Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Sjögren’s disease(formerly known as Sjögren’s syndrome) is anautoimmune diseasethat affects moisture-producing glands and tissues, causing dry eyes and mouth, joint pain, mouth infections, and other chronic (long-lasting) symptoms. Over time, the lack of moisture can start to damage the lungs, kidneys, and nervous system.
The diagnosis of Sjögren’s disease involves multiple tests, including blood tests that can detect autoimmune antibodies. The treatment may involve immunosuppressant drugs like steroids as well as eye drops and oral painkillers for symptomatic relief.
This article describes the symptoms and causes of Sjögren’s disease, including how this relatively common autoimmune disease is diagnosed and treated.
What Are the Symptoms of Sjögren’s Disease?
The symptoms develop when moisture-producing glands and tissues sustain damage due to the autoimmune assault. This includessalivary glandsthat produce saliva,lacrimal glandsthat produce tears, andmucosal membranesthat produce mucus.
The severity of symptoms can vary, with some people only experiencing eye irritation while others develop serious complications.
Common signs and symptoms of Sjögren’s disease include:
Complications
In some people, the decreased output of saliva and mucus can start to damage tissues and organs over time, leading to complications like:
Sjögren’s disease is closely associated and often co-occurs with other autoimmune diseases likelupus,celiac disease,multiple sclerosis,Hashimoto’s disease,rheumatoid arthritis,scleroderma, andpolymyositis.
Sjögren’s also appears to increase your risk ofnon-Hodgkin’s lymphoma, a type of cancer affecting the lymphatic system.
DefinitionsPrimary Sjögren’s disease is diagnosed if you do not have another autoimmune disease. Associated Sjogren’s disease is when it occurs along with another autoimmune disease.
Definitions
Primary Sjögren’s disease is diagnosed if you do not have another autoimmune disease. Associated Sjogren’s disease is when it occurs along with another autoimmune disease.
What Causes Sjögren’s Disease?
Sjögren’s disease is one of the most common autoimmune disorders affecting as many as four million people in the United States. Around 90% of cases are in females. People of any age can get it, although the average age of onset is around 40.
The cause of Sjögren’s disease is unknown. For reasons not entirely clear, the immune system will suddenly attack moisture-producing glands and tissues withchronic inflammation.
Genetics are thought to predispose certain people to Sjögren’s, with six gene mutations currently identified:RF5, STAT4, BLK, IL12A, TNIP1,andCXCR5.
Having these gene mutations does not mean you will automatically get Sjögren’s disease; other factors are thought to “trigger” the disease, including infection with theEpstein-Barr virusandhepatitis C virusand hormonal abnormalities likeestrogen deficiency(which starts to decline steeply in females after the age of 40).
Having another autoimmune disease also increases your risk of Sjögren’s disease.
How Is Sjögren’s Disease Diagnosed?
Because the symptoms are somewhat vague, it can take years for Sjögren’s to be diagnosed. Your symptoms, physical examination, specialized tests, and blood tests can help identify the condition.
Your healthcare provider or dentist may observe that your mouth is dry. Your nose and skin may appear dry and your eyes may also appear red or dry. Your joints may appear swollen, and you may have a rash on your skin.
Lab Tests and Procedures
Along with your symptoms and physical examination, several diagnostic tests may help confirm the diagnosis of Sjögren’s disease, including:
Blood Antibody Tests
Several blood tests can be used to identify certain antibodies commonly seen in people with Sjögren’s disease. These include:
How Is Sjögren’s Disease Treated?
There is no cure for Sjögren’s disease. However, the disease can be managed with medications that decrease the inappropriate immune response and over-the-counter or prescription remedies that help ease symptoms.
Treatment is tailored for each individual, depending on which parts of the body are affected and how severe the symptoms are. For example, joint pain or muscle pain may be managed with non-steroidal anti-inflammatory drugs (NSAIDs), while serious complications such as lymphoma need to be treated with chemotherapy.
Immunosuppressive therapy is often used to reduce the inflammatory process that damages the glands, joints, and organs in Sjögren’s—this approach is often referred to as disease-modifying therapy.
Relief of Oral Symptoms
Mouthwashes, lip balm, saliva substitutes, sprays, gels, and gum can relieve dry mouth and tenderness. Medication options may include saliva and mucus-stimulating drugs, such as:
Relief of Eye Symptoms
There are some practical ways to manage dry eyes. Sunglasses can protect your eyes from wind and drafts outdoors, and a humidifier can relieve dry air indoors to help alleviate the symptoms. Smoke and eye makeup can be irritating and should be avoided.
Artificial tears and eye ointments can help relieve chronic dry eyes. Medication options for dry eye associated with Sjögren’s disease may include:
Disease-Modifying Drugs
The risk of lung, kidney, blood vessel, or nervous system complications can be reduced with medications that suppress the immune system. They also treat severe flare-ups (exacerbation) in people with Sjögren’s disease.
Disease-modifying treatments include:
These medications can cause side effects, including an increased risk of infection.
Summary
Sjögren’s disease is an autoimmune disease that affects moisture-producing glands and tissues, causing dry eyes, skin, mouth, and nose as well as fatigue, joint pain, and blurred vision. Over the long term, the disease can cause damage to the kidneys, lungs, and nerves in some people.
Sjögren’s disease is diagnosed with multiple tests, including blood antibody tests. Certain disease-modifying drugs can temper the overactive immune response. Eye drops, oral pain relievers, and mucus-stimulating medications may help ease symptoms.
3 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Both T, Dalm VA, Van Hagen PM, Van Haele PL.Reviewing primary Sjögren’s syndrome: beyond the dryness - from pathophysiology to diagnosis and treatment.Int J Med Sci.2017;14(3):191-200. doi:10.7150/ijms.17718Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T.The diagnosis and treatment of Sjögren’s syndrome.Dtsch Arztebl Int. 2017;114(20):354-361. doi:10.3238/arztebl.2017.0354Sjogren’s Syndrome Foundation.About Sjogren’s syndrome | diagnosis.Additional ReadingAbout Sjogren’s Syndrome. Sjogren’s Syndrome Foundation.Carsons SE, Vivino FB, Parke A, Carteron N, Sankar V, Brasington R, et al.Treatment Guidelines for Rheumatologic Manifestations of Sjögren’s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain.Arthritis Care Res (Hoboken). 2017 Apr;69(4):517-527. doi: 10.1002/acr.22968. Epub 2017 Mar 3.Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S.Pulmonary manifestations of Sjögren’s syndrome.Eur Respir Rev. 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016.Rodrigues AR, Soares R.Inflammation in Sjögren’s syndrome: Cause or consequence?Autoimmunity. 2017 May;50(3):141-150. doi: 10.1080/08916934.2017.1280027. Epub 2017 Feb 14.
3 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Both T, Dalm VA, Van Hagen PM, Van Haele PL.Reviewing primary Sjögren’s syndrome: beyond the dryness - from pathophysiology to diagnosis and treatment.Int J Med Sci.2017;14(3):191-200. doi:10.7150/ijms.17718Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T.The diagnosis and treatment of Sjögren’s syndrome.Dtsch Arztebl Int. 2017;114(20):354-361. doi:10.3238/arztebl.2017.0354Sjogren’s Syndrome Foundation.About Sjogren’s syndrome | diagnosis.Additional ReadingAbout Sjogren’s Syndrome. Sjogren’s Syndrome Foundation.Carsons SE, Vivino FB, Parke A, Carteron N, Sankar V, Brasington R, et al.Treatment Guidelines for Rheumatologic Manifestations of Sjögren’s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain.Arthritis Care Res (Hoboken). 2017 Apr;69(4):517-527. doi: 10.1002/acr.22968. Epub 2017 Mar 3.Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S.Pulmonary manifestations of Sjögren’s syndrome.Eur Respir Rev. 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016.Rodrigues AR, Soares R.Inflammation in Sjögren’s syndrome: Cause or consequence?Autoimmunity. 2017 May;50(3):141-150. doi: 10.1080/08916934.2017.1280027. Epub 2017 Feb 14.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Both T, Dalm VA, Van Hagen PM, Van Haele PL.Reviewing primary Sjögren’s syndrome: beyond the dryness - from pathophysiology to diagnosis and treatment.Int J Med Sci.2017;14(3):191-200. doi:10.7150/ijms.17718Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T.The diagnosis and treatment of Sjögren’s syndrome.Dtsch Arztebl Int. 2017;114(20):354-361. doi:10.3238/arztebl.2017.0354Sjogren’s Syndrome Foundation.About Sjogren’s syndrome | diagnosis.
Both T, Dalm VA, Van Hagen PM, Van Haele PL.Reviewing primary Sjögren’s syndrome: beyond the dryness - from pathophysiology to diagnosis and treatment.Int J Med Sci.2017;14(3):191-200. doi:10.7150/ijms.17718
Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T.The diagnosis and treatment of Sjögren’s syndrome.Dtsch Arztebl Int. 2017;114(20):354-361. doi:10.3238/arztebl.2017.0354
Sjogren’s Syndrome Foundation.About Sjogren’s syndrome | diagnosis.
About Sjogren’s Syndrome. Sjogren’s Syndrome Foundation.Carsons SE, Vivino FB, Parke A, Carteron N, Sankar V, Brasington R, et al.Treatment Guidelines for Rheumatologic Manifestations of Sjögren’s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain.Arthritis Care Res (Hoboken). 2017 Apr;69(4):517-527. doi: 10.1002/acr.22968. Epub 2017 Mar 3.Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S.Pulmonary manifestations of Sjögren’s syndrome.Eur Respir Rev. 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016.Rodrigues AR, Soares R.Inflammation in Sjögren’s syndrome: Cause or consequence?Autoimmunity. 2017 May;50(3):141-150. doi: 10.1080/08916934.2017.1280027. Epub 2017 Feb 14.
About Sjogren’s Syndrome. Sjogren’s Syndrome Foundation.
Carsons SE, Vivino FB, Parke A, Carteron N, Sankar V, Brasington R, et al.Treatment Guidelines for Rheumatologic Manifestations of Sjögren’s Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain.Arthritis Care Res (Hoboken). 2017 Apr;69(4):517-527. doi: 10.1002/acr.22968. Epub 2017 Mar 3.
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S.Pulmonary manifestations of Sjögren’s syndrome.Eur Respir Rev. 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016.
Rodrigues AR, Soares R.Inflammation in Sjögren’s syndrome: Cause or consequence?Autoimmunity. 2017 May;50(3):141-150. doi: 10.1080/08916934.2017.1280027. Epub 2017 Feb 14.
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