Sjogren syndrome: symptoms, treatment, causes,diagnostics

Update: December 2018

Sjogren’s syndrome, or “dry syndrome” is manifested by a decrease
function of the glands of the external secretion, which is why there is a pronounced
dry skin, oral mucosa, eyes,
nasopharynx, trachea, vagina, as well as reducing the release
digestive enzymes by pancreatic cells, etc.

He often accompanies a number of autoimmune diseases of the connective
tissue – scleroderma, dermatomyositis, etc. – and is called
secondary sjögren’s syndrome. In the case of an independent disease
it is called the primary syndrome or Sjogren’s disease.

Epidemiological studies that were conducted in
recent years, made it possible to identify the presence of this pathology in
0.59 – 0.77% of the total population, among people over 50 years old – in 2.7%.
The incidence among women is 10 to 25 times higher than among
men.

Causes and mechanism of the disease

The most characteristic of the disease, Sjogren’s syndrome are
lesions of the salivary and lacrimal glands with a decrease in their function. how
the consequence is dryness and inflammation of the mucous membrane of the cavity
mouth (xerostomia), conjunctiva, keratitis (corneal inflammation
eye), keratoconjunctivitis.

The causes of Sjogren syndrome are not fully established. Most
accepted is the theory of the pathological immune response of the body.
It arises in response to damage to the cells of the glands by the retrovirus, in
particular human immunodeficiency virus cytomegalovirus VI
herpes virus, Epstein-Barr virus. Despite the similarities
immunological disorders and changes in the body with the defeat
direct evidence of the viral cause of the disease
received

The viruses themselves, as well as the glandular epithelial cells that they have changed
the immune system perceives as alien elements (antigens).
It produces antibodies on them that attack and gradually
destroy glandular tissue. The disease is often found as
marital, especially among the twins, which gives rise to the assumption
about the presence of genetic predisposition.

Thus, it is assumed that in the mechanism of occurrence and
The development of the disease is a combination of many factors:
Синдром Шегрена симптомы

  • Genetic.
  • Viral (possible).
  • Immune control by T-lymphocytes.
  • Immune regulation with the participation of sex hormones, what
    evidenced by a rare incidence in childhood (up to 20
    years old); and among children in 80% of the girls are sick.
  • The stress response of the body resulting from
    immune response.

Symptoms of Sjogren syndrome

All symptoms of Sjogren’s disease are combined into 2 groups:

  • Glandular – defeat epithelial secreting glands,
    accompanied by a decrease in their function.
  • Vnelezististye, or systemic – a variety of symptoms,
    testifying to the defeat of various systems of the body and not
    that are specific to the disease.

Permanent and mandatory symptom for diagnosis
Sjogren’s syndrome is the defeat of the lacrimal and salivary glands.
In the early stages of Sjogren’s disease, dry mucous membranes are felt.
patient, as a rule, only with excitement or physical
load, as the disease progresses, feeling of dryness
becomes permanent, forcing the patient to drink food, often
moisten the mouth and use a drop of artificial tear for
moisturizing the eyes.

Glandular symptoms

Symptoms of keratoconjunctivitis – a feeling of scratching, sand and burning
in the eyes, itchy skin in the eyelids and their redness, intermittent
accumulation in the corners of the eyes detachable white. Arise
decrease in visual acuity, narrowing of the palpebral fissure, pinpoints
hemorrhages and pinpoint infiltrates (edema) of the conjunctiva, increasing
sensitivity to bright light (photophobia) – unpleasant
sensations, lacrimation, pain and pain in the eyes (for more information,
dry eye: treatment, symptoms).

The result of deep dryness of the cornea is its clouding and
the formation of corneal trophic (malnutrition) ulcers.
Присоединение стафилококковой infections вызывает гнойный
conjunctivitis, suppuration of corneal ulcers followed by formidable
a complication in the form of its perforation (perforation). Occasionally
an increase in the lacrimal glands themselves.

Chronic parenchymal parotitis

It is the second permanent and mandatory symptom of the syndrome.
Sjogren and is characterized by a widespread salivary tissue damage.
glands. It is often preceded by stomatitis, redness and
dryness in the area of ​​the rim and the corners of the lips “zadyy”, caries of many teeth,
enlarged submandibular and cervical lymph nodes.

Further dryness of the oral mucosa and an increase
parotid salivary glands, much less often – palatine, sublingual and
подчелюстных glands. Dryness in the oral cavity occurs first with
slight psycho-emotional or physical exertion, but
afterwards it becomes permanent and leads to the need
moisturizing in the mouth while talking and eating, especially when
use dry foods.

The disease in 50% of patients proceeds with exacerbations, during
which the parotid glands enlarge, leading to a change
face contours (“Muzzle hamster”). Glands slightly painful
when palpating or painless at all. During remission, they
decrease, but after one of the next exacerbations remain
enlarged. In approximately 30% of patients, an increase in the glands does not occur.
only in the period of relapses, but gradually and constantly.

The mucous membrane of the tongue and mouth becomes dry and
red (lacquered), easily injured and sometimes bleed, and
saliva – scanty foamy or viscous.

In the absence of treatment in the future:

  • the symptoms described become more pronounced
  • nipples of tongue flatten and atrophy, appear on it
    folds, occurs glossitis (inflammation of the root of the tongue), because of what
    swallowing is very difficult
  • on the mucous membrane of the cheeks appear areas of keratinized epithelium, and
    saliva is completely absent
  • bacterial, fungal or viral attachment is possible
    infections
  • there are cracks and crusts on the lips
  • partially or completely loose teeth

On the listed group of symptoms based diagnosis
Sjogren’s syndrome, the rest of the symptoms may accompany
disease in certain variants, but are not decisive in
making a diagnosis.

Related signs of gland damage
  • Dryness of the upper respiratory tract mucosa

This leads to hoarseness, chronic rhinitis and
inflammation of the mucous membranes of the paranasal sinuses, otitis media and decrease
hearing Atrophy, dryness, redness and swelling of the mucous membrane
vaginas are the cause of chronic colpitis, followed by
burning, pain, itching, decreased sexual desire.

  • Frequent symptoms are dry skin, a decrease in
    sweating.

In 30% of patients there is a defeat of the sweat apocrine glands,
located in the armpits, lower parts of the anterior
abdominal wall, in the pubis and external genitalia. it
promotes skin flaking, pigmentation in these areas and
formation of abscesses and phlegmon when attaching a secondary
infections.

  • Symptoms of diseases of the digestive system (80%)

it такие заболевания, как хронический эзофагит, гастрит,
chronic cholecystopancreatitis (see pancreatitis: symptoms, treatment,
what can you eat with pancreatitis). They manifest belching, pain for
sternum during the passage of food through the esophagus, pain in the epigastric
area and right hypochondrium, nausea and sometimes vomiting. By reducing
secretion of digestive enzymes by pancreatic cells
there are intolerances of fat and dairy products, and
clinical picture of intestinal dysfunction.

Vegetal symptoms

These include bone pain without radiographic changes in
of them. 60% of patients have stiffness, pain and limitation.
movements in small joints mainly in the morning, less often – in
large joints. 5 – 10% have pain and a slight
weakness in the muscles; sometimes severe polymyositis can develop
(common inflammation of muscle groups).

In 50% of cases of Sjogren’s syndrome, tracheobronchitis occurs,
accompanied by coughing and shortness of breath, while radiographic
In 65% of patients with a survey, chronic interstitial
pneumonia or fibrosis of the lungs, much less – exudative
(effusion) and dry pleurisy (see pleurisy: symptoms, treatment).

Most patients show an increase in occipital,
submandibular, cervical and supraclavicular lymph nodes, in 30 – 35%
lymphadenopathy (swollen lymph nodes) is generalized
character. In this case, there is often an increase in the spleen and
the liver.

The same percentage suffers from vasculitis (inflammation of the internal
shells of arteries), which proceed according to the type of erased forms of the syndrome
Raynaud or obliterating atherosclerosis of the lower extremities.
The defeat of small-caliber vessels is manifested in the form of various
small-point or spotted skin rashes, accompanied by
itching, burning and fever, ulceration and
necrotic (dead) skin areas.

Common lesion of the peripheral nervous system
(polyneuropathy) is clinically manifested by disorder or loss
skin sensitivity on the hands and feet of the type of “gloves” and
�”Socks”, neuritis of the facial nerve, trigeminal
nerve damage to the vessels of the membranes of the brain and spinal cord.

Also, the survey allows to establish signs of damage
thyroid gland with a decrease in its function (approximately 10%),
tendency to allergic reactions to many foods,
drugs and household chemicals.

Diagnostics

The diagnosis of Sjogren’s disease is established on the basis of
two main diagnostic criteria – keratoconjunctivitis and
parenchymal parotiditis, but only after the exclusion of the third
criteria – systemic autoimmune diseases (systemic red
lupus, rheumatoid polyarthritis, etc.). In the presence of the third
The criterion is diagnosed as Sjogren syndrome.

Laboratory research methods are optional. They
allow for differential diagnostics and in some
degree to judge the degree of activity of the process:

  • high ESR
  • anemia, decrease blood leukocyte count and
    platelet count
  • increase in blood levels of immunoglobulins A, G and M
  • presence of rheumatoid factor
  • the presence of antibodies to the components of the cell nuclei determined
    immunofluorescent method.

The Schirmer test is also used to determine the level
reducing the discharge of tears after stimulation with ammonia,
Special ophthalmic staining of the cornea and conjunctiva
dyes followed by biomicroscopic examination
epithelium.

To study the function of the salivary glands are used:

  • unstimulated and stimulated sialometry –
    the amount of saliva released per unit of time
  • sialography – performed by x-ray after
    the introduction of a contrast agent in the ducts of the salivary glands; technique
    allows you to detect more than 1 mm in the gland cavity
  • microscopic examination of salivary gland biopsy

Sjogren’s Syndrome Treatment

Sjogren’s Syndrome Treatment проводится в зависимости от стадии
diseases and the presence of systemic manifestations.

In order to stimulate the function of the glands is carried out:

  • drip
  • subcutaneous administration of galantamine
  • as a symptomatic treatment prescribed “artificial
    tears “(drops in the eyes) – low viscosity – Lacrisifi
    (200-250 rubles), Natural Tear (250 rubles), medium viscosity
    Lakrisin, high viscosity Oftagel 180 rub, Vidisik 200 rub,
    Lakropos 150 rub.
  • vitamin therapy courses are offered with a strengthening purpose

In the initial stages in the absence of damage to other systems and
unexpressed laboratory changes are assigned to long courses
glucocorticosteroids (prednisone, dexamethasone) in small
doses.

If symptoms and laboratory findings are significantly
expressed, but no systemic manifestations, to corticosteroids
cytostatic immunosuppressive drugs are added –
cyclophosphamide, chlorbutin, azathioprine. Maintenance therapy
carried out by the same means for several years.

If there are symptoms of systemic lesions regardless of stage
diseases are immediately prescribed in high doses of corticosteroids and
immunosuppressants for several days with a gradual transfer
on maintenance doses.

With generalized polyneuritis, vasculitis, kidney damage and
other severe manifestations of the disease to the above treatment
such methods are added, extracorporal treatment – plasmapheresis,
hemosorption, plasma ultrafiltration.

The remaining drugs are prescribed depending on the complications and
associated diseases – cholecystitis (see what you can eat with
cholecystitis), gastritis, pneumonia, endocervicitis, etc.

In certain cases, dietary compliance is necessary.
nutrition and limitation of physical activity.

Sjogren’s syndrome is not threatening for the life of the patient,
but can reduce the quality of life and lead to disability.
Timely diagnosis and treatment in the early stages of the disease
able to significantly slow down the development of pathological
process, prevent severe complications and keep
working capacity.

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