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Sjogren’s Syndrome - Causes, Diagnosis, and Treatment

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Sjogren’s syndrome, also known as Gougerot-Sjogren syndrome and sicca syndrome, is a systemic autoimmune disorder that is characterized by dry eyes and dry mouth. Read the article to know more.

Medically reviewed by Dr. Sneha Kannan
Published At March 2, 2022
Reviewed At October 3, 2022

Education:

BDS

Professional Bio:

Dr. Anuthanyaa is a Dental Surgeon who has an impeccable interest in serving people. She graduated BDS from J. K. K. Nataraja Dental College and Hospital, Bhavani. She is a part of the medical reviewer team at iCliniq, a global telemedicine platform. She is actively involved in surgical procedures and has a proven record of managing patients utilizing exceptional communication skills.

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Education:

BDS

Professional Bio:

Dr. Sneha Kannan is a Dental Surgeon with 11 years of clinical experience in treating patients with various oral conditions. She graduated from SRM Dental College and Hospital, Chennai, in the year 2012 and then did courses in minor oral surgeries, dental emergencies, and rotary endodontics. She is the proprietor and Head Dental Surgeon of Crown Me White Dental Clinic, Coimbatore. In addition, Dr. Sneha is the Senior Medical Reviewer at iCliniq.

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Sjogren’s Syndrome - Causes, Diagnosis, and Treatment
Table of Contents

What Is Sjogren’s Syndrome?

Sjogren's syndrome is a condition consisting of a triad of conditions, like keratoconjunctivitis sicca, xerostomia, and rheumatoid arthritis. Subsequently, it has been found that some patients present only with dry eyes and dry mouth, while others also developed systemic lupus erythematosus, polyarteritis nodosa, polymyositis, or scleroderma and rheumatoid arthritis.

Sjogren's syndrome was clinically illustrated as an autoimmune disorder of exocrine glands, and it is of two types:

  1. Primary Sjogren's syndrome is associated with salivary and lacrimal dysfunction without any autoimmune condition.

  2. Secondary Sjogren's syndrome is characterized by:

  • Lacrimal gland involvement causing ocular dryness.

  • Salivary gland involvement causing decreased salivation (xerostomia).

  • Along with autoimmune disorders such as rheumatoid arthritis and systemic lupus erythematosus.

What Are the Causes of Sjogren’s Syndrome?

As it is an autoimmune disorder, the immune system attacks the body's own cells mistakenly. The clear cause is still unknown, but specific genes put people at higher risk of the disorder. Still, a triggering mechanism like an infection with a particular virus or bacteria is also necessary. Various causes of this disease have been suggested, such as,

  • Genetic.

  • Hormonal.

  • Infectious.

  • Immunological.

The immune system will first target the glands that produce tears and saliva. Still, it can damage other parts of the body (thyroid, joints, kidneys, lungs, liver, skin, and nerves). It may be possible that a combination of extrinsic and intrinsic factors plays a role in the etiology of this condition. An altered immunological response is the main intrinsic factor responsible for the disease. Cytomegalovirus, paramyxovirus, and Epstein-Barr virus have been implicated in the pathogenesis of this condition but have not been proven conclusively.

What Are the Clinical Manifestations of Sjogren’s Syndrome?

This disease occurs predominantly in postmenopausal women over 40 years of age, although children or young adults may also be affected. The female to male ratio is approximately 10:1. The typical features of the disease are dryness of the mouth and eyes as a result of the hypofunction of salivary and lacrimal glands. This often results in painful, burning sensations of the oral mucosa. In addition, various secretory glands of the nose, larynx, pharynx, and vagina are involved in this dryness. Dryness of the pharynx, larynx and nasal cavity may lead to pneumonia.

Lacrimal gland involvement gives rise to dryness of the eyes and continuous feeling of dirt or foreign body in the eye. Conjunctivitis and corneal ulceration may be indicative of Sjogren's syndrome. Xerostomia (severe dryness of the oral cavity) causes difficulty in speech, mastication, and deglutition. Saliva will appear thick and ropy, and during clinical examination by the dentist, the mirror head will get stuck to the tongue and mucosa of the oral cavity. The lips will appear dry and cracked with angular cheilitis, and the tongue appears depapillated and at times lobulated. Candidiasis and increased dental caries are most commonly observed.

Parotid gland enlargement, lymphadenopathy, purpura, Raynaud’s phenomenon, kidney involvement, and myositis are more frequently manifested in people without rheumatoid arthritis.

How Is Sjogren’s Syndrome Diagnosed?

Sjogren's syndrome can be challenging to diagnose because the symptoms vary from one person to another, and the symptoms can be similar to other diseases. The adverse effects of some medications also mimic symptoms of Sjogren's syndrome. So, it is diagnosed by the following tests that can help to rule out other conditions from Sjogren's syndrome.

1. Blood tests:

The doctor takes blood tests to check levels of different blood cells, antibodies, inflammatory conditions, and problems with the liver and kidneys.

2. Ophthalmic Tests:

  • Schirmer's Test - In normal patients, 15 mm of the filter paper gets wet when placed in the lower conjunctival sac for five minutes, but in the case of Sjogren's syndrome, only 5 mm of the filter paper gets wet.

  • Rose Bengal Dye Test - Denuded and damaged areas of the cornea can be visualized clearly with this dye.

  • Break Up Time Test - A slit-lamp is used, and an interval between complete blink and appearance of a dry spot on the cornea is noted.

3. Salivary Gland Tests:

  • Sialography - It shows thinning of the ducts and a decrease in the number of ductules. It appears as punctate, globular, and cavitary pseudo sialectasis. It is termed pseudo sialectasis because the appearance is due to the pooling of the dye in the periductal area and not because of the dilation of the ductules. The overall description is given by the term branchless tree with a fruit-laden appearance (Rankow).

  • Salivary Scintigraphy - It is the nuclear medicine test, and it involves the injection of a radioactive isotope into a vein, which is tracked for an hour to see when it arrives at the salivary gland.

  • MRI (Magnetic Resonance Imaging) - It shows the salt and pepper appearances of the enlarged salivary glands.

4. Biopsy

A lip biopsy is done to detect if there are any clusters of inflammatory cells, which is indicative of Sjogren's syndrome. A sliver of tissue will be removed from salivary glands in the lip and examined under the microscope.

What Are the Treatment Options for Sjogren’s Syndrome?

There is no satisfactory treatment for Sjogren's syndrome. Most patients are treated symptomatically and medical consultation for the prevention of pneumonia is needed.

1. To Manage Keratoconjunctivitis and Xerostomia - A regular examination by the ophthalmologist is needed. Keratoconjunctivitis is treated by a mixture of ocular lubricants such as artificial tears containing Methylcellulose, and xerostomia is treated by saliva substitutes with topical Methylcellulose (Oralube, Xero-Lube wet mouth) or such as those used in the treatment of a person with xerostomia secondary to radiation therapy.

2. Dental Considerations - Extensive dental caries are a common complication, so oral hygiene and frequent fluoride application are indicated to reduce this problem. Topical application of Nystatin and Clotrimazole is needed to control candidiasis. There is no specific treatment for the enlargement of salivary glands.

3. Surgery - Surgery has been employed but is generally recommended only in patients with discomfort. A minor procedure to close the tear ducts that drain tears from the eyes may help relieve the dry eyes. Silicone plugs or collagen are inserted into the ducts to help preserve the tears. Also, patients with Sjogren's syndrome may be on steroids or immunosuppressants for the treatment of systemic lupus erythematosus or rheumatoid arthritis; therefore, one must take precaution against infection preceding oral surgical procedures.

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Frequently Asked Questions

What Kind of Illness Is Sjogren's Syndrome?

Rheumatoid arthritis, keratoconjunctivitis sicca, and xerostomia are the three disorders that make up Sjogren's syndrome. Some patients experience crippling pain and exhaustion as significant symptoms of the condition.
- Rheumatoid arthritis - Rheumatoid arthritis is an autoimmune condition that develops when the body's own tissues come under attack by the immune system.
- Keratoconjunctivitis - It is a chronic, bilateral desiccation of the cornea and conjunctiva brought either by insufficient tear production or hastened tear evaporation. Regular symptoms include photosensitivity, burning, blurring, and a gritty, tugging, or alien body sensation.
- Xerostomia - A condition known as xerostomia occurs when there is insufficient saliva production by the salivary glands in the mouth, resulting in a dry mouth.

What Organs Are Impacted By Sjogren's?

Due to the hypofunction of the salivary and lacrimal glands, the disease's common symptoms include dryness of the mouth and eyes. In addition, Sjogren's syndrome can lead to dryness of the skin, nose, and vaginal area. It can also impact the kidneys, blood vessels, lungs, liver, pancreas, and brain, among other body parts.

Can the Patient Get Rid of Sjogren's?

Yes, Sjogren's syndrome has no permanent cure. The majority of patients receive symptomatic care the requirement for routine ophthalmological examinations. Methylcellulose-containing artificial tears are used to treat keratoconjunctivitis, while topical methylcellulose-containing saliva substitutes are used to treat xerostomia.

What Is the Average Lifespan of Those With Sjogren's Syndrome?

Sjögren's syndrome is undoubtedly going to have an impact on a patient's quality of life even though it is not typically regarded to be linked to complications that shorten life expectancy.

What Vitamins Aid Sjogren's Disease?

The pathogenesis of Sjögren's syndrome may involve vitamin D. In addition, people with Sjögren's syndrome have been discovered to have low levels of vitamin D, which are linked to extra-glandular symptoms like lymphoma or neuropathy, indicating a potential beneficial effect of vitamin D in Sjögren's disease.

What Sjogren's Symptoms Did the Patient Experience Initially?

The typical symptoms of the conditions are:
- Dryness of the lips and eyes.
- Dryness causes painful, burning sensations in the oral mucosa.
- A number of secretory glands in the vagina, larynx, pharynx, and nose contribute to this dryness. 
- Pneumonia may result from dryness of the pharynx, larynx, and nasal cavity. 
- Sjogren's syndrome may be indicated by conjunctivitis and corneal ulcers. 
- Difficulty in speech, chewing, and deglutition are all made more difficult by xerostomia (extreme oral dryness).

Who Is Susceptible To Sjogren's?

While Sjogren's syndrome can manifest at any age, most cases are detected in patients over 40. Women are far more likely to suffer from the illness. The impact of sex hormones on a woman's immune system may be related to this imbalance.

What Occurs If Sjogren's Disease Is Not Treated?

Patients with Sjogren's syndrome may experience irritation, a gritty sensation, or intense burning in the eyes if left untreated; dry eyes are more prone to infection and are sensitive to corneal damage. A dry mouth can make it difficult to eat and swallow dry foods. It can result in tooth loss, chipping, breakage, and dental caries.

What Kind of Blood Test Reveals Sjogren's?

Antinuclear antibody (ANA) immunofluorescence testing is crucial for diagnosing connective tissue disorders. Particular antibodies may indicate an autoimmune disease against immune system proteins that ordinarily attach to dangerous chemicals, which can be found through a blood test.

What Does A Positive Sjogren's Test Mean?

The presence of elevated Sjögren's antibodies against Sjögren's syndromes A and B is symptomatic of an inflammatory connective tissue illness. Elevated test results may be a sign of Sjogren's syndrome or another autoimmune condition like lupus or rheumatoid arthritis.

Does Sjogren syndrome Cause Pain?

Yes, the lacrimal and salivary glands' hypofunction causes dryness of the mouth, which leads to painful, burning sensations in the oral mucosa, and dryness in the eye lead to conjunctivitis and corneal ulcers.

When Does Sjogren's Syndrome Begin?

Sjogren's syndrome affects areas of the body that secrete fluids, such as saliva and tears. It typically manifests in adults between the ages of 40 and 60 and affects women far more frequently than males.

How Is Sjogren's Syndrome Naturally Treated?

- Self-care techniques work well for many Sjogren's syndrome symptoms.
- Patients can avoid having uncomfortable dry lips and eyes by boosting the humidity in the room and limiting their exposure to blowing air. Avoid, for instance, sitting near a fan or air vent and putting on goggles or other protective eyewear while outside.
- Avoid smoking because it might make the mouth dry and irritated.
- Drink plenty of liquids throughout the day, especially water. Avoid drinking alcohol or coffee because they can worsen dry mouth symptoms. Avoid drinking colas and several other sports drinks because the acid in these drinks might damage the teeth's enamel.
- Gum without sugar or hard candies with citrus flavors might encourage salivation.
- For the patient to breathe freely through their nose, a nasal saline spray can aid in moisturizing and clearing nasal passages. Increased mouth breathing may result from a dry, congested nose.
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Dr. Anuthanyaa. R from iCliniq
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