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Sjögren syndrome

Author: Dr Scott Barker, Dermatology Registrar, Greenlane Hospital, Auckland, New Zealand, 2006.


Sjögren syndrome — codes and concepts
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What is Sjögren syndrome?

Sjögren syndrome (SS) is an autoimmune disease that mainly affects secretory glands of the eyes and mouth, reducing production of tears and saliva (sicca symptoms) and resulting in dry eyes (called keratoconjunctivitis sicca) and dry mouth (xerostomia). 

Many patients with Sjögren syndrome also have features of other autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus or rheumatoid arthritis. In this case, they are said to have an overlap syndrome or the SS is said to be secondary to the other autoimmune condition. The diagnosis is Primary SS if other conditions are not present.

Who gets primary Sjögren syndrome?

Primary Sjögren syndrome is 9 times more common in females than males. It is especially common in older females. However, anyone can develop it. Up to 2% of older females have been found to have primary SS.

What is the cause of Sjögren syndrome?

The cause of Sjögren syndrome is not known. One theory is that viruses such as Epstein-Barr virus may play a role in triggering SS in people with a genetic background that predisposes them to SS. Once the condition has been triggered lymphocytes infiltrate into the secretary glands. This is a slow, low-grade inflammatory process that damages the cells of the glands and thus impairs the function of the glands.

What are the mucocutaneous features of Sjögren syndrome?

Eye problems in Sjögren syndrome

Patients with Sjögren syndrome usually describe dry or gritty eyes. They may also feel a burning discomfort, notice decreased tear production, increased eye fatigue, and sometimes intolerance of bright lights (photophobia). Increased dryness of the eyes damages the lining of the cornea at the front of the eye causing keratoconjunctivitis sicca.

Dry mouth

Xerostomia may develop very gradually over a number of years. When first noticed it may only be the sensation of dryness of the mouth. As it progresses patients may notice difficulty swallowing dry food, difficulty speaking for long lengths of time, problems with dentures and sometimes increased tooth decay. Oral thrush is also more likely to develop.

Skin signs

The most common skin manifestation of Sjögren syndrome is dry rough skin (xerosis), which is noted by more than half of patients. This may result in itch. Patients with SS also sweat less than people without the disease. Although these symptoms are common, most patients with SS are more bothered by the dry mouth and dry eyes. Vaginal dryness may also occur.

Vasculitis is potentially more serious. This develops in about 10% of patients with primary SS and affects the skin in half of them (cutaneous small-vessel vasculitis). Skin lesions usually present as multiple purple or reddish-brown spots. The colour is due to bleeding from small inflamed vessels. Vasculitic skin lesions are most commonly seen on the legs but can develop anywhere. They may present as red flat patches, raised lumps, blisters, ulcers, or raised urticaria-like weals (urticarial vasculitis).

Other skin changes reported in Primary Sjögren syndrome include:

Sjogren syndrome – cutaneous vasculitis
 other organs in Sjögren syndrome

What other organs can be involved in Sjögren syndrome?

Gastrointestinal system

Rarely the gastrointestinal system may be affected in Sjögren syndrome. The resulting dryness may inflame the lining of the oesophagus (oesophagitis) and the stomach (atrophic gastritis).

Lungs

Although Sjögren syndrome may affect the lungs it is rarely serious. The most common manifestation is a dry cough, due to dryness of the small airways.

Joints

Up to 60% of people with Sjögren syndrome will at some stage develop at least one episode of arthralgia or arthritis. Arthritis does not usually result in erosion of joint structures or in joint deformity.

Kidneys

Involvement of the kidneys is more common if Sjögren syndrome is present as part of overlap syndrome with systemic lupus erythematosus. It can affect the kidneys in various ways including interstitial nephritis, kidney stones or glomerulonephritis.

Vasculitis

Primary Sjögren syndrome vasculitis can damage kidneys, lungs, nerves and joints. Fevers and anaemia may also be present. Involvement of internal organs is more likely if the vasculitis is associated with cryoglobulins. Some patients with Primary SS and vasculitis have an underlying B-cell lymphoma (cancer of the lymphatic system).

Long term prognosis of Sjögren syndrome

In long term studies, the reduced glandular function in Sjögren syndrome has not been observed to improve. If arthritis, Raynaud phenomenon, or interstitial nephritis are present early in the course of the illness they can resolve with treatment and time. Worse prognostic factors are the presence of glomerulonephritis, decreased blood levels of C4 complement, vasculitis and cryoglobulinaemia.

How is Sjögren syndrome diagnosed?

In people with symptoms that are consistent with Sjögren syndrome, tests may be done to help confirm the diagnosis.

Schirmer test

This measures the wetting of a standardised tear strip applied between the eyeball and the lateral edge of the lower eyelid. The test is considered positive if the result is less than 5mm in 5 minutes.

Biopsy of salivary glands in the mouth

An infiltrate of typical inflammatory cells supports the diagnosis.

Anti-Ro (SSA) and anti-La (SSB) antibodies

In the correct clinical setting, the presence of these antibodies in the blood supports a diagnosis of Sjögren syndrome. If positive, this increases the risk of a baby having neonatal lupus erythematosus.

Other tests that are sometimes performed include the measurement of salivary flow, parotid gland sialography, and salivary scintigraphy.

What is the treatment of Sjögren syndrome?

The mainstay of treatment for the dry eyes, dry mouth and dry skin is to minimise symptoms by replacing the reduced secretions. There is no cure for Sjögren syndrome. Immune suppressive medication is reserved for cases with internal organ involvement, particularly severe kidney or lung disease.

Dry eyes

Artificial tears and ocular lubricants are most commonly used. In severe cases, these may be needed as often as every 30 minutes.

Dry mouth

Increased oral fluid is often taken by the patient as a method to try and relieve symptoms. Other measures include the use of artificial saliva which can come in the form of gels, pastilles or sprays. There is also a medication called pilocarpine which can increase secretion from the damaged glands, although it does not cure the problem or reverse damage to the glands. Pilocarpine can be taken as tablets with each meal and at bedtime to increase the saliva. Stop smoking, if relevant.

Dry skin

Moisturisers and emollients may be applied as required. Choose a non-soap cleanser. A standard water-based lubricant may be used to relieve dryness of the vagina.

Arthritis

Non-steroidal anti-inflammatory drugs are the first-line medication for sore joints. Prednisone may be prescribed for severe disease or if symptoms fail to improve with NSAID treatment. Hydroxychloroquine has also been found to be useful in some people.

Vasculitis

Systemic steroids (prednisone) and immune suppressant medications such as azathioprine may be necessary.

Patients with Sjögren syndrome should try to avoid medications with anticholinergic side effects (tricyclic antidepressants such as amitriptyline, antipsychotics and antihistamines), as these may aggravate sicca symptoms.

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