Sjögren's syndrome

Content

Introduction

Sjögren's syndrome (pronounced schiagren) is an autoimmune disease in which the body's defense system (immune system) recognizes glands that secrete liquids such as, for example, the lacrimal and salivary glands, as foreign, and attacks them causing inflammation This leads to a reduction in the production of tears and saliva and the appearance of dry eyes and mouth.

The glands that produce sweat (sweat) and those responsible for vaginal secretion may also be affected by the disease. Consequently, there may be a reduction in sweat and, again due to the lack of secretions, vaginal dryness, trachea and large bronchi, with the appearance of dry and irritating cough.

The causes of the disease remain unknown but research suggests that "autoimmunity is triggered by a combination of hormonal, genetic and environmental factors. It is hypothesized that a genetic predisposition plays a role in the reaction of the immune system, on the one hand, and on the other". other, triggering events such as, for example, viral infections.

Sjögren's syndrome is classified as:

  • primary, if it develops in people who do not have other autoimmune diseases
  • secondary if it occurs in combination with another autoimmune disorder such as lupus or rheumatoid arthritis

If dry eyes, mouth and vagina appear, it is advisable to contact the family doctor who will evaluate the complaints (symptoms) and, if he deems it necessary, will prescribe some specific tests.

There is no cure for Sjögren's syndrome but some treatments can help reduce ailments (symptoms): for example, dry eyes and mouth can be relieved with artificial tears and saliva. Furthermore, it is important to maintain good hygiene because the risk of developing infections is greater.

If left untreated, Sjögren's syndrome can cause permanent vision impairment or increase the risk of developing non-Hodgkin's lymphoma, a cancer of the lymph glands.

In women with Sjögren's syndrome there is a likelihood of having children with a temporary rash (lupus) or, in rare cases, with cardiac abnormalities; for this reason every pregnancy must be carefully followed (monitored).

Sjögren's syndrome mainly affects people between the ages of 40 and 60 without distinction of race and ethnicity. The vast majority, around 90% of cases, are women. Rarely, it occurs in children.

It is believed that 1-4 million people worldwide are affected by Sjögren's syndrome.

Symptoms

The most common symptoms (symptoms) of Sjögren's syndrome are dry eyes, mouth or vagina. These are very common problems in old age, also present in non-sick people.

In Sjögren's syndrome, dry mouth can cause:

  • tooth decay and gum disease
  • dry cough
  • difficulty swallowing (swallow) and chew
  • hoarse voice
  • difficulty speaking
  • swollen salivary glands (located between the jaw and the ears)
  • fungal infections in the mouth (thrush) which can occur with a coated or white tongue

Dry eyes can cause:

  • burning or pain in the eyes
  • itch to the eyes
  • the feeling of having sand in your eyes
  • sore eyelids and swollen
  • sensitivity to light (photophobia)
  • tired eyes
  • mucus secretions from the eyes

These disorders (symptoms) can get worse:

  • in a windy or smoky environment
  • in an air-conditioned building
  • on air travel

Other disorders (symptoms) of Sjögren's syndrome

In severe cases, the immune system can attack other parts of the body, causing:

  • dry skin
  • tiredness and fatigue
  • muscular pain
  • joint pain, stiffness and swelling
  • inflammation of blood vessels (vasculitis)
  • difficulty concentrating

Causes

In Sjögren's syndrome, antibodies, which are part of the defense system (immune system) and normally attack agents external to the body (for example, bacteria and viruses), begin to attack the healthy cells and tissues of the body, damaging the glands that produce liquids (exocrine).

Furthermore, according to some evidence, the immune system would also damage the nerves that control the glands, thus further reducing their ability to secrete liquids.

Saliva and tears play a key role in protecting the mouth and eyes, respectively.

Usually, tears are only noticed when crying but a thin layer, known as tear film, always clothes the eyes.

Tears are made up of a mixture of water, protein, fat, mucus, infection-fighting cells. They perform several important functions:

  • lubricate the eye
  • keep the eye clean and dust-free
  • protect the eye from infections
  • help stabilize vision

Saliva plays several roles, including:

  • keep your mouth and throat moist
  • help digest food: enzymes contained in saliva are able to digest starches
  • act as a natural disinfectantSaliva contains antibodies, enzymes and proteins that protect against some common bacterial and fungal infections

In Sjögren's syndrome, the immune system can also damage other parts of the body such as, for example, muscles, joints, blood vessels, nerves and, less frequently, some organs.

Primary Sjögren's syndrome

Primary Sjögren's syndrome is believed to be triggered by a combination of genetic and environmental factors.

Some individuals have specific genes that make them more vulnerable to developing autoimmunity. Over the course of their life, an environmental factor (such as a viral infection) can activate the immune system, causing the autoimmune disease to develop.

Female sex hormones (estrogen) also appear to play a triggering role. In fact, the symptoms (symptoms) of Sjögren's syndrome become evident in women especially around the period of menopause, when estrogen levels begin to decrease.

Secondary Sjögren's syndrome

When Sjögren's syndrome is associated with other autoimmune diseases, such as rheumatoid arthritis or systemic lupus erythematosus, it is called secondary Sjögren's syndrome.

Diagnosis

Sjögren's syndrome can be difficult to ascertain (diagnose) because the disorders (symptoms) it causes are similar to those of other diseases. If you have discomfort such as, for example, dry eyes, mouth or vagina, it is advisable to consult your family doctor who, after having evaluated them, if he deems it necessary to ascertain (diagnose) the cause, may prescribe specialist visits with:

  • dentist, for dry mouth
  • ophthalmologist, for dry eyes
  • gynecologist, for vaginal dryness

Industry experts have formulated a questionnaire, which is useful in case you suspect you have Sjögren's syndrome. If most of the questions are answered yes, the likelihood of being sick is greater:

  • do you have dry eyes every day, annoying and persistent for more than three months?
  • do you have the feeling of sand in your eyes?
  • do you need to use eye drops containing tear substitutes more than three times a day?
  • Have you felt like your mouth has been dry every day for more than three months?
  • do you have salivary glands (located between the jaw and ears) always swollen?
  • do you drink fluids frequently to help you swallow food?

Additional tests used to ascertain (diagnose) Sjögren's syndrome include:

  • break up time and Schirmer test
  • lip biopsy
  • blood analysis
  • salivary flow velocity

Break up time and Schirmer test

The break up time test and Schirmer's test are performed by an ophthalmologist.

The Schirmer test serves to measure the amount of tears produced and, therefore, the functionality of the lacrimal glands. In Schirmer's test, small strips of blotting paper are placed in contact with the lower lid. After five minutes the strips are removed to evaluate the amount of tears the paper has collected.

The break-up time test measures the effectiveness of the lacrimal glands and the consequent alteration of the fluid that bathes the eye (tear film). To do this, a dye (non-toxic) is dropped onto the surface of the eye: the distribution of the vital dye allows the ophthalmologist to evaluate the tear film, the evaporation time of the tears as well as the damage caused to the eye by dryness.

This test is also carried out using a slit lamp which allows to evaluate the severity of dryness and the possible inflammation of the external part of the eye as well. The slit lamp is a low power microscope with a high intensity light source. The specialist uses the slit lamp to examine the tear glands more closely.

Lip biopsy

It consists in the microscopic analysis of a fragment of salivary gland, obtained by taking a small piece of tissue (biopsy) of the lower lip. A local anesthetic is injected into the inner surface of the lower lip to numb the area before making a small cut .

The diagnosis is based, above all, on the demonstration of an infiltration of inflammatory cells in the salivary glands: the presence of lymphocytes (a type of white blood cell) in the tissue may indicate Sjögren's syndrome.

Blood analysis

Blood tests are done to look for Sjögren's syndrome-specific autoantibodies, known as anti-Ro / SSA and anti-La / SSB. They are present in only about 60% of sick people, therefore, it is possible to get a negative result but still have the disease.

Salivary flow velocity

It is a test that measures the amount of saliva produced by the salivary glands. For its execution it is required to collect the saliva in a container for a period of five minutes. The quantity produced is weighed and measured. If unusually low, it can indicate the presence of Sjögren's syndrome.

In case of detection (diagnosis) of Sjögren's syndrome, the reference specialist is the rheumatologist, competent in systemic autoimmune diseases.

Therapy

There is no definitive cure for Sjögren's syndrome, but pharmaceutical products are available that can help relieve the ailments (symptoms). Since the disease can manifest itself differently, the treatment plan will be tailored to the needs of each patient.

Eye care

Cases of mild to moderate dry eyes can be successfully relieved with eye drops made from artificial tears (a liquid that mimics tears) available at pharmacies without a prescription.

If you use eye drops regularly, more than three times a day, it must not contain preservatives: it is proven, in fact, that excess exposure to preservatives can damage the surface of the eye.

In case of irritated eyes, the use of eye drops containing corticosteroids for short periods may be recommended. The lowest effective dose will be prescribed for the shortest possible time since the use of corticosteroids, for long periods, can cause serious undesirable effects (side effects).

It is also advisable to wear glasses to reduce the evaporation of the tear film. In particular, specially made glasses, called wet chamber glasses envelop the eyes and help retain moisture and protect them from irritants.

A widely used technique is the "tip occlusion, which should help keep the eye protected from natural tears. This is a mechanical treatment that blocks the drainage of tears to keep them on the surface of the eye for as long as possible. Occlusion can be achieved by "plugs" of various nature (e.g. in collagen), or, by cauterization or laser. The most common type of tip occlusion is occlusive “plugs”.

Mouth care

A number of techniques can be used to keep the mouth moist, including:

  • maintain good oral hygiene to prevent tooth decay and gum disease
  • increase fluid intake
  • use sugar-free chewing gum to stimulate saliva production
  • suck on ice cubes to help lubricate the mouth and reduce dryness
  • regularly use mouthwashes to soothe the mouth and protect it from infections
  • stop smoking: Smoking irritates the mouth and increases the rate at which saliva evaporates

There are a number of saliva replacement products on the market that can help lubricate the mouth but cannot replace saliva in preventing infections.

They are available in the form of a spray, gel, or chewing gum. Your doctor or pharmacist can suggest the most suitable product.

Medicines for Sjögren's syndrome

There pilocarpine It is a medicine often used to treat ailments (symptoms) such as dry eyes and mouth: it stimulates the glands to produce more tears and saliva, respectively. Undesirable effects (side effects) of pilocarpine include:

  • excessive sweating
  • nausea
  • diarrhea
  • stomach ache
  • abdominal pain (stomach ache)
  • need to urinate often

For some people, the side effects are mild, for others, they outweigh the benefits.

Pilocarpine is contraindicated in the case of asthma or chronic obstructive pulmonary disease (COPD) and during pregnancy or breastfeeding.

L"hydroxychloroquine, is believed to slow the immune system's attack on the tear and salivary glands. It can also help reduce associated ailments (symptoms) such as muscle pain and stiffness. It should be taken for several weeks before you notice any improvement and it may take six months before getting the full benefit of the treatment. Undesirable effects (side effects) are rare and generally mild:

  • nausea
  • rash
  • loss of appetite
  • stomach cramps
  • He retched

In very rare cases, hydroxychloroquine can damage the retina of the eye. It is therefore advisable to undergo an eye examination to check the retina before starting the treatment and, subsequently, to regular periodic visits (usually at least once a year).

Breastfeeding women should not take hydroxychloroquine.

Treatment of other disorders of Sjögren's syndrome:

  • dry skin, it can be treated with specially designed soaps or creams recommended by your family doctor or pharmacist
  • vaginal dryness, it can be reduced by using a lubricant. Some women also use estrogen-based creams or hormone replacement therapy (HRT)
  • muscle and joint pain, can be treated with non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen

NSAIDs may increase the risk of stomach ulcers and internal bleeding, particularly if taken for a long time.

NSAIDs are not recommended for pregnant, breastfeeding, or people with cardiovascular or kidney disease.

Complications

Sjögren's syndrome is usually not life-threatening, but the complications it can cause can be serious.

Non-Hodgkin's lymphoma

It is estimated that Sjögren's syndrome sufferers are 44 times more likely to develop non-Hodgkin's lymphoma than people who do not have the syndrome.

The most important early disorder (symptom) of non-Hodgkin's lymphoma is a persistent and painless enlargement of a lymph node (gland) in the neck, armpits or groin.

If there is an enlarged lymph nodes, therefore, it is advisable to contact the family doctor who, if he deems it necessary, may prescribe further investigations.

Other disorders (symptoms) such as, for example, fever, night sweats, constant fatigue, unexplained weight loss, itching or red spots on the skin are not certain signs of lymphoma, but it is advisable to carry out further investigations to discover their origin.

Eye damage

If dry eyes are left untreated, inflammation and ulcers on the surface of the eyes (corneal ulcers) can develop which, if left untreated, could cause permanent damage or loss of vision.

Pregnancy

Women with Sjögren's syndrome who wish to become pregnant should contact their family doctor to check for the presence or absence of certain antibodies (anti-SSA / SSB) involved in Sjögren's syndrome. These antibodies can cause a temporary rash (lupus) in newborns and, in very rare cases, heart block in the fetus or heart defects at birth. The baby, therefore, may need specialized care both during pregnancy and after birth.

Other associated diseases and conditions

Some conditions and diseases have been linked to Sjögren's syndrome. They include:

  • peripheral neuropathy, a condition that often leads to loss of sensation in the hands and feet
  • Raynaud's phenomenon, restricted blood flow to the hands
  • kidney problems, such as inflammation or kidney stones
  • hypothyroidism, cause of fatigue and weight gain
  • irritable bowel syndrome, cause of abdominal pain and irregular bowel functioning
  • dental caries

Living with

Simple tips to help prevent many of the problems associated with Sjögren's syndrome include:

  • carry out a dental check-up every six months
  • practice good dental hygiene using regular brushing, flossing and mouthwash
  • avoid eating too many sweet foods as Sjögren's syndrome increases the risk of dental caries developing
  • do not use strong, fragrant soaps and prefer, instead, special creams and soaps
  • avoid dry environments, such as air-conditioned offices
  • don't drink too much alcohol
  • increase fluid consumption, especially water to reduce dry mouth
  • stimulate saliva production with sugar-free chewing gum or hard candy. Lemon juice in water can also help stimulate saliva production
  • avoid hot water for the bath or shower, if the skin is dry; do not rub the skin with the towel and apply a moisturizer while it is still wet
  • use rubber gloves for washing dishes or cleaning

Bibliography

NHS. Sjögren "s syndrome (English)

Mayo Clinic. Sjögren "s syndrome (English)

In-depth link

American College of Rheumatology. Sjögren "s Syndrome (English)

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