Charting the course of Sjögren's disease: A historical analysis of scientific progress.

Abstract

Sjögren's disease (SjD) bears the name of the Swedish ophthalmologist Henrik Sjögren. His 1933 doctoral thesis on keratoconjunctivitis sicca ignited interest, yet much remains under debate. SjD is an autoimmune disorder that primarily affects the exocrine glands, particularly the salivary and lacrimal glands, leading to a dryness of the eyes and mouth. Diagnostic criteria have evolved significantly, with over 10 classification criteria proposed since 1965. From its inception, SjD was often overshadowed by other rheumatic diseases, struggling for recognition as a distinct autoimmune entity. Early research in the 1960s and 1970s led to inconsistent nomenclature for autoantibodies, further complicating understanding. Although Sjögren studied biopsies early on, standardized approaches to biopsy sites and histological findings took considerable time to establish. Recent advancements, such as the introduction of salivary gland ultrasound as a non-invasive diagnostic tool, have offered new avenues for investigation. However, challenges persist in accurately assessing disease activity due to the wide range of manifestations. This narrative and historical review explores the evolution of Sjögren's syndrome as a distinct disease, tracing its history from initial descriptions to contemporary developments.