Sjögren disease, previously termed Sjögren syndrome, is a chronic, multisystem autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, primarily affecting the salivary and lacrimal glands. The infiltration leads to hallmark symptoms such as dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). The disease predominantly affects adult females, with a female-to-male ratio of 9:1. Common clinical manifestations include local complaints like asymptomatic glandular swellings and a variety of multiorgan manifestations, such as lymphadenopathy, neurologic disturbances and chronic pain. The current classification criteria encompass various clinical features and are based on five key tests: auto-antibodies [anti-SSA(Ro)] serology, Schirmer’s test, ocular staining score, unstimulated whole saliva flow rate and minor salivary gland biopsy. Microscopically, Sjögren disease is characterized by the infiltration of B and T lymphocytes surrounding the glandular parenchyma, often forming focus of lymphoid aggregates with more than 50 cells around ductal structures. Secondary lymphoid follicles and lymphoepithelial lesions may be observed in some cases. Patients with Sjögren disease have a significantly increased risk of developing non-Hodgkin lymphomas, and multidisciplinary care is often recommended for these individuals. Treatment protocols usually include the use of corticosteroids and immunosuppressive agents.

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Sjögren Disease

  • Luiz Alcino Monteiro Gueiros,
  • Silvia Ferreira de Sousa,
  • Felipe Paiva Fonseca

摘要

Sjögren disease, previously termed Sjögren syndrome, is a chronic, multisystem autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, primarily affecting the salivary and lacrimal glands. The infiltration leads to hallmark symptoms such as dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). The disease predominantly affects adult females, with a female-to-male ratio of 9:1. Common clinical manifestations include local complaints like asymptomatic glandular swellings and a variety of multiorgan manifestations, such as lymphadenopathy, neurologic disturbances and chronic pain. The current classification criteria encompass various clinical features and are based on five key tests: auto-antibodies [anti-SSA(Ro)] serology, Schirmer’s test, ocular staining score, unstimulated whole saliva flow rate and minor salivary gland biopsy. Microscopically, Sjögren disease is characterized by the infiltration of B and T lymphocytes surrounding the glandular parenchyma, often forming focus of lymphoid aggregates with more than 50 cells around ductal structures. Secondary lymphoid follicles and lymphoepithelial lesions may be observed in some cases. Patients with Sjögren disease have a significantly increased risk of developing non-Hodgkin lymphomas, and multidisciplinary care is often recommended for these individuals. Treatment protocols usually include the use of corticosteroids and immunosuppressive agents.