Predominant involvement of women, with female to male ratios over 10:1. Diagnosis most commonly established in the fifth and sixth decade of life, although symptoms of dryness may precede the diagnosis by many years. Affects individuals across the age spectrum, including children, but most commonly women in the perimenopausal years of life “Extraglandular” manifestations in approximately 50% of patients, including constitutional symptoms (such as fatigue and mild cognitive impairment) and other systemic manifestations, with involvement of diverse organ systems. Presence of anti-SSA (Ro) and anti-SSB (La) in 60-80% of patients [34-37]. Increased risk of B-cell non-Hodgkin lymphoma, particularly mucosa-associated lymphoid tissue lymphoma (MALT) and diffuse B-cell lymphoma. The relative risk of non-Hodgkin lymphoma ranges from 4.8 for primary and 9.6 for secondary SS [38], with an estimated lifetime risk of 5-10% [39].

Data from Refs 34-39