TY - CHAP M1 - Book, Section TI - Chapter 26. Primary Sjögren Syndrome A1 - Ramos-Casals, Manuel A1 - Brito-Zerón, Pilar A1 - Sisó-Almirall, Antoni A2 - Imboden, John B. A2 - Hellmann, David B. A2 - Stone, John H. PY - 2013 T2 - CURRENT Diagnosis & Treatment: Rheumatology, 3e AB - Sjögren syndrome (SjS) is a systemic autoimmune disease that presents with sicca symptomatology of mucosal surfaces.The main sicca features (xerophthalmia and xerostomia) are determined by specific ocular (rose bengal staining and Schirmer test) and oral (salivary flow measurement and parotid scintigraphy) tests.The histologic hallmark is a focal lymphocytic infiltration of the exocrine glands, determined by a biopsy of the minor labial salivary glands.The spectrum of the disease includes systemic features (extraglandular manifestations) in some patients, and may be complicated by the development of lymphoma.Patients with SjS present a broad spectrum of analytic features (cytopenias, hypergammaglobulinemia, and high erythrocyte sedimentation rate) and autoantibodies, of which antinuclear antibodies are the most frequently detected, anti-Ro/SS-A the most specific, and cryoglobulins and hypocomplementemia the main prognostic markers. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/19 UR - accessmedicine.mhmedical.com/content.aspx?aid=57272696 ER -