Navigating the complexities of IgG4-Related Disease: an expert’s insights on improving care and awareness
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Dr Alireza Meysami, a leading expert in IgG4-related disease, shares the challenges healthcare providers face in diagnosing and treating this multi-systemic condition, and the opportunities he sees for increasing education among the medical community. His insights offer a comprehensive view on the evolving landscape of this rare and misunderstood disease
Written by Emma Bishop, RARE Revolution Magazine
Interview with Dr Alireza Meysami, division head of rheumatology, Henry Ford Health, associate professor of medicine at Michigan State University and Wayne State University
Dr Alireza Meysami’s first interaction with IgG4-related disease (IgG4-RD) was during his residency in Boston, while working with Dr John Stone, who he describes as the “guru of IgG4-RD”. This encounter with his first IgG4 patient was in 2008-9 at a time when it was almost unheard of in the US. Fast forward six years and Alireza has established an IgG4-RD clinic at Henry Ford Health to treat patients and educate healthcare providers and rheumatology fellows, where he is building a patient registry and participating in multiple clinical trials.
Alireza notes that there are multiple challenges in treating a condition such as IgG4-RD, the first being the multi-systemic nature of the disease, making it difficult for a single specialist or primary healthcare provider (HCP), to recognise it. Additionally, the manifestations are not specific to IgG4-RD and can mimic other conditions, such as malignancies and infections, making it hard to fit the pieces of the puzzle together.
He explains that diagnosis of IgG4-RD can be difficult as there is no biomarker. “The diagnosis is usually a combination of clinical suspicion, radiographic findings, high IgG4 levels in the blood in maybe 75% of the cases and pathology findings from a tissue biopsy.” Often there is a delay in diagnosis or a misdiagnosis, because there’s no one biomarker for this condition.”
Treatment for IgG4-RD is complex, with no FDA approved medication. The first line of defense is steroids, which can have several side-affects and cannot be a long-term solution. Other immunosuppressive treatments require close monitoring and have their own complexities.
Lastly, Alireza notes that there is no clear guidance on how to monitor the disease activity and respond to treatment as the disease can present in unpredictable ways. “The disease is metachronous, which means that the relapse of the disease can happen in completely different places at different times—patients don’t always have a relapse in the same place they had it at the time of initial presentation. It makes it challenging to know what to look for and where to look for it.”
Alireza emphasises the importance of patients relaying all symptoms, however minor they may seem, to their doctor. “This can be a daunting question for providers to ask, as it may feel overwhelming to address so many symptoms at once. However, it is also one of the most critical questions to ask. Patients need to discuss the seemingly unrelated symptoms with their HCP, because sometimes symptoms such as dry eyes, fatigue or weight loss can open the door to this multi system organ involvement.” He notes that it can help HCPs recognise the disease earlier, leading to improved outcomes rather than permanent damage.
Effective communication among providers is also crucial, as patients often see different specialists citing different symptoms, and so building a strong network of communication can aid in early diagnosis.
Alireza concludes that in bringing seemingly unrelated symptoms to the attention of different providers builds awareness for doctors and improves the education of this multi-systematic condition. “Providers can look at these symptoms and think ‘should I consider a disease that can explain them all?’”
Alireza cites one of the challenges for improving education of IgG4-RD among HCPs as the very nature of the condition being rare. “Because doctors don’t encounter this disease regularly, they may not prioritise learning about such a rare condition, making it challenging to spark interest within the medical community.”
Another challenge is the lack of training doctors currently receive on IgG4-RD in their medical education. IgG4-RD is not a part of the curriculum of any specialty that Alireza is aware of. He notes that, because of the multi-systemic nature of the disease, it doesn’t clearly belong to any one specialty making it difficult to determine which departments it should extend to, and which specialty should be responsible for leading the education. Alireza raises the question: “Should we teach gastroenterology fellows about eye manifestations, ear manifestations or subglottic manifestations? They may not have the interest or the capacity to engage in such extended learning.”
Alireza sees grand round presentations as one of the main opportunities for educating doctors (grand rounds are formal medical education meetings where clinical cases, research or emerging medical topics are presented to a group of physicians, residents and students). He conducts grand rounds on IgG4-RD and says it is crucial to deliver the training to multiple specialties to help expose more providers to the condition. “It’s important not only to give grand rounds to your field of medicine, like rheumatology or internal medicine, but also to neurology, pathology, radiology, ENT and ophthalmology.” He has seen great success in his grand rounds and has received positive feedback and referrals from doctors who, upon hearing the case studies, have been prompted to think of a patient whose symptoms may point to IgG4-RD.
A third opportunity he identifies is the development of clinical guidelines and diagnostic criteria for IgG4-RD, providing a standardised approach for providers to recognise and manage the condition. Alireza believes there is a hesitation in creating these guidelines because, being a relatively newly described disease, it is still not well understood or widely accepted, but he hopes for preliminary guidelines and recommendations in the next couple of years. In the meantime, he says he, and others who treat IgG4-RD, form their own guidelines based on their experience treating patients with the condition—guidelines which evolve as they learn more about the disease and collaborate with others in the field.
Lastly publishing reports and case studies is vital to building an evidence base and educate the broader medical community. Publications and research studies can highlight the diverse clinical presentations, diagnostic approaches and management strategies for this condition, ultimately leading to better patient outcomes.
Alireza has plans to establish a centre of excellence for IgG4-RD at Henry Ford Health by 2025. His goal is to bring together providers from different disciplines who have an interest and expertise in IgG4-RD, to create a collaborative network of specialists providing comprehensive multidisciplinary care for patients. The aim is for the centre to have access to diagnostic tools, such as advanced imaging and specialised pathology services, to facilitate accurate diagnosis of IgG4-RD. This would help streamline the diagnostic process and avoid unnecessary invasive procedures or even surgery.
The centre will serve as a hub for research on IgG4-RD, including participation in clinical trials, which will allow the centre to contribute to the growing body of evidence and potentially offer patients access to new treatment options. It will play a key role in educating providers, not just within Henry Ford Health, but in the broader medical community, with grand rounds, workshops and educational initiatives to improve knowledge of the disease.
Alireza says the centre of excellence should meet certain criteria in terms of the number of the patients, clinical trial participation, publications and experts in that field to ensure it offers meaningful value and provides a high-level of care and expertise in the IgG4-RD field.
Alireza sees patient organisations as pivotal in providing patients with valuable education and resources about their condition, and in empowering them to feel more informed and engaged in their own care. He says patients can discuss the learning with their doctor, something that is especially beneficial when navigating life with a rare disease.
The rarity of IgG4-RD can cause patients to feel lonely and isolated, therefore connecting with others following the same path can be incredibly beneficial and promote enhanced well-being. “When patients are part of advocacy groups, like the newly formed IgG4Ward!, they are not alone. It fosters a sense of community and benefits them emotionally to be hopeful and help themselves. It also helps them advocate for themselves and for others, which I believe is very important.”
Alireza believes patient organisations offer a mutually beneficial relationship between patients and HCPs. They can serve as an educational resource for HCPs, who can learn from the patient experience and perspective, which can help improve recognition and management of IgG4-RD. The organisations can also facilitate referrals to providers who have expertise in the condition, helping to connect patients with the appropriate specialists.
April 4th is the annual awareness day for IgG4-RD, something Alireza is a staunch supporter of. “I am a big fan of awareness days. IgG4-RD is rare and under diagnosed, so an awareness day does a number of things. It’s an opportunity to raise awareness, educate, discuss diagnosis, treatment and the burden of the disease throughout society, to both our patients and healthcare providers.”
He feels the awareness day can provide an opportunity for people to engage in advocacy efforts, such as reaching out to lawmakers and policymakers to discuss the needs of the IgG4-RD community, helping to drive progress in areas like research, funding, access to care and recognition of the condition.
“The sense of unity and collaboration that comes from an awareness day unifies us all to try to address all rare diseases.”
Articles within this digital spotlight are for information only and do not form the basis of medical advice. Individuals should always seek the guidance of their medical team before making changes to their treatment.
This digital spotlight has been made possible with sponsorship from Amgen. All opinions are those of the contributor. RARE Revolution Magazine retains all copyright.
