SHYOGRENA SINDROM (H. S. Page of Sjogren, the Swedish ophthalmologist, a sort in
1899; syndrome; synonym: dacryosialoa-denopathia atrophicans, Guzhero — Hauera — Shegrena a syndrome, to Mick-licha — Shegrena a syndrome, Mikuli-cha — Guzhero — Shegrena a syndrome, Guzhero — Shegrena a syndrome, Haddena — Hauera — Shegrena a syndrome) — the symptom complex which is characterized by a combination of signs of defeat of excretory glands (is more often salivary and the lacrimal) with a number of autoimmune diseases.
Separate manifestations of Sh. of page were described at the end of 19 century. The filamentous keratitis, to-ry is found at a dry keratoconjunctivitis, is for the first time described by Leber (Th. Leber, 1882), a xerostomia — Hadden (W. Century of Hadden, 1888), increase salivary (parenchymatous parotitis) and the lacrimal glands of not tumoral origin without signs of decrease in their function — And. Mikulich (1892), an atrophy of the lacrimal, salivary, sweat and other glands with development of a xerophthalmus, a mouth, nasopharynx, vagina and skin — A. Guzhero (1925), a combination of a dry keratoconjunctivitis, defeat of sialadens to chronic polyarthritis — Hauer (A. W. M of Houwer, 1927). The symptom complex including a dry keratoconjunctivitis, a xerostomia (owing to defeat of sialadens), a pseudorheumatism and other symptoms, was for the first time in detail studied by the Swedish ophthalmologist Shegren (1933). Further defeats of the lacrimal, salivary and other excretory glands were described in combination with a system scleroderma (see), a system lupus erythematosus (see the Lupus erythematosus), a dermatomyositis (see), autoimmune gepatobiliarny diseases (chronic active hepatitis, primary biliary cirrhosis), Hashimoto's thyroiditis and other autoimmune diseases and also as an independent disease (see Shegren a disease).
In the International classification of diseases, injuries and causes of death of the IX review of Sh. of page it is included along with Shegren's disease in group of diffusion diseases of connecting fabric.
Highway of page is observed preferential at women at the age of 20 — 60 years (more often later there are 40 years). Cases of development of Sh. of page and at children are known.
According to various researchers, Sh. of page
5 — 10% of patients with diffusion diseases of connecting fabric are observed at 15 — 25% of patients with a pseudorheumatism (see. Collagenic diseases) and 50 — 100% of patients with autoimmune gepatobiliarny diseases. III. the page most often develops at patients chronic and subacute options of a system lupus erythematosus and a system scleroderma, and also seropositive highly active forms of a pseudorheumatism (see). At most of patients clinical manifestations of Sh. of page (dry keratoconjunctivitis, chronic parenchymatous parotitis, xerostomia, etc.) appear against the background of the developed clinical picture of a basic disease, on average in 5 — 10 years after its beginning. Approximately at a third of patients simultaneous development of signs of defeat of excretory glands and the specified diseases, and only in rare instances a dry keratoconjunctivitis is observed, defeat of sialadens and a xerostomia can precede development of a basic disease. Emergence of symptoms of pathology of excretory glands, as a rule, does not exert a direct impact on the current of a basic disease, and only supplements his clinical picture with manifestations not inherent usually, complicating treatment. The most expressed signs of defeat of excretory glands occur generally at patients with a pseudorheumatism and are much more rare at diffusion diseases of connecting fabric and autoimmune gepatobiliarny diseases; partly it can be explained more often and it is long the corticosteroid and cytotoxic immunodepressive therapy which is carried out at them suppressing expressiveness of manifestations of Sh. of page.
Diagnosis of III. the page is considered reliable in the presence of a combination of the dry keratoconjunctivitis and (or) chronic parenchymatous parotitis which is followed by a xerostomia to various autoimmune diseases. Therapy of patients with III. shall be page complex and include in addition to the means and methods influencing the current of a basic disease, therapy of a dry keratoconjunctivitis, xerostomia (see), parotitis (see) and other manifestations of defeat of excretory glands (it is detailed see Shegren a disease). High frequency of a medicinal allergy (30 — 50%) is characteristic of patients with Sh. of page that demands careful selection of drug treatment. As at patients with Sh. of page heavy complications from use of drugs of gold, D-Penicillaminum and levamisole develop more often, there is an opinion on inexpediency of use at their treatment of the specified means.
390 ShEGRENA — J1APCCOHA the SYNDROME
the Bibliography: See bibliogr. to the Art. of Shegren
a disease. V. I. Vasilyev.