Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8536866 | Pharmacology & Therapeutics | 2018 | 56 Pages |
Abstract
Primary Sjögren syndrome (SjS) is a systemic autoimmune disease that may affect 1 in 1000 people (overwhelmingly women) and that can be a serious disease with excess mortality due to severe organ-specific involvements and the development of B cell lymphoma; systemic involvement clearly marks the disease prognosis, and strongly suggests the need for closer follow-up and more robust therapeutic management. Therapy is established according to the organ involved and severity. As a rule, the management of systemic SjS should be organ-specific, with glucocorticoids and immunosuppressive agents limited to potentially-severe involvements; unfortunately, the limited evidence available for these drugs, together with the potential development of serious adverse events, makes solid therapeutic recommendations difficult. The emergence of biological therapies has increased the therapeutic armamentarium available to treat primary SjS. Biologics currently used in SjS patients are used off-label and are overwhelmingly agents targeting B cells, but the most recent studies are moving on into the evaluation of targeting specific cytokines involved in the SjS pathogenesis. The most recent etiopathogenic advances in SjS are shedding some light in the search for new highly-selective biological therapies without the adverse effects of the standard drugs currently used (corticosteroids and immunosuppressant drugs). This review summarizes the potential pharmacotherapeutic options targeting the main cytokine families involved in the etiopathogenesis of primary SjS and analyzes potential insights for developing new therapies.
Keywords
TFHAPCImmunocomplexTLRIL-1RAPRRTregMarginal zone B cellRCTIFN-IILC2iNKTTACITSLPBr3Plasmacytoid DCSJSOAS1Follicular helper T cellFDCNODmAbBCRSGECautoantibodiesPDCnatural killerRheumatoid arthritisRandomized controlled trialIL-1 receptor antagonistMonoclonal antibodyantigen-presenting cellsAPRILoligoadenylate synthetaseType I interferoninterleukinBAFFToll-like receptorTherapyRegulatory T cellinvariant natural killer T cellDendritic cellfollicular dendritic cellplasma cellHelper T cellSjögren syndromeCytokinesrheumatoid factorB-cell activating factorBiological agentsLymphotoxinThymic stromal lymphopoietinSystemic lupus erythematosusSLEGerminal centerGenome-wide association studiesGWASnon-obese diabetic mouseHPAtype 2 innate lymphoid cellPathogenesisB-cell receptorpattern recognition receptora proliferation-inducing ligand
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Authors
Soledad Retamozo, Alejandra Flores-Chavez, Marta Consuegra-Fernández, Francisco Lozano, Manuel Ramos-Casals, Pilar Brito-Zerón,