What Research Is Being Done on Sjögren's Syndrome?

Through basic research on the immune system, autoimmunity, genetics, and connective tissue diseases, researchers continue to learn more about Sjögren's syndrome. As they get a better understanding of the genes involved and which environmental factors trigger disease and how, they'll be able to develop more effective treatments. For example, gene therapy studies suggest that we may someday be able to insert molecules into salivary glands that will control inflammation and prevent their destruction. Other research focuses on how the immune and hormonal systems work in people who have Sjögren's and on the natural history of the disease (learning how it affects people by following those who have it).

This 2019 study has identified 4 subtypes of Sjögren's syndrome which could lead to personalized treatment for patients.

Scientists at Newcastle University, UK, have found there are at least 4 versions of primary Sjögren's syndrome by discovering 4 different types of SS each with their own unique patterns of clinical and biological characteristics. that may respond to different treatments.

This is the first study to report 4 different subtypes, breaking the traditional view that SS has only 2 main subtypes - primary and secondary. The subtypes were:

low symptom burden (LSB)
high symptom burden (HSB)
dryness dominant with fatigue (DDF)
pain dominant with fatigue (PDF)

Professor Fai Ng, from Newcastle University's Faculty of Medical Sciences,

said:

We are very excited about our findings. To date, we have no effective treatment for this condition and we believe our study may help significantly with drug developments into PSS.

One of the key barriers to research has been that the clinical presentations of patients with the condition differs markedly from patient to patient.

Knowledge of these subtypes will now help us to develop more personalised management plans for those with the condition, which in turn will help to improve people's quality of life.

This study suggests that patients responses to treatments may differ due to their subtype. It also means that we will have new, more targeted treatments. It may also explain why some patients do not respond to treatment.

Further research will focus on

the biology of each Sjögren subtype
the stability and long-term outcome of each subtype
proving the different responses to treatment of each subtype.

Source:

Newcastle University

Journal reference:

Tarn, J. R. et al. (2019) Symptom-based stratification of patients with primary Sjögren's syndrome: multi-dimensional characterisation of international observational cohorts and reanalyses of randomised clinical trials. The Lancet Rheumatology. doi.org/10.1016/S2665-9913(19)30042-6

Researchers are also looking into the use of the salivary stimulant pilocarpine for dry eyes. Other researchers are testing immune modulating drugs to treat the glandular inflammation. A drug called cevimeline has recently been approved for treating dry mouth. Work on developing an artificial salivary gland is in progress.

From Johns Hopkins Jerome L. Greene Sjögren’s Syndrome Center:

Many Sjögren’s patients with devastating neuropathies have delayed diagnoses, because the blood work does not show any evidence of abnormal antibodies.

Our preliminary work shows that nearly 50 percent of Sjögren’s patients with neuropathies lack these antibodies.

Using the expertise of the Rheumatology Core Laboratory, we are seeking to identify novel and previously unidentified antibodies, which will provide insight into relevant mechanisms, and suggest therapeutic opportunities.

Breakthrough study links Type 1 diabetes and Sjogren’s syndrome

Scientists at Sydney's Garvan Institute have identified a new group of immune cells that for the first time directly link two autoimmune diseases, Type 1 diabetes and Sjogren’s syndrome.

The National Institute of Dental and Craniofacial Research is conducting several studies on Sjögren's syndrome designed to help scientists better understand, manage, and treat the disease. Some focus on the disease's natural history, while others test potential new treatments. Talk to your doctor if you'd like more information about these clinical trials.

Sjögren's Syndrome International Collaborative Clinical Alliance (SICCA) is a worldwide registry that aims:

To develop standardized diagnostic criteria for Sjögren's syndrome (SS).

To oversee the collection, processing and storage of clinical data and biospecimens (tears, saliva, blood, minor salivary glands) from individuals with SS.

Central nervous system disease in primary Sjogren's syndrome is from Entrez Pub Med. De Backer and Dehaene present a review of the literature especially . Includes features that may help distinguish multiple sclerosis from primary SS.

NCBI Entrez pub Med 100's of medical research articles about Sjogren's Syndrome such as New Developments in Sjogren's syndrome & Primary Sjogren syndrome with involvement of the nervous system.

Androgens and Dry Eye in Sjögren's Syndrome an article from the New York Academy of Sciences 1999 Annals

'Celiac disease and markers of celiac disease latency in patients with primary Sjögren's syndrome' from The American Journal of Gastroenterology Volume 94 Issue April 1999

Dr Joanne Reed is on a mission – to track down the immune cells that ‘go rogue’ in people with Sjogren’s syndrome and lupus. In autoimmune disorders such as these, immune cells, called B cells, start to produce antibodies against the body’s own tissues, instead of against invaders.

Dr Reed’s work, at The GARVAN INSTITUTE OF MEDICAL RESEARCH In Australia, has been given a significant boost, with the award of a New Investigator Project Grant from the National Health and Medical Research Council. Worth over $900,000 over 4 years, the grant will support Dr Reed’s search for rogue B cells, and the self-reactive antibodies they produce, in patients with lupus and Sjogren’s.

She is seeking to understand how rogue B cells differ from their healthy cousins – and ultimately to develop new therapies that target rogue cells, but leave healthy immune cells untouched.

LATEST RESEARCH

Efficacy and safety of using topical cyclosporine A for treatment of moderate to severe dry eye disease

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Hospitalisation rates among patients with primary Sjögren’s syndrome: a population-based study, 1995–2016 (28 February, 2018)

Gabriel Maciel, Luisa Servioli, Carlotta Nannini, Alvise Berti, Cynthia S Crowson, Sara J Achenbach, Eric L Matteson, DiviCornec Article

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Molecular profiling and clonal tracking of secreted rheumatoid factors in primary Sjögren's syndrome. Arthritis Rheumatol. 2018 Apr 26. doi: 10.1002/art.40539. Article

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Ultrasound elastography in assessment of salivary glands involvement in primary Sjögren's syndrome.

Cindil E, Oktar SO, Akkan K, Sendur HN, Mercan R, Tufan A, Ozturk MA.

Clin Imaging. 2018 Apr 14;50:229-234. doi: 10.1016/j.clinimag.2018.04.011.

PMID:: 29689477

Research progress in renal injury relevant to primary Sjögren's syndrome. Zou Y, Ling G, Tian J, Chen J, Ge Y. Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2018 Mar 28;43(3):320-326. doi: 10.11817/j.issn.1672-7347.2018.03.014. Chinese.
PMID:

29701196
Article

The G-protein-coupled receptor ALX/FPR2 Regulates Adaptive Immune Responses in Mouse Submandibular Glands.

Wang CS, Baker OJ. Am J Pathol. 2018 Apr 20. pii: S0002-9440(17)31166-5. doi: 10.1016/j.ajpath.2018.04.003.

PMID: 29684359

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Increased frequency of CCR7+CD4+ T cells from patients with primary Sjögren's syndrome: An indicator of disease activity rather than of damage severity. Wu C, Yang P, Liu H, Xiao W, Zhao L.

Cytokine. 2018 Apr 20;110:9-17. doi: 10.1016/j.cyto.2018.04.015.

PMID:: 29684636 Article

Anti-IL-7 receptor-α treatment ameliorates newly established Sjögren's-like exocrinopathy in non-obese diabetic mice. Zhou J, Yu Q.

Biochim Biophys Acta. 2018 Apr 19. pii: S0925-4439(18)30142-X. doi: 10.1016/j.bbadis.2018.04.010.

PMID:: 29680668

B cells in the pathogenesis of primary Sjögren syndrome

Primary Sjögren syndrome (pSS) is characterized by B cell infiltrates in the salivary glands and an increased risk of B cell lymphoma. In this Review, the authors describe the myriad ways that B cells are involved in the pathogenesis of pSS.

Gaëtane Nocturne
& Xavier Mariette

Nature Reviews Rheumatology 14, 133–145 08 February 2018

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