This year, World Sjögren’s Day is on July 23. We spoke with Linda Stone of Sjögren Europe about the challenges of the chronic inflammatory immune disease, the empowerment of Sjögren’s patients, and the organisation’s plans for the awareness event.
Please tell us more about Sjögren Europe and its vision.
Sjögren Europe was formed in 2019 as a federation of properly constituted national Sjögren’s patient organisations.
Six of the seven Board members have Sjögren’s, and we are all active in our own national associations. We are also all active in differing ways in a variety of research projects. Our collective experience covers the range from project team through patient advisory group to patient subject. The members of the Board and the Medical Board are all volunteers. Funding comes solely from modest member fees plus donations and discretionary grants from Pharma industry.
Sjögren Europe’s purpose is “to increase visibility, attention and solutions for Sjögren’s Syndrome by promoting the advancement of knowledge, research, information, treatment and care, by fostering patient involvement and participation in scientific research, medical, health, political institutions, pharmaceutical industry and social areas, and by increasing awareness about Sjögren’s Syndrome at all levels.”
The ideal is for Sjögren Europe to be accepted at the table whenever and wherever within Europe Sjögren’s is being considered.
What exactly is Sjögren’s, and is there maybe an overlap with other diseases?
Sjögren’s is a chronic inflammatory immune disease which presents in diverse ways. The common recognised symptoms are dryness (especially eyes and mouth) and fatigue (approximately 70%), but this is too simplistic. Sjögren’s can develop to impact one or more of several major organ systems, for example, lungs, gastrointestinal system, bladder and peripheral nerves. Research is ongoing to see if phenotyping sufferers predicts the likelihood of developing such serious effects in the future. The different phenotypes may indicate the probability of response to therapy.
Currently, there is no agreement amongst patient groups about overlap syndromes. Is it a result of Sjögren’s or a separate disease? Similarly, there are a number of differing opinions within the relevant medical professionals.
What are the challenges of Sjögren’s, for example regarding diagnosis and treatment?
Receiving a diagnosis is challenging. The heterogeneous nature of Sjögren’s seriously complicates diagnosis, frequently leading to misdiagnosis and or significant delays in receiving appropriate care. There are not enough rheumatologists with an interest in, and knowledge of Sjögren’s to ensure that every patient receives the best care. Access to medication is variable from country to country as is access to additional care such as dentistry (lack of saliva results in vulnerability to tooth decay), ophthalmology (poor tear film leads to corneal damage), hydroxychloroquine monitoring, physiotherapy, etc.
Treatment is mainly symptomatic – eye drops, emollients, secretagogues, etc. Currently, there are no treatments to halt disease progression let alone reverse it or cure the sufferer. Research into potential new treatments is problematic. The heterogeneous presentation has caused difficulties in identifying trial outcomes that are measurable, reproducible and meaningful for both researcher and patient. Projects are still endeavouring to produce a useful set of Patient-Reported Outcomes (PROs) which will aid research into treatments to alleviate Sjögren’s.
What is important for empowering the patients to have the utmost quality of life possible?
Living with Sjögren’s is difficult, protecting dry eyes and mouth, moisturising dry skin, and coping with enveloping fatigue. Life is exhausting, and this is before even taking into account its long-term effects on the body together with the various diseases of age and the continual battle for access to appropriate care and treatment. Patient support groups play a significant role in guiding patients and empowering them to manage their disease and have the best possible quality of life.
The overall impact on family and personal life is immense. Socialising is limited by fatigue and difficulty coping with smoke and air conditioning. Photophobia is commonplace. Keeping sufficiently hydrated can be difficult but adequate fluid is essential to ensure that medication and food can be comfortably swallowed.
The variation in presentation and difficulty in achieving a diagnosis create a further challenge. Sjögren’s is not recognised by welfare authorities as a disease meriting disability benefit. This lack of recognition results in financial hardship for some severely affected sufferers at a time when they are most in need of help.
Why can’t Sjögren’s be regarded as a rare disease as such?
Sjögren’s is considered to be a rare disease by some, and not so by others. As knowledge of the prevalence and incidence varies from country to country, and estimates vary even more, it is difficult to be definite. What is agreed is the variation in presentation which could influence the view of clinicians.
What does Sjögren Europe plan for World Sjögren’s Day on July 23 to raise further awareness of the syndrome?
World Sjögren’s Day on 23 July is a wonderful opportunity to promote understanding of this devastating disease and help sufferers. This year, Sjögren Europe launched its campaign on 14 June with a webinar on fatigue. This has been followed up by a series of posts on social media. These posts are based on “15 Types of Fatigue” by Teri Rumpf PhD. The campaign will culminate on 23 July with the publication of a booklet, authored by Prof Rinie Geenen, offering advice and tips on how to manage Sjögren’s fatigue. This will then be augmented by a leaflet summarising Teri Rumpf’s 15 types of fatigue.
Many thanks for the interview and for your insights.