Primary Sjögren syndrome: an update on current pharmacotherapy options and future directions.
BACKGROUND
Primary Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by sicca features and systemic manifestations, and requires a multidisciplinary therapeutic approach.


METHODS
Treatment of sicca manifestations is symptomatic and is based on the administration of topical therapies (saliva substitutes and preservative-free artificial tears). In severe cases of keratoconjunctivitis sicca, topical cyclosporine A may be used. For patients with residual salivary gland function, stimulation of salivary flow with a sialogogue (pilocarpine or cevimeline) is the treatment of choice. The management of extraglandular features must be tailored to the specific organ(s) involved. Hydroxychloroquine may be appropriate for patients with fatigue, arthralgia and myalgia, while glucocorticoids and immunosuppressive agents should be reserved for severe systemic involvement (although no controlled trials in primary SS guide their use). RCTs have demonstrated the lack of efficacy of antitumor necrosis factor agents and promising results for B-cell depleting agents.


CONCLUSIONS
The overall low level of evidence in therapeutic studies in primary SS suggests that much larger trials of the most promising therapies are necessary. The use of drugs targeting molecules and receptors involved in the etiopathogenesis of primary SS may open up a new era in the therapeutic management of the disease, but the potential risks and benefits of these agents must be weighed carefully.
Servei de Malalties Autoimmunes, Hospital Clínic, Barcelona, Spain.
Pilar
P
Brito-Zerón
Antoni
A
Sisó-Almirall
Albert
A
Bové
Belchin A
BA
Kostov
Manuel
M
Ramos-Casals
D016572:Cyclosporine / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007638:Keratoconjunctivitis Sicca / Q000188:drug therapy* / Q000503:physiopathology; D058990:Molecular Targeted Therapy; D009883:Ophthalmic Solutions / Q000627:therapeutic use; D012464:Saliva, Artificial / Q000627:therapeutic use; D012859:Sjogren's Syndrome / Q000188:drug therapy* / Q000503:physiopathology
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D009883:Ophthalmic Solutions; D012464:Saliva, Artificial; D006886:Hydroxychloroquine; D016572:Cyclosporine