Abstract
Sjögren's syndrome is a chronic autoimmune disorder, characterized by lymphocytic infiltration and malfunction of the exocrine glands, resulting in dry mouth and eyes. The syndrome can present either alone (primary Sjögren's syndrome) or in the context of an underlying connective tissue disease (secondary Sjögren's syndrome). Systemic features, resulting from cutaneous, respiratory, renal, hepatic, neurologic, and vascular involvement, often occur. Two types of primary Sjögren's syndrome are currently recognized: a benign disease that affects quality of life, and a systemic syndrome associated with increased morbidity and mortality owing to a high risk of malignant transformation, and that requires close follow-up. Ocular involvement, manifested as keratoconjunctivitis sicca, is managed with local and systemic stimulators of tear secretion and supportive surgical procedures. Treatment of oral manifestations includes intense oral hygiene, prevention and treatment of oral infections, use of saliva substitutes, and local and systematic stimulation of salivary secretion. Cholinergic agents, such as pilocarpine and cevimeline, are helpful in patients with residual salivary function, and ciclosporin ocular drops seem to be of some benefit. Systemic immunosuppressives are reserved for treatment of severe extraglandular manifestations of Sjögren's syndrome. Anti-B-cell therapy is a new potential therapy for the glandular and extraglandular manifestations, such as glomerulonephritis or vasculitis, in addition to the management of lymphoma associated with Sjögren's syndrome. Induction of oral tolerance and gene-transfer modalities were recently attempted in animal models, with promising results.
Key Points
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Sjögren's syndrome is a chronic autoimmune disorder that primarily targets the exocrine glands and results in symptoms of dry eyes and dry mouth
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Extraglandular features, involving the skin, kidney, lung, liver, and nervous system, are common
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Among patients with Sjögren's syndrome, the presence of purpura, low complement levels, and cryoglobulinemia define a subgroup at high risk of developing lymphoma
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Treatment of ocular manifestations includes local and systemic stimulators of tear secretion and supportive surgical procedures
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Treatment of oral manifestations includes meticulous oral hygiene and routine dental care combined with local and systemic stimulation of salivary secretion and the use of salivary substitutes
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Management of extraglandular manifestations is mainly empiric: steroids and immunosuppressive agents are of some benefit, and anti-B-cell therapy is currently being investigated and shows promising results
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Written consent for publication was obtained from the patients or their responsible relatives. We would like to thank Dr E Kapsogeorgou for her assistance in the preparation of figures.
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Mavragani, C., Moutsopoulos, N. & Moutsopoulos, H. The management of Sjögren's syndrome. Nat Rev Rheumatol 2, 252–261 (2006). https://doi.org/10.1038/ncprheum0165
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DOI: https://doi.org/10.1038/ncprheum0165
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