Sjögren's syndrome

As an autoimmune condition, Sjögren's starts with the body's defences mistaking its own tissues for an enemy. Its particular targets are the glands that make tears and saliva: inflammation there reduces the moisture they produce and, in time, can impair how the glands work. What sets the process going is not fully understood, and no one cause has been identified. Several things are thought to play a part: the genes you carry, hormones, and possibly an infection in the past. Having a parent with Sjögren's does not mean a child will develop it.

It is a condition of women far more than of men, and one that often appears in middle age, though no age is exempt. In some people it is the only autoimmune condition present; in others it keeps company with lupus, systemic sclerosis or rheumatoid arthritis.

Dryness is the signature of Sjögren's, though it reaches beyond the glands. People may notice:

• Eyes that stay dry, gritty or sore
• Dryness in the mouth, dry food that is hard to swallow, or trouble with the teeth
• Painful, stiff joints
• Fatigue, sometimes a great deal of it
• Sometimes dryness elsewhere, or features of another autoimmune condition

The diagnosis rests on your own account and an examination, on blood tests for the antibodies typical of Sjögren's and for inflammation markers, and on measuring how much the eyes and the glands still produce; an ultrasound scan of the salivary glands is often added, and a small biopsy from the inside of the lip where the blood tests and the examination leave the diagnosis open. With so many possible causes of dryness, the assessment serves to establish whether Sjögren's is the cause and whether another condition is present alongside it.

If the joints are involved, ultrasound is a good way to gauge how inflamed they have become. Point-of-care ultrasound is part of the consultation with Dr Borukhson, so a joint that is troubling you can often be scanned before you leave; the ultrasound clinic page has the details of the scan.

There are four aims: dryness relieved, eyes and teeth protected, joint symptoms and fatigue kept in check, and any wider involvement watched for. When simple measures fall short, prescription treatments can be added to them: preservative-free lubricating drops, gels or a night-time ointment for dry eyes, saliva substitutes for a dry mouth and, where the glands still have some function left, a medicine such as pilocarpine, which encourages them to produce more moisture. Hydroxychloroquine is sometimes prescribed, under specialist supervision, for the joint pain and the fatigue, and it helps some people but not all. A short course of steroid tablets can ease severe joint pain, and a longer course is sometimes needed where the condition is more severe. Treatment that suppresses the immune system is generally kept back for organ involvement beyond the glands, which is less common, with methotrexate the usual choice where the joints are inflamed. Your plan is your own and is explained to you, and since Sjögren's is a lifelong condition, review at regular intervals remains part of it.

Sjögren's is a condition in which more than one specialty is sometimes needed, because the eyes, the mouth, the joints and, less commonly, other organs can each require expertise of their own. Dr Borukhson practises within a world-renowned tertiary centre, with ready access to consultant colleagues in the other specialties this condition may call on. Where it helps, she can bring those specialists in directly, and particularly complex cases can go to a multidisciplinary team meeting (MDT) with minimal delay. Whenever more than one kind of expertise is needed, the strands of your care can be drawn together and decisions reached promptly.

Much of your comfort comes from small daily habits. Dry eyes do better with regular lubricating drops, with breaks from screens, and with some shelter from wind and air conditioning. A dry mouth is helped by sipping water through the day and by chewing sugar-free gum to get the saliva flowing; careful dental care matters too, because with less saliva the teeth decay more easily.

• Drink enough, and go easy on caffeine and alcohol, which make dryness worse
• In heated or air-conditioned rooms, a humidifier puts some moisture back
• Pace yourself, so that gentle regular exercise, with rest between sessions, keeps fatigue within bounds
• Give up smoking: smoke dries the eyes, and stopping lowers the risk of lung complications

With the right routine and regular review, most people manage well over the years. The symptoms have their ups and downs, and your appointments are the moments at which treatment is adjusted and anything new beyond the glands is picked up. Tell your team early if you are thinking of a pregnancy: Sjögren's can raise the risk of miscarriage, and where anti-Ro or anti-La antibodies are present the pregnancy is monitored more closely and low-dose aspirin may be advised; how that care is arranged is set out in arthritis, autoimmune conditions and pregnancy.

A routine review is the right place for most changes, but a few call for quicker action.

Treat any of these as a 999 or A&E emergency: call 999 and do not drive yourself to A&E.

• Severe illness with suspected sepsis: a high fever with shaking, confusion, rapid breathing, or feeling dramatically worse over hours
• With a fever or feeling unwell, a rash that does not fade when you press it, a stiff neck with eyes that cannot bear bright light, or skin, lips or tongue turning blue, grey, pale or blotchy, since these can be signs of meningitis or sepsis; on black or brown skin the rash and the change of colour may be easier to see on the palms of the hands or the soles of the feet
• Chest pain that is crushing, tight or like a pressure, that spreads to an arm, the jaw, the neck, the stomach or the back, that comes with breathlessness, sweating, feeling sick or feeling light-headed, or that came on suddenly and does not go away
• Breathlessness that is severe, with gasping, choking or no breath to get words out; or coughing up blood
• Palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, since a heart whose muscle or rhythm is affected has to be checked at once
• Sudden weakness or numbness on one side, a drooping face or difficulty speaking, a sudden severe headache, sudden confusion or a seizure, or sudden loss of vision, any of which can be a stroke, even if it stops after a short time
• An eye that suddenly and completely loses its sight
• Sudden severe pain in the abdomen or back, since the pancreas can react to prednisolone
• Lips, mouth, throat or tongue that suddenly swell, a throat that feels tight, very fast breathing or a struggle to breathe or to swallow, skin, tongue or lips turning blue, grey or pale (on black or brown skin this may be easier to see on the palms of the hands or the soles of the feet), becoming suddenly very confused, drowsy or dizzy, or fainting and not being able to be woken, since this can be a severe allergic reaction, anaphylaxis, which can follow a dose of any medicine
• Vomiting blood, or bleeding from the back passage that is heavy or will not stop, most of all with faintness, confusion or pain in the abdomen, or vomit that is black or dark brown, since a stomach ulcer on prednisolone can bleed
• Thoughts of harming yourself or ending your life, which steroids can bring on

Any of these needs an assessment that is urgent, on the same day, from your GP, your rheumatology team or NHS 111:

• A fever, or feeling generally unwell, on immunosuppressant, biologic or steroid treatment, since an infection has to be treated early
• A joint that is hot and swollen, with a fever or feeling unwell, since an infected joint has to be ruled out at once, at A&E or through NHS 111
• A joint that has become hot and very swollen suddenly, or is worsening quickly, even without a fever, since an infected joint does not always announce itself with one; a joint that will take no weight at all, or that cannot be moved, is a 999 or A&E emergency, whether or not there is a fever
• A new red, painful eye, or sight that has blurred, dimmed or changed, which needs an eye casualty or urgent eye clinic the same day, since severe dryness can damage the surface of the eye, and untreated damage there can cost sight; on hydroxychloroquine, a change in sight is also one to report to the team that prescribes it; in pregnancy, any change in sight, blurring or flashing lights among them, is also for your midwife or maternity unit straight away, as the pregnancy line below says, since it can be a sign of pre-eclampsia
• New chest pain, new palpitations, or breathlessness that is new or getting worse; palpitations happening now that do not stop, or that come with chest pain, breathlessness, feeling faint or fainting, belong to the first list; in pregnancy, breathlessness that comes on suddenly, or when you lie down, or with dizziness, is for your midwife or maternity unit straight away, as the pregnancy line below says
• A persistent dry cough with a fever, with new breathlessness, or that is worsening over hours to days
• A marked change in mood or behaviour, confusion, or seeing or hearing things that are not there, on steroid treatment, since prednisolone can do this, the more so the higher the dose; confusion that comes on suddenly is a 999 or A&E emergency, as the first list says
• Unusual thirst or hunger, passing urine more often, or feeling sleepy or confused, on steroids, or swelling of the arms or legs, bruising or bleeding that is not normal, black or dark red stools, severe stomach pain, severe back pain, a severe upset stomach, being sick, or very bad dizziness or passing out, since each can be a steroid effect that needs checking without delay; pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the first list says; in pregnancy, bleeding that is not normal, and stomach pain that is severe, belong to the pregnancy line below
• Contact with measles, chickenpox or shingles, since these infections can be severe on steroids and a medicine to protect you may be needed
• Being near someone with measles, chickenpox or shingles, while you take a medicine that weakens the immune system, since measles and chickenpox can be dangerous when the immune system is weakened, and you can catch chickenpox from someone with shingles if you have never had chickenpox; NHS 111 can tell you what to do that day
• In pregnancy, any bleeding, stomach pain that is severe or does not go away after a rest, reduced baby movements, a severe headache or visual disturbance, sudden swelling of the face, hands, legs or feet, or breathlessness that comes on suddenly, or when you lie down, or with dizziness: contact your midwife or maternity unit straight away, whatever the time; bleeding that is heavy, or that comes with severe pain in the tummy, pain in the shoulder, or feeling sick, faint or dizzy, or passing out, is a 999 or A&E emergency: call 999 and do not drive yourself to A&E; pain in the abdomen or back that comes on suddenly and severely is a 999 or A&E emergency, as the first list says
• A high temperature in pregnancy, whatever medicines you are taking, since a fever can be a sign of an infection that needs checking; your midwife or maternity unit can advise

Do not wait for a scheduled appointment; contact your GP or specialist team promptly if you notice:

• Any lump or swelling that is new and stays, particularly around the jaw glands or in the neck
• Night sweats that drench you, weight you are losing without trying, or, outside pregnancy and off immunosuppressant, biologic or steroid treatment, a fever that keeps coming back, none of them explained
• A limb that has become numb, tingling or weak, the change coming on gradually
• A joint that has swollen gradually and is not hot

Each of these deserves a prompt check: a swollen gland that stays can be the first sign of lymphoma, which affects a small minority of people with Sjögren's over a lifetime, and the others can mark a change in the condition. A persistent dry cough on its own, or breathlessness that has drifted slowly, over weeks or months, is something to tell your team promptly, without waiting for a routine slot. Dryness, fatigue or joint pain that is steadily getting worse belongs at a routine review, where your plan can be looked at again. If you cannot tell how urgent something is, asking is never a mistake.

Dryness that will not go away, once joint pain and fatigue join it, raises two questions: whether Sjögren's is the cause, and whether another autoimmune condition is present alongside it. A specialist review can answer both and agree a plan with you.

Which tests confirm Sjögren's syndrome?

No single test confirms Sjögren's. What does is putting several findings together: what you describe, what the examination finds, what the blood tests show (specific antibodies and markers of inflammation), and how well the eyes and the glands turn out to be working. Because so many things dry the eyes and mouth, it takes that combination to show whether Sjögren's is the one responsible.

How does a first consultation go?

A careful history first, covering the dryness, any joint symptoms and the fatigue, then an examination, then a look at any blood results you already have and a request for those that are missing. If the joints are troubling you, inflammation in them can be looked for during the same appointment: point-of-care ultrasound is part of the consultation with Dr Borukhson. By the end you should know what is suspected and what happens next.

Does Sjögren's spread beyond the eyes and mouth?

For most people, no further than the joints and energy levels. Dry eyes and mouth, joint symptoms and fatigue are the main effects; involvement of other organs is less common, and across everyone with Sjögren's about one person in seven has serious involvement beyond the glands, which is partly why regular review exists: to catch it early and deal with it promptly.

How often should I be seen?

Because Sjögren's is a long-term condition, the reviews over time matter more than any one assessment. The spacing follows your symptoms and whether anything beyond the glands is involved, and it is decided with you when the plan is drawn up. If a second specialty is involved, the two sets of reviews can be coordinated rather than run in isolation.

Is Sjögren's ever part of a wider autoimmune picture?

Yes. Sjögren's can be the only autoimmune condition someone has, or one of several, with lupus or systemic sclerosis among the possible companions, and the assessment looks for a companion on purpose: finding one can change both the treatment and the monitoring.