Prognosis of patients with primary Sjogren's syndrome

The natural history of primary Sjogren's syndrome has been little studied. Some studies agree that, although it is not a benign disease, it is characterized by a steady evolution of the predominant symptoms (sicca and general manifestations). However, there are 2 main exceptions to this chronic course: the development of vasculitic manifestations, and the high incidence of lymphomas, both processes being related to the excess of mortality in patients with primary Sjogren's syndrome. Recent studies have prospectively analyzed the outcome of this disease in a large series of patients and identified those factors present at diagnosis prospectively associated with an adverse outcome. The main prognostic factors identified are severe involvement of the exocrine glands, vasculitis, hypocomplementemia and cryoglobulins at diagnosis. These features identify a specific subset of patients diagnosed with primary Sjogren's syndrome in whom a closer follow-up, and probably an earlier and more robust therapeutic management, should be mandatory.