Sjogren's Syndrome | Causes, Clinical features, Diagnosis & Treatment

Summary

Sjogren's syndrome is a chronic autoimmune disease that primarily affects the moisture-producing glands, leading to dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). It is categorized into primary and secondary forms, with the latter often associated with other autoimmune diseases like rheumatoid arthritis or SLE. The exact cause is unknown, but genetic and hormonal factors, along with environmental triggers, may play a role. Treatment is supportive, focusing on symptom relief through artificial tears and saliva, and managing complications like dental decay and secondary infections.

Takeaways

• 🕵️‍♂️ Sjogren's syndrome is a chronic systemic autoimmune disease recognized and named by Dr. Henrik Sjogren in 1933.
• 💧 The syndrome primarily affects exocrine glands, leading to decreased tear and saliva production, causing dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca).
• 🏥 There are two forms: primary Sjogren's syndrome, which only affects exocrine glands, and secondary Sjogren's syndrome, which occurs alongside another autoimmune disease.
• 🧬 The exact cause is unknown, but genetic factors, sex hormones, and environmental factors like viral or bacterial infections may trigger the disease.
• 👩‍⚕️ Sjogren's syndrome is more common in women, especially middle-aged, and is associated with female estrogen.
• 🔬 The disease process involves the activation of T cells and B cells, leading to the production of anti-nuclear autoantibodies, which contribute to glandular destruction.
• 👄 Oral symptoms include dry mouth, difficulty swallowing, and potential dental decay, while ocular symptoms involve dry, inflamed eyes with a sensation of dryness or foreign body.
• 🩺 Diagnostic tests include Schirmer's test for tear flow, sialometry for salivary flow, and blood tests for anti-SSA and anti-SSB autoantibodies.
• 💊 Treatment is supportive, aiming to reduce symptoms through artificial tears, saliva, medications to stimulate salivary flow, and oral hygiene to prevent dental decay.
• 🌐 The video encourages viewers to subscribe, like, share, and comment for more information and support, and provides a link to a Patreon page for additional resources.

Q & A

What is Sjogren's syndrome?

Sjogren's syndrome is a chronic systemic autoimmune disease that affects moisture-producing exocrine glands, primarily the salivary and lacrimal glands, causing dryness of the mouth (xerostomia) and eyes (keratoconjunctivitis sicca).

Who first recognized and named Sjogren's syndrome?

Sjogren's syndrome was recognized and named by Dr. Henrik Sjogren in 1933.

What are the two forms of Sjogren's syndrome?

The two forms of Sjogren's syndrome are primary Sjogren's syndrome and secondary Sjogren's syndrome.

How does primary Sjogren's syndrome differ from secondary Sjogren's syndrome?

In primary Sjogren's syndrome, only the moisture-producing exocrine glands are affected, and no other autoimmune disease is present. In secondary Sjogren's syndrome, patients experience another associated autoimmune disease in addition to Sjogren's syndrome.

What is the most common associated autoimmune disease in secondary Sjogren's syndrome?

The most common associated autoimmune disease in secondary Sjogren's syndrome is rheumatoid arthritis.

What are some possible triggers for the development of Sjogren's syndrome?

Possible triggers for Sjogren's syndrome include genetic factors, sex hormones, and environmental factors such as viral or bacterial infections.

Which gender is more commonly affected by Sjogren's syndrome and why?

Sjogren's syndrome is more common in women, likely due to the influence of female estrogen, and it mostly affects middle-aged women between 40 to 60 years.

What is the role of T cells in the pathophysiology of Sjogren's syndrome?

In Sjogren's syndrome, T cells abnormally activate and consider the nuclear components of the salivary cells as foreign antigens, leading to the production of cytokines and activation of B cells, which produce anti-nuclear autoantibodies.

What are the common oral symptoms of Sjogren's syndrome?

Common oral symptoms include a dry mouth, difficulty in swallowing, altered speech, difficulty wearing dentures, a fissured tongue, and red and painful oral mucosa due to secondary candidiasis.

How can the severity of dry eye in Sjogren's syndrome be assessed?

The severity of dry eye can be assessed using Schirmer's test, which measures the amount of tears produced over a period of time by placing a strip of paper under the eye.

What is the treatment approach for Sjogren's syndrome?

The treatment for Sjogren's syndrome is supportive, aiming to reduce symptoms. It includes the use of artificial tears for dry eyes, artificial saliva for dry mouth, medications to stimulate salivary flow, and oral hygiene practices to prevent dental decay.

Outlines

00:00

🧬 Understanding Sjogren's Syndrome

Sjogren's syndrome is a chronic autoimmune disease named after Dr. Henrik Sjogren, who recognized it in 1933. It primarily affects exocrine glands responsible for moisture production, such as the salivary and lacrimal glands, leading to dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). The disease is categorized into primary and secondary forms, with the latter often associated with other autoimmune conditions like rheumatoid arthritis or systemic lupus erythematosus (SLE). The exact cause remains unknown, but it's believed that genetic factors, sex hormones, and environmental triggers like infections might play a role. Women, particularly middle-aged, are more commonly affected. The pathophysiology involves the immune system mistakenly attacking the body's own cells, leading to inflammation and damage to the glands, which in turn causes the characteristic symptoms.

05:01

🩺 Diagnosis and Management of Sjogren's Syndrome

Diagnosing Sjogren's syndrome involves tests such as Schirmer's test for tear production and sialometry for salivary flow, along with blood tests to detect specific autoantibodies like anti-SSA and anti-SSB. A lip biopsy may also reveal immune cell infiltration in the salivary glands. Treatment is supportive, aiming to alleviate symptoms. For dry eyes, artificial tears are recommended, and for dry mouth, artificial saliva or medications like pilocarpine can stimulate saliva production. Maintaining oral hygiene and using fluoride applications can help prevent dental decay, a common issue due to reduced saliva. Anti-fungal therapy may be necessary for secondary infections like candidiasis. The video concludes by encouraging viewers to engage with the content through likes, subscriptions, and sharing, and invites questions and comments for further discussion.

Keywords

💡Sjogren's Syndrome

Sjogren's Syndrome is a chronic systemic autoimmune disease that affects the moisture-producing exocrine glands throughout the body, primarily the salivary and lacrimal glands. This leads to a decrease in tears and saliva production, causing dryness in the mouth (xerostomia) and eyes (keratoconjunctivitis sicca). The video explains that there are two forms of the syndrome: primary and secondary, with the latter being associated with other autoimmune diseases. The term is central to the video's theme as it is the main subject being discussed.

💡Exocrine Glands

Exocrine glands are glands that secrete their products, such as enzymes, sweat, or saliva, into ducts that lead to the body's surface or into a body cavity. In the context of Sjogren's Syndrome, these glands are affected, leading to a decrease in the production of moisture, which is crucial for the proper functioning of the mouth and eyes. The video emphasizes the role of exocrine glands in the development of the disease.

💡Xerostomia

Xerostomia refers to the dryness of the mouth that results from a decrease in saliva production. It is a primary symptom of Sjogren's Syndrome, as highlighted in the video. Patients with xerostomia may experience difficulty in swallowing, speaking, or wearing dentures, and it can lead to other oral health issues such as a fissured tongue and secondary infections.

💡Keratoconjunctivitis Sicca

Keratoconjunctivitis Sicca, often abbreviated as KCS or dry eye syndrome, is the dryness of the eyes due to insufficient tear production. This term is used in the video to describe the ocular symptoms of Sjogren's Syndrome, where patients complain of a scratchy sensation or a feeling of a foreign body in the eye, which can lead to blurred vision and discomfort.

💡Autoimmune Disease

An autoimmune disease is a condition in which the immune system mistakenly attacks the body's own healthy tissues. Sjogren's Syndrome is classified as an autoimmune disease because it involves the immune system attacking the exocrine glands. The video discusses how the disease can be primary, affecting only the exocrine glands, or secondary, where it is associated with another autoimmune disease.

💡Rheumatoid Arthritis

Rheumatoid Arthritis (RA) is a chronic inflammatory disorder that affects the joints and can lead to deformity and disability if not treated. The video mentions that in 15% of patients, Sjogren's Syndrome is associated with RA, indicating a connection between the two autoimmune conditions.

💡Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus, also known as SLE or lupus, is an autoimmune disease that can affect various parts of the body including the skin, joints, and internal organs. The video notes that secondary Sjogren's Syndrome may develop in 30% of patients with SLE, highlighting the overlap between these two autoimmune diseases.

💡Anti-SSA and Anti-SSB Antibodies

Anti-SSA and Anti-SSB antibodies are autoantibodies that are produced against SSA and SSB antigens, respectively. These antibodies are indicative of Sjogren's Syndrome and can be detected through blood tests. The video explains their role in the pathophysiology of the disease, where they contribute to the immune response that leads to glandular destruction.

💡Schirmer's Test

The Schirmer's Test is a diagnostic test used to measure tear production and is mentioned in the video as a method to assess the severity of dry eye symptoms in Sjogren's Syndrome. The test involves placing a strip of paper under the eye and measuring the amount of tears absorbed over a set period, which helps in diagnosing keratoconjunctivitis sicca.

💡Salivary Gland Biopsy

A salivary gland biopsy is a procedure where a small sample of tissue is taken from the salivary glands to examine under a microscope for signs of inflammation or other abnormalities. The video mentions this as a diagnostic tool for Sjogren's Syndrome, where the biopsy can reveal infiltration of immune cells, indicating the autoimmune nature of the disease.

💡Supportive Treatment

Supportive treatment refers to therapies aimed at managing symptoms and improving the quality of life for patients with chronic conditions like Sjogren's Syndrome. The video discusses various supportive treatments such as the use of artificial tears for dry eyes, artificial saliva for dry mouth, and oral hygiene measures to prevent dental decay, which are all aimed at reducing the symptoms of the disease.

Highlights

Sjogren's syndrome is a chronic systemic autoimmune disease named after Dr. Henrik Sjogren.

It primarily affects the salivary and lacrimal glands, leading to dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca).

There are two forms: primary Sjogren's syndrome and secondary Sjogren's syndrome, which is associated with other autoimmune diseases.

Primary Sjogren's syndrome only affects exocrine glands, while secondary involves another autoimmune disease.

Secondary Sjogren's syndrome is often associated with rheumatoid arthritis or systemic lupus erythematosus (SLE).

The exact cause of Sjogren's syndrome is unknown, but it's believed to involve genetic factors, sex hormones, and environmental triggers like infections.

Genetic factors may include malformed genes in immune cells, and sex hormones like estrogen, which may explain the higher prevalence in women.

The disease process begins with infection of salivary glands, leading to the exposure of nuclear components to antigen-presenting cells.

Abnormal activation of T cells and production of cytokines lead to the activation of B cells and the creation of anti-nuclear autoantibodies.

Autoantibodies target the glands, causing further destruction and reduction in salivary flow.

Symptoms vary, but common oral symptoms include a dry mouth, difficulty swallowing, and potential dental issues.

Ocular symptoms are characterized by dry eyes, scratchy sensation, and potential blurred vision.

Dryness can also affect other areas with exocrine glands, such as the skin, nose, sinuses, throat, ears, and vagina.

Schirmer's test and salivary flow measurement are used to assess tear and saliva production, respectively.

Blood tests can detect the presence of anti-SSA and anti-SSB autoantibodies, which are indicative of Sjogren's syndrome.

A lip biopsy can reveal infiltration of minor salivary glands with immune cells, which is characteristic of the disease.

Treatment is supportive, aiming to reduce symptoms, and includes artificial tears for dry eyes and artificial saliva for dry mouth.

Patients are at an increased risk of dental decay, and daily fluoride applications are recommended.

Anti-fungal therapy may be necessary to treat secondary candidiasis, a common complication of dry mouth.

Transcripts

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shogren's syndrome is a chronic systemic

and autoimmune disease that became

recognized and named by Dr Henrik

shogren in 1933.

the disease affects moisture producing

exocrine glands located all over the

body

primarily the salivary and the lacrimal

glands causing a decrease in tears and

saliva production resulting in dryness

of the mouth called xerostomia and

dryness of the eyes called zero of

thumbia

two forms of Sjogren's syndrome are

recognized so far the primary sjogren

syndrome and the secondary chagrance

syndrome

in primary sjogren syndrome which is

also known as zika syndrome only the

moisture producing exocrine glands are

affected and no other autoimmune disease

is present

however in secondary Sjogren's syndrome

patients experience another Associated

autoimmune disease in addition to

Sjogren's syndrome

the associated autoimmune disease in 15

of patients is rheumatoid arthritis in

addition secondary Sjogren's syndrome

may also develop in 30 percent of

patients with systemic lupus

erythematosis or SLE

coming to the causes of Sjogren's

syndrome the exact cause of Sjogren's

syndrome is not known however research

suggests that genetic factors and sex

hormones when combined with an

environmental Factor such as a viral or

a bacterial infection May trigger the

development of Sjogren's syndrome

the genetic factors can be the presence

of a malformed gene in the body's immune

cells and the sex hormone can be the

female estrogen indicating the condition

much more common in women than men

mostly affecting middle aged women

between 40 to 60 years

let's look into the pathophysiology of

Sjogren's syndrome the disease process

starts when salivary glands become

infected with a viral or a bacterial

infection this causes salivary cells to

break down and expose their nuclear

components to circulating antigen

presenting cells

these nuclear components are picked up

by the antigen presenting cells and are

presented to the cd8 or T cells

the T cells get abnormally activated in

patients with Sjogren's syndrome and

consider the nuclear components of the

salivary cells as foreign antigens the T

cells then produces cytokines and

further activate B cells which then

produce anti-nuclear autoantibodies or

anas against these antigens

the anti-nuclear autoantibodies are the

anti-ssa and the anti-ssb auto

antibodies produced against SSA and SSB

antigens together the anti-nuclear auto

antibodies and the T and B cells reach

the site of glandular destruction and

promote cellular breakdown by recreating

more immune cells and producing more

Auto antibodies in the area hence

resulting in further loss of the

secretary cells of the glands which

ultimately results in a reduction of

salivary flow

the severity of the disease differs in

individuals the principal oral symptom

of Sjogren's syndrome includes a dry

mouth

patients experience difficulty in

swallowing authored speech or difficulty

in wearing dentures

the dryness of the mouth reaches to the

extent the tongue becomes fissured and

Exhibits a trophy of the papillae

oral mucosa might become red and painful

as a result of secondary candidiasis

bilateral enlargement of the major

salivary glands which are usually

non-painful may also be observed

furthermore the reduced amount of saliva

production causes a lack of salivary

cleansing action which predisposes the

patient to dental decay the ocular

symptoms can be presented by the term

keratoconjunctivitis Seca

in keratoconjunctivitis Seeker the eyes

become dry which causes patients to

complain of a scratchy sensation or the

feeling of presence of a foreign body in

the eye the conjunctival epithelium

becomes inflamed resulting in blurred

vision and sometimes an aging pain in

the eyes

are the most common symptoms dryness can

also occur in areas where these Master

producing exocrine glands are located

such as Skin and mucosa resulting in dry

areas including the skin nose sinuses

throat ears and in women the vagina

schimmer's test can be used to assess

the flow of tears and is done by holding

a strip of paper under the eye and

measuring the amount of Tears over a

period of time

similarly salometry can be done to

measure salivary flow

and blood tests of course can be used to

indicate the presence of anti-ssa and

anti-ssv auto antibodies

lip biopsy indicates infiltration of

minor salivary glands with immune cells

the treatment for patients with sugar

syndrome is supportive and aims at

reducing the patient's symptoms dry eyes

are best managed with the periodic use

of artificial tears similarly artificial

saliva can be used for the treatment of

dry mouth

medications such as phylocarpine can be

useful to stimulate salivary flow

Sensations with children syndrome are at

an increased risk of dental decay daily

fluoride applications are indicated

symptoms can also be relieved by the use

of oral hygiene products like mouth

rinses and regular truth brushing

anti-fungal therapy is needed to treat

secondary candidiasis

so this was all about Sjogren's syndrome

I hope this video helps if you think

this video was really helpful please do

like subscribe share and comment if you

have any questions thank you for

watching