Sjogren's syndrome What we need to know

08.07.2024

Table of Contents

What is Sjögren's syndrome?

Sjogren's syndrome is an autoimmune disease in which the glands of the mucous membranes, which ensure the maintenance of moisture in these membranes, are damaged. The most typical symptom of Sjögren's disease is chronic, abnormal dryness of the eyes, mouth, nose, digestive system, vagina, and other mucous membranes of the body.

Types of Sjogren's syndrome

Sjogren's syndrome is classified into two main types:

Primary Shogen syndrome: which develops independently of other health pathologies.
Secondary Sjogren's syndrome: Development or provoking is caused by another disease/pathological condition.

How common is Sjogren's syndrome?

Sjogren's syndrome can develop at any age, but is more common in women over 40. The epidemiology of the disease in different geographical areas is different and depends on the diagnostic criteria.

A recent meta-analysis estimated the incidence of Sjogren's syndrome to be 0.04% per 100 population.

Sjogren's syndrome symptoms

The most typical symptoms of Sjogren's syndrome are dry mucous membranes:

Dry and itchy eyes
Dry mouth (xerostomia), ulcers on the mucous membrane and thickening of saliva
Dryness and roughness of the skin
Dryness of the nasal cavity and frequent bleeding
Dry throat and frequent, spasmodic cough
Thickening of the vaginal mucosa
Muscle and joint pain
Swelling and pain of lymph nodes
General weakness and easy fatigue
Difficulty swallowing (dysphagia)
Feeling dizzy (decreased concentration and thought processes)
Loss of taste
Tooth damage (caries) and loss
Rash on the skin
Neuropathy
Pain or burning along the esophagus
Decreased sensitivity

What causes Sjogren's syndrome?

Sjogren's syndrome is a disease with autoaggressive processes. The exact causes of the development of the disease have not been fully clarified. Primary Sjogren's syndrome is caused by any underlying cause, while secondary Sjogren's syndrome is caused by various provoking factors, such as: other autoimmune diseases, viral infections, and others.

The following viruses can cause Sjogren's syndrome:

Hepatitis C
Cytomegalovirus
Epstein-Barr virus
Human T-lymphotropic virus 1
COVID-19

Sjogren's syndrome can also provoke other autoimmune diseases, such as:

Rheumatoid arthritis
Psoriatic arthritis
Lupus and others

Genetics and epigenetic factors

Genetic factors play an important role in the pathogenesis of Sjogren's syndrome. Family case studies have shown that 1/3 of Sjogren's syndrome patients have the same or another autoimmune connective tissue disorder. The risk of developing Sjogren's syndrome is much higher in twins than in sporadic cases.

Among the pathogenic mechanisms involved in the development of Sjogren's syndrome, interferon regulatory factor 5 polymorphism is considered.

The role of epigenetic factors in the development of autoimmune diseases is also widely recognized. DNA methylation, modification of histones and non-coding RNAs leads to the modulation of gene expression and plays an important role in the development of Sjogren's syndrome.

Sjögren's syndrome risk factors

Anyone can develop Sjogren's syndrome, but certain conditions increase the risk of developing the disease, including:

90% of cases of the disease develop in women
People with other autoimmune diseases are at higher risk
The disease develops more often in people aged 45 to 55
The risk of developing the disease increases if biological relatives of the first or second degree also have the same diagnostics

Diagnosis of Sjogren's syndrome

Diagnosis of the disease is based on objective examination of the patient, anamnestic and laboratory research data.

Diagnosis of the disease naturally includes differential diagnostics to rule out or confirm other autoimmune diseases.

Studies usually include:

serological tests
eye examination
otolaryngological examination
urine analysis
Biopsy chest X-ray

autoantibodies

Typical diagnostic criteria for Sjogren's syndrome are:

Antinuclear Antibodies (ANA)
Rheumatoid factor (RF)
SSA/RO autoantibodies
SSB/LA autoantibodies

Antinuclear antibodies and rheumatoid factor are positive in 85% of patients with Sjogren's syndrome. SSA/RO (33–74%) and SSB/LA (23–52%) autoantibodies, which are the main immunological markers in the diagnostics of Sjogren's syndrome, are often observed in patients with a negative titer of antinuclear antibodies.

Studies have shown that the presence of these autoantibodies is associated with earlier development of the disease, longer duration and severity of damage.

ENA, or extractable nuclear autoantibodies, is a further extension of the positive rate of antinuclear research and is used to differentiate connective tissue autoimmune diseases.

Anti-SS-A – The most commonly detected ENA antibody. It is associated with systemic lupus erythematosus, its subacute subcutaneous course, neonatal lupus, etc.

Anti-SS-B – It is a typical marker of autoimmune connective tissue diseases. It occurs in Sjogren's disease, scleroderma, myositis, systemic lupus erythematosus.

Anti-RNP – Autoantibodies against ribonucleoproteins. It occurs in 45% of patients with systemic lupus erythematosus, however, it is also characteristic of other connective tissue diseases: Sjogren's syndrome, scleroderma and polymyositis.

Anti-SM- Smith's autoantibodies are formed against small nuclear ribonucleoprotein - Smith, which consists of B, B1, D, E, G protein subunits. Smith's autoantibodies are typical of systemic lupus erythematosus and are present in 30% of patients.

Anti-SCL-70 – Autoantibodies are present in 20% of patients with systemic scleroderma and often indicate a poor clinical prognosis.

Anti-JO-1 – It is an autoantibody characteristic of myositis, which occurs in patients with idiopathic inflammatory myopathies. These antibodies are directed against histidyl transport RNA synthetase and inhibit histidyl attachment to transport RNA during protein synthesis.

It is often associated with a form of polymyositis and dermatomyositis with lung damage. The anti-JO-1 antibody titer correlates directly with disease activity.

Differential diagnostics

A number of diseases and conditions with damage to the mucous membrane of the eye and mouth require differential diagnostics with Sjogren's syndrome:

Medications - Long-term use of anticholinergic drugs, antispasmodic agents, beta-blockers, antihistamines, diuretics, opioids is often associated with dryness of mucous membranes and skin. The condition is even worse if the patient has Sjogren's syndrome.
age – Saliva, tears, gastrointestinal secretions are much reduced in old age.
Neuropathy - Chronic neuropathies of various chronic diseases, which slow down the production and release of secretions by the glands (diabetes, Parkinson's disease, multiple sclerosis, etc.)
Dehydration - is also associated with a reduced amount of secretions released by the gland
Radiotherapy - Decreases salivary and lacrimal (as well as other exocrine) gland function
Tobacco consumption - slows down the secretion of saliva and disrupts its alkaline-acidic balance
Alcoholism - damages the mucous membrane of the oral cavity and the secretion of saliva.
Hepatitis C - causes enlargement of the salivary gland and decrease in saliva production
human immunodeficiency virus With lymphocytic infiltration, the use of antiretroviral drugs often reduces the activity of the salivary gland.
stress, depression, anxiety - Affects sympathetic activity leading to changes in salivation.
amyloidosis - Amyloid infiltration of salivary glands
Sarcoidosis - Granulomatous infiltration of tissues may become the cause of xerostomia - oral mucosa damage.

Treatment of Sjogren's syndrome

Most people with Sjogren's syndrome live without serious complications. There is no cure for Sjogren's syndrome, but symptomatic treatment can greatly reduce the risk of complications.

Depending on which gland the disease is mainly affected, various symptomatic remedies are used:

Moisturize the eye
Stimulation of saliva production
Mouthwashes to protect against infection
Vaginal moisturizers
Hormone therapy against inflammatory processes
Non-steroidal anti-inflammatory drugs – to relieve pain and inflammation
Immunosuppressants to manage autoimmune processes
Antirheumatic treatment

What are the complications of Sjogren's syndrome?

Infections of the mucous membranes of the mouth and eyes
Liver and kidney function disorders
lymphomas
Lung damage (which is often mistaken for pneumonia)
Neurological disorders - neuropathies, head and spinal cord damage

When to see a doctor?

When the listed symptoms appear, or during the aggravation/exacerbation of the existing symptoms, it is necessary to consult a specialist, who decides the issues of further diagnostics and treatment.