Sjogren's Syndrome: Causes, Symptoms, Risk Factors

Sjogren's syndrome, an immune system disorder, is most frequently characterized by dry mouth and dry eyes.

Along with lupus and rheumatoid arthritis, the condition usually coexists with other immune system disorders. Sjogren's syndrome often causes first-degree damage to the mucous membranes and glands that exude moisture from your mouth and eyes, resulting in decreased production of saliva and tears.

The majority of Sjogren's syndrome cases are diagnosed in persons over the age of 40, despite the fact that it can appear at any age. Compared to men, women have a substantially higher risk of developing the condition. 

Causes Of Sjogren's Syndrome

The condition known as Sjogren's syndrome is autoimmune. Your body's own cells and tissues are accidentally attacked by your immune system.

The cause of Sjogren's syndrome in certain patients remains unknown to scientists. A triggering mechanism, such as infection with a specific virus or strain of bacteria, appears to be required in addition to certain genes that increase a person's risk of developing the condition.

Your immune system targets the glands that produce saliva and tears first when you have Sjogren's syndrome. However, it can potentially harm other areas of your body, including:

• Joints
• Kidneys
• Thyroid
• Lungs
• Skin
• Liver
• Nerves

Symptoms Of Sjogren's syndrome

Sjogren's syndrome has two primary symptoms, which are:

• Dry eyes: It may hurt, itch, or feel like there is sand in your eyes.
• Dry mouth: It could be challenging to speak or swallow because your mouth may feel like it's filled with cotton.

Some Sjogren's syndrome sufferers also have one or more of the following conditions:

• Stiffness, edema, and joint pain
• Swollen salivary glands, especially those that are in front of and behind the jaw.
• Skin rashes or dry skin
• Vaginal dryness
• Chronic dry cough
• Prolonged tiredness

PATHOPHYSIOLOGY : SJOGREN’S SYNDROME

Mainly due to genetic and environmental conditions → autoantibodies form→ autoimmune lymphocytic exocrinopathy-> activates duct epithelium → lead to symptoms of dry eye, dry ,outh,dry skin, dry throat 

• HLA DR 3 : GENETICAL FACTOR
• ANTIBODIES: Anti M3 Muscarinic  , Anti Alpha Fodrin

Classification of Sjogrens is based on CTD & Pathology

1. Primary Sjogrens 
2. Secondary Sjogrens

Pathologically sjogrens shows  granular & extraglandular changes 

Extraglandular Sjogren's  may manifests to systemic illness

Predictors of Extraglandular Sjogrens

1. Low c3,c4 complement
2. Cryoglobulins
3. RF: POSITIVE
4. Anti Ro  : Positive

Risk factors Of Sjogren's syndrome

The majority of patients with Sjogren's syndrome have one or more of the following risk factors:

• Age: Typically, adults over the age of 40 are diagnosed with Sjogren's syndrome.
• Sex: Sjogren's syndrome is substantially more prevalent among females.
• The rheumatic disease: A rheumatic condition, such as lupus or rheumatoid arthritis, is frequently present in Sjogren's syndrome patients.

Criteria For Diagnosis Of Sjogrens Syndrome

According to 2016 American college of Rheumatology .

Risk Factors Of Sjogren's syndrome

The majority of patients with Sjogren's syndrome have one or more of the following risk factors:

• Age: Typically, adults over the age of 40 are diagnosed with Sjogren's syndrome.
• Sex: Sjogren's syndrome is substantially more prevalent among females.
• The rheumatic disease: A rheumatic condition, such as lupus or rheumatoid arthritis, is frequently present in Sjogren's syndrome patients.

Diagnosis Of Sjogren's Syndrome

Sjogren's syndrome can be challenging to diagnose because the signs and symptoms differ from person to person and can resemble those brought on by other disorders. A few Sjogren's disease symptoms and signs can sometimes be mistaken for side effects of certain drugs.

Tests can help diagnose Sjogren's syndrome and help rule out additional medical conditions.

Blood Test

Blood tests may be prescribed by your doctor to look for:

• Levels of various blood cell types
• Presence of common antibodies found in Sjogren's syndrome
• Indications of inflammatory disorders
• Signs of liver and kidney problems

Vision Examinations

Using a procedure known as the Schirmer tear test, your doctor can determine how dry your eyes are. Your lower eyelid is touched with a small piece of filter paper in order to determine how many tears you are producing.

A specialist in treating eye conditions (ophthalmologist) may also use a slit lamp, a magnification tool, to inspect the surface of your eyes. He or she might inject drops into your eye that help you view corneal damage more clearly.

Imaging

Your salivary glands can be tested using certain imaging techniques.

• Sialogram: A dye injection into the salivary glands in front of your ears can be detected with this specialized X-ray. This process demonstrates how much saliva enters your mouth.
• Scintigraphy of the saliva: This nuclear medicine test includes injecting a radioactive isotope into a vein, which is monitored for an hour to observe how rapidly it spreads throughout your salivary glands.

Biopsy

Additionally, your doctor might perform a lip biopsy in order to check for inflammatory cell clusters, which may be a sign of Sjogren's syndrome. For this test, a small piece of tissue is taken from the salivary glands in your lip and examined under a microscope.

Treatment Of Sjogren's Syndrome

The affected bodily parts determine how Sjogren's syndrome should be treated. By using over-the-counter eyedrops and drinking more water, many people are able to control the dry eye and dry mouth symptoms of Sjogren's syndrome. However, some people require prescription drugs or even surgery.

Medications

Your doctor may recommend drugs that:

• Reduce inflammation in the eyes: If you have moderate to severe dry eyes, your eye doctor may suggest prescription eyedrops such as cyclosporine (Restasis) or lifitegrast (Xiidra).
• Increase saliva production: Saliva and occasionally tears might be produced more often when using medications like pilocarpine (Salagen) and cevimeline (Evoxac). Sweating, stomach ache, flushing, and increased urination are examples of possible side effects.
• Address specific problems: Nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis medications may be helpful if you start to experience the symptoms of arthritis. Antifungal drugs should be used to treat oral yeast infections.
• Symptoms throughout the entire body should be treated: Treatment of Sjogren's syndrome frequently benefits from the use of the malaria medicine hydroxychloroquine (Plaquenil). Additionally, immune system suppressants such as methotrexate (Trexall) may be administered.

Surgery

• Punctal occlusion, a quick technique that seals the tear ducts that drain tears from your eyes, may help cure dry eyes. To assist in keeping your tears from drying out, collagen or silicone plugs are put into the tear ducts.

Complications

Your eyes and mouth are the most typical areas of Sjogren's syndrome problems.

• Cavities in teeth: You're more likely to have cavities if your mouth is dry because saliva helps shield the teeth from the bacteria that cause cavities.
• Candida infections: Oral thrush, a yeast infection of the mouth, is considerably more common in those with Sjogren's syndrome.
• Vision issues: Light sensitivity, impaired vision, and corneal damage are all symptoms of dry eyes.

Less frequent issues could have an impact on:

• Liver, kidneys, or lungs: Inflammation can result in kidney difficulties, hepatitis or cirrhosis in the liver, pneumonia, bronchitis, or other issues with the lungs.
• Lymphatic nodes: Lymphoma, a kind of lymph node cancer, can occur in a small percentage of Sjogren's disease patients.
• Nerves: Your hands and feet could have peripheral neuropathy, which causes numbness, tingling, and burning.

Frequently Asked Questions

1)Does Sjogren’s has permanent treatment?

There is currently no cure, except managing symptomatically and maintaining effective and health lifestyle adjustments

2)What  are the reasons for getting sjögren's syndrome?

The reason for getting sjogrens is unknown, but it is known due to the involvement of genetic and hormonal combinations & environmental factors.

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