different areas, and it's generally different departments and sometimes different NHS trusts that offer the services to patients from one locality. So it's helps to bring that together and that's really important in the experience of people with Behçets - to have ease of access.
 A fluid multi-level support rather than these kind of, maybe, slightly outdated ideas of people simply having depression or anxiety.

I think the other thing it offers is flexibility in that model, because you can move up and down it. So, again, it helps facilitate the patient through the process. 

In terms of therapy, there's very little that's directly related to Behçets, which is not a surprise given its rarity and the amount of psychological interest has been vested in it. But we did come up with some ideas. We generally adapt from other related areas and we've listed most common therapies that we use, like cognitive behavioural therapy, third wave therapies, like acceptance and commitment therapy, mindfulness and compassion focus, and various others. And there's probably not a great need to specifically look at its effectiveness in this group because we know it works and we know it can be adapted to all kinds of groups. So that's really to kind of caution against going into research into specific effectiveness/effectiveness-specific therapies for people with Behçets.

 I guess the other area is sometimes these listed services can be a bit simplistic, so we've listed EMDR. There's some evidence for it. It's great. We love that it's in there because, for us, a whole area is emerging that this is medical trauma. Not just people's lifelong personal traumas in their personal life, but also the trauma that they go through as they go on their journey through symptoms, to diagnosis, to treatment, to living their life. And just looking at something like an EMDR therapy, it's probably not going to be sufficient, so it needs more thinking, and I think that's why the service model lends itself to more integrated multi-level support, which I think is really helpful.
 
 This leads to where we might go next with this. The little bit of research in psychology tends to dive deep into very specific areas like EMDR or there's a small study on psychodynamic influences as well. And, to me, we have to take a step back in terms of research and actually see what does this field look like, what actually are the experiences, rather than presuming the making assumptions about very specific things. I would really welcome, in terms of future research for psychological direction, that it’s more qualitative, more finding out what the lived experience is like in specific areas. And I think medical traumas are a really interesting area for all of us to think about. 

The two things that have made the most major impact on psychological well-being for people with Behçets: number one is the change and reduction in symptom-to-diagnosis. That is absolutely crucial in reducing experience and build-up of trauma through people's lives. It renders it more amenable to treatment if it's caught earlier; worked with earlier. Then, I guess the second area, which costs no money at all, and is the medical trauma area, is how we, as professionals, traumatise our patients when we have consultations with them. And I think that's something we should all be thinking about - everyone, all professionals across the board need to think about that because it's pretty ubiquitous within the population. So these are things we can work on and highlight that I think would make actually a really big height.

Ernest Choy   18:32
 Great. So we've heard that Behçets is a rare but very complex and severe disease that requires multidisciplinary expert care. So we need a living guideline to address this complex issue, which is also fast moving in terms of having new treatments for the condition. But I want to come back to Rob and ask him, how would this guideline help clinicians in primary care to manage or identify individuals with Behçets disease?

Robert Moots   19:07
 Yeah, I mean Behçets, what a crazy disease - few people can pronounce it and even fewer understand it. So if you're watching this podcast in primary care, I'm really pleased. We're really pleased that you are, because we really want you to be able to understand more about this. Some people wrongly think it's almost impossible to have Behçets in the UK. I can tell you, we've got 700 patients just in the Liverpool Centre. So there are more patients around. And if nothing, I'd like these guidelines to be raising awareness, because the clever thing is thinking of Behçets. For the likes of us, we've all got loads of people with Behçets. It's you, in primary care, in other specialties, who sees a patient, thinks of the diagnosis and then be aware of the sorts of things that can be done; the specialists around that can help you, because that's really the crucial thing. And within the guidelines there's plenty of advice about relatively easy and straightforward ways to manage symptoms. Ulcers in the mouth, skin problems, genital ulcers. So all of those things don't really need high cost, fancy drugs. Just by thinking of the disease, just by being aware and providing simple treatments, that can provide a lot of help to patients. 

Furthermore, we've really emphasised the importance of having an appropriate expert, multidisciplinary team. So, not everybody with Behçets needs to be seen in a major centre, but, everybody with Behçets needs to have an appropriately trained specialist who can be taking care of them overall, and, certainly, in the centres, we're very keen to be liaising with patients’ primary care teams because it makes far more sense to us for patients to be cared for near to home rather than travelling up to hundreds of miles to see somebody, somewhere else. So for all of these reasons, I want you to know that primary care is really important and that we do hope that the guidelines will be helpful to you as you're looking after your patients because there'll be more of them than you can ever imagine.

Ernest Choy   21:21
 Ruth, what do you think the impact of this guideline will be on people with Behçets?

Ruth Murphy   21:26
 Well, as both Steve and Rob have said, there are a couple of themes which commonly recur when we speak to our patients. I think probably the commonest one is that people will say, why is it taking so long to diagnose me. And that is because it's actually a very difficult diagnosis to make and we emphasise that in this guideline. There isn't a blood test, there isn't a genetic test it. We're looking for a constellation of clinical features, which accrue over time. As Rob has said, many of them will be mouth ulcers, ano-genital ulcers, skin lesions. But, as Harry said, some of the presentations can be more severe and potentially lead to permanent damage if the eye is affected by occlusion of a vessel or inflammation of a vessel which helps with vision.

 So those sorts of things mean that, for the patient trying to navigate through what type of Behçets they've got, will they go from a low-grade but troublesome version, maybe a mucocutaneous type, or will they escalate to something which could cause permanent damage to something vital like the eye – it produces a lot of anxiety and we hope that these guidelines actually make it clear that we're trying to help the whole of the healthcare workforce understand more about this disorder. As Rob said, raise awareness generally. Patients hopefully will know that we are taking their concerns seriously. So we don't get trauma and stress because of delayed diagnosis, Not because people have missed the diagnosis, that can sometimes happen, but just because sometimes in the lower grade presentations of the disease, which really you know these forms of the disease also have massive impact on our patients, but it can take a long time to reach diagnostic certainty.

 We hope that the guidelines will reassure patients that we are taking them seriously by helping them and the people that look after them understand more about their condition, understand their need to understand that the problem does flare and settle. And so, sometimes when they've waited for an appointment, there won't be any symptoms to see. Understand that we need to evaluate the disorder when it flares but also throughout our follow-up consultations to determine whether there is still some low grade activity and maybe the medication needs to be fine-tuned. And to help all those diagnostic and management decisions, we actually do have an algorithm, which is quite detailed, in the guideline, and I'm hoping that the patients will see it. They have a plain language summary to explain the guidelines and they also can show that to the people looking after them - primary care, secondary care - make them aware of the guidelines and hopefully they'll be reassured that in this way we're trying to optimise their care.

Ernest Choy   24:51
 Wonderful. Thank you for that wonderful summary, Ruth. I want to thank all the panellists today for summarising what the new living guideline for Behçets highlighted and how it will contribute to improving the diagnosis and management of this very rare but very significant disease. Those of you who will have a chance to read the guideline will recognise the enormous amount of work that has gone into developing the guideline and the literature that covers, but there are also very succinct algorithms and summaries that will help you to manage patients with Behçets disease. And, with that, I want to thank all of you for listening. I hope you'll get a chance to read the guidelines which will be published by Rheumatology, Oxford, but also the British Journal of Dermatology, and they will be available at the associations’ websites.

 Thank you very much everybody and goodbye.