Information

Sjögren's Syndrome

Introduction

Henrich Sjögren was a Swedish eye doctor who described a 'syndrome' consisting of 3 features: dry eyes, dry mouth and aches and pains.

What Is It?

Sjögren's Syndrome is now recognised as being due to an over-active immune system - in some ways an older, less life-threatening, though troublesome, 'cousin' of Lupus. The over-active immune cells infiltrate and damage some of the glands of the body, notably the salivary glands (dry mouth) and lachrymal glands (dry eyes).

Dry Eyes
Often the patients complain of 'irritation' or 'scratchiness' of the eyes rather than dryness - indeed some patients don't realise until tested that their tear secretion is down. Another common complaint is of photophobia, or sensitivity to bright light.

Dry Mouth
This can be so severe that the patient is continually sipping water, even to the extent of taking jugs of water to bed. One of the complications of inadequate saliva is that dental and gum disease can occur, therefore regular, careful dentistry is important.

Other 'dryness'
Dryness of the throat can lead to a dry cough, and of the oesophagus to swallowing difficulties. Vaginal dryness can lead to discomfort, and to an increased risk of thrush. Some patients, probably through similar alterations in their bladder and urethral lining, develop recurrent (non-infective) cystitis.

Aches and Pains
These, a major feature of Sjögrens, are often wrongly ascribed to "M.E." or fibromyalgia. Occasionally, they flare into an attack of full-blown arthritis, clinically indistinguishable from rheumatoid arthritis, but fortunately, usually 'self-limiting' or reversible.

Other Features

As in Lupus, fatigue can be an overwhelming symptom. Also, as in Lupus, sun-allergic rashes can occur. Other 'auto-immune' diseases may be present, notably, thyroid problems (especially low or hypo-thyroidism), pernicious anaemia, lung fibrosis (rare) and fluid/electrolyte imbalance.

Allergy
One major feature of Sjögrens is allergy - allergy to drugs (almost 100% of Sjögrens patients are allergic to the antibiotic SEPTRIN), allergy to some vaccines, allergy to insect bites and even, in some patients, allergy to certain foods. I always advise my patients to keep a 'mental diary' watching out for food-related flares.

Congenital Heart Block
A very rare complication in the babies of some mothers with lupus and with Sjögrens (specifically limited to a sub-group of those with the antibody anti-Ro) is congenital heart block. The baby is born with a slow pulse rate and may require a pacemaker. Although this rare neonatal condition cannot as yet be prevented, international studies using a form of globulin 'immunisation' in pregnancy are underway.

The Cause of Sjögrens

Many patients have family histories suggesting a genetic origin. Others give a history of prolonged 'glandular fever' in their teens, hinting at a possible viral cause. In some cases, the immunological over-activity spills over into a low-grade lymphoma - fortunately rare and usually very responsive to treatment.

Tests

Dry Eye
A very simple clinical test, hooking a thin strip of blotting paper over the lower eyelid, is extremely valuable - soaking wet in normals, bone dry in Sjögrens. It always surprises me how few colleagues routinely use this important screening test.

Blood Tests
Sjögrens patients often have positive rheumatoid factor tests (±80%) and positive anti-nuclear factor (ANA) tests (±80%) though the specific test for lupus (anti-DNA) is negative. One of the more specific tests for Sjögrens is an antibody test called anti-Ro (see Leaflet on blood tests)

Treatment

'Artificial tears' (e.g. Hypromellose) help protect the eyes, especially in dusty environments. There are some 'artificial saliva' substitutes on the market and a tablet - pilocarpine - helps stimulate saliva in a proportion of patients.

The most effective drug for the aches and pains as well as the fatigue is the anti-malarial hydroxychloroquine (Plaquenil), which can be given over long periods of time. For acute flares, e.g. of arthritis, a short course of steroids (not long term) is usually effective.

For rarer, more severe cases, treatment with immuno-suppresive drugs (e.g. azathioprine, methotrexate and Mofetil) are used, as in lupus.

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