Sjögren’s Syndrome and Raynaud’s Phenomenon

This site is intended for healthcare professionals as a useful source of information on the diagnosis, treatment and support of patients with lupus and related connective tissue diseases.
Henrik Sjögren was a Swedish ophthalmologist who described the dry eyes and dry mouth that people with lupus and other forms of arthritis sometimes experience. For many people with lupus this is one of the most annoying features of the condition, all the more so because it is often not obvious to those around. The condition is caused by inflammation of the salivary and tear glands, which then fail to produce as much saliva and as many tears as before.

There are two types of Sjögren’s syndrome, primary and secondary. In Primary Sjögren’s syndrome there may be inflammation in other areas, such as the joints. Secondary Sjögren’s syndrome presents as a secondary diagnosis in a patient with another auto-immune disease condition, such as lupus or rheumatoid arthritis.

Symptoms of Sjögren’s syndrome

Often the first sign is grittiness and some redness in the eyes. There may also be some blurring of vision, which can be worrying, but settles once the eyes are moistened by blinking or adding eye drops. A dry mouth is common. Because saliva also protects against tooth decay the first sign can be worsening dental health. Sometimes the salivary glands can become swollen and painful. Lack of saliva can also lead to difficulty in swallowing, difficulty with dentures and Candida (thrush) in the mouth. Rarely, the salivary glands may enlarge. Occasionally the vagina may also be dry, sometimes leading to thrush and discomfort on sexual intercourse. Sometimes dry skin may also be a problem.

Diagnosing Sjögren’s syndrome

In order to diagnose Primary Sjögren’s syndrome, antibodies to both anti-Ro (SSA) and/or Anti- La (SS-B) should be present serologically, they can also be seen in those with Secondary Sjögren’s syndrome. Sometimes it may be necessary to undertake a lip biopsy under local anaesthesia, especially in patients who are seronegative for these antibodies. Ultra sound of the salivary glands is a simple noninvasive test. Occasionally a parotid sialography or a salivary scintingraphy helps in diagnosis. When assessing dry mouth, visual observation of the mouth is important with review of dental records including history of active or previous caries, gingivitis and any future planned dental treatments. Objective measurement of the eye includes the Schirmer’s test that measures the production of tears. A piece of sterile blotting paper is placed in the lower eyelid and tear response over a five minute time period is observed. Opticians and ophthalmologists can undertake more specific tests using slip-lamps to look for dryness on the surface of the eye. Patients should report dryness of the eyes and mouth for more than three months including the need to take fluid when eating in order to swallow food. Some patients also describe significant fatigue and arthralgias.

Treatments

Many patients respond to Hydroxychloroquine which can be effective for arthralgias and fatigue. Unfortunately the dryness is a very difficult symptom to treat. Simple replacements can be used to help ease symptoms, as can be seen below:-

1. Regular mouthwashes and excellent oral hygiene
2. Regular dentist and dental hygienist checks at least every 6 months
3. Sips of water
4. Chewing sugar free gum and using gel, saliva replacement or saliva stimulation tablets as needed
5. Regular applications of artificial tears and gel based products can help to keep the eyes moist at night
6. Temporary punctual plugs can help in some cases
7. Some patients may benefit from Pilocarpine although this is often limited due to the side effects

Raynaud’s phenomenon

Raynaud’s phenomenon is a vasospastic disorder of the peripheral circulation where the blood supply to the extremities, usually the fingers and toes is interrupted. The stimulus is usually a change in ambient temperature or stress. The extremities become white and dead-looking, may turn blue and then bright red, perhaps with considerable pain, numbness or tingling. Primary Raynaud’s can occur in isolation and is prevalent in the general population, with up to 20% of women suffering from this phenomenon. It can also present as a secondary diagnosis to conditions such as lupus, but also more commonly seen in scleroderma and also seen in rheumatoid arthritis. It can be hereditary and can vary in intensity.

Treatment approaches can include the following:-

• Avoiding dramatic changes in temperature and planning for colder weather
• Hand warmers and thick gloves
• Layers of clothing to maintain inner heat
• Stop smoking and avoid caffeine
• There is some evidence that fish oils supplements that contain long-chain omega-3 fatty acids can improve symptoms
• Drug approaches can include calcium channel blockers, ACE inhibitors, Fluoxetine and in those who develop secondary critical digital ischaemia, intravenous prostacycline can be of benefit

As Raynaud’s is seen as one of the presenting features of Scleroderma, more information is available from Scleroderma & Raynauds UK (SRUK).