Abstract
Background: Sjögren's syndrome is one of the most common inflammatory rheumatic diseases. The prevalence in Germany is at least 0.4%.
Method: The overview work is based on a selective literature research in PubMed. Attention is drawn to the renewed classification criteria and current therapeutic recommendations.
Results: The clinical picture of Sjögren's syndrome includes a wide range of manifestations, from the local infestation of exocrine glands with the leading symptoms of keratoconjunctivitis sicca and xerostomia to extraglandular systemic involvement of various organs. In addition, fatigue determines the quality of life of patients. Serologically, antinuclear autoantibodies anti-Ro / SSA and anti-la / SSB can be detected alongside rheumatoid factors. As a histological marker, a focal lymphocytic infiltration in unobtrusive azines is impaired, with the disease having a substantially increased risk for non-Hodgkin's lymphoma of the B-cell line (approximately 5% of the patients). The primary Sjögren syndrome (pSS) can be distinguished from a secondary form (sSS) Which can occur as a companion in the context of other autoimmune diseases. A sSS coexists in particular with systemic lupus erythematosus (15-36%), rheumatoid arthritis (20-32%) and limited and progressive systemic sclerosis (11-24%). Therapeutically, a basic therapy is reserved for patients with systemic participation, but the data on the efficacy is limited. The complexity of the disease requires interdisciplinary care depending on the clinical manifestation. However the data on the effectiveness is limited. The complexity of the disease requires interdisciplinary care depending on the clinical manifestation. However the data on the effectiveness is limited. The complexity of the disease requires interdisciplinary care depending on the clinical manifestation.
Conclusion: Improving the quality of life, drug and non-drug control of disease activity and the risk of lymphoma are the main focus of the interdisciplinary care of patients with Sjögren syndrome. Future therapeutic approaches must take into account the heterogeneity of the disease.
Method: The overview work is based on a selective literature research in PubMed. Attention is drawn to the renewed classification criteria and current therapeutic recommendations.
Results: The clinical picture of Sjögren's syndrome includes a wide range of manifestations, from the local infestation of exocrine glands with the leading symptoms of keratoconjunctivitis sicca and xerostomia to extraglandular systemic involvement of various organs. In addition, fatigue determines the quality of life of patients. Serologically, antinuclear autoantibodies anti-Ro / SSA and anti-la / SSB can be detected alongside rheumatoid factors. As a histological marker, a focal lymphocytic infiltration in unobtrusive azines is impaired, with the disease having a substantially increased risk for non-Hodgkin's lymphoma of the B-cell line (approximately 5% of the patients). The primary Sjögren syndrome (pSS) can be distinguished from a secondary form (sSS) Which can occur as a companion in the context of other autoimmune diseases. A sSS coexists in particular with systemic lupus erythematosus (15-36%), rheumatoid arthritis (20-32%) and limited and progressive systemic sclerosis (11-24%). Therapeutically, a basic therapy is reserved for patients with systemic participation, but the data on the efficacy is limited. The complexity of the disease requires interdisciplinary care depending on the clinical manifestation. However the data on the effectiveness is limited. The complexity of the disease requires interdisciplinary care depending on the clinical manifestation. However the data on the effectiveness is limited. The complexity of the disease requires interdisciplinary care depending on the clinical manifestation.
Conclusion: Improving the quality of life, drug and non-drug control of disease activity and the risk of lymphoma are the main focus of the interdisciplinary care of patients with Sjögren syndrome. Future therapeutic approaches must take into account the heterogeneity of the disease.
Original language | English |
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Pages (from-to) | 354-361 |
Journal | Aerzteblatt |
Volume | 114 |
Issue number | 20 |
DOIs | |
Publication status | Published - 15 Mar 2017 |