RT Book, Section A1 Tanikawa, Akiko A2 Kang, Sewon A2 Amagai, Masayuki A2 Bruckner, Anna L. A2 Enk, Alexander H. A2 Margolis, David J. A2 McMichael, Amy J. A2 Orringer, Jeffrey S. SR Print(0) ID 1162817456 T1 Sjögren Syndrome T2 Fitzpatrick's Dermatology, 9e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9780071837798 LK dermatology.mhmedical.com/content.aspx?aid=1162817456 RD 2024/03/28 AB AT-A-GLANCEA chronic, multisystem autoimmune disease, characterized by chronic inflammation involving the exocrine glands.Salivary and lachrymal glands are affected predominantly, leading to dry mouth and dry eyes.May occur alone (primary Sjögren syndrome, pSS), or may coexist with other systemic connective tissue disorders (secondary Sjögren syndrome).Extraglandular manifestations of primary Sjögren syndrome include fatigue, Raynaud phenomenon, purpura, arthritis, vasculitis, interstitial pulmonary disease, peripheral or central neuropathy, and autonomic nervous dysfunction.Patients with systemic manifestations are at higher risk of developing lymphoma.Treatment of sicca symptoms is mainly symptomatic, whereas management of extraglandular manifestations is similar to that of other autoimmune diseases.Both innate and adaptive immunity contribute to pSS pathogenesis and pSS is associated with upregulation of the Type 1 interferon signaling pathway.