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Sjogren Syndrome

Sjogren Syndrome

What is Sjogren Syndrome

Sjogren syndrome is a long-lasting inflammatory autoimmune disorder that causes few moisture productions from the exocrine gland, primarily the eyes and the mouth. It is a rheumatic disease because patients may have inflammations, pain, swelling, and stiffness in the joints, ligaments, bones, and other muscles. Sjogren syndrome is an autoimmune disease because our immunity loses its function and attacks healthy cells instead of protecting invading bacteria or viruses. White blood cells typically protect us from germs, attacks on the glands in charge of making moisture, and insufficient moisture production when this attack occurs. This inflammatory disease can also affect many different parts of the body and mostly affect the tear and saliva glands. Most patients report that they noticed irritations, painful burning sensations in the eyes, dry mouth, swelling of the glands around the face and neck. Some patients have also complained of dryness in the nasal tract, vagina, and skin. Swallowing difficulty and acid reflux have also been common presentations by patients. As reported by the Cleveland Clinic, more than one million people diagnosed with Sjogren syndrome, about 90% of the affected people are women. It is most frequently common among middle-aged women. Sjogren syndrome can be classified into either primary form when there is no other associated illness or disease. A secondary type observed among patients with autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissues, Hashimoto thyroiditis, primary biliary cirrhosis, or chronic autoimmune hepatitis.

Causes of Sjogren Syndrome

Usually, the immune system produces a protein called antibodies that protects our body from any foreign attack like inflammatory destructions, our cells such as those seen in germs, bacteria, viral, and bacterial infections. When the immune system is overwhelmed, there is a trigger of the inflammatory response, which causes the white blood cells to attack and damage its moisture-producing glands. The leading cause of Sjogren syndrome is still not well established, but the following factors below can contribute to the syndrome

  • Abnormal immune response
  • Inheritance: some people may have a genetic or inherited factor that may make them more likely to have Sjogren syndrome than others.
  • Environment: Smoking or exposure to a low humidity environment avoided as they exacerbate Sjogren syndrome factors.
  • Sex hormones
  • Medications: Tricyclic antidepressants and antihistamines can cause Sjogren syndrome symptoms, without manifestations of inflammation or damage. Exposure to radiation treatment to head and neck can cause severe dry eyes and mouth.

Clinical Symptoms presented

Clinical presentations can vary among patients. The symptoms can be grouped into two subtypes namely,

  • Glandular symptoms:
    • Inflammation of the salivary glands: decreased production of saliva, also known as xerostomia (dry mouth), salivary gland pains.
    • Dysphagia: Some patient presents with difficulty to swallowing, also reported are sensitivity to spicy food, altered taste sensations, and speech difficulties.
      Dental caries, dental erosions, salivary gland swelling lead to oral infections like candida ulcers.
    • Parotitis: inflammation seen in one or both parotid glands, a significant type of salivary gland located either side of the face. If abnormal and persistent enlargement of one gland may indicate a tumor, a proper evaluation will be needed to rule out any malignancy like cancer.
    • Inflammation of the lacrimal glands will lead to decreased secretions of tears
    • Redness, itching, burning of eyes, in advanced cases the cornea may be severely damaged, vision impairment.
    • Sensation of foreign body in the eyes
    • Sicca syndrome: This is a combination of the dry mouth and dry eyes.
    • Dryness: Vaginal dryness and lack of moisture at the respiratory tract can cause cough.
  • Other non-glandular symptoms:
    • General symptoms: arthralgias, excessive fatigue
    • Skin manifestations: Many people with Sjogren syndrome can see different skin changes, seen in the form of body areas that are harder or tighter. Since the skin becomes tight, the ability to move might be restricted. Furthermore, the finger may become highly sensitive to cold and change color with severe or emotional stress (symptoms of Raynaud’s phenomenon). The symptoms observed because the blood vessels are narrow due to spasm.

Diagnosis of Sjogren syndrome

Diagnosing Sjogren syndrome can be complicated because it’s an autoimmune disease that can mimic other forms of autoimmune diseases. To avoid the challenges, providers have mapped out a few measures to treat.

  • Blood test: Monitoring of levels of different types of blood cells, checking for the presence antibodies common in Sjogren syndrome, which includes Anti-Ro/SSA antibody and anti-La/SSB antibody; target ribonucleoprotein antigens (Ro/La) of epithelial cells, especially of the salivary glands. Also, to be monitored are the levels of Antinuclear antibodies and Rheumatoid factor.
  • Eye Examination: Most cases providers measure the eyes’ dryness with a test known as the Schirmer tear test. This test conducted using a small piece of paper placed under the lower eyelid to measure the degree of tear production. Another examination uses the magnifying device called the Slit Lamp, which helps the ophthalmologist take a closer look at the different structures in the front and inside of the eye. The exam helps determine the health of the eyes and detect eye disease like in the case of cornea damage.
  • Imaging: This procedure can help check the saliva gland’s functions using a sialogram, an x-ray that detects how much saliva flows into your mouth. Another test is the salivary scintigraphy, which involves an injection into the vein of a radioactive isotope, then tracked over an hour to see how quickly it will arrive in all salivary glands.
  • Biopsy: This is a lip biopsy done to detect inflammatory cells, usually taken from the labial salivary glands. Positive findings may shoe destructions of minor salivary glands like fibrosis, parenchymal atrophy.

Management and treatment

Sjogren syndrome is a chronic disease that can affect both physical and mental health. Treatment of Sjogren syndrome depends on the part of the body affected. The key to the management and care is for providers to find what works best to reduce the severity of a particular symptom and manage or prevent further complications, whatever treatment initiated, potential risk, side effects, and benefits discussed between the patient and provider.

Some patients can manage dry mouth and dry eyes using over the counter eye drop, while in some case-patients’ needs prescriptions. The following are some of the treatments of Sjogren syndrome.

  • In some practices, patients who are experiencing dry eyes can apply artificial eye drop that can keep the eyes from drying out. You will need to use the drops throughout the day, in contrast to gels that you can put on your eyes at night. The gel’s advantage is you stick it on the eyes and don’t have to apply drops throughout the day. According to WebMD, if the artificial tears are not improving symptoms, providers can prescribe the following medications, Cequa, Lacrisert, and Restasis. The drugs to treat dry eyes are Pilocarpine (Salagen), Cevimeline (Evoxac), and Supersaturated calcium phosphate rinse (NeutraSal). It is also essential to know that wearing of contact lenses is not recommended if you have a dry eye. Make sure you consult your provider.
  • Decrease eye inflammations: The eye doctor may recommend topical immunosuppressive drugs like cyclosporin, which works by increasing the quantity of tears made.
  • Arthralgias and arthritis: Acetaminophen or non-steroidal anti-inflammatory drugs taken for the cases of arthralgia. This medication will also help to control the pain.
  • Skin and Vaginal dryness: In dryness, patients should use skin creams like Eucerin or Lubriderm, a skin lotion to help with dryness. Vaginal lubricants like Replens can also be applied; vaginal estrogen creams used among postmenopausal women.
  • Maintaining muscle strength and through physical therapy and mild exercises.
  • Improve emotional stress through counseling and other measures available.
  • If all less invasive tests and treatments cannot resolve symptoms, surgery will become an option. For example, if patients developed finger ulcers, which may lead to gangrene, the better option will be amputation.

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