title: Treatment of primary Sjögren syndrome.
abstract: Primary Sjögren syndrome (pSS) is a progressive autoimmune disease characterized by sicca and systemic manifestations. In this Review, we summarize the available data on topical and systemic medications, according to clinical signs and disease activity, and we describe the ongoing studies using biologic drugs in the treatment of pSS. Expanding knowledge about the epidemiology, classification criteria, systemic activity scoring (ESSDAI) and patient-reported outcomes (ESSPRI) is driving active research. Treatment decisions are based on the evaluation of symptoms and extraglandular manifestations. Symptomatic treatment is usually appropriate, whereas systemic treatment is reserved for systemic manifestations. Sicca is managed by education, environment modification, elimination of contingent offending drugs, artificial tears, secretagogues and treatments for complications. Mild systemic signs such as fatigue are treated by exercise. Pain can require short-term moderate-dose glucocorticoid therapy and, in some cases, disease-modifying drugs. Severe and acute systemic manifestations indicate treatment with glucocorticoids and/or immunosuppressant drugs. The role for biologic agents is promising, but no double-blind randomized controlled trials (RCTs) proving the efficacy of these drugs are available. Targets for new treatments directed against the immunopathological mechanisms of pSS include epithelial cells, T cells, B-cell overactivity, the interferon signature, proinflammatory cytokines, ectopic germinal centre formation, chemokines involved in lymphoid cell homing, and epigenetic modifications.
authors.affiliation: EA2216, INSERM ESPRI ERI29, Laboratoire d'Immunothérapie et Pathologies lymphocytaires B, Université de Brest, Labex 'Immunotherapy, Graft, Oncology', 29609 Brest Cedex, France.|Department of Rheumatology, Centre Hospitalier Universitaire de la Cavale Blanche, 29609 Brest, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Saraux
authors.affiliation: Department of Rheumatology, Centre Hospitalier Universitaire de la Cavale Blanche, 29609 Brest, France.
authors.forename: Jacques-Olivier
authors.initials: JO
authors.lastname: Pers
authors.affiliation: EA2216, INSERM ESPRI ERI29, Laboratoire d'Immunothérapie et Pathologies lymphocytaires B, Université de Brest, Labex 'Immunotherapy, Graft, Oncology', 29609 Brest Cedex, France.|Department of Rheumatology, Centre Hospitalier Universitaire de la Cavale Blanche, 29609 Brest, France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Devauchelle-Pensec
mesh_terms: D018771:Arthralgia / Q000188:drug therapy / Q000209:etiology; D001168:Arthritis / Q000188:drug therapy / Q000209:etiology; D001691:Biological Therapy; D003449:Cryoglobulinemia / Q000188:drug therapy / Q000209:etiology; D015352:Dry Eye Syndromes / Q000188:drug therapy / Q000209:etiology; D005221:Fatigue / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology; D058990:Molecular Targeted Therapy; D009135:Muscular Diseases / Q000188:drug therapy / Q000209:etiology; D009395:Nephritis, Interstitial / Q000188:drug therapy / Q000209:etiology; D009422:Nervous System Diseases / Q000188:drug therapy / Q000209:etiology; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy*; D012871:Skin Diseases / Q000188:drug therapy / Q000209:etiology; D014987:Xerostomia / Q000188:drug therapy / Q000209:etiology