Overview
Introduction
Sjögren disease, formerly often called Sjögren syndrome, is a chronic systemic autoimmune exocrinopathy.
Sjögren disease, formerly often called Sjögren syndrome, is a chronic systemic autoimmune exocrinopathy. Focal lymphocytic inflammation progressively injures the lacrimal and salivary glands; B-cell hyperactivity and type I interferon signalling sustain that injury and explain the dry eyes and dry mouth. The process is not confined to secretory glands. The same B-cell activation can produce inflammatory joint, pulmonary, renal, neurologic, cutaneous, or haematologic disease and contributes to excess risk of B-cell non-Hodgkin lymphoma. A useful clinical model is to separate three questions: how much secretory function has been lost, whether another organ is active or threatened, and whether a change suggests lymphoma or an alternative diagnosis. Dryness severity alone does not measure systemic activity. A patient with modest sicca symptoms may have clinically important renal tubular acidosis or interstitial lung disease, while severe dryness may require intensive local care without systemic immunosuppression. Canadian practice uses the 2016 ACR/EULAR classification criteria and EULAR management recommendations; there is no separate Canadian diagnostic threshold.