Syndrome of Goujerot-Sjögren

The Syndrome of Gougerot-Sjögren or syndrome of Sjögren is a auto-immune Maladie systemic characterized by an attack of the Glande S exocrines, in particular of lachrymal and salivary glands. The disease touches 0,2% of the population, more often the women than the men. Although often primitive (i.e. isolated), it is in 50% of the cases associated with another auto-immune disease: érythémateux Lupus disseminated, Polyarthritis rhumatoïde.

Diagnosis

The most frequent attack is that of salivary glands, appearing by a Xérostomie (the mouth is dry permanently). The attack of lachrymal glands involves a dryness of the tunics protecting the eye (Conjonctive, and especially Cornée), which can involve corneal ulcerations, conjunctivites. The xerosis (very dry Peau) is rarer, just as the attack of the Poumon (lymphoid pneumopathy), which appears by a Toux dries chronic, sometimes very invalidating. This specific table is often enriched by systemic signs having nothing to do with an attack of glands, but rising from the autoimmune character of the disease:

Signs of Vascularity (reached vessels): Syndrome of Raynaud, Purpura, peripheral neuropathy;
Arthralgies (joint pains) in connection with an inflammatory arthritis;
Sometimes, signs of an associated auto-immune disease (érythémateux Lupus disseminated, Polyarthritis rhumatoïde, Thyroïdite, etc)

Complementary examinations

Assessment of the glandular anomalies: the test of Schirmer explores the production of the tears. The test of the “Station-wagon-up time” usefully supplements it by evaluating the time of rupture of lachrymal film. The objective measurement of salivary flow and the test with sugar explore the production of saliva
Bilan of the immunizing anomalies: the research of the factor rhumatoïde is often positive (reactions of Latex and Waaler-Rose), the antibodies anti-SSA and anti-SSB are generally positive, the remainder of the immunological assessment is generally negative.
It has only few inflammatory Syndrome there: CRP generally normal, but acceleration of the Sedimentation test by Hyper-gamma-globulinémie polyclonale, etc
the diagnosis of certainty is brought by the anatomo-pathological analysis of a Biopsie of additional salivary glands if there does not exist anti-SSA or anti-SSB anticoprps in the serum.

Treatment

The treatment of the syndrome of primitive Gougerot-Sjögren is only palliative: one uses artificial tears, artificial saliva, cough mixtures, and Savon S will surgras for the cutaneous xerosis. Only one drug was effective against the dryness: the chlorydrate of pilocarpine .

See too

External bonds

AFGS, French Association of Gougerot Sjögren and the dry syndromes
Article of the site Orpha.net
www.sjogren.ch, French Association of the Syndrome of Sjögren

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