[Primary Gougerot-Sjogren's syndrome].
Sjogren's syndrome is characterized by the association of a Sicca syndrome prevailing at the ocular and oral level and of extra-glandular involvement of immuno-inflammatory mechanism: nonerosive polyarthritis, Raynaud's phenomenon, cutaneous and (or) neurological vasculitis, pulmonary involvement and interstitial nephropathy. In the typical forms, the biological signs associate a polyclonal hypergammaglobulinemia, sometimes a cryoblogulinemia, rheumatoid factor and anti SSA and anti SSB ANA. The diagnosis is confirmed by minor salivary gland biopsy showing a lymphoid infiltrate in clusters. These biological anomalies, the presence of major salivary gland enlargements and extra-glandular manifestations, characterize the progressive forms of the disease with a high risk of evolution towards malignant lymphoma. Antimalarial drugs are used in the treatment of polyarthritis, corticosteroids and immunosuppressive agents in serious extra-glandular involvement.
Hatron
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PY
Service de médecine interne Hôpital Claude-Huriez CHRU 59037, Lille. pyhatron@chru_lille.fr
Fauchais
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AL
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000962:Antimalarials / Q000627:therapeutic use; D001706:Biopsy; D018450:Disease Progression; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation; D008223:Lymphoma / Q000209:etiology; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D013256:Steroids
D000893:Anti-Inflammatory Agents; D000962:Antimalarials; D007166:Immunosuppressive Agents; D013256:Steroids