Chapter 156 Sjögren Syndrome Abraham Gedalia Sjögren syndrome is a chronic, inflammatory, autoimmune disease characterized by progressive lymphocytic and plasma cell infiltration of the salivary and lacrimal glands. It is rare in children and predominantly affects middle-aged women. Epidemiology Sjögren syndrome typically manifests at 35-45 yr of age, with 90% of cases among women. It is uncommon in the pediatric age group. The mean age at diagnosis in children is 9-10 yr; 75% are girls. The disease can occur as an isolated disorder, referred to as primary Sjögren syndrome (sicca complex), or as a secondary form in association with other rheumatic disorders. Most commonly, it accompanies systemic lupus erythematosus, scleroderma, or mixed connective tissue disease and usually precedes the associated autoimmune disease by several years. Etiology and Pathogenesis The etiology of Sjögren syndrome is complex and includes genetic predisposition and possibly an infectious trigger. Lymphocytes and plasma cells infiltrate salivary glands, forming distinct periductal and periacinar foci that become confluent and may replace epithelial structure. This autoimmune exocrinopathy results in xerophthalmia (dry eyes, or keratoconjunctivitis sicca) and xerostomia (dry mouth). Several genes regulating apoptosis influence the chronicity of lymphocytic infiltration. Clinical Manifestations International classification criteria have been developed for the diagnosis of Sjögren syndrome in adult patients (Table 156-1 Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window) Related Related posts: Rumination, Pica, and Elimination (Enuresis, Encopresis) Disorders Adolescent Pregnancy Yersinia Fever without a Focus Stay updated, free articles. Join our Telegram channel Join Tags: Nelson Textbook of Pediatrics Expert Consult Jun 18, 2016 | Posted by admin in PEDIATRICS | Comments Off on Sjögren Syndrome Full access? Get Clinical Tree
Chapter 156 Sjögren Syndrome Abraham Gedalia Sjögren syndrome is a chronic, inflammatory, autoimmune disease characterized by progressive lymphocytic and plasma cell infiltration of the salivary and lacrimal glands. It is rare in children and predominantly affects middle-aged women. Epidemiology Sjögren syndrome typically manifests at 35-45 yr of age, with 90% of cases among women. It is uncommon in the pediatric age group. The mean age at diagnosis in children is 9-10 yr; 75% are girls. The disease can occur as an isolated disorder, referred to as primary Sjögren syndrome (sicca complex), or as a secondary form in association with other rheumatic disorders. Most commonly, it accompanies systemic lupus erythematosus, scleroderma, or mixed connective tissue disease and usually precedes the associated autoimmune disease by several years. Etiology and Pathogenesis The etiology of Sjögren syndrome is complex and includes genetic predisposition and possibly an infectious trigger. Lymphocytes and plasma cells infiltrate salivary glands, forming distinct periductal and periacinar foci that become confluent and may replace epithelial structure. This autoimmune exocrinopathy results in xerophthalmia (dry eyes, or keratoconjunctivitis sicca) and xerostomia (dry mouth). Several genes regulating apoptosis influence the chronicity of lymphocytic infiltration. Clinical Manifestations International classification criteria have been developed for the diagnosis of Sjögren syndrome in adult patients (Table 156-1 Only gold members can continue reading. Log In or Register to continue Share this:Click to share on Twitter (Opens in new window)Click to share on Facebook (Opens in new window) Related Related posts: Rumination, Pica, and Elimination (Enuresis, Encopresis) Disorders Adolescent Pregnancy Yersinia Fever without a Focus Stay updated, free articles. Join our Telegram channel Join Tags: Nelson Textbook of Pediatrics Expert Consult Jun 18, 2016 | Posted by admin in PEDIATRICS | Comments Off on Sjögren Syndrome Full access? Get Clinical Tree
