TY - CHAP M1 - Book, Section TI - Primary Sjögren Syndrome A1 - Ramos-Casals, Manuel A1 - Brito-Zerón, Pilar A1 - Sisó-Almirall, Antoni A2 - Stone, John H. Y1 - 2021 N1 - T2 - Current Diagnosis & Treatment: Rheumatology, 4e 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 tests (corneal stainings and Schirmer test) and oral investigations (salivary flow measurement and parotid scintigraphy).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 is complicated by the development of lymphoma in a small percentage of patients.Patients with SjS can be characterized by a broad spectrum of laboratory 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 - McGraw Hill CY - New York, NY Y2 - 2024/04/19 UR - accessmedicine.mhmedical.com/content.aspx?aid=1180196959 ER -