Sjogren’s Syndrome: Symptoms, Causes, Risks, Complications, Diagnosis and Treatment

0
185
Sjogren's Syndrome

1Overview

The immune system plays a pivotal role in the body by protecting it from harmful foreign intruders such as disease-causing pathogens. When the immune system detects something wrong in the body, it sends out cells to detect and destroy foreign substances that are deemed harmful to the body. However, this mechanism may malfunction, leading to autoimmunity.

Autoimmunity is described as the destruction of healthy cells in the body by the immune system, believing the healthy cells are harmful. Today, there are many autoimmune diseases and their exact etiology are still unclear.

One of the disorders affecting the immune system is Sjogren’s syndrome, a disorder characterized by two distinct symptoms – dry mouth and dry eyes. Specifically, in this condition, the body’s immune system attacks the glands that produce tears and saliva. However, Sjogren’s syndrome can also affect other parts of the body like the throat, skin, nose, joints, blood vessels, kidneys, nerves and digestive organs.

Moreover, the condition often accompanies other autoimmune diseases such as lupus and rheumatoid arthritis. Despite the fact that you can get this disorder at any age, people who are older than 40 years old are at a higher risk of developing the illness. Also, it’s more common in women.

2Symptoms

There are two main symptoms of Sjogren’s syndrome:

Dry mouth – The mouth may feel like it’s full of cotton. This makes it hard for the patient to speak or to swallow. Moreover, eating is also affected because saliva is needed for the digestion of food.

Dry eyes – The eyes may feel dry, itchy and gritty. Some people feel as if sand is in their eyes.

Aside from these two hallmark symptoms, other symptoms include:

  • Dry skin
  • Tiredness or persistent fatigue
  • Joint swelling, stiffness, and pain
  • Dry and itchy skin
  • Vaginal dryness
  • Rashes
  • Swollen salivary glands
  • Persistent dry cough

3Causes

Sjogren’s syndrome is an autoimmune disorder. This means that the immune system targets the body’s healthy cells and tissues. Up to date, scientists are still baffled on the exact cause of autoimmunity. However, some people are highly predisposed to autoimmunity than others.

In some cases, the scientists believe that autoimmunity happens when a certain trigger like an infection affects the body. Also, certain genes may make some people susceptible to the condition. Hormones may play a pivotal role in the development of autoimmune disorders. For instance, the female hormone estrogen can increase the risk of developing Sjogren’s syndrome since it’s more common in women than in men. In fact, the ratio of the disease when it comes to gender is 9:1 (9 women is to one man).

Sjogren’s syndrome is caused by the immune system damaging the glands that produce tears and saliva. However, the immune cells can also attack other parts of the body such as the joints and nerves. Other parts of the body that can be affected include the thyroid, kidneys, liver, lungs, and skin.

There are two types of Sjogren’s syndrome – primary and secondary. Primary Sjogren’s syndrome means that there is no other autoimmune disease while secondary Sjogren’s syndrome happens in conjunction with other autoimmune illnesses like lupus, and rheumatoid arthritis.

4Risk Factors

Sjogren’s syndrome develops in some people who have risk factors including:

  • Family history – Genetics play an important role in the development of an autoimmune disease like Sjogren’ syndrome. Some people whose family members have other autoimmune diseases are at a higher risk of the illness too.
  • Sex – Females are at an increased risk of developing the condition than males.
  • Age – Older people (those who are 40 years old and above) are at a heightened risk of developing Sjogren’s syndrome.
  • However, in some people, the disease develops as a single autoimmune disease (primary Sjogren’s syndrome).

5Complications

Due to the effects of Sjogren’s syndrome to the body, the patient may experience some complications due to the persistent dryness of some parts of the body.

The most common complications of the disorder happen in the mouth and eyes, including:

Dental caries or cavities – Saliva helps protect against tooth decay and gum disease. Saliva also films the teeth enamel, protecting it from bacteria that cause tooth decay. Aside from these, it helps in the digestion of food in the mouth. When there is a dry mouth, it may lead to the formation of dental cavities. In some cases, the infection may even lead to gum disease and periodontitis.

Problems with visionPeople with dry eyes may lead to an increased risk of serious eye infections. Also, it may lead to the thinning, scarring and perforation of the cornea. People with dry eyes may also experience light sensitivity and blurred vision.

Yeast infections – Dryness in the mouth may eventually lead to oral thrush, which is defined as the yeast infection in the mouth. This can be pesky because they return even after being treated.

Other Complications:

Lymphoma People with Sjogren’s syndrome have an increased risk of developing non-Hodgkin lymphoma compared to individuals who are healthy. In fact, an estimated 5 percent of patients with Sjogren’s syndrome will have some form of lymphoid cancer. Those with more severe cases of the syndrome are at a higher risk of having lymphomas than those with just mild or moderate cases.

The most common lymphomas that could develop are salivary B cell lymphomas and diffuse large B-cell lymphoma.

Lung problems – The inflammation of the lungs may cause pneumonia, bronchitis and other lung problems.

Kidney and liver problems – People with Sjogren’s syndrome may have kidney problems and liver issues including cirrhosis and hepatitis.

Nerve issues – Another complication linked to the syndrome is the development of numbness, burning and tingling in the hands and feet, dubbed as peripheral neuropathy.

Pregnancy problems – Most women with Sjogren’s syndrome can give birth to healthy babies. However, those mothers who are planning a pregnancy, talk with your doctor about the possible complications such as a rash on the baby that lasts for a few weeks and a serious heart problem.

more complications:

  • Reynaud’s phenomenon
  • Irritable bowel syndrome
  • Hypothyroidism
  • Vasculitis or the inflammation of the blood vessels
  • Central Nervous System (CNS) complications like myelitis, Neuromyelitis Optica, and Primary-Progressive Multiple Sclerosis.

6Diagnosis

Sjogren’s syndrome is hard to detect because the signs and symptoms vary from one person to another. Here are the diagnostic procedures for this condition:

Blood tests – The doctor may request blood tests to check for the presence of antibodies for Sjogren’s syndrome, the problem in the liver and kindness, the presence of inflammatory conditions, and the levels of the various types of blood cells.

Eye tests – The doctor will observe the dryness of the eyes using a test dubbed as Schirmer tear test. Also, a test will also be used. In this test, the doctor will look at the layer of tears that form in the eyes.

Moreover, the doctor will do a test to examine the layer of tears that form across the front of the eyes. To do this, he will use harmless colored drops to make the tears easier to see. A special microscope is used. If the layer of tears appears patchy, this could signal the presence of the syndrome.

Biopsy – The doctor will perform a biopsy by removing a piece of lip tissue. In people with Sjogren’s syndrome, white blood cells are released by the immune system and these can form inside the cells where saliva is produced. For biopsy to be performed, tissue from the inside of the lips is removed. This tissue is examined under a microscope to see any abnormalities.

Imaging – Imaging tests can be done to check the function of the salivary glands. One test, called Sialogram, is a special type of X-ray that can see the salivary glands by using a special dye that’s injected into them.

Another test called salivary scintigraphy is done by the injection of a radioactive isotope into a vein. The test will see if this isotope will arrive in the salivary gland and how fast it could reach them.

Other tests – The doctor may request for the patient to undergo a spit test to see how much saliva he or she can produce in a five-minute period. Another test is used to measure how many tears the person can produce. Strips of paper are placed in the lower eyelid for five minutes.

7Treatment

The treatment of Sjogren’s syndrome depends on the parts of the body involved or affected. Many individuals may manage dry mouth or dry eyes caused by the condition by just sipping water frequently or using over-the-counter eye drops. However, it is advised to seek the help of a doctor first and using medicines which were prescribed. Do not self-medicate.

Medications – Medications are given to patients depending on their symptoms.

To increase the production of saliva – To increase saliva production, the doctor can prescribe drugs such as pilocarpine (Salagen) and cevimeline (Evoxac). Sometimes, this can also help increase the production of tears.

Reduce inflammation of the eyes – To reduce eye inflammation, the doctor may prescribe eye drops such as lifitegrast (Xiidra) and cyclosporine (Restasis).

Treat complications – For patients who developed arthritis symptoms, the use of nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce pain and swelling. For oral thrush, the doctor may prescribe antifungal medicines.

Treat systemic symptoms – One drug that’s used to treat malaria, hydroxychloroquine (Plaquenil) can help treat Sjogren’s syndrome. Other drugs, which can be used to suppress the immune system, can be prescribed. One example of this type of medicine is methotrexate (Trexall).