What is Sjögren's syndrome (sicca, dry)? Symptoms, Treatment, Diet

Sjogren or Sjögren's syndrome is a chronic autoimmune rheumatic disease in which the external secretory glands are affected. These are mainly the lacrimal (tears) and salivary (saliva) glands.

Hence the typical symptoms, for which the disease is also referred to as dry syndrome.

Sjögren syndrome = SjS = Sicca syndrome / complex - sicca = dry ⇒ dry syndrome. 

It is a multisystem disease that results in the malfunction of the exocrine glands, which is associated with other problems manifested in different parts of the human body.

Multisystem means that it affects multiple organ systems. 
Exocrine glands are glands with external secretion. 
External secretion = secretion is secreted through an outlet onto the surface of the epithelium, into a hollow organ or onto the surface of the body.

It mostly affects women.

In addition, this disease often runs together with other rheumatic and autoimmune diseases.

Accordingly, it is divided into primary and secondary forms.

The disease itself is characterized by a diverse course, which is highly individual. New data are emerging in the study of the syndrome and it is branching out into multiple subtypes, which helps in targeting treatment.

The disease is long-standing, i.e. chronic, is classified as a rheumatic disease and its exact cause is unknown, and therefore a complete cure is not possible.

Treatment aims to prevent progression and alleviate the discomfort, which often reduces the quality of life and, in the presence of another syndrome with a worse course, disables the affected person.

From the history of the disease we know that it is named after the Swedish ophthalmologist Henrik Samuel Conrad Sjögren.

Further, other scientists, physicians have also dealt with it and refined the information about its manifestations. They found that it affects several areas of the body. Subsequently, they reclassified it as a systemic autoimmune disease.

Want to know more about Sjogren's syndrome?
What are the anticipated risks of occurrence?
How does it manifest?
And what is its available treatment?
Read on.

About Sjögren's syndrome

Sjogren's syndrome is a chronic systemic autoimmune inflammatory connective tissue disease with a rheumatic basis that affects the endocrine glands.

Autoimmune = immunity pathologically attacks the body's own cells.

The glands are affected by a chronic inflammatory process. It results in reduced function and secretion of, for example, tears, saliva and other secretions according to the damage to the gland in question. The damage may even reach levels of complete dysfunction.

It predominantly affects women, 9 to 13 times more often than men.
This is between 0.08% and 3% of the female population, and approximately 1.2% of the total population.  

It is reported that there is usually a more pronounced onset of the first symptoms in postmenopausal women. Hormonal changes are therefore also considered to be a predisposing factor.

However, it is a chronic disease.
It was therefore concluded that the onset of the flare-up was already around the age of 20.
Occurrence in childhood is also described.
It has an age range of 15 - 65 years.

It mainly affects the lacrimal and salivary glands. Subsequently, a disorder of the glands of the gastrointestinal tract at the level of the stomach or pancreas is also described.

It also affects the secretory glands in the respiratory tract. It is thus behind the development of frequent nosebleeds - epistaxis or inflammation of the nose and nasopharynx (rhinopharyngitis), due to dry mucous membranes of the nose. 

It is also associated with recurrent bronchitis (chronic bronchitis). The secretion of the respiratory mucosa has a defensive and cleansing function and its deficiency due to reduced production is the basis of the problem = frequent morbidity.

An example is the problem of dry skin and reduced sweating or sebum production. Dry vagina may appear as an issue occurring among women. 

From the above information, it has a variety of manifestations that occur in different combinations.

Sjogren's syndrome can run its own course,
in which case it is referred to as primary Sjogren's syndrome
or Sjögren disease. 
The manifestation is only hypofunction or affunction of the exocrine glands.

Hypofunction = reduced function, 
afunction = total malfunction. 

The second form is secondary Sjogren's syndrome.

The secondary form often occurs with other rheumatic diseases, such as:

• rheumatoid arthritis
• systemic lupus erythematosus
• scleroderma
• Raynaud syndrome
• but also polymyositis, primary biliary cirrhosis or chronic active hepatitis

SjS is reported to be the second most common autoimmune disease.

In general, it is known that autoimmune diseases often run in combination as systemic connective tissue diseases. It is reported that about 10% of people with SjS also have another systemic connective tissue disease.

Mixed connective tissue disease - Sharp's syndrome - mixed connective tissue disease - MCTD - syndrome with symptoms of SLE - systemic lupus, SSc - systemic scleroderma and PM - polymyositis.

What causes Sjogren's syndrome?
We do not know.

The basic component is an immunity disorder, i.e. autoimmunity. This is a condition where the defence system attacks the body's own cells, more specifically connective tissue. A multifactorial influence is also reported.

Risk factors for SjS include:

• genetic predisposition
• presence of HLA antigens
• being female
• hormonal influence and female hormones
• external influences
  • viral disease, such as infection with Epstein-Barr virus, cytomegalovirus or retrovirus

The inflammatory process attacks the body's own cells, i.e. connective tissue cells, more specifically the exocrine glands.

During this process, several morbid changes occur in the structure of the glands and their ducts. Infiltration with an inflammatory infiltrate, the components of which are components of immunity, antibodies.

Glands and their ducts initially expand, gradually atrophy, narrow. There is also remodelling into non-valuable tissue - connective tissue, which is afunctional = does not fulfil its function.

The risk is also the reversal of the process to malignant growth = tumour growth.

As mentioned, SjS often occurs together with other autoimmune diseases. Their common feature is that they are long term and progressive.

The progressive course is due to worsening of the discomfort progressing over time. Therefore, in this group of diseases, early detection is important, followed by early treatment, which seeks to reduce the impact of the autoimmune disease processes and symptoms.

Symptoms in Sjogren's syndrome are divided into two groups. These are difficulties that are associated with impaired glandular function = glandular manifestations.

Gland = Lat. glandula.

There are also signs and symptoms outside of the gland - extraglandular symptoms.

Extra = outside.

Damage to the lacrimal and salivary glands is typical. Plus, damage to other glands such as the mucus glands of the respiratory tract, digestive system, stomach, pancreas, small intestine or female reproductive system, skin and sweat glands, and also the thyroid gland is a possibility.

Extraglandular symptoms:

• weakness
• fatigue
• reduction in performance
• concentration and memory disorders
• headaches
• increased body temperature
• joint pain - arthalgia
• joint inflammation - arthritis
• swelling and stiffness of the joints
• muscle pain - myalgia
• peripheral neuropathy - tingling, paresthesias and other sensory disturbances
• Raynaud syndrome - up to 80 % of cases
  • discolouration of the extremities of the body, mostly fingers and toes, pale to purple colour
  • underbleeding is provoked by cold, stress, emotional stress
• loss of appetite
• weight loss
• depression and anxiety
• food intolerance
• irritable bowel syndrome and other digestive problems such as bloating
• drug intolerance
• liver dysfunction
• lung involvement and the presence of shortness of breath, pneumonia
• deterioration of kidney function - approximately 15-25% of cases, examples include diabetes insipidus and renal failure
• purpura - reddening of the skin especially in the lower limbs, shins
• damage to blood vessels, vasculitis - inflammation of blood vessels
• lymphadenopathy, enlargement of lymph nodes
• risk of malignant relapse (pseudolymphoma, lymphoma and others)

Table: symptoms of glandular involvement

Diagnosis of the disease is difficult because of the wide variety of symptoms.

It is not always thought of as an autoimmune disease. Initially, there may be general discomforts such as fatigue, weakness and decreased performance. And this can delay the detection of the disease for several months.

What is important is the medical history, that is, what the person describes. Also, clinical and laboratory examination is complementary. Examination of tears, tear film by an ophthalmologist and evaluation of saliva production.

There are several ways of assessing SjS, one of which is classification criteria. 

American-European Consensus Criteria:

1. ocular symptoms
   • Symptoms of dry eyes for at least 3 months
   • A foreign body sensation in the eyes
   • Use of artificial tears 3 or more times per day
   • at least one affirmative answer
2. oral symptoms
   • Symptoms of dry mouth for at least 3 months
   • Recurrent or persistently swollen salivary glands
   • Need for liquids to swallow dry foods
   • at least one affirmative answer
3. ocular signs - objective
   • Abnormal Schirmer’s test
   • Positive vital dye staining of the eye surface
   • dry eye score
   • at least one affirmative answer
4. histopathology of the salivary gland
   • signs of sialoadenitis - inflammation of the salivary gland
5. salivary gland involvement - objective
   • unstimulated salivary secretion
   • sialography with finding of inflammation
   • salivary scintigraphy
   • at least one affirmative answer
6. Autoantibodies
   1. the presence of antibodies in the serum of Ro - SSA or La - SSB

For primary SjS, the presence of 4 of the 6 criteria is required if 4 or 6 are positive, or the presence of 3 of the 4 objective criteria (3,4,5 and 6).

In secondary SjS, the presence of 2 of 1 or 2 criteria, plus any 2 of 3, 4, and 5 criteria, is confirmed.

Exclusion criteria:

• past head and neck radiation treatment
• hepatitis C
• AIDS
• lymphoma
• sarcoidosis
• Graft versus host disease - post-transplant complication
• current use of anticholinergic drugs

Laboratory tests: sedimentation FW, CRP, rheumatoid factor, ANA, HLA antibodies, and others

The course of the disease can be typical when it is characterized by the appearance of difficulties from the involvement of the glands. The other side consists of general systemic problems.

In SjS, the symptoms are varied and individual, even in combinations of multiple symptoms.

Primarily it is the lacrimal and salivary glands. Their dysfunction is manifested by dry eye and dry mouth as well as other symptoms as described in the table.

An exception is not the association of other complaints, namely extra-pruritic and general. These worsen a person's state of health and psychological condition. 

The disease is long-term, so its course is chronic, prolonged and develops over several years. With the onset in youth and the onset of more severe difficulties in later life.

From this point of view, early recognition of the manifestations and early treatment are also very important.