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Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome
Sjögren syndrome is an autoimmune disorder in which the glands that produce tears and saliva are destroyed. This causes dry mouth and dry eyes. The condition may affect other parts of the body, including the kidneys and lungs.
The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most often in women ages 40 to 50. It is rare in children.
Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder.
Secondary Sjögren syndrome occurs along with another autoimmune disorder, such as:
Dry eyes and dry mouth are the most common symptoms of this syndrome.
Mouth and throat symptoms:
Other symptoms may include:
A complete physical exam will be done. The exam reveals dry eyes and dry mouth. There may be mouth sores, decayed teeth or gum inflammation. This occurs because of the mouth dryness. Your health care provider will look in your mouth for fungus infection (candida). Skin may show a rash, the lung exam may be abnormal, the abdomen will be palpated for liver enlargement. The joints will be examined for arthritis. The neuro exam will look for deficits.
You may have the following tests done:
The goal is to relieve symptoms.
Disease-modifying antirheumatic drugs (DMARDs) similar to those used for RA may improve the symptoms of Sjögren syndrome. These include tumor necrosis factor (TNF) inhibiting drugs such as Enbrel, Humira or Remicaide.
Some things you can do to ease symptoms include:
Talk with your dentist about:
To prevent dental decay caused by mouth dryness:
The disease is most often not life-threatening. The outcome depends on what other diseases you have.
There is a higher risk for lymphoma and early death when Sjögren syndrome has been very active for a long time, as well as in people with vasculitis, low complements, and cryoglobulins.
Complications may include:
Call your provider if you develop symptoms of Sjögren syndrome.
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Mariette X. Sjögren syndrome. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 268.
Seror R, Bootsma H, Saraux A, et al. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis. 2016;75(2):382-389. PMID: 25480887 www.ncbi.nlm.nih.gov/pubmed/25480887.
Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2016;55(3):450-460. PMID: 26412810 www.ncbi.nlm.nih.gov/pubmed/26412810.
Turner MD. Oral manifestations of systemic diseases. In: Flint PW, Haughey BH, Lund V, et al, eds. Cummings Otolaryngology. 6th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 14.BACK TO TOP
Review Date: 4/8/2019
Reviewed By: Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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