Sjögren’s syndrome

What is Sjögren’s syndrome?

Sjögren's syndrome is a chronic systemic autoimmune disease, in which moisture-producing glands become inflamed.

White blood cells penetrate the moisture-producing glands, such as the salivary glands in the mouth and the lachrymal glands in the eyes.
The white blood cells damage the glands, causing them to produce less glandular fluid, and the fluid they do produce is of a lower quality.

This causes dryness, such as dry eyes and a dry mouth. These are called sicca complaints. Sicca is the Latin term for dry.

Fatigue, dry skin, inflammation and muscle and joint pain are other common Sjögren’s syndrome complaints.
The muscle and joint complaints can vary from mild to severe.

Other glands and internal organs, such as the kidneys and the liver, can also become inflamed.

Primary and secondary form

Sjögren's syndrome is divided into primary and secondary forms. Those who have the primary form of Sjögren's syndrome, have no other disease. Those who have the secondary form, have another systemic disease as well, possibly Rheumatoid Arthritis (RA) or Systemic Lupus Erythematosus (SLE).

People with Sjögren's syndrome are slightly more at risk of developing non-Hodgkin's lymphoma, which is a malignant disease of the lymph nodes (cancer).

How common is Sjögren's syndrome?

It is not entirely clear how many people suffer from Sjögren's syndrome; however, it is estimated to be the second most common rheumatic autoimmune disorder, behind rheumatoid arthritis. The disease can develop at any age, but is most commonly diagnosed in people around middle age, so between 45 and 60 years old. Sjögren's syndrome is rare in children and adolescents. Why that is, is not exactly known (yet).

Symptoms related to Sjögren’s syndrome

The most common symptoms are:

Dry eyes
Because the lachrymal glands are inflamed, they produce less or poorer quality tear fluid, causing dry, itchy and sandy eyes.
 

Dry mouth
Sjögren's syndrome can cause a reduction in saliva production or the production of poorer quality saliva, due to the inflamed salivary glands. As a result, the mouth and throat are usually dry and chewing and swallowing can be difficult.
 

Dry vagina and skin
The inflammation can also cause the glands in the skin and the vagina to function poorly, causing dryness. The skin can become dry, scaly and itchy. Vaginal dryness is more likely to cause fungal infections and can cause pain and irritation. Women can therefore experience pain during sex.
 

Internal organs
Sjögren's syndrome can affect internal organs, namely: 
 

Oesophagus and stomach: A dry oesophagus can cause heartburn or difficulty swallowing solid food.

Liver: Liver complaints are quite rare. They usually do not cause any noticeable complaints at first. Very occasionally, the skin or the whites of the eyes may turn yellow, and upper abdominal pain can occur as well as intense fatigue.

Kidneys: Kidneys usually do not cause noticeable complaints at first, but high blood pressure and fluid retention can occur at a later point in time. Only if the kidney function deteriorates sharply, you will be able to notice that you urinate less.

Kidney stones: These can occur more often causing pain near the kidneys.

Lungs: You may experience a gruelling, dry tickly cough and sometimes shortness of breath. The lungs can become inflamed.

Heart: The protective membrane enclosing the heart (pericardium) can become inflamed.

Bladder: A dry vagina and urinary tract can make you more susceptible to bacterial bladder infections.

Blood vessels: Sometimes the blood vessels become inflamed, which usually causes an itchy rash, often on the lower legs.
 

Muscular and joint complaints
About 80% of people with Sjögren's disease complain of painful and/or inflamed joints. Two types of joint complaints are associated with Sjögren’s syndrome:
 

Arthralgia, which only causes painful joints

Arthritis, which causes painful as well as inflamed joints

Fatigue
Profound fatigue occurs in about 95% of people with Sjögren's syndrome. Profound fatigue can be very debilitating. It can be so severe that it becomes impossible to lead a normal life and function at work, ultimately resulting in full disablement. The fatigue can suddenly worsen. Chronic inflammation may be the underlying cause of this severe fatigue. Anaemia can exacerbate the fatigue.
 

Flu-like feeling
Many people with Sjögren's syndrome often feel as if they have the flu. An increase in body temperature, fever, accompanies the feeling.
 

Damaged nerves
Sjögren's syndrome can also affect the nerves. If the narrow nerve fibres are damaged, you can experience complaints such as intense shooting, burning and stabbing pain, numbness, tingling and itching. This is called Small Fibre Neuropathy. Autonomous functions can be affected as well, in which case you may suffer from palpitations, changes in sweat patterns, a decreased libido, intestine/bladder/stomach problems or fluctuating blood pressure.