Sjogren’s Disease—Aspects of Clinical Disease Beyond Dry Eyes/Mouth

Primary Sjogren's Disease (SjD) is characterized by features of dryness arising from inflammation in the secretary glands, particularly the salivary and lachrymal glands. Generalized symptoms of fatigue and limb pain are very common but, in addition, about 40% of patients have one or more features of organ-specific systemic disease. This review goes through the background of SjD including diagnosis and classification, epidemiology, impact and investigations such as ultrasound and lip biopsy. It then focuses in detail on each of the systemic organ-specific features, principally using the European League against Rheumatism (EULAR) Sjogren's Syndrome Disease Activity Index (ESSDAI) along with some non-ESSDAI domains, before concluding with comments on disease heterogeneity, treatment, vaccination, pregnancy and surgery along with observations on patient perspectives. The aim is to provide a general overview of these aspects of the disease to complement other chapters in this monograph.