Posted on Dec 07, 2020, 6 p.m.
Sjögren’s syndrome (pronounced “show-grins,” sometimes abbreviated SS) is an autoimmune disorder that predominantly affects women, particularly of perimenopausal age. Mayo Clinic explains that, in Sjögren’s syndrome, “mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first — resulting in decreased tears and saliva.” Some researchers believe that dysfunction in producing one or more hormones (such as DHEA, testosterone, or estrogen) may be at the root of the problem.
Symptoms of Sjögren’s Syndrome
Primary symptoms of Sjögren’s are dry eyes and dry mouth. However, other systemic features may also be present. These symptoms include:
- Dry mouth – May have the sensation of cotton or chalkiness
- Dry eyes – May burn, itch, or feel gritty
- Dry throat and lips
- Dryness in the nose
- Changes in sense of taste or smell
- Swollen salivary glands in the neck and face, “particularly the set located behind your jaw and in front of your ears,” states Mayo Clinic.
- Skin dryness, rashes, or sensitivity to UV light
- Persistent dry cough or shortness of breath
- Prolonged fatigue
- Trouble concentrating or remembering things
- Vaginal dryness in women
- Swelling, pain, and stiffness in the joints
As the disease progresses, complications may occur such as a higher tendency for developing dental cavities, oral yeast infections (thrush), or vision problems such as light sensitivity, blurred vision, and even corneal damage. Although less common, other health-related complications may arise:
- Lung issues, potentially leading to pneumonia or bronchitis
- Diminished kidney function
- Hepatitis or cirrhosis in the liver
- Lymph node problems, including lymphoma
- Peripheral neuropathy, in which the nerves cause feelings of numbness, tingling, or burning in the hands or feet
- Irritable bowel syndrome (IBS)
- Interstitial cystitis
Who Is at Risk?
While scientists are still not certain what causes Sjögren’s syndrome, a few factors may predispose a person to develop it. Age, sex, and preexisting autoimmune conditions all influence risk levels. Women are more afflicted than men, and most women are diagnosed between 40 and 50 years of age, leading some researchers to believe there may be a link to menopause.
Additionally, Sjögren’s may occur with other autoimmune disorders such as Hashimoto’s thyroiditis, rheumatoid arthritis, or lupus. Sometimes a particular infection—either viral or bacterial—may trigger the condition. Researchers suggest this may be linked to a genetic predisposition; WebMD explains that, while white blood cells are normally responsible for protecting the body against germs, a specific “faulty” gene may cause them to target healthy cells in the glands that produce tears and saliva instead.
Sjögren’s not only may occur with other autoimmune conditions, but its symptoms also mimic a variety of other diseases—such as chronic fatigue syndrome (CFS) or fibromyalgia—making it more difficult to diagnose. In addition to considering the possible symptoms, your healthcare practitioner may conduct tests to help determine a potential diagnosis before developing a treatment plan. For example, blood testing may be used to measure antibodies or proteins that are present in Sjögren’s patients.
Could Hormone Deficiency Increase Sjögren’s Risk?
One theory as to what causes Sjögren’s syndrome suggests that it may be related to hormone imbalance.
As estrogen levels drop during menopause, the likelihood of Sjögren’s increases. Dr. Konttinen and his colleagues note that estrogens seem to play a role in protecting cells in the secretory glands that produce tears and saliva. As estrogen levels decrease, that protection diminishes, potentially leading to increased cell death in these tissues.
Androgens, such as testosterone and dehydroepiandrosterone (DHEA) also may be important hormones in protecting the body against the risk of Sjögren’s. In a study published in the Scandinavian Journal of Rheumatology, Dr. Pauliina Porola et al. suggest that “the driving factor behind Sjögren’s syndrome could be lack of androgens,” drawing a correlation between the hormonal changes in women at menopause and the higher incidence of Sjögren’s.
This may explain why men are less likely to develop this condition, as they generally have higher testosterone levels than women, even as they grow older. It may be the lack of this “safety net,” that explains why women are more likely to develop Sjögren’s, particularly as the production of other hormones, such as estrogen and DHEA decrease with age.
DHEA is a hormone produced mostly by the adrenal glands. It functions as a precursor to the sex hormones testosterone and estrogen. Patients with Sjögren’s syndrome have been shown to have lower levels of DHEA-S (a more stable, easily measured form of DHEA), than healthy individuals of the same age both in their serum and in their saliva. Additionally, a study by Drs. Porola and Mikael Laine et al. showed that patients with Sjögren’s syndrome while able to convert DHEA into testosterone in the salivary glands are less able to convert testosterone into its more potent form, dihydrotestosterone (DHT).
Conventional treatments for Sjögren’s syndrome typically involve easing the discomfort of dry eyes and mouth with artificial tears and artificial saliva preparations or with prescription medications, such as those prescribed for rheumatoid arthritis. However, these treatments focus on symptom management rather than addressing the underlying cause of the disease. As researchers continue to study the causes, effects, and potential treatments, we look forward to increasing our understanding of how hormone balance plays a role, in hopes of better assisting individuals with this challenging condition.
This article was written by the staff at Women’s International Pharmacy and Edited by Michelle Violi, PharmD – Women’s International Pharmacy.
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This article is not intended to provide medical diagnosis, advice, treatment, or endorsement
- Mayo Clinic Staff. Sjogren’s Syndrome. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/symptoms-causes/syc-20353216. Last accessed September 2020.
- Porola P, et al. Androgen deficiency and defective intracrine processing of dehydroepiandrosterone in salivary glands in Sjögren’s syndrome. J Rheumatol. 2008 Nov;35(11):2229-35. doi: 10.3899/jrheum.080220.Epub 2008 Oct 1.
- Porola P, et al. The influence of sex steroids on Sjögren’s syndrome. Ann N Y Acad Sci. 2007 Jun;1108:426-32. doi: 10.1196/annals.1422.045.
- WebMD Medical Reference. Sjogren’s Syndrome. WebMD. https://www.webmd.com/a-to-z-guides/sjogrens-syndrome#1. Reviewed by Hansa D. Bhargava, MD on June 18, 2020. Last accessed September 2020.
- Konttinen YT, et al. Sjögren’s Syndrome and extragonadal sex hormones formation: A clue to better disease control? J Steroid Biochem Mol Biol. 2015 Jan;145:237-44. doi: 10.1016/j.jsbmb.2014.08.014.Epub 2014 Aug 23.
- Konttinen YT, et al. Sex steroids in Sjögren’s syndrome. J Autoimmun. 2012 Aug;39(1-2):49-56. doi: 10.1016/j.jaut.2012.01.004.Epub 2012 Feb 1. Last accessed September 2020.
- Laine M, Porola P, et al. Low salivary dehydroepiandrosterone and androgen-regulated cysteine-rich secretory protein 3 levels in Sjögren’s syndrome. Arthritis Rheumatol. 2007 Aug:56(8):2575-2584. https://doi.org/10.1002/art.22828. Accessed November 2020.