Introduction: History of Sjögren’s Syndrome

1

Introduction

Sjögren’s syndrome (SS) is a complex disorder characterized by both organ-specific and systemic manifestations, potentially expanding to lymphoproliferative complications. In addition to occurring as a primary or secondary disease, SS can occur in association with organ-specific autoimmune diseases, as well as in overlapping complex entities with the major connective tissue diseases.

SS is named after the Swedish ophthalmologist Henrik Sjögren, who presented his doctoral thesis in 1933. However, the historical traces of SS can be found in a number of case reports published during the 19th century before Henrik Sjögren. Over the years, SS has been described first as an organ-specific disorder involving salivary and lachrymal glands, and then as a systemic autoimmune disease displaying significant extraglandular manifestations.

Norman Talal has suggested that the history of SS can be subdivided into three phases : firstly, the initial definition of SS through a series of reports documenting its glandular manifestations; secondly, beginning in the 1950s, the second definition of SS as an autoimmune disorder potentially involving different organs and systems; and thirdly, since the 1980s, the era of molecular biology.