Preface

Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease associated primarily with symptoms of the eye and mouth dryness. In the literature, the term "autoimmune epithelitis" is also used to describe this disease, drawing attention to the central role of the epithelial cells in its pathogenesis.

Although exocrine glands are the main target of pSS, epithelial tissue, which constitutes a component of other organs such as kidneys, liver, lungs, or biliary ducts, is also affected, which leads to various extraglandular manifestations of the disease. The autoimmune process, which underlies the pathogenesis of pSS through the overstimulation of B cells, results in an increased risk of the development of lymphomas. Diagnosis of pSS is often very distant in time from the emergence of this condition, while effects of the long-term autoimmune processes taking place meanwhile can be irreversible.

The classification criteria of pSS were modified in recent years, in particular in light of the expanding knowledge of immunology and because of the separate group of Ig-G4-related diseases. Diagnosis of pSS must be established taking into account other epithelial autoimmune diseases, such as primary biliary cholangitis, which may coexist with pSS or occur independently mimicking pSS symptoms.

The aim of this book is to present pSS as a complex disease entity, as well as to present other autoimmune diseases in which the autoimmunity affects mainly epithelial cells and exocrine glands. The book combines the expertise of specialists in different fields of medicine—ophthalmologists, laryngologists, rheumatologists—to discuss various problems associated with Sjögren's syndrome and other autoimmune diseases with autoimmune epithelitis.

> **Dr. Maria Maślińska** National Institute of Geriatrics, Rheumatology and Rehabilitation, Early Arthritis Clinic, Warsaw, Poland

**1**

Section 1

Interdisciplinary Aspects

of Primary Sjogren's

Syndrome
