Sjogren’s syndrome

Sjogren’s syndrome is an auto immune disorder and mainly affects the moisture-producing cells and glands of body. The disease is characterized by dry mouth, eyes, and skin and vaginal dryness. Numbness of muscles, gastric ulcer and joint pain and lymphoma are other symptoms. This disease affects the mucous membrane and moisture-secreting of eyes and mouth which results in decreased amount of tears and saliva. Usually the age of onset is above 40.

What causes Sjogren’s syndome?

This disorders results from a combination of both genetic and environmental factors. We can say this is a multifactorial condition. Mostly, some kind of ‘trigger’ results in onset of disease. Genetic variations and inheritance increase the risk factors. While, sometimes bacterial infections may trigger the disease symptoms. The chances of association of nervous and endocrine system with onset of this autoimmune disorder are an area of research for now.

Signs and symptoms

The major symptoms of Sjogren’s syndrome are:

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  • Dryness of mouth: It feels as if mouth is full of cotton and the person feel difficulty in speaking and swallowing.
  • Dryness of eyes: The eyes feel itchy and gritty.

Other associated symptoms are:

  • Dry skin and skin rashes
  • Joint pain, swelling and stiffness
  • Swelling in salivary glands
  • Vaginal dryness
  • Dry coughing

Complications

This immune system disorder mainly affects eyes and mouth. However, the symptoms may get severe and these complications occur:

  • Vision problem: Dry eyes become gritty and it can lead to increased light sensitivity, cornea damage and blurry vision.
  • Dental cavities: The problem with salivary glands may lead to denatal cavities. This is because the saliva and its enzyme protect the teeth from bacteria under normal conditions.
  • Fungal infections: Dryness of skin and mouth increase the chances of fungal infection such as yeast infection.
  • Lymphoma: This is rare but some people may have swollen lymph nodes- lymphoma.

Moreover, dryness of mucous membranes and glands may affects the other vital parts of body such as kidney, brain and its nerves and heart. However, these are rare cases.

Genetic basis of Sjogren’s syndrome

This autoimmune disorder is associated with genetic polymorphism of human leukocyte antigen DR (HLA-DR). High levels of interleukin 1 antagonist, IL-1RA in cerebrospinal fluid are observed in this disorder. The enhanced activity of interleukin is followed by auto-regulatory up-regulation of IL-1RA. It reduces the normal binding of interleukins with receptors of brain cells. Low levels of IL-1 in saliva results in dryness of mouth and other symptoms such as systemic sclerosis. In some cases, there is production of auto-reactive antibodies that may lead to self reactive immune system mediated loss of glands and body cells. Loss of lacrimal fluid and saliva are symptoms.

Microchimerism factors (fetal cells in maternal blood circulations), genetic tendency, viral and pathogenic infection in body induce this autoimmune response. Epstein barr virus, human T cell leukemia virus and hepatitis virus are among causative agents of this disorder.

Genetic defects along with other hormonal factors trigger infiltration of lymphocytes including CD4+ B & T cells and the plasma cells. The results are glandular dysfunction of salivary and lacrimal glands.

Inheritance

Sjogren’s syndrome does not appear to be genetically inherited but researchers suggest a genetic presupposition. This means that a person may not inherit the disease but he/she does high risks of getting this disease. The studies reveal the familial clustering and close association of autoimmune disorder’s genes. In some studies, the person suffering with Sjogren’s syndrome has relatives with autoimmune disease like this one. Howver, this is not a rule of thumb that relatives of a patient with Sjogren’s syndrome must have it. Some may enjoy perfect health.

Risk factors

These few factors influence the onset of disease:

  • Age of person: Usually, the age of onset is 40 years.
  • Gender: This disease is more common in women as compared to men.
  • Medical history: If a person is immune-compromised or has rheumatoid arthritis or lupus, the chances of getting Sjogren’s syndrome are even high.

Diagnostic tests

Like many other diseases, the symptoms of Sjogren’s syndrome vary from person to person. Moreover, its diagnosis is difficult because a person may develop these symptoms as side effects of some other medication.  The diagnostic tests for Sjogren’s syndrome are:

1.Blood testing

Blood testing is performed to check:

  • The presence of antibodies in blood of person
  • Levels of blood cells of all types
  • To confirm the Inflammatory reactions in body
  • Indicate the conditions of lymph nodes, liver and other organs

2. Schirmer tear test/ Eye test

Doctors perform this test to check for dryness in eyes. For the test, the doctor’s place a small piece of filter pare under lower eyelid to estimate the eye production. Eye specialist or ophthalmologist examines the eye surface with a device- slit lamp. The result help in diagnosis of Sjogren’s syndrome.

3. Biopsy

Bioposy is performed to check the presence of clustered inflammatory cells. For the test, the doctors remove a small shard of salivary gland and perform microscopic examination.

4. Imaging test

Imaging test is to check the functioning of salivary glands. These are of two types:

  1. Sialogram: In this imaging, X-rays are directed on salivary glands, in front of ears. This estimates the amount of saliva in mouth.
  2. Salivary scintigraphy: The doctors inject a radioactive isotope in vein of the person. Its tracking up to its reaching to salivary glands in mouth helps in diagnosis.

Treatment options

The treatment of Sjogren’s syndrome depends on the area/organ of infection. its management is possible by application of eye drops and keeping body hydrated. The food and drug administration FDA has approved a medicine Pilocarpine HCl for curing the dryness of mouth in Sjogren’s patients. The MG1 Pharma is manufacturer of this medicine.

1. Drugs and medication

  • Dryness of eyes: ophthalmologists suggest cyclosporine (Restasis) or lifitegrast (Xiidra) eye drops to reduce the eye dryness and inflammation.
  • Salivary glands: The recommended medicines for mouth dryness are pilocarpine and cevimeline.
  • Other complications: In case the patient already have inflammatory arthritis or similar condition, the doctors recommend non-steroidal anti-inflammatory drugs (NSAIDs). Similarly, antifungal antibiotics treat the fungal infections in mouth and skin. As Sjogren’s syndrome is auto-reactive immune system disorder, doctors also recommend immune suppressor drugs such as methotrexate (Trexall).

2. Surgery

In some cases, the surgeons perform a minor surgery to seal and close off the tear ducts that drain tears from eyes, making them dry. For the purpose, doctors use silicon and collagen plugs to close the ducts.

Prognosis

The Sjogren’s syndrome mainly affects the eyes and slivery glands which are not life threatening. However, the symptoms may become severe and life threatening such as systematic lupus erythematous and lympho-proliferative cancer-lymphoma.

bottom line

Sjogren’s syndrome is auto-immune disorder which affects the mucous membrane and salivary glands. Genetic and environmental factors influence the onset of disease. A few treatment options are available for curing the symptoms and to prevent the complications.

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