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Sjögren’s Disease (SjD) is a chronic autoimmune disorder that predominantly affects women but may also occur in men and children, often with distinct clinical presentations. This review aimed to describe the main clinical findings, complications, and therapeutic approaches of SjD in these two underrepresented populations and to compare them with the classical phenotype observed in adult women. A total of 117 studies were analysed, comprising 74 adult male cases and 65 paediatric patients. In men, glandular manifestations were frequent but often incomplete, with isolated oral and/or ocular symptoms associated with significant systemic involvement, particularly neurological (38.2%), pulmonary (31.1%), and nephro–urological (21.6%) complications. In children, enlargement of the major salivary glands was the most common manifestation (75.4%), followed by musculoskeletal (44.6%), haematological (44.6%), cutaneous (29.2%), renal (20.0%), and neurological (13.8%) involvement. Complete sicca syndrome was less frequent in both groups; however, isolated glandular symptoms and early systemic manifestations were common and clinically relevant. SjD in men and paediatric patients demonstrates greater clinical heterogeneity and a higher burden of systemic manifestations compared with the classical adult female phenotype, underscoring the need for heightened clinical awareness and tailored diagnostic strategies in these populations.
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Lameira et al. (2026) studied this question.
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