For many people with lupus having Sjögren’s syndrome, characterised by persistent dry eyes and dry mouth as well, 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.

Sjögren’s syndrome can be diagnosed before or after lupus. In primary Sjögren’s syndrome (where it appears and is diagnosed as a stand-alone condition) there may be inflammation in other areas, such as the joints. Secondary Sjögren’s syndrome presents after a patient has been diagnosed with another autoimmune disease condition, such as lupus or rheumatoid arthritis.

Symptoms of Sjögren’s syndrome

Often the first symptom is grittiness and/or some redness in the eyes. There may 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, speaking, difficulty with dentures, candida (thrush) in the mouth, or the salivary glands may enlarge. The vagina may also be dry and we should routinely ask gently, as patients rarely volunteer this symptom. It can lead to thrush and discomfort on sexual intercourse and there are treatments that can be offered. Sometimes generalised dry skin may also be a problem and again we should be asking routinely in case the patient has not linked their separate symptoms.

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. In patients who are seronegative for these antibodies, a lip biopsy can be taken under local anaesthesia. Ultrasound of the salivary glands might be possible instead. 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, where 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.

We can remind and encourage patients they should let us know of persistent dryness of the eyes and mouth (present 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 arthralgia.

Treatments

Some patients respond to hydroxychloroquine which can be effective for arthralgia and fatigue, but is now understood to have a placebo effect for the direct condition. Rituximab is likely to increase Sjögren’s symptoms. However, these treatments can be used to help ease symptoms:-
1. Regular mouthwashes and excellent oral hygiene
2. Use of a high-fluoride toothpastes (i.e. 5,000 ppm) GPs can be reluctant to continue treatment, however when aware of the diagnosis dentists are very likely to support and prescribe.
3. Regular dentist and dental hygienist checks at least every 6 months.
4. Sipping water regularly and frequently, and getting in the habit of always having water to hand and within immediate reach.
5. Chewing sugar-free gum, especially those containing xylitol as this is also anti-dental caries.
6. Using gel, saliva replacement or saliva stimulation tablets if helpful.
7. Temporary punctal plugs can help in some cases.
8. Some patients may benefit from pilocarpine although this is often limited due to the side effects, as it can increase systemic sweating.
9. Using eyelid warmers (such as microwave wheat-bag or gel pack). This helps warm the eyelids and the natural oils, making them more viscous and able to move more easily to fulfil their natural role.
10.Wearing normal, everyday glasses and sun-glasses reduces the rate of evaporation from the eye by up to 30%, so can also be helpful.
11. Regular applications through the day of artificial tears and gel or ointmentbased products can help to keep the eyes moist. If this application is greater than six times each day, these drops MUST be preservative free, as the preservatives can accumulate in the eye and be toxic.
12. Eye drops containing hyaluronic acid supports the natural tear production.
13.Guar gum or liposomal sprays can be used to support natural eye oils and reduce evaporation.

Raynaud’s phenomenon

Raynaud’s phenomenon is seen in some people with lupus where the fingers or toes change colour as a response to the cold and stress. The usual colour changes seen most prominently in Caucasian skin tones are white, blue and then red (when pain can be felt at this point) though do remember the colours appear less pronounced in other skin-tones.

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, sometimes as little as 5°C or 10°C, or stress. The extremities become white and deadlooking, 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 10% of women suffering from this phenomenon. It can also present as a secondary diagnosis, to conditions such as lupus, but more commonly seen in scleroderma and also seen in rheumatoid arthritis. It can be hereditary and can vary in intensity. Treatment approaches are mainly proactive. By keeping the patient’s core temperature higher, this then encourages blood flow to be maintained to the extremities. There is much we can support the patient to do for themselves:-
• Avoiding dramatic changes in temperature and planning for colder weather.
• Hand warmers and thick gloves.
• Boots and thick socks.
• Wear a hat keeping the head covered, and add a scarf.
• Multiple layers of clothing to maintain inner heat, and reminding that natural fibres tend to help regulate and maintain a more even body temperature more easily.
• Stop smoking, as this has a direct vasoconstrictive action.
• Avoid caffeine, again this is a vasoconstrictor.
• Consider if temperature respondent, colour changing nail varnish would help as an additional reminder of hand temperature (especially as for many, having cold hands is “just normal”).
• Some find help in acupuncture.
• Some find Sudafed (pseudoephedrine) can make symptoms worse, and so can be better avoided.
• There is some evidence that fish oil supplements that contain long-chain omega-3 fatty acids can improve symptoms.
• Drug approaches can include calcium channel blockers, such as nifedipine, amlodipine, or felodipine, ACE inhibitors, such as lisinopril, enalapril, or ramipril, fluoxetine, sildenafil, or iloprost, can be of benefit.
• There are some medical procedures available, such as nerve surgery or Botox injections, however, these seem to have very variable response and reliability rates, and as yet remain more experimental.

Other autoimmune conditions that can occur with lupus

Lupus patients are at increased possibility of other auto-immune conditions such as;
• Hypothyroidism
• Diabetes Mellitus
• Pernicious Anaemia
• Multiple Sclerosis
• Autoimmune Hepatitis
Each of these conditions should be screened for annually, or when a patient with lupus is symptomatic, during their SARD clinic appointment as they can occur concurrently.