Shegren's disease

Description:

Shegren's disease (primary syndrome of Shegren) - a general autoimmune disease, characterized by a chronic inflammation of the excretory glands cosecreting preferential IgA, first of all salivary and the lacrimal with gradual development of their secretory insufficiency in combination with various system manifestations. It is described by the Swedish ophthalmologist Shegren in 1933.

Along with a disease allocate Shegren's syndrome (a secondary syndrome of Shegren) - defeat of the lacrimal glands with development of a dry keratoconjunctivitis and sialadens with development of a chronic sialadenitis and xerostomia at autoimmune diseases - a pseudorheumatism, a system lupus erythematosus, a system scleroderma, chronic active hepatitis, primary biliary cirrhosis, an autoimmune thyroiditis, etc.

The disease and Shegren's syndrome occur at women at 20-25 times more often than at men, usually at the age of 20-50 years.

Symptoms Shegren's Disease:

  1.
      Defeat of the lacrimal glands, corneas, conjunctivas, xerophthalmia in the form of a dry keratoconjunctivitis that is shown by feeling of a foreign body in an eye, a photophobia, burning in eyes, reddening of eyes, lack of tears, erosion and opacification of a cornea.
  2.
      Defeat of sialadens - parotitis with decrease in secretion of saliva, dryness in a mouth (xerostomia). Parotid, submandibular glands increase, saliva is not enough, it dense, develops a cheilitis, a glossitis, caries of teeth, stomatitis.
  3.
      Defeat of other excretory glands with depression of function that is shown by a xeroderma, vaginas, a gullet, a nose, a throat, tracheitises, bronchitis, a dysphagy, rhinitises, gastritis with secretory insufficiency, up to an akhiliya, chronic pancreatitis with decrease in vneshnesekretorny function.
  4.
      Polyarthralgias or development of polyarthritis as a pseudorheumatism.
  5.
      System manifestations: fever, lymphadenopathy, vasculites, miozita, increase in a liver, spleen, damage of kidneys, Reynaud's syndrome.

The clinic of a disease of Shegren depends on current option. At a chronic current signs of defeat of excretory glands prevail, at subacute - in addition kidneys, lungs are involved in process, the necrotizing vasculitis, defeat of a nervous system (peripheral polyneuropathy, neuritis, tserebrovaskulita) develop, the gepatosplenomegaliya develops less often.

On skin of shins, feet, hips, a stomach, a trunk there can be rashes as a hemorrhagic vasculitis which cornerstone increase in content in blood and adjournment in walls of capillaries is at - or cryoglobulins Rash is followed by an itch and painful burning. The Temorragichesky vasculitis caused preferential by cryoglobulins is followed by emergence of bubbles and pustules with hemorrhagic contents, there is an accurate communication of rashes with overcooling.

The thyroid gland can be involved in pathological process (a hypertrophic form of an autoimmune thyroiditis with the subsequent development of a hypothyroidism).

Reasons Shegren's Disease:

Causes of illness are unknown. The role of a hereditary factor is supposed, HLA B8, DW3, DW2 antigens often are found in patients. The role of a viral infection, in particular retroviruses which cause an expression of HLA of DR antigen and a translocation of autoantigens on membranes of epithelial cells with the subsequent activation of V-lymphocytes against the background of insufficiency of T-suppressors (Jacobsson) is not excluded.

The major pathogenetic factor is development of autoimmune reactions with the advent of antibodies to fabric of channels of salivary, lacrimal and other excretory glands (a pancreas, to covering cells of a stomach). The autoimmune nature of a disease is confirmed by extensive lymphoid infiltration struck the lacrimal and sialadens, and also detection not only organospetsifichesky (to the lacrimal and sialadens), but also organonespetsifichesky autoantibodies (the Russian Federation, anti-nuclear antibodies, antibodies to SA/Ro and SS-B/La antigens). Lymphoid infiltrates at Shegren's disease produce a large number interleykina-2, interleykina-6 and interleykina-10, the autoimmune reactions promoting development. At the generalized course of a disease (at 30% of patients) muscles (miositis), kidneys (intersticial nephrite), vessels (a productive and destructive vasculitis) are involved in process.

Treatment Shegren's Disease:

Treatment of a disease of Shegren has to be complex and directed as to suppression of immune disturbances in an organism, and on clinically demonstrated defeats of separate bodies and systems. The leading role in treatment belongs to the rheumatologist who performs complex therapy together with the oculist, the stomatologist, and if necessary and with other specialists.

As preventive measures dispensary observation at the rheumatologist with attraction is applied to joint therapy of the stomatologist, the oculist, the gynecologist, etc. Constant reception of maintenance doses of medicines, small doses of hormones (5 mg), tsitostatik (Chlorbutinum, Cyclophosphanum) and course preventive treatment by symptomatic means for the purpose of achievement of a condition of clinical remission are necessary.

Sanitation of the centers of persistent infection (adenoid disease, sinusitis, carious teeth, stomatitis) is important. It is recommended to avoid overcoolings, catarrhal diseases and a psychological overstrain. Primary prevention of a disease Shegrena is not developed.

In the period of an exacerbation of a disease restriction of speech loadings because of a xerostomia is recommended (dryness in a mouth). For reduction of a photophobia widespread among patients with primary syndrome of Shegren, glasses wearing with tinted windows is recommended (in bright sunny days).

Medicamentous therapy at a disease Shegrena is based on corticosteroid hormones and cytostatics.

Local therapy of dental and ophthalmologic displays of a disease of Shegren is of great importance. As a result of reduction of amount of saliva and its protective properties of manifestation of a xerostomia at most of patients are aggravated with the accompanying oral cavity candidiasis. Irrigation of an oral cavity of 2% by solution of bicarbonate soda, greasing by Unguentum Nystatini is applied to fight against candidiasis, nystatin on 500 000 PIECES of 4-6 times a day is appointed (or levorinum, дифлкжан). It is not necessary to swallow of tablets of antifungal antibiotics, and it is necessary to rassasyvat in a mouth. At emergence of herpetic defeat of mucous it is applied oxolinic or metiluratsilovy ointments, an acyclovir.

For treatment of parenchymatous parotitis it is possible to use compresses from 30% of solution of Dimexidum. They are imposed daily on area of parotid and submaxillary sialadens for 30-40 min.

Essential value has also timely treatment of carious teeth and prosthetics of an oral cavity.

Local therapy of ophthalmologic displays of a disease Shegrena is directed to overcoming a gipolakrimiya, improvement of regeneration of an epithelium of a cornea and conjunctiva, prevention of consecutive infection. As artificial tears 0,5% methyl cellulose solution are used. It is dug in by from 3 to 12 times a day depending on weight of defeats. Solutions of albucid, levomycetinum, Furacilin are applied to fight against an infection.

At Shegren's disease the atropinsoderzhashchy and antihistaminic drugs capable to reduce secretory function of excretory glands are contraindicated.

Non-drug treatment. Medical diet.

Physiologically full-fledged diet with enough vitamins is appointed. 5-6-times food is recommended. Use of chewing gum is shown.

Sanatorium treatment at Shegren's disease  - a gastrointestinal profile.