Myasthenia Gravis Learning Zone
Transcript: Implementing recent gMG guidelines
Professor Andreas Meisel
Interview recorded June 2024. All transcripts are created from interview footage and directly reflect the content of the interview at the time. The content is that of the speaker and is not adjusted by Medthority.
So, my topic is implementing recent guidelines for generalised myasthenia gravis to improve patient care. So what has been changed over the last almost seven years, is that we have drugs and it's a door opener for the new world and treatment of myasthenia gravis was eculizumab, which was approved in Europe in 2017. Then we had in 2022, a new class, with the FCN inhibitor, which was opened by efgartigimod, which was, by the way, myasthenia gravis is the first disease where this drug has been introduced. Ravulizumab is the further candidate which has been introduced at the end of 2022, which has a difference in terms of frequency the drug has been given compared to eculizumab. We have in class, in addition, zilucoplan approved as beginning or the end of 2023 and efgartigimod, we have since end of last year, not only intravenous application but also subcutaneous application. And the further candidate in the FCN class is rozanolixizumalumab, which has been approved as the beginning of this year. I will just give a short overview on how we have just in Germany, dealed with a new treatment choices we have. I want to show here, first of all, because I think there are a lot of information you probably can't get in time here, you will find some main information in the publication, which has been done in the last year, "Guidelines for the management of myasthenic syndromes", which was published in Therapeutic Advances and Emerging Disorders. And the main starting point for us was that the therapeutic goal is to achieve the best possible disease control while storing the patient's quality of life. And that is not new in a way, but when I say that the aim is to achieve it as fast as possible, then it is a bit more, stronger than it was in the past.
And this there was a consensus, which is a strong consensus. Important for this is that we have an ongoing assessment in this patient, and I want to read it here what we have written in the guidelines, this assessment is based on the current severity of disease, which takes into account the current status according to the Myasthenia Gravis Foundation of America, classification, which differentiate between mild, moderate from highly active disease including refactory disease. And this is different to what we had before the last part of the sentence that we differentiate between a mild, moderate to highly active disease, and how we do that, the determination of disease activity that is based on the severity of clinical symptoms and the duration tendency to regress on one hand, on the other is we should take into account clinical residual symptoms, present number of crisis like exacerbation or crisis, and the intensity of treatment we need for these patient. That's not very specific. We have done a bit more specifically, but we can, we don't talk about show it here in detail, discuss in detail, but there we have not so much evidence that we have real markers how sharply differentiate between mild moderate to highly active disease, we can only have clinical parameters for that. What is new in the German guideline is that I only want to focus here on the AChR antibody positive generalised myasthenia gravis since that is where we have the new approvals for the most components I mentioned before.
And what you can see here is that the mild moderate disease activity, which is that first choice of treatment, is still the old world using steroids, azathioprine, a steroid we interpret as first choice in Germany and thymectomy in these patient, of course we are talking about a disease modifying therapies, it's important to know that pyridostigmine as symptomatic treatment is highly important still even in this world of changing opportunities. Second choice mostly in Germany is mycophenolate mofetil and cyclosporine A and methotrexate are not so often used, at least in Germany. What is important now for when talking about the new drugs that these drugs should use, as given the approval add on to the standard treatments for patients which have been labelled by high disease activity or severity, including the so-called refractory cause of the disease. And what we have here as for choice as complementy inhibitors, eculizumab, ravulizumab and FCN modulator, efgartigimod as mentioned earlier, we have also zilucoplan, and rozanolixizumalumab, which is the guideline changes in progress. But probably we will see soon that these drugs will also be part of the guideline at the classes mentioned here. We also labelled in the guideline as choice rituximab, which is I think much more stronger recommended in the Scandinavian guidelines. Second choice is that we use an interval a few of patients still intravenous immunoglobulin leads. So what does it mean in the area of trial therapy, which we can help patients reach elevated treatment goals, including best possible disease control. Of course, we want to achieve at best, complete stable remission or pharmaco remission, which is minimal symptoms expression. We want to have the highest quality of life in this patient and participation in life, and this not on cost of steroids, so we want to have either steroid dosages, which are lower five or 7.5 milligramme prednisone or pushing cut off levels. This is important treatment goal.
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