Written by Geoff Case, RARE Revolution Magazine

Sjögren’s disease is both under-recognised and misunderstood, says Janet Church, president and CEO of the Sjögren’s Foundation. Here, she explains the urgent need for the misconceptions around the disease to be dispelled.

Janet Church, 62, the president and CEO of the US-based Sjögren’s Foundation, is quick to make an essential point about Sjögren’s—a disease that has affected her since childhood but was only diagnosed when she was 41.

“Sjögren’s is not a rare disease,” she says. “It’s had that label because people have never heard of it, but just because people don’t know the word doesn’t mean there’s a low prevalence.” Janet believes that Sjögren’s is one of the “most prevalent rheumatic diseases” and that many people are living with the disease without a diagnosis.

Janet also believes that Sjögren’s has “absolutely” been trivialised over the years. “It is not widely taught in medical schools, and many doctors are unfamiliar with it,” she says. Compounding that problem is the current deficit of rheumatologists in the healthcare workforce,^1,2 and, as she believes, the tendency for Sjögren’s to be considered a mere “nuisance” that causes only dry eyes and dry mouth, by those doctors who have been incorrectly trained about the disease.

Accurate knowledge of the disease has now grown among rheumatologists, Janet thinks, but there is still a lack of awareness among primary care doctors and the specialists that many patients need.

“Over the years, we’ve learned so much more about the disease. The disease is a serious and systemic disease, and so many people suffer because they are not getting correct treatment.”

Although Sjögren’s can affect anyone at any age, more women than men have the disease. Janet says that it is not uncommon for symptoms of Sjögren’s disease to worsen for women around menopause. Some doctors make the assumption that the menopause is the reason for their quality-of-life change, or mistakenly diagnose a different disease with similar symptoms.

“A patient really has to push for a diagnosis and they may not do that, so then they just continue to suffer.”

Sjögren’s varied symptoms means that a doctor may refer a patient to several different specialists, depending on the most prominent symptom. For example, patients with postural tachycardia syndrome (PoTS) are often referred to a cardiologist or a neurologist, and if those doctors do not know that Sjögren’s is a potential cause of the issue, they may not refer them to a rheumatologist for appropriate treatment. (The Sjögren’s Foundation has recently been working with neurologists to highlight the high prevalence of neurological symptoms in Sjögren’s patients.)

Janet knows of many people with Sjögren’s who were wrongly diagnosed with another rheumatic disease. “It’s also not uncommon that a patient diagnosed with one rheumatic disease may also have Sjögren’s,” she says, “but the doctor does not continue with Sjögren’s tests once there is a positive diagnosis of another disease.”

’It is essential that every doctor understands Sjögren’s disease, Janet says, so they can consider it as a potential diagnosis, order appropriate tests, and if the results are positive for Sjögren’s, treat the patient to help manage the disease and the patient’s symptoms. “Caring for a Sjögren’s patient is different than caring for a patient with other diseases,” she affirms.

The Sjögren’s Foundation website has an online training course on Sjögren’s disease, which gives continuing medical education (CME) credits to medical practitioners in the United States.

Janet believes that one of the most damaging misconceptions about Sjögren’s disease, which often delays a diagnosis, is that it is an “old lady disease” that only affects menopausal women. In fact, “it can affect anyone, men too, at any age”, she says. “Not enough is yet known about the disease and how it progresses. There are many people, including me, who believe that the disease can have a quick onset for some people, and a long, progressive onset for others.”³

In her own case, Janet is convinced that she has had Sjögren’s for a very long time. Looking back at her childhood health history, she can identify “oddities that no one could explain” that align with the known presentations of childhood Sjögren’s.

Janet believes that other misconceptions arise from the very words that are used to name the disease. She says that the term “Sjögren’s syndrome”, used for decades to describe the disease, tends to trivialise it.

She explains that many patients object to the term “syndrome” because it focuses attention on the presence of signs and symptoms rather than the serious and damaging disease that causes them. “Sjögren’s is not a collection of symptoms. Our quality of life is incredibly impacted by this disease—some more than others, but we don’t know if those others will become more highly impacted over time.”

Janet explains how the terms “primary” and “secondary” have often been attached to a person’s diagnosis of Sjögren’s disease. This is “incredibly misleading” and not done for any other autoimmune disease, she says.

It is frustrating, she adds, that if somebody is diagnosed with Sjögren’s first, they are said to have “primary Sjögren’s”, but if they are later diagnosed with another rheumatic disease, their Sjögren’s becomes “secondary Sjögren’s”. This is another way, she believes, that the disease is minimised.

“Rheumatic diseases are horrible and one shouldn’t be judged against the other. You have a disease, or you don’t… and if you have multiple diseases, they all need to be appropriately treated.”

Janet explains that this distinction has also affected our understanding of how common the disease is, because people with another autoimmune or immune-mediated disease have been discounted when the total number of Sjögren’s patients is calculated, especially in the US.

In recent years, the international Sjögren’s community has been transitioning the nomenclature to refer to Sjögren’s as a “disease” rather than a “syndrome”. The decision about whether to use the umlaut (ö) or the possessive apostrophe (’s) rests with national organisations.

“However, this is a time of great hope,” comments Janet. “There is significant research occurring to discover more about Sjögren’s. What we know about the disease today may drastically change within the next five years. It is my hope that every Sjögren’s patient will have a better quality of life in the future.”

References

[1] Battafarano DF, Ditmyer M, Bolster MB, et al. 2015 American College of Rheumatology Workforce Study: Supply and Demand Projections of Adult Rheumatology Workforce, 2015-2030. Arthritis Care Res (Hoboken). 2018;70(4):617-626. doi:10.1002/acr.23518

[2] Miloslavsky EM, Marston B. The Challenge of Addressing the Rheumatology Workforce Shortage. J Rheumatol. 2022;49(6):555. doi:10.3899/jrheum.220300

[3] Melissaropoulos K, Bogdanos D, Dimitroulas T, Sakkas LI, Kitas GD, Daoussis D. Primary Sjögren’s Syndrome and Cardiovascular Disease. Curr Vasc Pharmacol. 2020;18(5):447-454. doi:10.2174/1570161118666200129125320

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