ITT:Breckinridge | Sjögren’s Syndrome | No tears for the unknown. |

Nathan Kalim
1/11/2016
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Sjögren’s Syndrome (SS) initially described and named by Swedish ophthalmologist Henrik Sjögren in 1933. Henrik discovered a connection among those that suffer from Sicca Syndrome; Arthritis, Keratoconjunctivitis Sicca or commonly called Dry Eye, Xerostomia or Dry Mouth. If gone untreated for extended time may experience debilitation symptoms, estimated 0.01%-0.1% of the population has this ailment. With its overlapping symptoms SS can be difficult to diagnose.
Etiology (Origin of the Disease) The exact origin of SS remains currently unknown, but believed to be caused due to multiple factors within the body and genetic history. Specific sources of SS factors are possible genetic, environmental, hormonal and viral influences, research published in 2010 by H.J. Chang. Those diagnosed with SS show nearly a 12% relation with a family member that also has SS diagnoses. Statistics show that SS typically shows signs in the years 40-60, and is 10 times more prominent in women verses men. SS can be discovered in all ages but very rarely seen in young children or teenagers.
Due to SS vague and seemingly petty symptoms, SS commonly goes several years without a proper diagnosis. Symptoms are often ignored or perceived as other ailments or even drug medication side effects, resulting in its commonly prolonged treatment. SS diagnosis is increasingly difficult do to its relation as an autoimmune disorder and often is found as a side diagnosis with other autoimmune diseases. In blood tests those with SS exhibit atypical proteins that are an indication of autoimmune etiology.
Pathology (how it affects the body) Primary ailments of SS are difficulty swallowing, speech, smell, sight and maintaining connective tissue lubrication. This dryness is caused by Lymphocyte infiltration within various glands that produce connective lubrication such as salivary glands and tear ducts. This infiltration is caused by T and B Lymphocytes, causing the release of pro-inflammatory cytokines, causing tissue dysfunction and decrease in lubrication production, gradually stopping entirely. If gone untreated systemic issues can arise from head to toe. According to Baldini, Talarico, Tzioufas, and Bombardieri (2012), approximately half the cases of SS occur alone, or called Primary Sjögren’s. SS that presents with another autoimmune disorder it is called Secondary Sjögren’s. Secondary Sjögren’s is commonly seen paired with Rheumatoid Arthritis (RA) or Systemic Lupus Erythematosus (SLE). Autoimmune disorders can exacerbate or mask symptoms for speedy and accurate diagnosis and treatment.
Signs & Symptoms Primary Sjörgren’s exhibits three primary symptoms; dry eye, dry mouth, and complications with the parotid glands. The Parotid Glands are a pair of large salivary glands located along jaw in front of each ear, in SS, parotid glands become inflamed and tender. The dry mouth symptom causes various problems with swallowing, tasting food and drink, as well as affecting sense of smell. The production of saliva helps protect dental area from drying out and rotting, resulting in dental cavities, tooth breakage and tooth lose. Secondary Sjögren’s causes exponential difficulties in both in identification and treatment. In conjunction with other autoimmune disorders there are a multitude of systemic system failures and malfunctions. The most common is chronic inflammatory responses, but can escalate to include arthralgia, arthritis, fibromayalgia, neuropathy, Raynaud’s Phenomenon (Sensation in of cool or numbness), vasculitis, pulmonary disease, biliary cirrhosis, anemias, renal disease, alopecia, and lymphoma.
Quality of Life Untreated this ailment can cause great discomfort and pain, lose of functional systems and even body parts. This strain even results into psychological symptoms of despair and depression. SS in primary or secondary diagnosis can result in further infections and complications, resulting in further changes of infections.
Available Treatments Without a direct cause of the disease, SS is difficult to treat directly, but indirectly treatment is aimed at symptom management. Patient and Physicians commonly need to coordinate with multiple specialists to cover all the symptoms and potencial complications that may arise. Not all treatment is pharmacological, some are holistic or even avoiding situations. Common practice for dry eyes and mouth is to avoid dry, smoky and windy areas that may cause further moisture lose. Patients are encouraged to used humidifiers in their homes, eye shields and punctal plugs or tear duct plug to help retain any moisture. Recently the autoimmune portion of SS has been treated with the administration of monoclonal antibodies, essentially a clone of a specific parent antibody cell; e.g. Rituximab or Rituxan. Holistic options include adding Omega 3, White Willow Bark, Turmeric, Maritime Pine Bark, Green Tie and Resveratrol into a dietary regiment. For dry mouth, throat lozenges, sugar free candy, and gum chewing is encouraged to help stimulate salivation. Fluroide gel may be used to help assist with salivation, as well as a safe guard to teeth decay, Fluoride helps protect the teeth from effects of acid and bacteria. Patients are discouraged from consuming diuretics such as caffeine and alcohol, which will cause the patient to lose crucial moisture. Avoiding nicotine habits will help maintain good oral hygiene and hydration. Those with SS that suffer from fatigue, low impact exercise, good nutritional diet and appropriate rest will help maintain energy levels and manage symptoms. Those with joint and muscular pain are often encouraged to take Acetaminophen (Tylenol) or Nonsteroidal Anti-Inflammatory Drugs (NSAIDs).
Progression and Prognosis Those Diagnosed with SS have a very debilitating disease that can run rampant and ruin one’s life. In 2011, professional tennis player Venus Williams was forced to with draw from the Women’s Open tournament. Her symptoms become so severe fatigue, joint pain, swelling and stiffness; Venus was diagnosed at age 31. Venus as since continued her professional tennis carrier. As long as the symptoms of SS are properly managed and caught early enough, a patient can continue to have a normal and comfortable modified life. This would require a patient to maintain treatment plan; diet, exercise, environmental factors, as well as keeping assigned treatment team informed of any new symptoms. Deviation of treatment plan may cause symptoms to return, worsen or even develop new symptoms. Growing awareness of SS has caused great improvements of assessment and diagnosis, and often early detection.

(American Psychological Assoc.)
References
Tanner, K., Pierce, J. L., Merrill, R. M., Miller, K. L., Kendall, K. A., & Roy, N. (2015). The Quality of Life Burden Associated With Voice Disorders in Sjögren’s Syndrome. Annals Of Otology, Rhinology & Laryngology, 124(9), 721-727 7p. doi:10.1177/0003489415579911
(American Psychological Assoc.)
References
Catanzaro, J., & Dinkel, S. (2014). Sjögren’s Syndrome: The Hidden Disease. MEDSURG Nursing, 23(4), 219-223 5p.
(American Psychological Assoc.)
References
Brown, T. P. (2013). Sjögren's syndrome. Magill’S Medical Guide (Online Edition),