Sjogren’s (“SHOW-grins”)

Sjogrens

Sjogrens is a chronic autoimmune disease in which people’s white blood cells attack their moisture-producing glands. Today, as many as four million Americans are living with this disease.

Although the hallmark symptoms are dry eyes and dry mouth, Sjögren’s may also cause dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients may also experience extreme fatigue and joint pain and have a higher risk of developing lymphoma.

With upwards of 4,000,000 Americans suffering from Sjögren’s, it is one of the most prevalent autoimmune disorders. Nine out of 10 patients are women.

About half of the time Sjögren’s occurs alone, and the other half it occurs in the presence of another autoimmune connective tissue disease such as rheumatoid arthritis, lupus, or scleroderma. When Sjögren’s occurs alone, it is referred to as “Primary Sjögren’s.” When it occurs with another connective tissue disease, it is referred to as “Secondary Sjögren’s.”

All instances of Sjögren’s are systemic, affecting the entire body. Symptoms may remain steady, worsen, or, uncommonly, go into remission. While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning. Early diagnosis and proper treatment are important — they may prevent serious complications and greatly improve a patient’s quality of life.

Since symptoms of Sjögren’s mimic other conditions and diseases, Sjögren’s can often be overlooked or misdiagnosed. On average, it takes nearly seven years to receive a diagnosis of Sjögren’s. Patients need to remember to be pro-active in talking with their physicians and dentists about their symptoms and potential treatment options.

Symptoms

  • generalized dryness, typically including xerostomia (dry mouth)
  • keratoconjunctivitis sicca (dry eyes)
  • skin, nose, and vaginal dryness

The hallmark symptom of Sjögren’s syndrome is a generalized dryness, typically including xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes), part of what are known as sicca symptoms. Sicca syndrome also incorperates vaginal dryness, chronic bronchitis and lacks signs of arthritis. Sjögren’s syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, peripheral nervous system (distal axonal sensorimotor neuropathy) and brain.

Sjögren’s syndrome is associated with increased levels in cerebrospinal fluid (CSF) of IL-1RA, an interleukin 1 antagonist. This suggests that the disease begins with increased activity in the interleukin 1 system, followed by an auto-regulatory up-regulation of IL-1RA to reduce the successful binding of interleukin 1 to its receptors. It is likely that interleukin 1 is the marker for fatigue, however, increased IL-1RA is observed in the CSF and is associated with increased fatigue through cytokine induced sickness behavior. On the other hand, Sjögren’s syndrome is characterized by decreased levels of IL-1ra in saliva, which could be responsible for mouth inflammation and dryness. Patients with secondary Sjögren’s syndrome also often exhibit signs and symptoms of their primary rheumatic disorders, such as SLE, Rheumatoid Arthritis or Systemic Sclerosis.

 

Resources

Resources

  • Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA (February 2006). “Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982-2003”. Rheumatology (Oxford) 45 (2): 187–91. doi:10.1093/rheumatology/kei107. PMID 16332955.
  • García-Carrasco M, Ramos-Casals M, Rosas J, et al. (July 2002). “Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients”. Medicine (Baltimore) 81 (4): 270–80. PMID 12169882.
  • Harboe E, Tjensvoll AB, Vefring HK, Gøransson LG, Kvaløy JT, Omdal R. (2009). Fatigue in primary Sjögren’s syndrome—a link to sickness behaviour in animals? Brain Behav Immun.23(8):1104–8. doi:10.1016/j.bbi.2009.06.151 PMID 19560535
  • Perrier S, Coussedière C, Dubost JJ, Albuisson E, Sauvezie B. (1998) IL-1 receptor antagonist (IL-1RA) gene polymorphism in Sjögren’s syndrome and rheumatoid arthritis. Clin Immunol Immunopathol. 87(3):309-13> http://www.ncbi.nlm.nih.gov/pubmed/9646842
  • Priori R, Medda E, Conti F, et al. (2007). “Risk factors for Sjögren’s syndrome: a case-control study”. Clin. Exp. Rheumatol. 25 (3): 378–84. PMID 17631733.

Treatments

There is neither a known cure for Sjögren’s syndrome nor a specific treatment to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes. Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface for a longer time. Additionally, cyclosporine (Restasis) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline (Evoxac) and pilocarpine. Nonsteroidal anti-inflammatory drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed, and sometimes IVIG (intravenous immunoglobulin). Also, disease-modifying antirheumatic drugs (DMARDs) such as methotrexate may be helpful. Hydroxychloroquine (Plaquenil) is another option and is generally considered safer than methotrexate.[15] Salagen, a man-made form of pilocarpine, can be used to help produce tears as well as saliva in the mouth and intestines. It is derived from the Jaborandi plant.

In the advanced stage, despite the use of tear replacement drops, the eyes always feel burning, scratchy, sore. The sufferer is always aware of some discomfort, the eyes feel worse in the morning and late evening. The use of tear replacement drops becomes tedious and ineffective. At this point it may be appropriate to consider punctal plugs.

Each eye has two sites at the inner corner of each eyelid where tears drain from the eye. The upper eyelid ‘puncta’ drains approximately 40% of the tears away and the lower puncta drains away the remaining 60% of the tears. If there is a problem with the quantity of tears, as there is in Sjögren’s disease, plugging the lower puncta can result in the tears that the patient has remaining on the eye longer.

Punctal plugs can be inserted into the lower or upper tear drainage canals of the eyes. The procedure takes only a few minutes and is painless. It can be done in the optometrist or ophthalmologist’s office. Generally, collagen plugs are inserted first. These plugs will dissolve within a few days, so it gives the patient a chance to see if there is any improvement in comfort. Generally, the improvement is immediate. The patient may proceed to use of permanent plugs, although these too can be removed if necessary.

Preventive dental treatment is also necessary (and often overlooked by the patient), as the lack of saliva associated with xerostomia creates an ideal environment for the proliferation of bacteria that cause dental caries (cavities). Treatments include at-home topical fluoride application to strengthen tooth enamel and frequent teeth cleanings by a dental hygienist. Existing cavities must also be treated, as cavities that extend into the tooth can not be effectively treated through teeth cleaning alone, and are at a high risk of spreading into the pulp of the tooth, leading to the loss of vitality and need for extraction or root canal therapy. This treatment regimen is the same as that used for all xerostomia patients, such as those undergoing head and neck radiation therapy which often damages the salivary glands, as they are more susceptible to radiation than other body tissues.

Unfortunately, many patients, not realizing the need for dental treatment, do not see a dentist until most of their teeth are beyond the point of restoration. It is not uncommon for a dentist to see a xerostomia patient with severe, untreatable cavities in almost every tooth. In severe cases, the only viable treatment may be to extract all of the patient’s teeth and treat with prosthetics such as dentures or implants.

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