Edited by Amelia Gross.
What is Sjogren’s Syndrome?
Within the realms of the medical field, Sjogren’s syndrome, pronounced “show-gren’s”, stands as a chronic autoimmune disorder that mainly targets a person’s throat and eyes, more specifically the salivary glands and tear ducts, resulting in a decrease in saliva and tear production. Although Sjogren’s syndrome may be developed at any age, it is most commonly diagnosed in people aged 40 and above. According to Mayo Clinic (2022), the condition is much more common among women who account for 90% of diagnosed cases. It leads to the question: why are women more susceptible to this syndrome?
Here enters VGLL3 (Vestigial-like-family-member 3), a unique transcription factor that accounts for many other autoimmune diseases like Systemic Lupus Erythematosus (SLE) and Scleroderma. Females tend to have a greater number of VGLL3, but is still present in both men and women in the largest organ of the body, as it stands first in line to defend against antigens due to it containing a sensitive indicator of autoimmune dysfunction.
Figure 1. Comparison of VGLL3 in males and females associated with autoimmune diseases. Retrieved from: https://www.nature.com/articles/cmi201728
Yet, despite scientific scrutiny of hormonal and genetic factors contributing to Sjogren’s, the specific causes and genre-biased targeting of Sjogren’s remain uncertain. Some suspect it is due to the evolutionary adaptation of a stronger immune system; this increased autoimmune response in the epidermis has manifested harmful and life-threatening autoimmune disorders, including Sjogren’s syndrome.
What are the common symptoms?
The most common symptoms of Sjogren’s include dry eyes and dry mouth, whereby a person experiences a burning sensation or itchiness in the eyes and finds it difficult to swallow or speak. Sjogren’s also shares similar symptoms to those with SLE, including butterfly rash, discoid rash, photosensitivity, and mucosal ulcers.
Other more common symptoms may include:
Joint pain, swelling, or stiffness
Dental cavities and ulcers
Abnormality of taste
Swollen lymph nodes
Prolonged fatigue
Acid reflux
Muscle weakness
Sjogren’s syndrome and pregnancy
Women who are pregnant and have Sjogren’s are able to have healthy babies. However, it is always a good idea to visit a doctor to ensure safety as the complications may include a rash on the baby for a few weeks, neonatal lupus, and more severely, heart problems. This is due to the high prevalence of anti-SS-a and anti-SS-b antibodies in the bloodstream, found in autoimmune diseases, in which anti-SS-a antibodies are correlated with congenital heart block. The chances of CHB in an offspring are around 1%-2% (Gupta, S. & Gupta, N., 2017).
Sjogren’s has also been associated with a drop in oestrogen levels during menopause, which can result in reduced effectiveness in managing inflammation. On the other hand, during pregnancy, the high levels of oestrogen and progesterone can offer protection against autoimmune disorders. However, the transition into menopause and the post-partum period may increase the risk of developing autoimmune disorders due to the sudden drop in hormone levels.
What happens if Sjogren’s is left untreated?
If left untreated, some serious cases of Sjogren’s may cause complications. For example, one’s eyesight may be permanently damaged due to the development of ulcers on the surface of the eye (cornea) if tear ducts are not properly treated. The immune system may also attack other parts of the body, increasing the risk of developing other closely related autoimmune diseases such as rheumatoid arthritis and systemic erythematosus lupus. Additionally, patients with Sjogren’s are 5 times more likely to develop non-Hodgkin lymphoma, a type of cancer affecting the lymph glands; while the chances are relatively low, at around 5%, the risk should not be ignored. There may also be an increased risk of tooth decay and oral infections due to the altering of protein profiles in saliva, thus changing the composition of it. Sjogren’s may likely attack connecting tissues and organs, leading to a complication known as “extraglandular involvement”. For instance, connecting tissues may be deeply inflamed, compromising strength and flexibility throughout the structures of the body.
As we've seen, early diagnosis and management are crucial to mitigate the potential complications of untreated Sjogren's Syndrome. Now, let's explore the available treatment options and strategies for managing this condition.
How to treat Sjogrens?
Currently, there is no medication that can help slow down Sjogren’s, however, a patient can work with their doctor to target the symptoms, like using artificial tears and a saliva production stimulator for dry eyes and mouth. Patients with Sjogren’s are also likely to experience eye infections (blepharitis) thus are encouraged to use eye drops specific to treating inflammations and warm compresses to relieve discomfort. According to the Sjogren’s Syndrome Foundation, it has been proven that Hydroxychloroquine (Salagen), originally used to treat malaria, is also helpful in long-term treatment of Sjogren’s. Similarly, NSAIDS (Non-steroidal anti-inflammatory drugs) are often first in line to be used in order to reduce high inflammation, which is very common amongst Sjogren’s cases. These types of medication can, more often than not, be found over the counter, such as aspirin and ibuprofen. To prevent specific symptoms such as tooth decay, patients are advised to brush and floss their teeth regularly, specifically using toothpaste that is designed for people with dried mouth, for example, containing fluoride to prevent cavities.
References:
Sjogren’s Syndrome Foundation (n.d) Understanding Sjogren’s - Treatment. Retrieved from https://sjogrens.org/understanding-sjogrens/treatment
Mayo Clinic Staff. (2022, August 8). Sjogren’s syndrome - Diagnosis and treatment. Retrieved from https://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/symptoms-causes/syc-20353216
Gupta, S., & Gupta, N. (2017). Sjogren’s Syndrome and Pregnancy: A Literature Review. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5267941/
Liang, Y., Tsoi, L. C., Xing, X., Beamer, M. A., Swindell, W. R., Sarkar, M. K., Berthier, C. C., Stuart, P. E., Harms, P. W., Nair, R. P., Elder, J. T., Voorhees, J. J., Kahlenberg, J. M., & Gudjonsson, J. E. (2017). A gene network regulated by the transcription factor VGLL3 as a promoter of sex-biased autoimmune diseases. Nature Immunology, 18(2), 152–160. https://doi.org/10.1038/ni.3643
Liang, Y., Kahlenberg, J. & Gudjonsson, J. A vestigial pathway for sex differences in immune regulation. Cell Mol Immunol 14, 578–580 (2017). https://doi.org/10.1038/cmi.2017.28
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