Sjogren Syndrome(SS) is an autoimmune disease that causes lymphocytic infiltration of exocrine glands, primarily lachrymal and salivary glands. Simply put, it is a chronic immune disorder in which white blood cells of the patient attack saliva and tear glands. At times, glands that keep the vagina moist are also attacked resulting in vaginal dryness.
In normal cases, humans' immune system generates antibodies to attack harmful material as virus and bacteria. Putting the cart before the horse, the immune system in SS patients starts producing autoantibodies and autoreactive cells that start attacking those very body parts that they are supposed to protect. SS causes cause persistent mouth infections, debilitating fatigue, difficulty in eating and swallowing, joint pains, blurred vision, and swollen parotid glands. It, in severe cases, may also damage vital organs of the body Around 90 percent of SS patients are females.
Most of the SS patients also have sicca symptoms as dry eyes, dry mouth, dry skin and parotid gland enlargement. Pathophysiology of SS is complex, and its etiology is still not fully discovered. However, scholarly studies identified that a combination of viral infections and/or stress activates the epithelial cell and leads to upregulation of toll-like receptors(TLR) in patients with the predisposed genetic background. Modifications in extracellular matrix and interactions among cells trigger alterations in glandular architecture that initiate the disease. Upregulation of TLR causes T-cell activation and secretes inflammatory cytokines. In advanced stages that follow activation of BAFFA and proliferation of B-cells trigger gland destruction; germinal centers form that may ensue lymphoma.
SS, mostly, affects women after 40 years of age. However, it may cause debilitating issues and own set of problems if occurs a woman during pregnancy. Research studies have revealed that spontaneous abortions and miscarriages were frequent in women with secondary SS that coexisted with positive serological tests for syphilis, anti-erythrocyte antibodies, and thrombocytopenia. However, a particular research concluded that SS alone does not affect the outcome of pregnancy. Exceptions include women with serological and hematological abnormalities linked to lupus anticoagulants. However, another research, comparing pregnancy outcomes in primary SS women with the general population, revealed that pSS women experience pregnancy later in their lives. Additionally, they are likely to deliver a baby with lower birth weight and other minor abnormalities. Other aspects including miscarriages and Apgar score did not differ between pSS women and normal females.
Research studies, though, have identified that SS, in extremities, can cause congenital heart defects in the newborn. It may result in the death of the infant or fetus. Around 2 percent of Ro-positive mothers get affected by this complication. The underlying mechanism behind congenital heart block and sjogren syndrome is due to the presence of La and Ro antigens apoptotic blebs. Press et al. revealed surveyed 64 children with CHB and identified that 60 percent mothers were Ro or La positive.
Diagnosis of SS is strongly suggested in patients who exhibit one or more SS symptoms. There are high chances of its occurrence if the patient tests positive for antibodies to anti-SS-A or anti-SS-B antigen or who have a positive biopsy for salivary gland. It is to be noted, though, that positive for antibodies does not directly indicate SS; results may come positive in people with other diseases and even in healthy persons. SS has a wide spectrum of clinical manifestations that make the diagnosis difficult and delayed. However, there are some tests including Schirmer test and sailography that help clinicians judge the presence of SS in a person.
Still, SS does not have proven cure. Different treatment strategies focus on prevention and relieving symptoms. For example, dry eyes can be successfully treated with artificial tears eye drops. Likewise, there are various glasses available to protect eyes with tear evaporation. Increasing the fluid intake, maintaining good oral hygiene, sucking ice cubes can help lubricating the mouth by preventing dryness. There are a number of saliva substitutes are available that may be used after consulting with a pharmacist. In addition to these natural cure techniques, some medication involving Pilocarpine, Hydroxychloroquine are prescribed to treat the disorder. Vaginal dryness is also treated using hormonal replacement therapy. Plasmatherapies and dexamethason are safe modalities in high-risk pregnancies for the birth of a child with congenital heart block.
The occurrence of Sjogren during pregnancy may raise mild to severe complications though the severity is quite rare. Otherwise, pregnancy complexities include delivery of babies with low birth and the like.
Most of the treatment therapies emphasize relief and prevention. Nevertheless, some medication and hormonal therapies have been helpful in treating the disorder. The existing research points out to the necessity of further research so as to unearth hidden aspects regarding the disorder.
References
Feist, E., Dorner, T., Waqenmann, A., Rhode, W., Burmester, G., & Hiepe, F. (2006). Sucessful Therapeutic Management of Risk Pregnancy in Primary Sjogren Syndrome with Plasmapheresis and Dexamethasone. Z Rheumatology , 127-32.
Hussein, S. Z., Jacobsson, L. T., & Lindquist, P. G. (2010). Pregnancy and Fetel Outcome in Women with Primary Sjogren's Syndrome with Women in the General Population: A Nested Case- control Study . Rheumatology .
Moutasim, K., Shirlaw, P., Escudier, M. P., & Timothy, P. (2009). Congenital Heart Block Associated with Sjogren Syndrome: Case Report. International Archives of Medicine .
Nikolov, N. P., & illei, G. G. (2009). Pathogenesis of Sjogren Syndrome. Curr Opin Rheumatology , 465-470.
Soyfoo, M. S., & Cogan, E. (2012). Diagnostic and Prognostic Features of Sjogren Syndrome. Insights and Pespectives in Rheumatology .
Takaya, M., Ichikawa, Y., & Shimizu, H. (1991). Sjogren Syndrome and Pregnancy. Tokai J Exp Clin Med , 83-88.
