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Sjogren Syndrome
Author: Darren Phelan, MD, Medical Director, Department of Emergency Medicine, Sierra Nevada Memorial Hospital
Updated: Nov 20, 2009

Introduction

Background
Sjögren syndrome is a chronic autoimmune disorder characterized by xerostomia (dry mouth), xerophthalmia (dry eyes), and lymphocytic infiltration of the exocrine glands. This triad is also known as the sicca complex.

Sjögren syndrome displays a wide spectrum of severity and can go undiagnosed for several months to years. The Swedish physician Henrik Sjögren first described the syndrome in 1933.1

For more information, see Medscape's Sjögren's Syndrome Resource Center
Pathophysiology

Sjögren syndrome can occur as a primary disease of exocrine gland dysfunction or in association with several other autoimmune diseases (eg, systemic lupus erythematosus [SLE], rheumatoid arthritis, scleroderma, systemic sclerosis, cryoglobulinemia, polyarteritis nodosa). These primary and secondary types occur with similar frequency, but the sicca complex seems to cause more severe symptoms in the primary form.

Virtually all organs may be involved. The disease commonly affects the eyes, mouth, parotid gland, lungs, kidneys, skin, and nervous system.

Frequency

United States
Sjögren syndrome is estimated to be the second most common rheumatologic disorder, behind only SLE.

International
Comparative studies between different ethnic groups have suggested that Sjögren syndrome is a homogeneous disease that occurs worldwide with similar prevalence and affects 1-2 million people.

Mortality/Morbidity
• Although the disease process rarely shortens a patient's lifespan, morbidity can be devastating. Ocular dryness can lead to chronic keratoconjunctivitis and corneal ulcers. Oral dryness leads to caries, fissures, candidal infections, and difficulty speaking and swallowing food. These patients often are undiagnosed or misdiagnosed for years. They often feel clinicians are uneducated or unsympathetic to their disease.
• As a result of the lymphocytic infiltration, 10% of patients may develop pseudolymphoma, a lymphoproliferative process. Approximately 10% of these patients can develop non-Hodgkin lymphoma (1% of all patients with Sjögren syndrome).

Sex
The female-to-male ratio of Sjögren syndrome is 9:1.2

Age
Peak incidence occurs in the fourth and fifth decades of life.

Clinical

History
Onset of Sjögren syndrome is insidious, and symptoms may be mild.
• Dry eyes and/or mouth (required for the clinical diagnosis); dry mouth is shown in the image below
• Sjögren syndrome. Dryness of the mouth and tongue due to lack of salivary secretion is characteristic of xerostomia associated with Sjögren syndrome. Mouth dryness may produce a deep red tongue, as shown here, and dental caries are cSjögren syndrome. Dryness of the mouth and tongue due to lack of salivary secretion is characteristic of xerostomia associated with Sjögren syndrome. Mouth dryness may produce a deep red tongue, as shown here, and dental caries are common.
• Musculoskeletal (60%)
o Fatigue (60%)
o Arthralgias (60-70%)
o Myalgias (20-30%)
• Dry skin (40%)
• Vaginal dryness (40%)

Physical
The Sj ö gren syndrome may be mild or hard to detect clinically.
• Keratoconjunctivitis
• Salivary insufficiency
• Vasculitis (25%)
• Lymphadenopathy (20%)
• Polyneuropathies (10-15%)
• Renal involvement
o Glomerulonephritis
o Interstitial nephritis
• Parotid gland enlargement (usually bilateral),3 shown in the image below
•

Sjögren syndrome. Marked bilateral parotid gland enlargement in a patient with primary Sjögren syndrome. Sicca syndrome is a common clinical finding.