Written by Geoff Case, RARE Revolution Magazine

Speaking with RARE Revolution Magazine at the first Sjögren’s conference for patients, Dr Chiara Baldini, assistant professor at the University of Pisa, explains how scientific breakthroughs are shaping care pathways in Sjögren’s disease.

Hello, Chiara. Firstly, what sparked your interest in Sjögren’s?

After medical school, my residency was in rheumatology, and my mentor was Professor Stefano Bombardieri, who was heavily involved with research around the classification criteria for Sjögren’s, so I was literally breathing Sjögren’s while I was there. This is why my interest became so great, and it was natural for me to become involved in research into Sjögren’s and with the clinical aspects, too. Each step of my academic career was focused on Sjögren’s research.

What do we know about how Sjögren’s affects quality of life for patients?

The main symptoms are chronic pain, dryness and fatigue, and these are the unmet needs in clinical practice. These really affect quality of life: you’re tired, you’re in pain, you can’t do the activities of daily life, including work activities, and you cannot sleep well.

Pain, dryness and fatigue are the main symptoms affecting patients’ quality of life; however, Sjögren’s is much more than that. Between those symptoms and the most serious complications, which in the vasculitis phenotype of the disease escalate in severity up to lymphoma, Sjögren’s is a disease that can affect any of the organs and systems in the body: the liver, the kidneys, the central neurological system, the peripheral neurological system, the digestive system, and so on. And in each case, there is an impact on quality of life. For example, without saliva you can’t eat properly, and that digestion problem compromises everything.

So, how Sjogren’s affects a patient’s quality of life is complex because of the symptoms, but also because it is a systemic disease that that may affect multiple organs in many different ways. Each patient’s health is different.

What gaps exist in the care patients receive?

There are a lot of gaps, and the first one is the diagnostic delay. Patients undergo a long journey between the onset of signs and symptoms and finally receiving a correct diagnosis. In most cases an invasive procedure (a biopsy), is needed to make that diagnosis.

When you do have the proper diagnosis, you start another journey, which is the follow-up. Because of the varied nature of the disease, you may have to manage your life around different tests and around appointments, not only with a rheumatologist but also with an ophthalmologist, an oral health specialist, a neurologist, a urologist, and, for many women, a gynaecologist. So it’s complicated. There is no smooth pathway to take care of your disease.

“You have so much to do to get the therapies you need for the disease.”

Then the most important unmet need is therapy. We have drugs that partially relieve symptoms, but we don’t have effective treatments that prevent the damage caused by the disease. So the disease is slowly progressive, despite the treatment that patients are receiving, and sometimes there may be adverse effects arising from the drugs, too. If you know that a drug is not controlling the symptoms properly and there is damage from the disease, it’s not easy to accept these.

Also, most drugs treat just a small part of the entire disease, like dryness, for example, but other symptoms are still there and are not addressed. So, I think that the most important gap is meeting the therapeutic needs that patients have.

What improvements have there been in the understanding of Sjögren’s?

In the last 10 to 20 years, we have gained many new insights. When I started to work with patients with Sjögren’s, textbooks had a small paragraph describing “Sjögren’s syndrome” as characterised by dry eyes and dry mouth and a list of possible other symptoms, and that was it.

Now, we have a much better insight into the pathogenesis of Sjögren’s and its progression. Importantly, we know more about the early phases of the disease. The more we know about this, the more we can do to target the pathophysiological mechanisms underlying the disease. We also have better information about the risk factors for developing lymphoma. And we can now also stratify patients by their clinical phenotype because we have a better understanding of the complexity of the disease.

In the past, Sjögren’s was the Cinderella in the rheumatological field. In the scientific world and in the community, it was a poorly understood disease. Today, we are here at the first conference organised by Sjögren’s patients—and RARE Revolution is here, too. This shows how Sjögren’s is much better known now.

Finally, a big step forwards is how we are developing therapeutic approaches with many novel drugs; there are many clinical trials underway.

What are the latest developments in Sjögren’s?

We have a totally new approach to the disease, based on the improvements that have been made. This new approach means looking at patients from a holistic perspective. It means not only considering the dryness of the eyes or mouth but trying to elaborate therapeutic strategies, and following up in a way that considers all the aspects that affect patients’ lives. It means fostering a multidisciplinary approach and turning scientific developments into improvements in the quality of clinical care.

“It’s not just about making the developments; it’s about translating them from the bench to bedside.”

It means having Sjögren’s clinics everywhere that are focused on the disease. It also means fast-tracking patients towards diagnosis, with protocols involving other specialists in every diagnosis.

Randomised clinical trials are probably the most concrete development that we have. In these trials we have many biological drugs with different targets, so we have the prospect of potentially having treatments that are not one-size-fits-all but individualised to the patient.

If I had to highlight one development, I would start with how we’re doing research: we are collaborating internationally on very big projects. And this is crucial. With the results of basic and clinical research, we are improving the quality of care. New therapeutic strategies coupled with better clinical phenotypes mean that we are moving towards an individualised therapeutic approach.

We are here in Athens at the first conference for Sjögren’s patients. Can you share some of your personal highlights?

This is the first conference organised by patients and this is a revolution. It puts patients, doctors and pharma in the same room, at the same level, so they can discuss the unmet needs and what we can do together to address them. There have been so many highlights!

Yesterday, we shared our knowledge about ongoing and completed research projects and heard how patients have been empowered by being involved in these projects. We discussed clinical phenotyping, the necessity project, biomarkers and composite endpoints for clinical trials. We had talks on the general aspects of the disease, including some of the key themes, particularly fatigue and pain. We had the opportunity of combining the perspectives of doctors and patients to focus on the unmet needs that patients have around mental health and fatigue, and this was really important. I think working together successfully means having effective communication, so using the same language is key. If I am saying “pain”, “fatigue” or “mental health” and the patient has a different view of what these mean, we’re not talking the same language. That is a problem.

“I think that one highlight of this conference has been our working together and trying to give words the same meaning for everyone going forwards.”

We have also discussed physiotherapy and the role of a complementary approach. I think that another of the highlights of the conference was the attention paid to mental health. Alongside the problems around dryness, glandular involvement or single organ involvement, there’s well-being. Coralie Bouillot highlighted the strong relationship between depression, anxiety and disease activity. They are one problem and they should not be separated.

One speaker suggested we worked in a more multidisciplinary way on this and that having a psychologist on the team would help patients come to terms with the chronic nature of the disease and would enable us to develop strategies to support patients’ well-being.

“Therapy in Sjögren’s is not just about drugs; it’s about taking a broader perspective of a patient’s daily life, thinking about exercise and thinking about mental health.”

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The interview transcript was edited for length and clarity.

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