Rheumatoid Arthritis Health Center

Font Size

Arthritis: Sjögren's Syndrome

Sjögren's syndrome is a chronic disorder that causes insufficient moisture production in certain glands of the body. It occurs when a person's normally protective immune system attacks and destroys moisture-producing glands, including salivary glands and tear glands. The lungs, bowel, and other organs are occasionally affected.

Sjögren's syndrome is named after the Swedish eye doctor, Henrik Sjögren, who first described the condition.

Sjögren's syndrome may cause the following symptoms:

Extremely dry eyes causing a feeling of grit or sand in the eyes or a burning redness.
Extremely dry mouth and throat causing difficulty with chewing and swallowing, decreased sense of taste, difficulty speaking, an increase in dental cavities, and dry cough or hoarseness.
Enlarged parotid glands (located at the angle of jaw) and sometimes infection of these glands.
Excessive fatigue
Aches and pains in muscles and joints

Less common features of Sjögren's syndrome are:

Irritation of the nerves in the arms, hands, legs or feet (neuropathy).
Thyroid gland abnormalities
Skin rashes
Memory loss or confusion.
Numbness or tingling.
Gastrointestinal problems.
Inflammation of the lungs, kidneys, liver or pancreas.
Cancer of the lymphatic tissue (lymphoma) occurs in less than 1% of patients.

What Causes Sjögren's Syndrome?

Normally, the immune system (the body's defense against invaders) protects the body from infection and foreign substances such as bacteria and viruses.

In autoimmune diseases, such as Sjögren's syndrome, the immune system triggers an inflammatory response when there are no foreign substances to fight off. This inflammatory response causes the body's white blood cells to attack and destroy certain moisture-producing glands.

The exact cause for the abnormal immune response in Sjögren's syndrome is unknown. Some theories suggest that a virus or bacteria may alter the immune system, causing it to attack the glands. Some people may have a genetic or inherited factor that makes them more likely to develop Sjogren's syndrome.

What Are the Forms of the Disease?

Sjogren's syndrome occurs in two basic forms: primary Sjögren's syndrome - the disease by itself and not associated with any other illness; and secondary Sjögren's syndrome - disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, lupus, or vasculitis.

As many as 4 million people in the U.S. have Sjögren's syndrome. More than 90% of these are women. The disease can affect people of any race or age, although the average age of onset is in the late 40s.

How Is Sjögren's Syndrome Diagnosed?

The diagnosis of primary Sjögren's syndrome is based on several factors, including:

Presence of dry eyes and mouth. An ophthalmologist can detect dry eyes by measuring tear production or carefully examining the cornea of the eye.
Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms. Examples include the presence of auto-antibodies in the blood, known as anti-SSA or anti-SSB (also called anti-Ro or anti-La).
Biopsy of the inner lip (performed in some cases to prove the diagnosis of primary Sjögren's syndrome). The biopsy may show inflammation damaging salivary glands.

Secondary Sjögren's syndrome is generally diagnosed when someone with an established autoimmune disease, such as rheumatoid arthritis or lupus, develops extreme dryness of the eyes and mouth. This diagnosis only rarely requires a lip biopsy.

1 | 2

Next Page >

WebMD Medical Reference provided in collaboration with the Cleveland Clinic

Take Charge of Your RA Treatment

Click to View Medication Guide

Important Safety Information you should know about HUMIRA® (adalimumab).

Serious infections have happened in patients receiving HUMIRA. These infections include TB (tuberculosis) and infections caused by viruses, fungi, or bacteria that have spread throughout the body. Some patients have died from these infections.

Before starting HUMIRA:

Tell your doctor if you think you have an infection, are being treated for an infection, have signs of an infection (such as a fever, cough, or flu-like symptoms), have any open sores on your body, have warm, red, or painful skin, get a lot of infections or have infections that keep coming back, have or had hepatitis B infection, take the medicine Kineret (anakinra), have TB or have been in close contact with someone who has TB, have lived in an area where TB or histoplasmosis is common, or were born in, lived in or traveled where there is more risk for getting TB. Your doctor should test you for TB before starting HUMIRA. If your doctor prescribes any medicine for the treatment of TB, you should start taking it before starting HUMIRA and take the full course of TB medicine prescribed.

Tell your doctor if you have any numbness or tingling, or have a disease that affects your nervous system such as multiple sclerosis or Guillain-Barré syndrome, have heart failure or other heart conditions, are scheduled for major surgery, are pregnant, become pregnant, plan to become pregnant or are breastfeeding. Tell your doctor if you are allergic to HUMIRA or any of its ingredients or are allergic to rubber or latex. The needle cover of the prefilled syringe and the pen contain dry natural rubber.

Also, tell your doctor if you have recently received or are scheduled for any vaccines. Except for live vaccines, patients may still receive vaccines while on HUMIRA. It is recommended that children with juvenile idiopathic arthritis be brought up to date with all immunizations prior to starting HUMIRA.

After starting HUMIRA:

Call your doctor right away if you get an infection, or any sign of an infection including a fever, feeling very tired, cough, flu-like symptoms, warm, red or painful skin or if you have any open sores on your body. HUMIRA can make you more likely to get infections or make any infection that you may have worse.

Possible side effects of HUMIRA:

Serious side effects, which sometimes lead to death, have happened in patients taking HUMIRA.

- Serious infections. These infections include TB (tuberculosis) and infections caused by viruses, fungi, or bacteria. Your doctor will examine you for TB and perform a test to see if you have TB. If your doctor feels that you are at risk for TB, you may be treated with medicine for TB before you begin treatment with HUMIRA and during treatment with HUMIRA. Even if your TB test is negative your doctor should carefully monitor you for TB infections while you are taking HUMIRA. Patients who had a negative TB skin test before receiving HUMIRA have developed active TB. Tell your doctor if you have any of the following symptoms while taking or after taking HUMIRA: cough, low-grade fever, weight loss, or loss of body fat and muscle.
- Certain types of cancer. There have been cases of certain kinds of cancer in patients taking HUMIRA or other TNF blockers. Patients with RA, especially more serious RA, may have a higher chance for getting a kind of cancer called lymphoma. Some patients receiving HUMIRA have developed types of cancer called non-melanoma skin cancer (basal cell cancer and squamous cell cancer of the skin), which are generally not life threatening if treated. Tell your doctor if you have a bump or open sore that doesn't heal.
- Allergic reactions. Signs of a serious allergic reaction include skin rash, a swollen face, or trouble breathing.
- Hepatitis B virus reactivation in patients that carry the virus in their blood. Tell your doctor if you have any of the following symptoms: feel unwell, poor appetite, fatigue, fever, rash or joint pain.
- Nervous system problems. Signs and symptoms include: numbness or tingling, problems with your vision, weakness in your arms or legs, and dizziness.
- Blood problems. Symptoms include a fever that does not go away, bruising or bleeding very easily, or looking very pale.
- New heart failure or worsening heart failure you already have. Symptoms include shortness of breath or swelling of your ankles or feet, or sudden weight gain.
- Immune reactions including a lupus-like syndrome. Symptoms include chest discomfort or pain that does not go away, shortness of breath, joint pain, or rash on your cheeks or arms that gets worse in the sun.

Call your doctor or get medical care right away if you develop any of the above symptoms. Your treatment with HUMIRA may be stopped.

Common side effects of HUMIRA are: injection site reactions (redness, rash, swelling, itching or bruising), upper respiratory infections (sinus infections), headaches, rash and nausea.

These are not all the side effects with HUMIRA. Ask your doctor or pharmacist for more information.

Information You Should Know About HUMIRA® (adalimumab).

HUMIRA is used to reduce the signs and symptoms of moderate to severe rheumatoidarthritis in adults, may prevent further damage to your bones and joints, and may help your ability to perform daily activities. HUMIRA can be used alone or with methotrexate or with certain other medicines. HUMIRA is used to reduce the signs and symptoms of moderate to severe polyarticular juvenile idiopathic arthritis in children 4 years of age and older. HUMIRA can be used alone or with methotrexate or with certain other medicines. HUMIRA is used to reduce the signs and symptoms of psoriatic arthritis in adults, may prevent further damage to your bones and joints, and may help your ability to perform daily activities. HUMIRA can be used alone or with certain other medicines. HUMIRA is used to reduce the signs and symptoms of ankylosing spondylitis in adults. HUMIRA is used to reduce the signs and symptoms of moderate to severe Crohn's disease in adults who have not responded well to conventional treatments. HUMIRA is also for these adults with moderate to severe Crohn's disease who have lost response or are unable to tolerate infliximab. HUMIRA is used to treat moderate to severe chronic (lasting a long time) plaque psoriasis in adults who are under the ongoing care of a physician, have the condition in many areas of their body and who may benefit from taking injections or pills (systemic therapy) or phototherapy (treatment using ultraviolet light alone or with pills).

HUMIRA is taken by injection.

64S-154126