Sjögren's syndrome
Sjögren's syndrome is an autoimmune disease in which the immune system turns on the body's own fluid-making glands, chiefly the tear glands and the salivary…
On this page
Medicines commonly prescribed for Sjögren's syndrome
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: TABLET, 5 mgActive substance: pilocarpineManufacturer: Merus Labs Luxco Ii S.À.R.L.Prescription requiredDosage form: ORAL SOLUTION/SUSPENSION, 4 mg/mlActive substance: prednisoloneManufacturer: Laboratorio Aldo Union S.L.Prescription requiredDosage form: TABLET, 400 mgActive substance: hydroxychloroquineManufacturer: Laboratorios Gebro Pharma S.A.Prescription required
Sjögren's syndrome is an autoimmune disease in which the immune system turns on the body's own fluid-making glands, chiefly the tear glands and the salivary glands. Hence its two best-known features: dry eyes and a dry mouth. But reducing the illness to dryness gets it wrong, because alongside it there is almost always a draining tiredness and aching joints, and in some people the lungs, kidneys and nerves are drawn in as well. Two things are talked about unforgivably little: without protection the dryness wrecks the teeth within a few years and damages the cornea, and a parotid gland that stays enlarged on one side calls for a separate look.
What kind of illness this is
Small glands that make moisture are scattered all over the body: the tear glands around the eyes, the salivary glands in the mouth and cheeks, and further glands in the nose, throat, airways, skin and vagina. In Sjögren's syndrome immune cells gather in those glands and gradually replace their working tissue. The glands shrink and put out less and less, which is why the dryness turns up in several places at once rather than in the eyes alone.
The disease can stand alone, or it can be a second condition added to another autoimmune illness — most often rheumatoid arthritis, lupus or scleroderma. Treatment and follow-up are much the same either way.
It affects mostly women, and the diagnosis usually comes in middle age, though it appears earlier too. Quite why the immune system behaves like this is not known: the current view points to a mixture of inherited susceptibility, hormonal background and perhaps an earlier infection as the trigger.
The course is long and runs in waves. Life expectancy for most people with Sjögren's syndrome is the same as for anyone else — what is at stake is the quality of daily life and the prevention of complications, not a threat to life.
Dry eyes and dry mouth: what it feels like
The dryness of this illness is nothing like ordinary tired eyes at the end of the day. It is constant, and it has a character of its own:
- a gritty feeling, as if something were in the eye, with burning, redness and heavy lids by evening;
- paradoxical watering: an eye irritated by dryness reflexively produces a thin, watery tear that does not help;
- stinging in wind, in air conditioning, in the dry air of an aircraft, and above all at a screen, where people blink half as often;
- vision that blurs every so often and clears again with a blink;
- a mouth so dry that dry food is hard to swallow without a drink, and talking for any length of time is an effort;
- a tongue that goes dry, smooth and shiny, cracked lips, sore splits at the corners of the mouth;
- a changed sense of taste, dentures that no longer stay put, and getting up in the night for water.
Saliva is not simply moisture. It washes away food debris, neutralises acid and carries substances that hold bacteria and fungi in check. When there is too little of it, thrush takes hold easily: the tongue reddens and burns and a white coating appears. It treats well, but it comes back as long as the dryness is not under control.
What comes besides the dryness
This is the part that most often drops out of the conversation, and it troubles people no less than the rest:
- fatigue — not "a bit tired" but nothing left in the tank after an ordinary day; for many it is the hardest symptom of all;
- aching, stiff joints, usually the hands, sometimes with swelling;
- vaginal dryness and pain during sex — rarely asked about, and straightforward to sort out;
- dry skin, nose and throat: crusting in the nose, a hoarse voice, a dry cough that drags on for months;
- swelling of the parotid glands, giving puffed-out cheeks; usually on both sides, coming and going;
- Raynaud's phenomenon — fingers going white and numb in the cold;
- numbness, tingling or burning in the feet and hands;
- a fine red rash on the lower legs that does not fade when pressed.
Less often the disease reaches internal organs: lung tissue, with breathlessness and a persistent cough, the kidneys, the liver, the thyroid gland. That is why review with a rheumatologist is not a formality — blood and urine tests once a year catch such things before they announce themselves.
A word on pregnancy: the antibodies found in this condition cross the placenta and, rarely, disturb the conduction of the impulse in the baby's heart. This is not a bar to pregnancy, but it is a reason to mention the diagnosis early, so that the baby's heart can be watched specifically.
Teeth: the part people learn about too late
This is the most practical section on the page. With a markedly dry mouth, decay advances fast and not where anyone expects it: it runs along the gum line and around the necks of the teeth, ringing them, and it often does not hurt until the tooth breaks down. People who have had no trouble with their teeth for decades lose them one after another within a couple of years — almost always because nobody told them that a dry mouth needs protection of its own.
So the dentist here is not about appearances but part of treating the illness:
- a check-up every three to six months, not once a year;
- high-fluoride toothpaste prescribed by the dentist, and fluoride varnish applied in the surgery;
- brushing twice a day, and cleaning between the teeth without fail;
- sugar-free gum or sweets, ideally with xylitol: chewing on its own gets saliva moving;
- small sips of water through the day instead of sugary or fizzy drinks;
- alcohol-free mouthwashes; the alcohol-based ones dry things out further;
- telling the dentist the diagnosis: with a dry mouth the approach to fillings and dentures is different.
Juice, lemon and sour sweets "to bring the saliva up" are a poor idea: they do stimulate the flow, but the acid dissolves enamel that has nothing left to defend it.
How the diagnosis is made
Dry eyes and a dry mouth happen to a great many people and on their own settle nothing. The doctor's task is to work out whether an autoimmune process sits behind them. The work-up usually goes like this:
- blood tests: specific antibodies, markers of inflammation, rheumatoid factor, full blood count and biochemistry;
- at the eye clinic, measuring tear production with a paper strip tucked under the lower lid and examining the cornea with staining drops that show up damaged areas;
- measuring saliva by collecting it into a container over a set time;
- an ultrasound scan of the salivary glands, painless and very informative;
- a biopsy of the minor salivary glands from the inner surface of the lip under local anaesthetic, if the picture is still unclear;
- a urine test and a chest X-ray or scan where organ involvement is suspected.
Reaching the diagnosis often takes years: the complaints look unrelated and the person is passed between specialists. One simple thing helps — tell the doctor about all the symptoms at once, not only the one that brought you in today.
Treatment and daily care
There is as yet no way to switch the autoimmune process off for good, but the disease can be managed well and most people carry on with ordinary life.
For the eyes. Artificial tears are the foundation; where they are used many times a day, preservative-free drops are the ones to pick, because the preservative irritates the eye in its own right, and at night a gel or ointment is more comfortable. If that is not enough, the ophthalmologist adds anti-inflammatory drops or fits tiny plugs into the tear ducts so that the tear does not drain away so fast.
For the mouth. Saliva substitutes as a spray or gel, frequent sips of water, humidified air in the bedroom. Where working gland tissue remains, a doctor may prescribe medicines that stimulate the production of saliva and tears; the effect is noticeable, but they bring sweating and flushing, so the dose is built up gradually.
For the joints, the fatigue and the internal organs. For joint pain and marked fatigue rheumatologists use the antimalarial drug hydroxychloroquine, and short courses of steroids during flares. Where the lungs, kidneys or nerves are involved, drugs that damp down the immune system are added. Ordinary painkillers ease the joints but do nothing to the disease itself.
For everything else. Vaginal dryness responds to long-acting moisturisers and a water-based lubricant during sex. Wash the skin with mild soap-free products and put an emollient on straight out of the shower. And giving up smoking has a direct point here: smoke dries out both the mouth and the airways.
What makes the dryness worse
Before concluding that the disease has taken off, it is worth checking far more prosaic causes. A great many medicines dry the mucous membranes as a side effect, and someone with Sjögren's syndrome feels that many times more than anyone else. The list includes:
- antihistamines, including those inside cold remedies;
- antidepressants, especially the older generations, and some drugs for anxiety;
- diuretics;
- a number of blood pressure medicines;
- treatments for an overactive bladder, and antispasmodics;
- strong painkillers of the opioid group;
- decongestant nasal sprays and vitamin A-derived acne treatments.
Nothing should be stopped on your own initiative — but do take the doctor a complete list of what you take, including anything bought over the counter. Often there is an alternative, and the dryness eases noticeably without any new treatment at all.
From the surroundings, the drying culprits are air conditioning and central heating, the dry air of an aircraft, smoke, dust and wind, alcohol and a great deal of coffee, and breathing through the mouth at night with a blocked nose.
When to see a doctor without delay
Most of this illness is dealt with by planned appointments, but a few situations are not plannable.
Persistent enlargement of a parotid or submandibular gland on one side is something to say plainly. The gland swelling that belongs to this disease is symmetrical and comes and goes. If instead one gland has grown and has not gone down for weeks, or has become hard or lumpy, it must be investigated. In Sjögren's syndrome the risk of lymphoma, a tumour of lymphatic tissue, is higher than in people without it, and it arises most often in exactly this place, a salivary gland. The absolute numbers stay small, and the great majority of such lumps turn out not to be a tumour. But this is checked straight away rather than in six months' time: lymphomas of this kind grow slowly and respond well to treatment when they are found early. For the same reason, show a doctor any new lymph nodes that stay up, night sweats, unexplained weight loss and a rash on the lower legs that will not clear.
Other signs also mean seeking help without delay:
- pain in the eye, discomfort in bright light, redness or loss of vision — a dry cornea is easily damaged, and an ulcer on it is treated urgently;
- increasing breathlessness, or a cough that changes and worsens;
- sudden weakness, numbness or loss of power in an arm or a leg;
- a painful, hot, swollen gland with fever, which suggests a bacterial infection in it;
- white patches and burning in the mouth that do not settle on their own.
Online consultation
People live with Sjögren's syndrome for years, and most of the questions come up between appointments — which is exactly what is convenient to settle online.
An online appointment can go through the combination of complaints and judge whether an autoimmune cause of the dryness is worth pursuing and which tests to start with; interpret results you already have; review the medication list and pick out what is adding to the dryness; put together a daily routine for eyes and mouth that actually works; plan the visits to the eye clinic and the dentist; and talk through pregnancy in advance.
It helps to note down when the dryness began and what came first, how the fatigue is changing, whether there is joint pain and dryness elsewhere, and to bring a list of all medicines and your latest results.
Examination of the cornea, ultrasound of the glands and biopsy are done in person. And if a lump in a gland has appeared on one side and stayed, if the eye hurts or vision drops, or if breathlessness is building, book a face-to-face appointment and do not let it wait.
This material is for information only and does not replace medical advice.
Consult with a doctor about Sjögren's syndrome
Discuss your symptoms and possible next steps with a doctor online.
Online doctors for Sjögren's syndrome
Discuss your symptoms and possible next steps for Sjögren's syndrome with a doctor online.
