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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.

Raynaud's phenomenon must systematically lead to the research of a professional cause, mainly if the patient is a manual worker. The most frequent professional cause is vibrations disease which involves different occupations, especially woodcutters. In this disease, Raynaud's phenomenon may be associated with neurological or rheumatic manifestations. Prevention relies on tool improvement and reduction of exposure-time. The more uncommon hammer's syndrome is characterized by the onset of a terminal cubital artery aneurysm, resulting from repeated trauma on the palm of the hand. Raynaud's phenomenon is severe, with frequent trophic complications related to embolic migration towards digital arteries. Improved prevention explains that acrosyndromes related to vinyl chloride exposure are now very rare. Finally, a Raynaud's phenomenon may be the first symptom of a systemic sclerosis induced by silicate exposure.

During the past few years, changes in the spectrum of serious forms of systemic lupus erythematosus have been observed, and generally, the prognosis has improved. This results from a better understanding of the mechanisms of some visceral involvements (especially cerebral and cardiovascular), from the characterization of the antiphospholipid antibodies syndrome, and from a better management. As a result, kidney and central nervous system involvements are not the main causes of mortality any more. However, increasing survival leads to a higher frequency of some late complications, especially related to atherosclerosis.

Drug-related lupus accounts for about 10 p. 100 of all lupoid diseases. The main responsible drugs are antihypertensive, antiarrhythmic and anticonvulsant agents, phenothiazines and beta-blockers. Iatrogenic lupus has a better prognosis than idiopathic lupus, since renal and neurological lesions are rare and, when present, repress after the responsible drug is withdrawn. The presence of antinuclear antibodies is indispensable to the diagnosis. The detection of anti-histones and denatured anti-DNA in the absence of anti-native DNA and/or anti-SM is the usual immunological pattern of drug-related lupus.