Lupus (SLE)

Lupus is an autoimmune condition. The immune system exists to defend the body, but in lupus it makes antibodies against the body's own tissues, and the inflammation those antibodies drive is what harms the joints, skin, blood and internal organs. Why it begins is still an open question. The likeliest account is that two things have to coincide: a genetic susceptibility, and something that sets it off (sunlight, a viral infection, certain medicines or a shift in hormones among the candidates), rather than any one cause acting alone.

Men are affected far less often than women, and the condition usually starts somewhere between the teens and the forties. People of African, Caribbean, South Asian and East Asian heritage are more likely to develop lupus, and in them it can be more severe. The risk may be higher if lupus has already appeared in your family, and lupus often has another autoimmune condition alongside it, Sjögren's syndrome for one, or the clotting tendency of antiphospholipid syndrome.

Lupus varies widely from person to person, and not everyone has the same set of them. The features seen most often are:

• Painful, swollen joints and aching muscles
• Tiredness that does not lift
• Rashes, classically the one that spreads across the cheeks and nose, and skin that reacts badly to sunlight
• Ulcers in the mouth, thinning hair, or fingers that lose their colour in the cold
• Fevers, swollen glands, or weight loss with no obvious reason

The symptoms tend to ebb and flow, flaring and then easing, and the full picture can take time to build, which is one reason lupus can be hard to recognise.

Lupus has no single defining test. The diagnosis rests on the whole rather than on any part: the way the symptoms fit together, the examination, and the blood and urine tests, which include a search for particular antibodies. The pattern can take time to form and it overlaps with other conditions, so it takes a specialist's judgement to turn it into a diagnosis.

When the joints are involved, ultrasound can give an accurate picture of how inflamed they are. Point-of-care ultrasound is part of the consultation with Dr Borukhson; what that involves is explained on the ultrasound clinic page.

Treatment is there to do four things: quieten an immune response that has become overactive, ease the symptoms, protect the organs lupus can harm, and cut the number of flares. How that is done depends on which parts of the body are involved, so plans differ widely from person to person and are reviewed regularly as time goes on.

For most people the cornerstone is hydroxychloroquine, a tablet that quietens the condition and reduces flares, and most stay on it long term. Its common side effects are the likes of an upset stomach or a headache, and its serious ones are for the most part rare. An eye check goes with it, yearly once you have taken it for five years, and from earlier for those at higher risk, because, rarely, it can affect the retina after years of use. A short course of corticosteroid can settle a flare. When the condition is more active, or internal organs are involved, immunosuppressant medicines such as methotrexate, azathioprine or mycophenolate, or biologic therapies, may be added; these can raise the risk of infection and need regular blood test monitoring, which is why they are prescribed under specialist supervision and discussed with you in full. Two further facts argue for specialist input over the years: lupus is a long-term condition that can change, and both the condition and some of its medicines need careful planning around pregnancy. You and Dr Borukhson settle the plan together.

Because lupus can involve so many different organs, there are times when more than one specialty has a part to play in your care. 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.

For most people lupus runs as a long-term condition that flares now and then and stays quiet in between, and with the right treatment and review most people live full and active lives. A handful of habits are worth the trouble.

• Protect yourself from ultraviolet light, which can trigger both rashes and flares: high-factor sunscreen, shade and clothes that cover you all help.
• Give up smoking, if you smoke: it makes lupus worse and can blunt the effect of hydroxychloroquine.
• Stay gently and regularly active, rest enough to keep fatigue in check, and keep your vaccinations up to date, checking with your team which are right for you; a fever, or contact with measles, chickenpox or shingles, while you take a medicine that lowers your defences is a same-day matter, as the next section says.

Learn what your own flares feel like as they begin, and treat eye checks, blood tests and urine tests as fixed points in the diary. If a pregnancy is in your plans, say so early; arthritis, autoimmune conditions and pregnancy explains what to expect.

Lupus also carries a somewhat higher risk to the heart and blood vessels over the years, which is one reason blood pressure and cholesterol deserve checking alongside the usual blood and urine tests. Keeping the inflammation well controlled is one of the best ways to lower that risk, and our guide to inflammation and your heart explains the simple checks worth asking about.

Planned review copes with most of what lupus does; a few things need prompt attention.

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, though fingers or toes that change colour in the cold, as they do in Raynaud's, are not what is meant; 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; coughing up blood; or chest pain that catches as you breathe in, since any of these can mean a clot on the lung, and clots form more readily in lupus, above all in people who carry antiphospholipid antibodies
• 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
• A blackout, since lupus can, uncommonly, inflame the heart itself
• 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, though in lupus inflammation of the brain itself can look the same
• 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, though fingers or toes that change colour in the cold, as they do in Raynaud's, are not what is meant
• 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 high fever needs the same answer with or without treatment, because lupus makes infection likelier and a fever can also mark a flare
• 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
• New chest pain, new palpitations, or breathlessness that is new or getting worse, since the lining of the heart or of the lungs can be inflamed; 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
• Marked swelling of the face or ankles, or frothy or bloody urine, either of which can mean the kidneys are involved
• A calf that is swollen, hot or painful, or a limb that is painful, swollen or discoloured, which can mean a clot; with breathlessness, chest pain, palpitations or feeling faint, this is a 999 or A&E emergency: call 999 and do not drive yourself to A&E
• A headache that is new and persistent, or that keeps getting worse, with none of the signs above, since where antiphospholipid antibodies are present a clot can form in the veins that drain the brain, and lupus itself can reach the brain
• 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, as does an eye that light hurts, since lupus can, rarely, inflame the eye, and inflammation that goes untreated 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
• 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, 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

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.

A flare of joint pain in which no joint is hot or changing fast, a rash or mouth ulcers, or steadily increasing fatigue, is a reason to book a routine but timely review with your rheumatology team, so that treatment can be adjusted before it gets worse. In pregnancy a new rash is a different matter: ring your midwife or maternity unit, or your GP, straight away, since the cause may be an infection that could harm the baby, and not your lupus; our pregnancy guide covers contact with a rash illness too, and says what else to do, which rash needs 999 or A&E, and how soon a flare should be seen in pregnancy. If you cannot tell how urgent something is, asking is never a mistake.

Because lupus is complex and takes so many forms, getting the diagnosis right, and keeping the plan fitted to you and under regular review, matters enormously. When joint pain, fatigue, rashes and other symptoms arrive together and no cause has yet been found, a specialist review is how the picture begins to make sense.

What does it take to diagnose lupus?

No one test can either confirm lupus or exclude it. Instead three things are weighed side by side: the pattern your symptoms make, what the examination shows, and blood and urine tests, among them tests for particular antibodies. Point-of-care ultrasound is part of the consultation with Dr Borukhson, and if your joints are involved the scan can help to show whether they are inflamed.

Why is lupus sometimes slow to diagnose?

Because lupus varies so much between people, because several other conditions share its features, and because the whole picture can be slow to declare itself. One appointment may settle the question, or it may be the start of a run of them, each adding results and any new symptoms to the picture. The aim is a diagnosis that is secure, even if it takes more than one visit.

Will I be on treatment long term?

For most people, yes: hydroxychloroquine, whose serious side effects are for the most part rare, keeps the condition quieter and flares fewer, and so it is continued for the long term. Everything else is added as and when it is needed, whether that is a short course of steroids to bring a flare under control, or immunosuppressants and biologics when the condition is more active or internal organs are involved. Plans are individual, and yours is looked at again whenever circumstances change.

How will my treatment be monitored?

That depends on the medicines. With hydroxychloroquine the checks are on the eyes, from five years of use and yearly after that, sooner for some, since in rare cases it can affect the retina after years of use. With immunosuppressant and biologic medicines they are regular blood tests, together with reporting a fever or feeling unwell to your GP, your rheumatology team or NHS 111 the same day, since an infection on these medicines has to be treated early, and with the signs of sepsis in the first list under When to seek help it is a 999 or A&E emergency. Beyond the medicines, regular specialist review has a value of its own, because lupus can change its behaviour over the years.

Does lupus affect plans for a pregnancy?

It does, and so do some of the medicines that treat it, which is why pregnancy in lupus is planned rather than left to chance. Mention it to your specialist as soon as you begin to think about having a child, so that your treatment can be looked at and any change made well ahead of time. Pregnancy is among the strongest arguments for continuing specialist input in lupus.