Multidisciplinary specialists discuss the diagnosis and management of IgG4-related disease
In this Roundtable discussion presented by Rare Disease 360, 3 clinicians from different subspecialty groups discuss their best practices for diagnosing and managing patients with IgG4-related disease.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Welcome and thank you for joining us on a peer discussion about the diagnosis and management of IgG4-related disease, some really practical aspects. I’d like to invite my 2 colleagues today. First, Guy, could you introduce yourself?
Guy Katz, MD:
Sure. Thanks, Emma. My name is Guy Katz. I’m a rheumatologist and investigator at the Massachusetts General Hospital (MGH) and Harvard Medical School. I work on clinical and translational research, and I do a lot of work with the MGH IgG4-related disease registry. My main research and clinical focus is in IgG4-related disease, and I’m involved in clinical trials and other clinical and translational research in the disease. Excited to be chatting with the 2 of you today.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Thank you, Guy. Phil, can you introduce yourself?
Phil Hart, MD:
Thanks, Emma. My name is Phil Hart. I’m a gastroenterologist with a subspecialty focus in pancreatology at the Ohio State University in Columbus. My initial exposure to IgG4-related disease was in the pancreas clinic about 15 years ago during training. At that time, we were managing the disease that we just called autoimmune pancreatitis, which obviously we now recognize as the pancreas’ manifestation of IgG4-related disease. My clinical and research interests are broad, but still pancreas-centric. Really glad to be talking about this today with you guys.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Fantastic. I’m Emma Culver, and I’m a consultant gastroenterologist and hepatologist working in Oxford at the John Radcliffe Hospital. Here, I manage the national service for IgG4-related disease, including some multidisciplinary work. I see a bit of a mix of both gastro and hepatology and also the systemic disease, and like Guy, very much involved in the clinical trials.
If I could just start by asking you generally, when you have to identify and diagnose patients with IgG4-related disease in your clinical experience, Guy, you can talk me through first a little bit about that, about how you do it and the presentations you see?
Guy Katz, MD:
Sure. I think one of the interesting and important things about IgG4-related disease is that it really can be identified by any clinician of almost any specialty because it presents so variably. It can even be identified by non-clinicians, whether that be pathologists or radiologists. There are a lot of things that can be seen that really point to the diagnosis. I think the most important thing is recognizing what the typical manifestations of the disease actually are, and that’s really what leads to the diagnosis most often.
In particular, in the head and neck, looking for orbital inflammation, looking for enlargement of the glands, the lacrimal glands, the parotid glands, submandibular glands. Those are essentially the main things that can be seen on routine physical exam. Almost everything else requires some degree of imaging to be able to identify, whether that’s any number of potential manifestations within the chest, large vessel vasculitis, autoimmune pancreatitis, which I’m sure we’ll talk about a lot over the next half hour or so, biliary disease, retroperitoneal fibrosis, renal disease.
It can affect almost any part of the body. I think the most important thing is recognizing those typical manifestations either on physical exam or on imaging. In most cases, biopsy of some sort is helpful in making the diagnosis whenever that’s possible, in part to look for the classic histologic features of IgG4-related disease and in part to exclude some of the important mimicking conditions.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Great, thank you. As you said, you’ll see a lot of head and neck disease particularly and you have to do imaging. Phil, can you tell me a little bit about how they come to you? Because obviously as a pancreatologist, they may present to you in a very different manner.
Phil Hart, MD:
Yeah, no, that’s exactly the case, and this is one of the things that makes this disease challenging, but I think also interesting in that there are classic presentations and those classic presentations differ based on our medical or surgical subspecialty practices. The patients who primarily come to a GI or a pancreas clinic, those are where there’s pancreas and biliary disease, which if we look at the broader disease entity, the multi-organ syndrome of IgG4-related disease, it’s roughly about half a patients who will have pancreas or biliary involvement. When that is the case, on imaging, this has a distinctive appearance with the diffuse enlargement or the so-called sausage-shaped pancreas. That’s not universal. But when that’s present, that’s helpful. From a clinical presentation standpoint, the most common presentation is with jaundice. Sometimes these patients will present with acute pancreatitis, although that’s less common and it generally radiographically at least looks different from other causes of acute pancreatitis.
Sometimes patients will initially present with abdominal pain, although that’s not a common symptom that is persistent. Then lastly, sometimes for patients they may present with isolated insufficiencies, be it endocrine with diabetes or exocrine with exocrine pancreatic insufficiency manifest by the symptom of steatorrhea and sometimes with really profound weight loss with or without jaundice.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Thank you. I mean, you’re right. It sounds like this can be both an indolent disease, which it can be, so it can be very slow. But as you say, with patients who present with jaundice and even acute pancreatitis, they can also present to the emergency department. I think when clinicians are managing this, it’s important to remember they don’t just rock up to your outpatient department.
They may also come to the emergency department, and that might be the first mode of presentation, which takes me a little bit to think about diagnosis because we know that we’re not great at diagnosing these patients partly obviously because it’s a rare disease and also because of the modes of presentation. Do you have any thoughts really about how we improve that time to diagnosis?
Is there anything that you can think about that we would guide our clinical colleagues on or think about how we might improve the flow to improve our diagnostic times? In some series, the mean time to diagnosis has been anywhere up to 3 years. Guy, from your perspective, is there anything you’ve found within the Mass General or even in your clinical experience that might potentially shorten that time schedule?
Guy Katz, MD:
Absolutely. I think that’s one of the biggest challenges that we have in IgG4-related disease right now is identifying the disease before it causes really severe irreversible damage in particular in the pancreas, as Phil was talking about. I think some of the things that are important are… The main one from my perspective I think is awareness of the diagnosis and recognition of the diagnosis. I think, in general, gastroenterologists are very good at identifying autoimmune pancreatitis.
I think, in general, rheumatologists are getting better at recognizing some of the other manifestations of the disease, but there’s still work to be done on many specialists understanding the disease and recognizing it. I think in part that’s because we talk about the disease as a rare disease, which it is, but it’s also not as rare as people give it credit for. The most recent estimates for how common it is suggest that it’s about as common as ANCA-associated vasculitides. But it definitely gets a lot less attention on differential diagnoses and in discussions in general.
I think the idea of shifting our thought process from thinking of it as a zebra that really doesn’t need to be entertained in the differential diagnosis to a very important element of the differential diagnosis for a lot of these different organ manifestations is one of the most important things for clinicians and radiologists and pathologists to be thinking about. Then increasing our education and our visibility for specialists of all kinds.
This is something that oculoplastic surgeons need to be aware of, neurologists need to be aware of, ENTs need to be aware of, pulmonologists need to be aware of, allergists, and the list goes on and on. I think increasing the awareness of the disease and the understanding of how it presents is going to be a really important feature and way that we can reduce the time to diagnosis.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Thank you. Phil, I guess the other problem we have really is thinking about how we distinguish it from cancer and the surgical MDTs. Do you have any thoughts about how that can be optimized for both our surgical and medical colleagues?
Phil Hart, MD:
That’s one of the things that is just critical is that when we are thinking about this, even though it has historically been considered a zebra in Guy’s words, I think one of the areas of interest from gastroenterologists is that we are hoping to find this zebra because the other possibilities, the horses, so to speak, are mostly bad things. Pancreatic cancer, plantar carcinoma affecting the bile ducts, primary sclerosing cholangitis, these either bad diseases or diseases for which there’s no active treatment.
From a gastroenterologist standpoint though, I would echo Guy’s sentiment that I still think that there are opportunities for increased awareness. While I think most gastroenterologists because of multiple reasons are very familiar with the term sausage-shaped pancreas and that rings the bell very loudly, identifying that on scans is not always possible and doesn’t always occur.
For gastroenterologists, I do think there’s still an opportunity to identify the other organs that can be involved, either that can be picked up with abdominal imaging, which we essentially universally have, or even the head and neck manifestations that could be visible just by talking to a patient and doing a very quick and focused physical exam. I think at the end of the day though, one of the biggest challenges that we face in the field is that we don’t have an accurate diagnostic biomarker.
The serum IgG4 level is helpful when it’s positive and when it’s combined with other diagnostic information, be it on imaging, other organ involvement, or with histologic findings. But for the pancreaticobiliary diseases, we can see false positives in about 10% of patients with the diseases that we’re most confused by, pancreatic cancer, PSC, and cholangiocarcinoma. I think that’s a huge opportunity for further research and to understanding the mechanisms of disease and hopefully to be able to identify a more accurate diagnostic biomarker.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Yeah, great points. I mean, I must admit, I tend to say to my clinical fellows there are some simple red flags for diagnosis when I think about this, and you’ve beautifully highlighted them between you. I always say there is this demographic where it’s more frequent in males between the ages of 40 and 60, but that’s not everyone because, of course, you can have that wide spread of age and particularly not necessarily for head and neck. There is this association with allergy and ATP that we see in up to half of patients. Taking an allergic and atopic history is quite nice. Also, we know about 15% of patients will have other autoimmune diseases.
Again, when you’re taking the history and you’re thinking about it, think of somebody who might have another autoimmune disease because they may overlap, and then obviously characteristic, as you said, multiple organ enlargement, so masses, strictures, and multiple organs make you think about the disease before you even get to the point of thinking of the IgG4, maybe the IgE level and complements, which actually sit really nicely as diagnostic markers together and then think of sampling.
I think all clinicians of any specialties, you need to think about these red flags and in particularly these flags about thinking across the organs. If there was multiple organs involved, there’s a history of autoimmunity, there’s ATP in a certain age group, you need to be thinking about the condition, obviously not just other autoimmune conditions and cancer. Obviously, once we diagnose, which we know is a challenge, we can start thinking about treatment options.
Treatment options will vary not just in terms of individual practices, but also in terms of whether you’re a pancreatologist or gastroenterologist and maybe whether you’re a rheumatologist. Again, if I could ask you, Guy, to speak to a little bit what you give in terms of treatment, how you prioritize patients, and talk a little bit about the pathway of management for me.
Guy Katz, MD:
Sure. As you mentioned, there’s a lot of variability in how people treat this disease both between centers and among different countries and based on availability of different treatment options. In many cases, glucocorticoids are considered to be the first line of treatment. This is certainly true for anyone with severe or organ-threatening disease, and that’s going to be the only way to quickly get the disease under control.
But the nice thing about IgG4-related disease is that it is really universally responsive to glucocorticoids. A good 4 to 6 weeks of 40 mg or above will put the disease in or close to remission in most people, and then that usually would need to be tapered over another couple of months. The only treatment that has been shown to be effective in the absence of concomitant glucocorticoids is B-cell depletion.
B-cell depletion, like glucocorticoids, is essentially or close to universally effective in the disease as well, but doesn’t work quite as quickly. You can expect that it’ll take a couple of months for the disease to really start to respond to the treatment. Usually by about 3 to 6 months, many patients have had a significant improvement. Sometimes you need another dose at the 4- to 6-month mark to really put people into remission.
The other treatment options are conventional synthetic DMARDs, in particular, mycophenolate and azathioprine are commonly used. The evidence for them is not as strong as it is for B-cell depletion in particular, given some recent data that I’m sure we’ll discuss in not so long. But in many cases, conventional synthetic DMARDs such as mycophenolate or azathioprine can be used as a steroid-sparing agent and to prevent relapses in the disease.
That can be effective in some patients, just not as universally effective as you can expect B-cell depletion to be. Now, that being said, B-cell depletion always works, but the disease almost always relapses. That is something that can be expected and these are patients that need to be followed long-term with physical exam, with imaging in many cases, and with the labs that Emma mentioned earlier to follow and see when the disease comes back and retreat them. That’s my bird’s-eye view. I’m happy to discuss in more detail, but wonder if the 2 of you have something to add to that.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Yeah, I’m going to say, Phil, I suppose adding on with regard to immunosuppression, but also thinking about more broadly not just obviously our acute management, but thinking also about our chronic management of therapy. It’d be nice to hear your views.
Phil Hart, MD:
Yeah, no, I agree with Guy’s approach and I think that’s even for patients with predominantly pancreaticobiliary disease, that’s the general approach that we would adhere to for disease control of IgG4-related disease. With the patients with predominant or GI involvement, even if it’s not the dominant manifestation of the disease, there are a number of other things though that come into consideration, particularly as it relates to pancreatic function.
The situation is that these patients are at high risk for developing diabetes and/or excretive pancreatic insufficiency, even if IgG4-related disease is otherwise effectively controlled. As an example, in patients who are treated with corticosteroids, while we expect that to result in normalization or restoration, resolution rather, of the diffused pancreatic enlargement, at least a third of patients will develop severe pancreatic atrophy on imaging.
These patients are and stay at high risk for developing both endocrine and exocrine pancreatic disease. Even in patients who otherwise have inactive involvement related to IgG4-related disease, we closely monitor these patients with lab screening. We also will routinely use DEXA imaging to screen for metabolic bone disease, which is common in the broader patient population with chronic pancreatic disease.
Then also in patients with biliary involvement, particularly if there’s involvement of the proximal biliary tract, some of these patients can develop fibrotic type strictures that, again, even in the absence of active inflammation can lead to organ dysfunction and even in that setting to secondary biliary cirrhosis, which can then create its own issues. All that to say, even once the acute disease is in remission for gastroenterologists seeing patients with pancreas and/or biliary involvement, there’s still work to be done as it relates to monitoring for other complications related to the disease.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Thanks. I think you make some really valid points there, both of you. I’m actually going to touch on 2 of those points just to draw them out. Because Guy, I mean, I know obviously Phil has beautifully there talked about what we do for pancreatic disease and some of the more damage limiting management strategies. Do you find that for other manifestations of the disease, including things like head and neck or retroperitoneal, there’s anything else that you can do to mitigate damage, or in terms of medications that you might offer or give?
Because I think we have to remember that this is not just a gastro or even rheumatological disease. This is obviously a disease that affects many organ systems. Is there any other strategies that you use or anything else you can add to what Phil nicely described?
Guy Katz, MD:
Absolutely. I think that Phil, you did such a great job of highlighting how important the pancreas and biliary system is in the disease, both in terms of how common it is and how common damage is in that part of the body. You can make similar statements about almost any part of the body that’s involved in IgG4-related disease. It’s just not quite as common or profound as what we tend to see with the pancreatobiliary manifestations.
I think the highlight is that this is a disease that really requires multidisciplinary care. I, as a rheumatologist, am not the right person to be placing ureteral stents, nor am I the right person to be doing corrective surgeries to fix ptosis and other damage manifestations of different organ manifestations. I work with a team of other specialists who help with all of the different manifestations and the different forms of damage that can happen in the disease.
Some that I’d highlight, you mentioned retroperitoneal fibrosis, I think that’s an area where we need better therapies. Because even when we do a really good job of controlling the inflammation, there’s often residual fibrosis and stricturing that happens. Where we see that most frequently is in the ureters. So many people will have hydronephrosis at presentation as a result of retroperitoneal fibrosis.
Very often with treatment we’re able to resolve that to the point that ureteral stents or other interventions can be taken away. But sometimes that’s not the case. Sometimes patients will need to have stents replaced every few months. If they’re taken out, the hydronephrosis recurs. Having urology help with that. Similarly, when there’s orbital inflammation, that can occasionally lead to compression of the optic nerve or can lead to diplopia if the orbital muscles are involved or ptosis as I mentioned before.
Once the inflammation is resolved, sometimes these are things that can be dealt with surgically. We do work with a number of surgical colleagues. Another example is sinus involvement where there’s very often we can do a great job of controlling the disease, but there’s a chronic sinusitis that happens in particular in people who’ve had erosive disease where that needs to be treated topically with topical steroids and irrigation and other things that are done from the sinus perspective.
We work with ENTs for that. I could go on with just about any organ manifestation. You can imagine that when there’s long-standing inflammation or fibrosis or both in any part of the body, local structures can get obstructed or the organ might not function as it’s supposed to, and that’s where the specialist for that particular part of the body needs to be involved to help with management.
Emma Culver, BSc, MBChB, DPhil, FRCP:
I think you’re completely right, and actually what we really want to try and do is avoid that damage and fibrosis in the first instance. We have our challenges with diagnosis, and then we have our first and second line therapies. You mentioned a few moments ago, Guy, I think it’s really important when we think about our B-cell therapies, we want to think about how they might work and how important B cells are in the disease potentially to control that inflammation and maybe even mitigate and prevent against that damage management that we might have to do.
Can you talk a little bit more about how you see the role of B cells in this disease and how going forward we might be able to target them better through some of the clinical trials that we’re currently looking at?
Guy Katz, MD:
Sure. B cells were recognized to be very important to the disease very soon after the pathophysiology was starting to be investigated and researched, and it initiated when we realized that patients with IgG4-related disease have a really robust immunoglobulin production. Their total IgG is very often quite elevated. Obviously, they have often an elevated serum IgG4, but other IgG subclasses can be elevated as well, IgG1 and IgG3 in particular.
It speaks to a certain degree of antibody production. In biopsies of patients with IgG4-related disease, in lesional tissue, you can see a large number of B cells and plasma cells, other cells within the B-cell lineage. You see a lot of those cells on biopsy, suggesting that they’re doing something. That was the rationale for initially trying B-cell depletion, which has been used for over a decade in the disease successfully.
But the first large clinical trial in the disease was only published last week, which we can talk about in just a minute. Interestingly, when B cells deplete, as you would expect, B cells in the blood do deplete very, very rapidly, but there have been studies that have looked at before and after treatment with rituximab that have shown that not only B cells are being depleted from the tissue, but also T cells.
We have quite a diverse inflammatory infiltrate, multiple types of B cells, multiple types of T cells, eosinophils, other cells that are involved in IgG4-related disease tissues. It’s really interesting that when you deplete a B cell, the T cells also are absent from tissue. Rituximab or other B-cell depleting agents really shouldn’t do that directly. It speaks to the role of B cells as antigen presenting cells that are helping activate T cells.
When you take away the B cells, the T cells are also not being activated. It really does seem like B cells are really central to the pathophysiology of IgG4-related disease and probably explains why B-cell depletion is so incredibly effective in the disease. I mentioned that recent clinical trial was done. Emma, do you want to speak to that a little bit?
Emma Culver, BSc, MBChB, DPhil, FRCP:
I was just going to add to your nice comment about B cells and more importantly, as you said, rituximab or B-cell depletion, at least CD19 B-cell depletion, depleting some of these T cells, because we’ve talked a little bit there about fibrosis. I think the other really interesting thing about B-cell depletion is it also seems to have an indirect effect on fibrosis markers, not just in terms of thinking about fibroblast or myofibroblast number and size, but there seems to be some early evidence of possible regression of that fibrosis, which is a really exciting thing that we see in IgG4-related disease that hasn’t been well described in lots of other conditions.
Emanuel Della Torre did a lovely paper looking directly at B-cell fibrosis and B-cell-related pathogenesis central to IgG4 disease and saw that these B cells were not only, as you said, antigen presenting, but also stimulated lots of different chemokines and lots of other fibrotic markers.
I think we have to think about the role of B-cell depletion, as you say, not just depleting, but T cells and potentially some of the fibrosis itself, which opens us really nicely to think about the role of more deeper B-cell depletion or different kinds of B-cell depletion with regards to clinical trials.
Guy, I know you were doing a great job. Do go ahead, talk us through this clinical trial that you mentioned, the MITIGATE trial, that’s just recently been published.
Guy Katz, MD:
Sure. This was one of the most exciting periods in IgG4-related disease since it was discovered. Just last week in The New England Journal of Medicine, the MITIGATE trial was published. It was the largest clinical trial in IgG4-related disease to date; randomized, placebo-controlled trial of inebilizumab, an anti-CD19 humanized antibody, for the treatment of IgG4-related disease; 135 patients were in the trial, about half and half placebo and inebilizumab.
All of the primary endpoints and key secondary endpoints were met. There was an 87% reduction in risk of flare, which was the primary endpoint. Overall, the safety profile looked very good. There was a strong reduction in the total use of glucocorticoids in the patients with inebilizumab compared to placebo. It was a 52-week trial. There’s only so much that can be gleaned in terms of long-term safety from a trial like that. But in terms of the efficacy, it was what we expected to see given our previous experience using rituximab and anti-CD20 agent. But it did confirm that B-cell depletion seems to be extremely effective in the disease.
Emma Culver, BSc, MBChB, DPhil, FRCP:
I guess the advantages of such a trial and the publication of such a trial is that obviously we would hopefully move to an approved therapy rather than an off-label therapy, which rituximab currently is. I think as you nicely spoke to, this is a CD19 agent, so it’s depleting some of the pro-B cells, but also some of the plasmablast in the plasma cells that we believe is integral into the disease.
Potentially, a deeper B-cell depletion, and also it’s humanized rather than a chimeric antibody where we know there is some hypersensitivity and potentially anaphylaxis. Having a humanized therapy is a fantastic adjunct. I think on top of that, obviously we have a drug that’s been shown in trial, and we do have a number of other drugs in clinical trials as well, which I think it’s important to remember.
We have drugs at the moment in clinical trials, including the INDIGO trial, which has just recently closed to recruitment, which again is another multicenter trial that was looking at a B-cell inhibitor in the disease overall and thinking very much again at targeting those patients who have active and also relapsing disease with a subcut infusion. Hopefully we’ll see the results of that in the new year.
Wwe’ve had some phase 2 trials as well looking at signaling pathways for B cells and thinking about targeting different types of T cells. I think this is a really exciting time for IgG4-related disease when we think about not just B-cell trials, our fantastic MITIGATE trial that potentially will revolutionize the therapy in this disease, but also how we might inhibit and target signaling pathways going forward for different patients.
Phil, how do you think this will affect… Because obviously we know that 50% of the patients in this trial have pancreatic disease. How do you think this is going to affect the GI community when we think about managing patients with the disease when often steroids is the only 1 drug that is given before we think about escalating therapy?
Phil Hart, MD:
I think time will tell how this affects the treatment strategies, treatment paradigms by gastroenterologists. My hope is that to our comments from earlier that there’s need for increased awareness. My hope is that the clinical trial publication will be a catalyst to that and to really put this at the front of people’s minds, both gastroenterologists as well as primary care physicians as well for that matter. Even in the study results, they can just see very easily the wide distribution of organ involvement. But so for gastroenterologists, again, I think this will be helpful, again, to raise awareness and to be confident that we have active treatment that we can rely on. It’s as rigorously demonstrated to be effective as I think will be feasible. Then the next step really falls back to the gastroenterologist to carefully think through this possibility when we’re evaluating patients in our clinics or in the hospital. To ask the question, could this patient have IgG4-related disease? To Guy’s comment from earlier, this is going to, again, hopefully be a reminder that this is not something that gastroenterologists can do alone. We have to have rheumatologists. We have to have ophthalmologists. We have to have all of these subspecialists, particularly the diagnostic ones, pathologists and radiologists. My hope is, again, that this will really catalyze and really help to foster increased awareness to identify patients who can benefit from therapy.
Emma Culver, BSc, MBChB, DPhil, FRCP:
It sounds like you both agree. I mean, this should be multidisciplinary, shouldn’t it? We need to be moving forward-thinking about how we do best multidisciplinary care for patients, both in terms of diagnosis, but also when we start thinking about management from both active disease and also we talked about fibrosis and damage.
This needs to be multidisciplinary. Do you think that the future really will be multidisciplinary teams, whether it is virtual, whether it is in person managing these patients together, or do you think that that’s too far-fetched to imagine in this current scenario and setup?
Guy Katz, MD:
Well, maybe I’m an optimist, but I would love to think that this is going to be a multidisciplinary disease. At least in our center, it already is. I know that at your center, you have a wonderful multidisciplinary clinic. We have very great working relationships with our pancreatologists and our ophthalmologists and ENTs who all have expertise in the disease.
Of course, that’s not necessarily going to be possible everywhere, but as long as there are people that are thinking about it and considering the different things that need to be done, I think that’s the most important thing. Probably the most important interdisciplinary relationship is between gastroenterology and rheumatology. I think that’s in part now that we have clinical trial level data and hopefully an approved treatment soon, B-cell depletion is just not something that most gastroenterologists have comfort with.
It’s not something that’s used for other GI immune-mediated diseases. That’s where I think teaming up with rheumatologists is going to be important. Rheumatologists also have a little bit more training in looking for organ involvement outside of the GI system. I think sometimes when there’s subtle presentations elsewhere, it can be helpful to have that multidisciplinary approach.
I always trust my GI colleagues’ insight more than my own when it comes to the pancreas and managing exocrine pancreatic insufficiency and how to interpret changes in imaging and all of these things that can be very challenging to understand as you follow these patients long-term. I don’t think this is something that I can do by myself. I don’t think it’s something that any of us can do by ourselves. I have already really enjoyed my multidisciplinary team and think that that’s the way forward.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Yeah, I agree. You have to lean on team members. As you alluded to, Guy, very much in the UK at least, we’ve run a national MDT meeting from 2016, and predominantly that was because of the diagnostic challenges we had and then the management decisions and obviously no approved therapy and having to make decisions with limited evidence sometimes.
It works quite nicely in our national health system to get referrals and to discuss virtually with multiple different specialists that are beautifully represented across the country. But as you rightly say, that doesn’t always work perfectly for every single institute. You’ve got to work what’s best for your institute, but make sure that you lean on your colleagues and that you involve them because no one can manage it on their own.
Is there anything else that either of you want to add to this really great discussion, because it’s been a great discussion about diagnostic and management, both strategies and also dilemmas? Anything else you’d like to add before I bring it to a close?
Phil Hart, MD:
Emma, I think this topic of multidisciplinary approach really just can’t be overstated, both from diagnostic evaluation as well as from a treatment standpoint. I would add too, related to some of the challenges for physicians practicing where healthcare is not organized in a centralized manner, one of the other ways is that the reality is that not every physician works in a practice where assembling a multidisciplinary team is feasible due to multiple reasons.
Because of that, it’s also perfectly fine for a gastroenterologist who has less accumulated experience to refer to another gastroenterologist or a rheumatologist to another rheumatologist. I think that is a model that doesn’t take all of the programmatic building that is really for some individuals and for some healthcare systems may be difficult to justify due to the low case volume.
But that I think is at least a temporary workaround in situations where there’s diagnostic uncertainty and/or where there’s just a difficult to treat patient, where there are questions that come up is to refer to a different center.
Emma Culver, BSc, MBChB, DPhil, FRCP:
Fantastic comment. Yeah, I think that’s really important. Thank you very much, everyone, for joining us. I think it’s been a great discussion amongst peers about our diagnostic challenges that all of us have, our management strategies, which are our current management strategies, but now also the opportunities for the future with new clinical trial data that’s come out, with clinical trials that are ongoing and hopefully we’ll receive the results of in the next few years, and also in the way that we strategically manage patients as a multidisciplinary group. It’s been great that you joined us. Thank you very much Phil, and thank you, Guy, for that great discussion.
