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 Sj ö gren syndrome is 9:1.2
Age
Peak incidence occurs in the fourth and fifth decades of life.
Clinical
History
Onset of Sj ö gren 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 common.
- Musculoskeletal (60%)
- Fatigue (60%)
- Arthralgias (60-70%)
- 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
- Glomerulonephritis
- 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.
More on Sjogren Syndrome |
 Overview: Sjogren Syndrome |
| Differential Diagnoses & Workup: Sjogren Syndrome |
| Treatment & Medication: Sjogren Syndrome |
| Follow-up: Sjogren Syndrome |
| Multimedia: Sjogren Syndrome |
| References |
| Next Page » |
References
Carsons S. Sjögren's Syndrome. In: Firestein GS, Budd RC, Harris ED, McInnes IB, Ruddy S, Sergent JS, eds. Kelley's Textbook Of Rheumatology. Vol 2. 8th ed. Philadelphia: W.B. Saunders Company; 2006:chap 69.
Mavragani CP, Moutsopoulos HM. The geoepidemiology of Sjögren's syndrome. Autoimmun Rev. Nov 7 2009;[Medline].
Isenberg DA. Systemic lupus erythematosus and Sjögren's syndrome: historical perspective and ongoing concerns. Arthritis Rheum. Mar 2004;50(3):681-3. [Medline].
Moutsopoulos, HM. Sjögren's syndrome. In: Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 17th ed. New York: McGraw-Hill; 2008:chap 317.
Ramos-Casals M, Brito-Zeron P, Garcia-Carrasco M, Font J. Sarcoidosis or Sjogren syndrome? Clues to defining mimicry or coexistence in 59 cases. Medicine (Baltimore). Mar 2004;83(2):85-95. [Medline].
Weber J, Keating GM. Cevimeline. Drugs. 2008;68(12):1691-8. [Medline].
Pijpe J, Meijer JM, Bootsma H, van der Wal JE, Spijkervet FK, Kallenberg CG, et al. Clinical and histologic evidence of salivary gland restoration supports the efficacy of rituximab treatment in Sjögren's syndrome. Arthritis Rheum. Nov 2009;60(11):3251-6. [Medline].
Talal N. Sjögren's syndrome: historical overview and clinical spectrum of disease. Rheum Dis Clin North Am. Aug 1992;18(3):507-15. [Medline].
Further Reading
Keywords
Sjogrens syndrome, Sjogrens symptoms, Sjogrens causes, Sjogrens treatment, Sjogren's, Sjogrens, Sjogren's syndrome, autoimmune disease, Sjögren syndrome, Sjögren's syndrome, sicca complex, Gougerot-Sjögren disease, sicca syndrome, Sjögren disease, Sjögren's disease, xerostomia, dry mouth, xerophthalmia, dry eyes, systemic lupus erythematosus, SLE, rheumatoid arthritis, scleroderma, systemic sclerosis, cryoglobulinemia, polyarteritis nodosa, rheumatologic disorder
