Primary Sjögren's Syndrome

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KEY CLINICAL POINTSPRIMARY SJÖGREN'S SYNDROMEA diagnosis of primary Sjögren's syndrome is often made on the basis of a classic triad of symptoms: dryness of the mouth and eyes, fatigue, and pain. Systemic complications, which are present in 30 to 40% of patients, may provide the first clues to the disease.The risk of B-cell lymphoma is markedly increased (by a factor of 15 to 20) among patients with primary Sjögren's syndrome, as compared with the general population.Treatment relies on muscarinic agonists (pilocarpine hydrochloride and cevimeline hydrochloride). The main adverse effect of these agents is sweating, which can be minimized by gradual escalation of the dose.Hydroxychloroquine is often used for arthralgia with or without synovitis and purpura, although benefits have not been shown in a randomized, controlled trial.In the absence of trials involving patients with primary Sjögren's syndrome, severe organ manifestations are treated with immunosuppressive agents (including prednisone, methotrexate, mycophenolate sodium, azathioprine, and cyclophosphamide), in accordance with guidelines for systemic lupus erythematosus and other connective-tissue diseases.

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