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Autoantibodies in Sjögren’s Syndrome and Laboratory Markers

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Connective Tissue Disease

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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Correspondence to Athanasios G. Tzioufas MD .

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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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