Sjogren's is a chronic disorder of the immune system. It is an autoimmune condition, meaning that healthy tissues and cells are mistakenly attacked by the immune system.
It happens when white blood cells infiltrate saliva glands, tear glands, and other exocrine tissues, leading to decrease in tear and saliva production. This can lead to dryness in the mouth, eyes, skin, nose, upper respiratory tract, and vagina.
Primary Sjogren's develops alone, but secondary Sjogren's co-occurs with another condition, such as lupus. The symptoms are similar, and both can be severe.
Sjogren's is a serious condition, but timely treatment can mean that complications are less likely to develop, and tissue damage is less likely to occur. Once treated, an individual can usually manage the condition well.
Sjogren's can develop at any age, but most diagnoses occur after the age of 40 years. In 90 percent of cases the patient is female, but it also affects, and can be under-diagnosed, in males.
It affects around 0.1 to 4 percent of people in the United States.
Fast facts on Sjogren's
- Sjogren's is an autoimmune disorder that affects moisture-producing glands.
- The condition affects 0.1 to 4 percent of people in the U.S., and 90 percent of people with Sjogren's are women.
- It causes the eyes and mouth to dry out, and can also lead to tooth decay, recurring oral thrush, and a persistent dry cough.
- Sjogren's affects multiple systems and can be difficult to diagnosis. However, blood tests, ophthalmological testing, and measuring the salivary flow rate can help identify the condition. A biopsy of the lower lip can be important in the diagnosis, especially if the hallmark autoantibodies SSA (Ro), and SSB (La) are negative, which can occur in up to 30% of biopsy positive cases.
- Prescribed eye drops, artificial tears, and moisture chamber spectacles can help to relieve dry eyes.
- Other medications can help to stimulate the flow of saliva. Chewing sugar-free gum can help lubricate the mouth, and numerous saliva substitutes are available to try.
Females may also report vaginal dryness.
Other symptoms can include:
- tooth decay, and eventual loss of teeth
- persistent dry mouth
- persistent dry cough
- problems chewing and swallowing
- hoarse voice
- difficulty speaking
- swollen salivary glands
- recurring oral thrush, a fungal infection in the mouth
The signs and symptoms associated with dry eyes include:
- a sensation in one or both eyes similar to the irritation caused by foreign material, such as sand or gravel
- tired-looking and heavy eyes
- an itchy sensation
- constant feeling of irritation in the eyes
- persistent dry eyes
- discharge of mucus from the eyes
- photophobia, or sensitivity to light
- stinging or burning eyes
- swollen and irritated eyelids
- blurred vision
Smoking, air travel, overhead fans, and air-conditioned or windy environments can exacerbate symptoms.
In some cases, the patient's immune system attacks other parts of the body, causing the following signs and symptoms:
- general tiredness
- brain fog
- aching muscles
- inflammation of joints, as well as stiffness and pain
- swelling in the salivary glands between the jaw and ears
- peripheral neuropathy, or numbness and occasional pain in the arms or legs
- small fiber neuropathy, which can be diagnosed by a skin biopsy
- lung disease (bronchiolitis, interstitial lung disease, cystic lung disease)
- Raynaud's phenomenon, in which the hands feel painful, cold and numb
- vasculitis, where the blood vessels become inflamed
There is no special diet for Sjogren's, unless the doctor suggests one.
Alcohol should be avoided, however. People may also wish to avoid foods that irritate the mouth, such as spicy or acidic items.
Sugar-free lozenges (xylitol) may help keep the mouth moist.
To make food easier to swallow:
- use sauces, olive oil, or dressings to lubricate food
- have a drink alongside your food
- use a drinking straw to make swallowing easier
- put cucumber in a sandwich, to add moisture
- increase fluid intake
The best option is to choose a balanced diet that is high in fresh fruit and vegetables and low in saturated fats and sugar. Some foods are believed to trigger an inflammatory reaction, such as artificial sweeteners. These may be best avoided.
Keeping a food diary may help pinpoint any specific foods that may cause a reaction or worsen symptoms.
Sjogren's treatment aims to lubricate the affected areas and prevent the onset of complications.
Treatment options include:
- Medications to stimulate saliva flow: These include pilocarpine and cevimeline. They have a short-term impact, limited to a few hours only, so several doses a day are usually required.
- Artificial saliva: Saliva substitutes and mouth-coating gels can relieve dryness of the mouth. They are available as sprays, pre-treated swabs, and liquids. These can be important at night, as the mouth becomes dryer during sleep.
- Artificial tears: Artificial tears can help to lubricate the eyes, and are available over-the-counter (OTC). Prescription options are available that reduce the need for artificial tears, such as cyclosporine ophthalmic emulsion and hydroxypropyl cellulose pellets.
- Prescription eye drops: These include cyclosporine and lifitegrast.
- Moisture chamber spectacles: These are special glasses that keep out irritants and retain moisture.
- Prescription expectorants and throat medications: In cases of respiratory dryness, drugs such as those used to stimulate saliva flow, as well as linseed extract, sorbitol, xylitol, or malic acid, can moisturize the area.
- Nonsteroidal anti-inflammatory drugs (NSAIDs): Anti-inflammatory drugs such as aspirin, naproxen, and ibuprofen can provide relief for people with Sjogren's who are experiencing joint pain.
- Disease-modifying antirheumatic drugs (DMARDs): In cases where the joint pain is accompanied by fatigue and rashes, DMARDs may relieve symptoms. Examples include hydroxychloroquine or methotrexate. If Sjogren's seems to be affecting the muscles, nerves, lungs, or kidneys, stronger DMARDs, corticosteroids, or rituximab may be prescribed.
- Antifungal medication: If oral yeast infections are occurring, medication may be prescribed to combat the fungus.
- Vaginal lubricants: For vaginal dryness, water-based vaginal lubricants can provide a solution, especially during sexual intercourse.
- Punctual occlusion: When all conservative treatment options have been exhausted, this surgical option seals the tear ducts with small plugs to reduce the drainage of tears from the eye. This keeps the eye moist for longer. Temporary silicone plugs are likely to be used until the procedure is confirmed successful.
- Autologous eye serum: In severe cases of eye dryness, eye drops can be made from the person's blood serum.
Here are some easy ways to keep the mouth lubricated.
- consuming more liquids.
- rinsing the mouth regularly to prevent infection and soothe the area
- maintaining excellent oral and dental hygiene
- topical fluoride gels or varnish
- quitting smoking, as smoke irritates the mouth and speeds up the evaporation of saliva
- chewing sugar-free gum, which stimulates the production of saliva
- applying coconut oil to the dry areas, as it is both moisturizing and anti-microbial
As Sjogren's signs and symptoms are similar to some other health conditions, it can sometimes be hard to diagnose. The patient may be seeing various health professionals, perhaps a dentist for dry mouth and tooth decay, a gynecologist for vaginal dryness, and an ophthalmologist for dry eyes, a pulmonologist for shortness of breath and chronic cough.
Some medications can also cause symptoms similar to those in Sjogren's.
The criteria for a definitive diagnosis to be included in a clinical trial include:
- the need to apply eye drops containing tears more than 3 times a day
- persistent dry eyes and mouth for over 3 months
- evidence of dry eye in ophthalmological test results
- measuring salivary flow rates
- SSA or SSB autoantibodies in blood
- a lip biopsy showing focal lymphocytic inflammation
Sjogren's presents in different ways for different people. A doctor may order some further tests, including:
Blood tests: Sjogren's triggers the presence of special antibodies in the blood. As these antibodies only show up in about 60 to 70 percent of people with Sjogren's, a negative result does not mean the disease can be ruled out. This frequently causes confusion in initial diagnosis.
Ophthalmological tests: An ophthalmologist can carry out the Rose Bengal, Lissamine green, and Schirmer eye tests. Dyes and blotting paper are used to expose dry areas on the eye.
Salivary flow rate: The doctor measures the weight of saliva produced in a cup over 5 minutes. If insufficient saliva is produced, this may indicate Sjogren's.
Sialogram: A dye is injected into the parotid glands. An X-ray is then taken to determine how much saliva flows into the patient's mouth. This practice is increasingly being used less.
Salivary scintigraphy: A radioactive isotope is injected and tracked with imaging to measure salivary gland function.
Chest X-ray or CT-scan: This determines the presence of inflammation in the lungs, which can be caused by Sjogren's.
Skin biopsy with nerve fiber stain: This determines the presence of small fiber neuropathy (SFN).
Urine testing: Urine is sampled and tested to determine whether the kidneys have been affected.
People with Sjogren's may be more susceptible to a rash, especially after spending time in the sun.
Skin problems that occur with the condition may present as:
- xerosis, or dry, rough skin
- small "blood spots" or purpura on the lower legs due to vasculitis, or inflammation of the blood vessels
- vasculitic skin lesions that may appear as weals, lumps, blisters, or ulcers
- red, ring-shaped lesions with a pale area in the middle, known as annular erythema
Using a strong moisturizer can help prevent dry skin. If vasculitis occurs, the doctor may prescribe drugs to suppress the immune system, such as cyclophosphamide.
Sjogren's comes with serious complications if left untreated, including:
- an increased risk of lymphoma and multiple myeloma
- oral yeast infections
- dental cavities
- vision problems
- inflammation leading to bronchitis, pneumonia, and other lung problems
- kidney function problems
- autoimmune hepatitis or cirrhosis in the liver
- giving birth to a baby with heart problems or lupus
- peripheral neuropathy
- interstitial cystitis of the bladder
It is important to seek treatment for Sjogren's as early as possible to prevent these.
The causes of Sjogren's remain largely unknown.
Studies have indicated that a viral or bacterial infection may trigger the disease, but that the underlying cause is primarily genetic and environmental. The nervous system and the endocrine, or hormone-producing, system have also been implicated in the onset of Sjogren's.
An environmental factor may change the immune system and cause immune problems later on, such as infection with hepatitis C or the Epstein-Barr virus.
As the majority of people who develop Sjogren's are female, one theory is that estrogen, a female hormone, plays an important role. However, this is still not understood.
Menopause is the most common time for a diagnosis of Sjogren's to be made. Some studies theorize that estrogen protects against Sjogren's, and falling levels of the hormone may alter immune function and trigger the condition.
There is no cure for Sjogren's. However, by successfully managing the dryness of the affected regions and treating any affected organs, a person with the condition can maintain a good quality of life.
There are currently a number of clinical trial programs focused on developing new therapies for Sjogren's.