Abstract
The treatment of primary Sjögren's syndrome (SS) is based principally on the management of sicca features and systemic manifestations. Sicca manifestations are treated symptomatically through administration of topical therapies, such as saliva substitutes and artificial tears; in patients with residual salivary gland function, stimulation of salivary flow with a sialogogue is the therapy of choice. The management of extraglandular features must be tailored to the specific organ or organs involved; however, limited data have been obtained from controlled trials in SS to guide the treatment of systemic symptoms using therapies including antimalarials, glucocorticoids, immunosuppressive drugs and biologic agents. Nevertheless, randomised controlled trials of biologic agents that target molecules and receptors involved in the aetiopathogenesis of primary SS have initiated a new era in the therapeutic management of the disease, although the potential risks and benefits of these agents must be carefully considered. In this Review, we analyse the evidence regarding the efficacy of the therapeutic agents currently available to treat the manifestations of SS. On the basis of this evidence, we provide guidance on the use of these agents in different clinical scenarios.
Key Points
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The primary therapeutic approach to sicca manifestations in Sjögren's syndrome (SS) should be symptomatic relief, using artificial tears and saliva substitutes
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The treatment of choice for patients with moderate or severe oral dryness and residual salivary gland function is an oral muscarinic agonist
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Patients with severe or refractory keratoconjunctivitis sicca might require the addition of topical cyclosporine A to suppress the underlying inflammation
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The management of extraglandular features must be tailored to the specific organ involved, mainly using corticosteroids and immunosuppressive agents
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Controlled trials of biologic agents in patients with SS have demonstrated the lack of efficacy of TNF blockade, whereas more promising results have been obtained with B-cell-targeted therapies, especially rituximab
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The overall low level of evidence from therapeutic studies in primary SS underscores the need for considerably larger trials
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Acknowledgements
M. Ramos-Casals, P. Brito-Zerón and A. Sisó-Almirall are supported by funding from Grants La Marató de TV3 (071810) and Fondo de Investigaciones Sanitarias (080103). The authors wish to thank David Buss for his editorial assistance.
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M. Ramos-Casals, P. Brito-Zerón and A. Sisó-Almirall contributed equally to researching the data for the article. M. Ramos-Casals, P. Brito-Zerón and X. Bosch contributed equally to the writing of the article. All authors provided substantial contributions to discussion of the content and reviewing and/or editing the manuscript before submission.
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Ramos-Casals, M., Brito-Zerón, P., Sisó-Almirall, A. et al. Topical and systemic medications for the treatment of primary Sjögren's syndrome. Nat Rev Rheumatol 8, 399–411 (2012). https://doi.org/10.1038/nrrheum.2012.53
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DOI: https://doi.org/10.1038/nrrheum.2012.53
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