Sjogren syndrome
Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome; Sjögren syndrome; Sjögren's syndromeSjögren 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.
Autoimmune disorder
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 autoimmune d...
Read Article Now Book Mark ArticleCauses
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:
- Rheumatoid arthritis (RA)
Rheumatoid arthritis
Rheumatoid arthritis (RA) is a disease that leads to inflammation of the joints and surrounding tissues. It is a long-term disease. It can also aff...
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Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It c...
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Scleroderma
Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the cells that line the ...
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Polymyositis
Polymyositis and dermatomyositis are rare inflammatory diseases. (The condition is called dermatomyositis when it involves the skin. ) These disease...
Read Article Now Book Mark Article - Hepatitis C can affect the salivary glands and looks like Sjögren syndrome
- IgG4 disease can look like Sjögren syndrome and should be considered
Symptoms
Dry eyes and dry mouth are the most common symptoms of this syndrome.
Eye symptoms:
- Itching eyes
Itching eyes
Eye burning with discharge is burning, itching, or drainage from the eye of any substance other than tears.
Read Article Now Book Mark Article - Burning sensation in eyes
- Feeling that something is in the eye
Mouth and throat symptoms:
- Difficulty swallowing or eating dry foods
- Loss of sense of taste
Loss of sense of taste
Taste impairment means there is a problem with your sense of taste. Problems range from distorted taste to a complete loss of the sense of taste. A...
Read Article Now Book Mark Article - Problems speaking
- Thick or stringy saliva
- Mouth sores or pain
- Teeth decay and gum inflammation
- Hoarseness
Hoarseness
Hoarseness refers to difficulty making sounds when trying to speak. Vocal sounds may be weak, breathy, scratchy, or husky, and the pitch or quality ...
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Other symptoms may include:
- Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
Read Article Now Book Mark Article - Fever
- Change in the color of hands or feet with cold exposure (Raynaud phenomenon)
- Joint pain or joint swelling
Joint swelling
Joint swelling is the buildup of fluid in the soft tissue surrounding the joint or the joint itself.
Read Article Now Book Mark Article - Swollen glands
Swollen glands
Lymph nodes are present throughout your body. They are an important part of your immune system. Lymph nodes help your body recognize and fight germ...
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- Numbness and pain due to neuropathy
- Cough and shortness of breath due to lung disease
- Irregular heartbeat
- Nausea and heartburn
- Vaginal dryness or painful urination
Exams and Tests
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 neurologic exam will look for deficits due to nerve damage.
Mouth sores
There are different types of mouth sores. They can occur anywhere in the mouth including bottom of the mouth, inner cheeks, gums, lips, and tongue....
Read Article Now Book Mark ArticleYou may have the following tests done:
- Complete blood chemistry with liver enzymes
- Complete blood count with differential
- Urinalysis
- Antinuclear antibodies (ANA) test
Antibodies
An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...
Read Article Now Book Mark ArticleANA
The antinuclear antibody panel is a blood test that looks at antinuclear antibodies (ANA). ANA are antibodies produced by the immune system that bind...
Read Article Now Book Mark Article - Anti-Ro/SSA and anti-La/SSB antibodies
- Rheumatoid factor
Rheumatoid factor
Rheumatoid factor (RF) is a blood test that measures the amount of the RF antibody in the blood.
Read Article Now Book Mark Article - Anti-cyclic citrullinated peptide (CCP) antibody
- Test for cryoglobulins
- Complement levels
- Protein electrophoresis
- Test for hepatitis C and HIV (if at risk)
- Thyroid tests
- Schirmer test of tear production
Schirmer test
The Schirmer test determines whether the eye produces enough tears to keep it moist.
Read Article Now Book Mark Article - Imaging of the salivary gland: by ultrasound or by MRI
- Salivary gland biopsy
Salivary gland biopsy
Salivary gland biopsy is the removal of cells or a piece of tissue from a salivary gland for exam.
Read Article Now Book Mark Article - Skin biopsy if a rash is present
- Examination of the eyes by an ophthalmologist
- Chest x-ray
Treatment
The goal is to relieve symptoms.
- Dry eyes may be treated with artificial tears, eye-lubricating ointments, or cyclosporine liquid.
- If Candida is present, it may be treated with sugar-free miconazole or nystatin preparations.
- Tiny plugs can be placed in the tear drainage ducts to help the tears stay on the surface of the eye.
Oral medicines such as pilocarpine or cevimeline that cause increased saliva production can be useful in dryness when local therapy is not helping.
Disease-modifying antirheumatic drugs (DMARDs) similar to those used for RA may improve the symptoms of Sjögren syndrome. These include Rituximab and tumor necrosis factor (TNF) inhibiting drugs such as etanercept (Enbrel), adalimumab (Humira) or infliximab (Remicade).
Some things you can do to ease symptoms include:
- Sip water throughout the day
- Chew sugarless gum
- Avoid medicines that can cause mouth dryness, such as antihistamines and decongestants
- Avoid alcohol
Talk with your dentist about:
- Mouth rinses to replace minerals in your teeth
- Saliva substitutes
- Drugs that help your salivary glands make more saliva
To prevent dental decay caused by mouth dryness:
- Brush and floss your teeth often
- Visit the dentist for regular checkups and cleanings
Outlook (Prognosis)
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.
Lymphoma
Hodgkin lymphoma is a cancer of lymph tissue. Lymph tissue is found in the lymph nodes, spleen, liver, bone marrow, and other sites.
Read Article Now Book Mark ArticlePossible Complications
Complications may include:
- Damage to the eye
- Dental cavities
- Kidney failure (rare)
Kidney failure
Acute kidney failure is the rapid (less than 2 days) loss of your kidneys' ability to remove waste and help balance fluids and electrolytes in your b...
Read Article Now Book Mark Article - Lymphoma
- Pulmonary disease
- Vasculitis (rare)
- Neuropathy
- Bladder inflammation
When to Contact a Medical Professional
Contact your provider if you develop symptoms of Sjögren syndrome.
References
Baer AN, Verstappen GM. Sjögren syndrome. In: Hochberg MC, Gravallese EM, Smolen JS, van der Heijde D, Weinblatt ME, Weisman MH, eds. Rheumatology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 146.
Mariette X, Nocuturne C. Sjogren syndrome. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 252.
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 pubmed.ncbi.nlm.nih.gov/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 pubmed.ncbi.nlm.nih.gov/26412810/.
Turner MD. Oral manifestations of systemic diseases. In: Flint PW, Francis HW, Haughey BH, et al, eds. Cummings Otolaryngology: Head and Neck Surgery. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 12.
Antibodies - illustration
Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.
Antibodies
illustration
Antibodies - illustration
Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.
Antibodies
illustration
Review Date: 4/30/2023
Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, New York, NY, and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.