Sjögren's syndrom – the review of the latest diagnostic guidelines essential for otolaryngologists

Abstract

Sjogren's syndrome (SS) is a complex connective tissue disease with autoimmune background and high clinical, radiological and molecular heterogeneity. SS is typically manifested by sicca syndrome, characterized by dry eyes and dry mouth due to autoimmune-induced inflammation of the lacrimal and salivary glands. Complications of sicca syndrome are dental caries, oral candidiasis, dysosmia, dysgeusia, difficulties in swallowing and chewing. SS may coexist with other diseases of rheumatoid and autoimmune etiology. SS is linked to a 16-fold increased risk of non-Hodgkin lymphoma. Early diagnosis results in inappropriate treatment and may slow down the course of the disease and limit extraglandular involvement. Due to diverse clinical phenotypes and symptomatology, establishing the diagnosis is often difficult. In 2016 the American-European Consensus Group (AECG) and European League Against Rheumatism (EULAR) proposed a classification system that defines SS as a systemic disease. Diagnostic tools in establishing SS diagnosis are serological tests, ultrasonography, Schirmer’s test, unstimulated whole saliva flow rate, and Ocular Staining Score. The complete curing of SS is still not possible. As a complex multisystem disease, SS requires multidisciplinary cooperation and individual diagnostic and therapeutic approaches in patients. Therapy is focused on the treatment of symptoms and prophylaxis of complications. The laryngological treatment of oral cavity symptoms in SS includes supervision of proper oral hygiene habits and adequate fluids supplementation. The EULAR Sjögren's syndrome disease activity index (ESSDAI) and Clinical Oral Dryness Score(CODS) is used to monitor disease progression and treatment effectiveness.