Robert W. Hoffman

Sjögren’s Syndrome

The disease entity now widely recognized by the name Sjogren’s syndrome (SS) was first fully characterized by Henrik Sjogren, who in 1933 described 13 women with xerostomia, keratoconjunctivitis, and rheumatoid arthritis (RA) (1). Although SS is now widely recognized, the differences between classification criteria for the disease that was proposed by investigators from the United States, Europe, and Japan have yet to be resolved (2–6). The disease is characterized by signs and symptoms attributable to infiltration and destruction of lacrimal and salivary glands by inflammatory cells, resulting in ocular and oral dryness. Systemic symptoms including fatigue are now also recognized to be significant features of SS. Extraglandular features of SS that may occur include: arthralgia, arthritis, Raynaud’s phenomenon, myalgia, pulmonary disease, gastrointestinal (GI) disease, leukopenia, anemia, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, and lymphoma (7,8). SS can occur as a primary disease entity or be secondary to another disease, most commonly a rheumatic disease. SS appears to be a common disease having prevalence similar to that of RA. Prevalence rates of 0.05–3.00% have been published using different criteria for disease classification among different study populations (9–12). Features indistinguishable from classically defined primary SS have been recently reported associated with viral infections with hepatitis C virus (HCV) (13,14), the human immunodeficiency virus (HIV) (15), and the human T cell lymphotrophic virus (HTLV) (16).