Sindrome di Sjögren

Che cos'è la sindrome di Sjögren?

Sjögren's syndrome is a condition that causes the tear and saliva glands to stop working. It causes dry eyes and a dry mouth. It can also affect other organs including the lungs, kidneys, skin and the nervous system.

Sjögren's syndrome is an autoimmune disease. It is caused by the body's immune system attacking the glands that make tears and saliva, meaning that they stop working.

In Sjögren's syndrome, these autoantibodies attack the cells of certain glands. The effect is that these glands cannot release their normal secretions. This means that the symptoms of Sjögren's syndrome are mainly due to dryness and lack of gland secretions.

Like many autoimmune conditions, we don't know exactly why some people develop Sjögren's syndrome. Some people seem more likely to get it because of their genes, but it can affect anyone.

Sjögren's syndrome is sometimes divided into primary Sjögren's syndrome and secondary Sjögren's syndrome.

Primary Sjögren's syndrome

This describes Sjögren's syndrome which occurs by itself. About one in a thousand people have this.

Secondary Sjögren's syndrome

This describes Sjögren's syndrome which occurs alongside another autoimmune disease such as artrite reumatoide oppure lupus eritematoso sistemico. This is sometimes called Sjögren-overlap syndrome.

Primary Sjögren's syndrome affects around 1 to 5 out of every 10,000 people. Secondary Sjögren's syndrome affects people with another autoimmune disease; for example, between 1 in 10 and 1 in 4 people with rheumatoid arthritis also have secondary Sjögren's syndrome.

Most people who have the disease are women. People usually first start noticing symptoms when they are in their 20s or 40s, but many people have symptoms for a number of years before the diagnosis of Sjögren's syndrome is made.

The two symptoms that everyone with Sjögren's syndrome will notice are:

• Bocca secca (xerostomia).

• Occhi secchi (xerophthalmia). Eyes may feel gritty and uncomfortable.

The dryness of the eyes, mouth and other body parts is known as sicca syndrome. As well as Sjögren's syndrome, sicca syndrome can also be caused by radiotherapy treatment and other diseases such as sarcoidosi e emocromatosi.

Bocca secca

This can lead to:

• Swallowing problems and the feeling of something getting stuck in the throat on swallowing (dysphagia).

• Thrush (a yeast infection) in the mouth.

• Loss of taste.

• Tooth decay and malattia gengivale (gingivitis). Saliva contains anti-infective agents, so when saliva production is reduced, infection in the mouth is more likely.

Dryness in other body parts

• Secchezza vaginale can cause discomfort when having sex (dyspareunia).

• Dryness of the upper airways (trachea and bronchus) can lead to a dry cough and chest infections.

• Dry skin can occur.

Altri sintomi

You may notice the following occurring:

• Stanchezza.

• Muscle aches and aching joints. Sjögren's syndrome often affects people who have other autoimmune diseases that affect the joints, such as rheumatoid arthritis and systemic lupus erythematosus.

• Swelling of the salivary glands, including the parotid glands (located in both cheek areas, just in front of the ears) and those under the jaw and in the neck area.

Some people who have Sjögren's syndrome develop complications such as:

• Infection of the salivary glands.

• Ulcere corneali: dry eyes can lead to infection and the development of ulcers on the surface of the eyes. If not treated, this can lead to loss of vision.

• Pancreatite: this is inflammation of the pancreas gland, characterised by severe pain in the upper part of the stomach (abdomen).

• Neuropatia periferica: this causes loss of sensation in fingers, hands, arms, toes, feet, and legs.

• Cranial neuropathy: this causes loss of sensation in parts of the face.

• Problemi renali: Sjögren's syndrome can progress to affect the kidneys. It can cause kidney inflammation, disruption in body fluid balance, calcoli renali and, if untreated, loss of kidney function.

• Linfoma non-Hodgkin:

  • People with Sjögren's syndrome are more likely to develop non-Hodgkin's lymphoma, a type of blood cancer.

  • The most common symptom of this is painless swelling of lymph nodes.

  • If you are diagnosed with Sjögren's syndrome, you should watch for the development of any lumps or swellings in your neck, groin or under your arms and report anything abnormal to your doctor.

• Parotid gland tumours: these may be more common in Sjögren's syndrome. If you notice a hard/firm swelling in either of your parotid glands (located in the cheek area as described above), you should seek an urgent appointment with your doctor.

• Aborto ricorrente: Sjögren's syndrome can cause recurrent miscarriage. Recurrent miscarriage is the term used when a woman has three or more miscarriages in a row. This is because of a link between Sjögren's syndrome and a condition called sindrome da anticorpi antifosfolipidi.

• Medication reactions: people with Sjögren's syndrome may be more prone to developing side-effects when they take certain medicines - for example, antibiotic medicines.

• fenomeno di Raynaud: the extremities of the body, usually the fingers and toes, change colour and may become painful, usually due to exposure to the cold.

There are a number of investigations that your doctor may do to help diagnose Sjögren's syndrome.

• The Schirmer tear test: this measures the amount of tears that you form. A piece of filter paper is placed under the lower lid of your eye and left for five minutes. In Sjögren's syndrome, tear production is reduced.

• Esame con lampada a fessura: a dye is used to stain the eye temporarily. The doctor will look into your eyes, using a special lamp, to see if there is reduced tear production or eye damage.

• Salivary gland biopsy: one of the tiny salivary glands may be removed from your lower lip for examination. This is a simple procedure and can be done using an injection of local anaesthetic. Special dyes and staining are then used in the laboratory to look for signs of Sjögren's syndrome in this gland.

• Saliva collection: you may be asked to collect the saliva that you make over 10 minutes. A reduced volume suggests Sjögren's syndrome.

• Esami del sangue: your doctor may also want you to have some blood tests that may suggest inflammation or signs of an autoimmune disease.

Some other tests may be arranged if the diagnosis is not clear or your doctor suspects a complication. For example:

• Un ecografia to look at your internal organs and lymph glands.

• Tests on your urine to look at your la funzione renale.

There is currently no cure for Sjögren's syndrome. Treatment is aimed at controlling and relieving symptoms such as the reduction in tears and saliva.

You may be referred to a number of different specialists when you are diagnosed with Sjögren's syndrome, depending on the parts of your body that it affects.

Joint specialists (rheumatologists) are the main doctors who have specialist knowledge of Sjögren's syndrome. This is because of the association of Sjögren's syndrome with other diseases that affect the joints, such as rheumatoid arthritis and systemic lupus erythematosus.

However, you may also be referred to a dentist, an eye specialist, a lung specialist or a kidney specialist. Your GP will continue to provide support for you and will usually prescribe your medication under the guidance of these other specialists.

Treatment of dry eyes

• Avoid situations that make dry eye symptoms worse. These may include:
  

  • Low humidity and air conditioning.

  • Wind.

  • Dust and smoke (so, if appropriate, try to stop smoking if you are a smoker).

  • Contact lenses.

  • Prolonged reading or staring at a computer screen or television. This makes us blink less often so our eyes don't stay as moist.

• Glasses: special glasses can help to keep in moisture and reduce eye dryness.

• Artificial tears: these provide lubrication and come in the form of eye drops. Preservative-free eye drops such as ipromellosa help to reduce the risk of developing eye irritation. Paraffin eye ointments are helpful for use at night as they are longer-lasting. However, if used during the day, paraffin ointment may cause blurred vision.

• Tear duct treatment: if drops alone do not work, you may need some special treatment to the tear ducts in your eyes by an eye specialist. An procedure called diathermy is used to close up the tear ducts.

Treatment of dry mouth and related symptoms

• Misure generali that can help include:
  

  • Sipping water throughout the day.

  • Keeping your teeth, gums and mouth as clean and healthy as possible. Brush your teeth regularly, use dental floss and a mouth wash.

  • Visiting your dentist regularly.

  • Using Vaseline® for dry, cracked lips.

  • Chewing sugar-free chewing gum.

• Saliva substitutes: artificial saliva can be used to keep the mouth moist and comes in the form of a spray, gel, liquid, lozenge or pastille.

• Saliva stimulants: in some people with Sjögren's syndrome, the saliva glands are only partially affected and can be stimulated to make more saliva. Pilocarpine tablets are sometimes prescribed, which stimulate saliva production.

Treatment of other symptoms

• Moisturisers and special bath additives can be used for dry skin.

• Lubricants may be needed when you have sex.

• Farmaci antinfiammatori non steroidei (FANS) can be taken to help joint and muscle pains.

Treatment of complications

If Sjögren's syndrome progresses to involve organs such as the skin, lungs, kidneys and lymph glands, you may need to take some other medication. Such medication may include:

• Steroidi: these are tablets taken by mouth that help to reduce inflammation. They may be prescribed if your symptoms are particularly bad.

• Immunosuppressive agents: these are medicines that damp down the abnormal antibody production in Sjögren's syndrome. Names include metotrexato, penicillamina e idrossiclorochina. As with steroids, they are reserved for severe cases as they do have side-effects and you will need close monitoring with blood tests while you are taking them. You may be prescribed one of these medicines if your kidneys or lungs are affected, or if you develop pseudolymphoma.

• A medicine called rituximab may be used if there is a poor response to treatment with steroids and other immunosuppressive agents.

Sjögren's syndrome is not usually life-threatening. Some people may only notice mild symptoms such as mildly dry eyes and a mildly dry mouth. Other people develop more irritating and disabling symptoms affecting their eyes, mouth, vision and eating and can also have uncomfortable joint pains and tiredness.

Sometimes symptoms can go away for long periods of time (go into remission). Some people develop more serious problems such as the kidney and lung problems described above.

Some people with Sjögren's syndrome develop another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus or polymyositis.

As mentioned above, a small number of people with Sjögren's syndrome develop lymphoma, most commonly non-Hodgkin's lymphoma. This lymphoma can usually be treated and your doctor will review you regularly to look for signs of this.

Can you live a long life with Sjögren's syndrome?

Yes, most people with Sjögren’s syndrome can live a normal lifespan. While the condition can cause ongoing discomfort and complications, symptoms are usually manageable with treatment and regular monitoring.

What are three signs of Sjögren's syndrome?

The three most common signs of Sjögren’s syndrome are dry eyes, a dry mouth, and fatigue. Some people may also experience joint pain, dry skin, or swelling of the glands around the face and neck.

Is Sjögren's syndrome serious?

Sjögren’s syndrome can range from mild to serious. While many people only experience dryness and fatigue, in some cases it can affect organs like the lungs, kidneys, or nerves, requiring ongoing medical care.

Is Sjögren's syndrome genetic?

Sjögren’s syndrome isn’t directly inherited, but genetics can play a role. Having a family member with an autoimmune condition may increase your risk, especially when combined with environmental triggers or hormonal factors.