Sjögren's disease

Definition

Sjögren's disease, also termed Sjögren's syndrome, is a systemic autoimmune disorder characterized by lymphocytic infiltration of salivary and lachrymal glands. Chronic inflammation results in the progressive destruction of these glands, leading to keratoconjunctivitis sicca and xerostomia, the characteristic symptoms of Sjogren's disease.

Latest Research and Reviews

  • Reviews |

    Ectopic lymphoid structures have been identified in the target organs of a subset of patients with rheumatic autoimmune diseases. This Review describes these structures and summarizes our current understanding of how they form, function and contribute to autoimmunity.

    • Michele Bombardieri
    • , Myles Lewis
    •  & Costantino Pitzalis
  • Reviews |

    Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands, resulting in the development of sicca symptoms and systemic disease. In this Primer, the mechanisms as well as the diagnosis and management of SjS are described.

    • Pilar Brito-Zerón
    • , Chiara Baldini
    • , Hendrika Bootsma
    • , Simon J. Bowman
    • , Roland Jonsson
    • , Xavier Mariette
    • , Kathy Sivils
    • , Elke Theander
    • , Athanasios Tzioufas
    •  & Manuel Ramos-Casals
  • Reviews |

    This Review discusses the current treatment of the manifestations of primary Sjögren syndrome and summarizes ongoing clinical trials. The authors examine the status of biologic therapies, the challenges relating to clinical trial design and potential targets for the future therapies.

    • Alain Saraux
    • , Jacques-Olivier Pers
    •  & Valérie Devauchelle-Pensec
  • Reviews |

    Kim et al. hypothesize that IL-21 is crucial to the pathogenesis of primary Sjögren syndrome (pSS), focusing on evidence of an interplay between the innate and adaptive immune responses mediated by the effects of IL-21 on immune cells. This new perspective might ultimately offer insight into novel approaches to the treatment of pSS.

    • Seung-Ki Kwok
    • , Jennifer Lee
    • , Di Yu
    • , Kwi Young Kang
    • , Mi-La Cho
    • , Hae-Rim Kim
    • , Ji Hyeon Ju
    • , Sang-Heon Lee
    • , Sung-Hwan Park
    •  & Ho-Youn Kim
  • Reviews |

    Both sicca and systemic symptoms affect quality of life in patient with Sjögren's syndrome, and their management is complicated by the low level of evidence supporting the available therapeutic options. Here, the authors comprehensively review the data on the efficacy of treatments for the glandular and extraglandular manifestations of this disease. Ramos-Casals and colleagues also recommend treatment approaches tailored to different clinical scenarios that patients with Sjögren's syndrome can present.

    • Manuel Ramos-Casals
    • , Pilar Brito-Zerón
    • , Antoni Sisó-Almirall
    • , Xavier Bosch
    •  & Athanasios G. Tzioufas

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