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Sjogren's Syndrome (Sjogren's Disease)

A fatigued woman with eye drops.

Sjogren's is a heterogenous autoimmune disease that can affect a variety of different organ systems. Since its presentation can vary widely from patient to patient, Sjogren’s can be difficult to diagnosis. As a result, it commonly remains either undiagnosed or is diagnosed only years after the onset of symptoms. There is currently no cure for Sjogren’s, but early recognition is crucial to prevent diagnostic delay that may result in damage and to evaluate an individual patient's symptoms holistically to determine optimal therapies for them.

What is Sjogren’s syndrome?

Sjogren’s syndrome (Sjogren’s disease) is a chronic autoimmune condition. It is marked by inflammation in several glands in the body. Glands producing saliva and tears are most often affected. Although best known as “Sjogren’s syndrome,” there is a movement among many doctors and researchers, including the Sjogren’s foundation, to remove “syndrome” from the name and instead call it “Sjogren’s disease.” Sjogren’s, indeed, is a serious and distinct autoimmune disease rather than merely a collection of symptoms as the word “syndrome” may imply.

Like many other autoimmune diseases, most people with Sjogren’s are women. Sjogren’s can affect people of all ages and may accompany other autoimmune diseases like lupus or rheumatoid arthritis. There are two forms of Sjogren's: primary and secondary.

  • Primary Sjogren's occurs when your experience dry eyes and a dry mouth, known as sicca symptoms. You may also experience fatigue and joint pain.
  • Secondary Sjogren's occurs when you experience sicca symptoms and have another associated autoimmune disease. In some people, Sjogren's syndrome may precede lupus by many years. More often, however, it appears late in the course of lupus. Sjogren's syndrome occurs in approximately 20% to 30% of people with lupus, while only in about 1% to 3% of the general population. Secondary Sjogren's may also be associated with rheumatoid arthritis or scleroderma.

What causes Sjogren’s?

Like most autoimmune diseases, the precise cause of Sjogren’s is unknown but is likely the result of interactions between genes and the environment. Some people may have a genetic predisposition to autoimmune disease but other factors such as hormonal effects or environmental contributors, viruses or stress are likely necessary to trigger disease. Some data suggests there is a central role of the type I interferon (IFN) system in the development of many autoimmune disorders, including Sjogren's.

What are the signs and symptoms of Sjogren’s?

The symptoms of Sjogren’s can vary greatly from patient to patient. Its hallmarks are dry eyes and dry mouth (also called sicca symptoms). Patients also commonly develop fatigue and joint pain. Sjogren’s can affect most organs in the body, including the lungs, kidneys, nervous system, blood vessels and gastrointestinal tract, though these manifestations are rare. It is important to remember that many things, including medications, aging and environmental factors can also cause dry eyes and dry mouth, so evaluation by the appropriate medical specialists is key.

How is Sjogren’s syndrome diagnosed?

There is no single test to confirm a diagnosis of Sjogren’s. Because symptoms can be nonspecific and involve several organ systems, it is often misdiagnosed or goes undiagnosed for several years. A diagnosis requires:

  • symptoms, such as sicca (dryness of the eyes and/or mouth)
  • a positive autoantibody test or gland biopsy
  • objective evidence of gland dysfunction

An eye doctor can perform several tests to show lacrimal (tear) gland dysfunction in people with dry eyes. These include a direct measure of tear production (called a Schirmer’s test) or use of special eye drops to identify dry spots on the surface of the eye.

Tests can be performed on the salivary gland to see saliva flow through the gland (sialography) or to find problems with the gland’s structure. In some cases, a biopsy of a salivary gland, usually from the lip, is needed to make the Sjogren’s diagnosis. A positive biopsy will show evidence of inflammation in the gland.

What is the treatment for Sjogren’s?

Treatment is generally aimed at controlling symptoms and targeting specific organ involvement. Rheumatologists, ophthalmologists, dentists and other specialists such as neurologists or pulmonologists often work together to manage Sjogren’s and monitor response to therapy. Various eye drops and oral lubricators can address sicca symptoms. Routine oral hygiene is important.

When there is evidence of organ involvement or more severe disease, therapy to suppress the immune system in an attempt to reduce inflammation and quiet disease is often necessary. This could include corticosteroids or other medications aimed at targeting inflammation.

Many prescription and over the counter medications can also exacerbate sicca symptoms.

All people with Sjogren’s should have their medication list reviewed by their doctor to ensure they are not on medications that can make dryness worse. This includes discussion of all over-the-counter medication, including antihistamines or sleep aids.

Over-the-counter eye drops can help with mild cases of dry eye. (Drops should be preservative-free artificial tears.) Longer-lasting lubricating ointments or gels can be helpful overnight. There are also prescription-strength eye drops that an ophthalmologist might prescribe if the over-the-counter drops are not enough. Eye doctors sometimes insert punctual plugs, which block the normal drainage of tears and prolong the time that tears remain on the surface of the eye. Dry, windy or polluted environments can worsen dry eye. Exposure to these environments should be limited when possible. Wearing sunglasses to protect the eyes, especially on windy days, can be helpful as well.

The management of dry mouth includes staying well hydrated, using lubricating agents such as over-the-counter alcohol-free mouthwashes or artificial saliva and avoiding medications that can make dry mouth worse. Chewing sugar-free gum or sucking on sugar-free candies can help stimulate saliva flow. (Look for products that contain Xylitol.)

There are two prescription medications (cevimeline and pilocarpine) that can help some people with dry mouth. Sleeping with a humidifier may also be helpful to ease the sicca symptoms. People with Sjogren’s should not smoke cigarettes as this can make dry eye and dry mouth worse. Exposure to secondhand smoke should also be avoided.

While at present, there are no therapies approved specifically for severe Sjogren’s, there are several therapies currently under investigation.

In recent years, medications targeting B cells, a specific type of white blood cell, have been studied in Sjogren’s. These medications may be helpful in controlling certain severe types of the disease. Ongoing research is aimed at finding new therapies to help treat all forms of this complex disease.

Sjogren's Syndrome (Sjogren's Disease) Patient Stories

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References

  • Baer AN, Hammitt KM. Sjögren's Disease, Not Syndrome. Arthritis Rheumatol. 2021 Jul;73(7):1347-1348. doi: 10.1002/art.41676. Epub 2021 Jun 1. PMID: 33559389.
  • Mavragani CP, Sagalovskiy I, Guo Q, Nezos A, Kapsogeorgou EK, Lu P, Liang Zhou J, Kirou KA, Seshan SV, Moutsopoulos HM, Crow MK. Expression of Long Interspersed Nuclear Element 1 Retroelements and Induction of Type I Interferon in Patients With Systemic Autoimmune Disease. Arthritis Rheumatol. 2016 Nov;68(11):2686-2696. doi: 10.1002/art.39795. PMID: 27338297; PMCID: PMC5083133.
  • Rodríguez-Carrio J, Burska A, Conaghan PG, Dik WA, Biesen R, Eloranta ML, Cavalli G, Visser M, Boumpas DT, Bertsias G, Wahren-Herlenius M, Rehwinkel J, Frémond ML, Crow MK, Ronnblom L, Vital E, Versnel M. Association between type I interferon pathway activation and clinical outcomes in rheumatic and musculoskeletal diseases: a systematic literature review informing EULAR points to consider. RMD Open. 2023 Mar;9(1):e002864. doi: 10.1136/rmdopen-2022-002864. PMID: 36882218; PMCID: PMC10008483.

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Reviewed and updated by Lindsay S. Lally, MD

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