Sjögren’s Syndrome- A Rare Autoimmune Disorder

Sjögren’s Syndrome- A Rare Autoimmune Disorder

Sjögren’s is a chronic condition that is regarded as an autoimmune disorder. It means that sometimes healthy and functional cells and tissues of the body are considered for an attack by your own immune system.

The white blood cells, that make up immune system attack the saliva glands, tear glands, and other tissues. It causes a decrease in tear and saliva production leading to dryness in eyes, skin, mouth, nose, vagina and upper respiratory tract.

The occurrence of Sjögren’s syndrome also leads to other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, primary biliary cholangitis and others

There are two stages of Sjögren’s syndrome. Primary Sjögren’s syndrome develops all by itself. A secondary stage of Sjögren’s syndrome occurs with other conditions, making the health status of the patient even worse. An example of it is lupus. Both may look similar but lead to a severe condition. In the USA, 0.1- 4.0% population is affected by Sjögren’s syndrome. 

Sjögren’s syndrome is a serious problem but only a timely diagnosis and treatment can make it less complicated. Once it is diagnosed and the treatment has started, it is less likely to cause severe damage.

There is no age and specific sex as the target of Sjögren’s syndrome. But most of the diagnosis evident in older age patients. It is surprising that more than 90% of the patients are females. It is less common in men.

What causes it?

As it is regarded as autoimmune disease, our own body cells cause Sjogren’s syndrome. The defensive white blood cells become aggressive and that start attacking the moisture producing glands of the body.

The obvious cause of Sjögren’s is not yet known and need research. It is estimated that viral/bacterial strains, environmental factors and hormonal changes in women may cause it.

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Symptoms of Sjögren’s syndrome

The characteristic feature of Sjögren’s syndrome is an inability to produce moisture wherever it is naturally present. It includes eyes, mouth etc. As most of the patients are women, it is more likely to cause a vaginal dryness.

Other symptoms include the following.

  • tooth decay,
  • a dry cough
  • difficulty in chewing and swallowing
  • hoarse voice
  • inability to speak
  • swollen salivary glands
  • recurring oral thrush,
  • dry eyes
  • itchy eyes
  • excessive discharge of mucus from the eyes
  • sensitivity to light
  • burning in eyes
  • blurred vision

When the condition of Sjögren’s syndrome becomes severe and it attacks other parts of the body too, following are the common signs that show up.

  • Fatigue
  • Tiredness
  • vasculitis
  • Brain fog
  • painful muscles
  • Raynaud’s phenomenon
  • stiffness and inflammation of joints
  • occasional pain in arms and legs
  • lungs problems

Related: Pain in lower right abdomen- why do you feel it?

How to diagnose Sjögren’s syndrome?

The Sjögren’s signs are not very differential. They may look similar to many other health conditions. That is why the diagnosis of Sjögren’s syndrome is difficult.

Usually, the patient ends up meeting a number of health professionals with different specializations. For example, he may see a dentist for the dental problems, a gynecologist for vaginal dryness, and an ophthalmologist for dry eyes.

Some of the medicines may also cause these effects, which look like symptoms of Sjögren’s syndrome. The doctor will ask a number of questions and test various medicines to finally conclude the condition with Sjögren’s syndrome. Some of the screening tests may confirm it. These tests are as follows.

  • Blood tests

In Sjögren’s syndrome, the body triggers the presence of a special type of antibodies in the blood. These antibodies show up in the majority of the patients. A negative test doesn’t mean that it is not Sjögren’s syndrome. Sometimes it confuses the diagnosis but other tests may help to diagnose it better.

  • Ophthalmological tests

An ophthalmologist will test the patient for Rose Bengal, Lissamine green, and SSchirmer’seye. These tests use different dyes and blotting paper to identify the dryness in eyes.

  • Salivary flow rate

In this test, the doctor will measure the weight of saliva produced within a time span of 5 minutes.

  • Sialogram

In this test, a dye is used to the parotid glands. Then an X-ray is used to determine how much saliva flows into the patient’s mouth.

  • Salivary scintigraphy

In this test a radioactive isotope is used and traced via imaging for measuring the salivary gland function.

  • Chest X-ray or CT-scan

These tests show the inflammation in lungs, which may be due to Sjögren’s syndrome.

  • Skin biopsy with nerve fiber stain

This test is used to determine the presence of Small Fiber Neuropathy (SFN).

  • Urine tests

Urine samples are tested for analyzing kidney functions.

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Treatment for Sjögren’s syndrome

The only thing that works for Sjögren’s treatment is to consider what it makes the body deficient. It means that lubricating and moisturizing the affected areas that become dry may help in condition.

The doctor may prescribe you the following types of treatments.

  • Medications to stimulate saliva flow– examples pilocarpine and cevimeline
  • Artificial saliva producers
  • Artificial tears
  • Prescription eye drops – examples cyclosporine and lifitegrast
  • Moisture chamber spectacles
  • Prescription expectorants and throat medications
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)– examples aspirin, naproxen, and ibuprofen
  • Disease-modifying antirheumatic drugs (DMARDs)– examples hydroxychloroquine or methotrexate
  • Antifungal medication
  • Vaginal lubricants
  • Punctual occlusion
  • Autologous eye serum

Dietary precautions for Sjögren’s syndrome

There are no as such dietary precautions for Sjögren’s syndrome. However if you have other health issues when Sjögren’s syndrome shows up, your doctor may prescribe you any special dietary tips. Otherwise, the patient doesn’t need it.

The only thing, which should be limited, is alcohol. Using less spices or oils in food is also helpful for overall health. You may use lozenges to retain the moisture of mouth.

Some tips that may make swallowing of food easier for the patient are as follows.

  • Use sauces, olive oils and other such things along with food. They will lubricate the solid food and help you to chew and swallow it.
  • Remember to drink water while you eat.
  • Add a straw to your glass/bottle; sipping makes the water intake easier.
  • Use ingredients that have plenty of water content in it i.e. cucumbers, tomatoes.
  • Use liquid diet.
  • Increase your fluid intake.

The concept of balanced diet is helpful that includes both vegetables and fruits. These food ingredients are low in saturated fats and are also sugar free. Remember there are some foods that may cause inflammation. Try to avoid such ingredients.

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Keep a history of your food intake. It will help you to point if any food is causing inflammation or allergy to you or is making the Sjögren’s signs worse.

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Rash in Sjögren’s syndrome

One must know that if they are suffering from Sjögren’s disease, they are at a high risk of rash. It is highly common when they are exposed to sun. Other skin conditions that may show up, along side rash are as follows.

  • Xerosis (dry, rough scaley skin)
  • Purpura (small blood spots) or lower legs
  • Vasculitis (inflammation of the blood vessels)
  • skin lesions (weals, lumps, blisters, ulcers)
  • Annular erythema (ring-shaped red lesions with a pale area in the middle)

What are the complications of Sjögren’s syndrome?

Sjögren’s syndrome may result into serious complications when the patient doesn’t take it serious. If left untreated, it may cause the following problems.

  • Lymphoma
  • Multiple myeloma
  • yeast infections
  • tooth cavities
  • vision problems
  • inflammation of lungs causing bronchitis, pneumonia etc
  • Kidney problems
  • Liver problems i.e. autoimmune hepatitis or cirrhosis
  • Birth defects in baby
  • peripheral neuropathy
  • interstitial cystitis of bladder

It is necessary to seek medical assistance as soon as the symptoms of Sjogren’s syndrome show up. Only a timely diagnosis can prevent the risks associated with the complications of it.

 

Nancy Walker

Nancy holds a Medicine degree and a Masters of Science MS in Infectious Disease and Global Health (MS-IDGH) from Tufts University. She worked as a lecturer for three years before she turned towards medical writing. Her area of interest are infectious diseases; causes, mechanism, diagnosis, treatments and prevention strategies. Most of her writings ensure an easy understanding of uncommon diseases.

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