Sjögren’s syndrome

Czech version

Authors: O. Kryštůfková
Authors‘ workplace: Revmatologický ústav Praha
Published in: Čes. Revmatol., 25, 2017, No. 1, p. 26-43.
Category:

Overview

Sjögren’s syndrome (SS) is a chronic, systemic autoimmune disease that affects exocrine glands. The dominating feature is mucosal dryness, but the clinical spectrum includes wide range of systemic complications. The heterogeneity of SS manifestations leads to continuous searching for appropriate diagnostic and classification criteria and improvement of the disease activity assessment approaches which could cover the whole symptomatology of disease. The diagnostic criteria are missing, but we have several systems for classification of SS. American-European Consensus Group (AECG) criteria consisting of both subjective symptoms and objective signs have been widely accepted and used in clinical practice and clinical trials since 2002. The alternative classification criteria including minimal set of three objective features were proposed by the American College of f Rheumatology (ACR) in 2012. New set of classification criteria for primary SS was recently developed and published last year, using guidelines from the ACR and the European League Against Rheumatism (EULAR) and includes features of both AECG and ACR criteria. The diversity of the glandular and extraglandular manifestations is also a reason for the failure of many clinical studies searching for the target therapy of SS. We are currently dependent on the symptomatic treatment of mucosal symptoms and in cases of severe organ involvement on the procedures established in other systemic rheumatic diseases.

Key words:
Sjögren’s syndrome, classification criteria, diagnostic criteria

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