[Primary Gougerot-Sjogren's syndrome]

Abstract

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.