Abstract
Sjögren’s syndrome (SS) is a systemic autoimmune condition with various clinical pictures, extending from mild exocrinopathy to severe, systemic disease. Noteworthily, the development of non-Hodgkin B-cell lymphoma is observed in about 5 % of patients with SS. A plethora of autoantibodies is detected in the sera of patients with SS. These autoantibodies do not only indicate the B-cell hyperactivity that characterizes the disease, but also have diagnostic and clinical utility. The responses against the protein components of the Ro/La ribonucleoprotein complex are of high importance for the diagnosis of the disease. Furthermore, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies indicates a severe disease with early onset, significant salivary hypofunction, and high prevalence of extraglandular manifestations. Among the other autoantibodies described in the sera of SS patients, cryoglobulins characterize patients with adverse prognosis and prompt to future lymphoma development, while anti-cyclic citrullinated peptide autoantibodies are associated with articular involvement. Finally, various histologic and laboratory parameters, such as infiltration by immune cell and low C4 serum levels, are associated with a more severe disease phenotype and lymphoma development.
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Abbreviations
- ACA:
-
Anti-centromere antibodies
- AMA:
-
Antimitochondrial antibodies
- ANA:
-
Antinuclear antibodies
- Anti-CCP:
-
Antibodies against cyclic citrullinated peptides
- Anti-CAII:
-
Anti-carbonic anhydrase II
- ASMA:
-
Anti-smooth muscle antibodies
- MRs:
-
Muscarinic receptors
- NLS:
-
Neonatal lupus syndrome
- RF:
-
Rheumatoid factor
- SLE:
-
Systemic lupus erythematosus
- SS:
-
Sjögren’s syndrome
- SSc:
-
Systemic sclerosis
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Kapsogeorgou, E.K., Tzioufas, A.G. (2016). Autoantibodies in Sjögren’s Syndrome and Laboratory Markers. In: Roccatello, D., Emmi, L. (eds) Connective Tissue Disease. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-24535-5_21
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