Systemic Lupus Erythematosus:
Recognising Flares, Treatment & Daily Life

1. What is systemic lupus erythematosus?

Systemic lupus erythematosus (SLE) is an autoimmune condition: the immune system makes antibodies against components of the cell nucleus. The immune complexes that form are deposited in tissues and set off inflammation there — the skin, joints, kidneys, blood count, the sac around the heart, the lining of the lungs and the nervous system can all be affected. Hence “systemic”.^¹

The best-known sign is the redness across the cheeks and the bridge of the nose, the butterfly rash — though by no means everyone has it. What is characteristic is the course in flares, and every flare can leave traces on the organs. Those affected are predominantly women from young adulthood onwards — which is why contraception and any wish to have children belong in the conversation here from the very start.^¹

2. Forms of lupus

“Lupus” is an umbrella term. The distinction matters, because the course, the monitoring and the treatment differ.^²

• Cutaneous lupus: stays confined to the skin — with scarring patches (discoid) or light-sensitive, ring-shaped patches (subacute cutaneous). Some people go on to develop a systemic form.
• Systemic lupus (SLE): the classic form — from mild courses with joint and skin complaints through to severe ones with kidney or nervous system involvement.
• Drug-induced lupus: a lupus-like picture caused by certain substances — described for individual blood pressure and heart rhythm medicines, anti-tuberculosis drugs and some biologics, among others. It usually resolves once the medicine is stopped.

Often there is also an antiphospholipid syndrome, a clotting disorder caused by autoantibodies. It raises the risk of thrombosis and pulmonary embolism and of complications in pregnancy.

3. Symptoms and consequences

Lupus is known as a “chameleon”, because hardly any two courses look alike. What is typical is several complaints sitting side by side.^¹,²

General, skin and joints

• Fatigue — a deep exhaustion that sleep does not put right; the article on persistent tiredness helps with putting it in context.
• Butterfly rash and light sensitivity — redness across the cheeks and the bridge of the nose, skin reactions or a full flare after sun exposure; other causes of a skin rash need to be told apart from it. Added to this are ulcers of the mucous membranes, diffuse hair loss, Raynaud's phenomenon and fever without signs of infection.
• Migrating joint pain with morning stiffness. Unlike rheumatoid arthritis, lupus does not as a rule destroy the joints — telling it apart from other forms of inflammatory rheumatic disease nevertheless remains important.

Internal organs

• Kidneys (lupus nephritis) — the involvement that matters most for the outlook; it runs without pain for a long time and shows up first in the urine.
• The sac around the heart and the lining of the lungs — chest pain that changes with breathing.
• Blood count and nervous system — anaemia or low platelets; problems with concentration through to rarer, severe events.

The most important consequence of untreated activity is permanent organ damage. The aim is therefore: lastingly low disease activity on as little steroid as possible.

4. Causes and flare triggers

Why the immune system loses its tolerance towards the body's own tissue is, according to current knowledge, not conclusively understood. An interplay of predisposition and outside factors is assumed.^²

• Predisposition and hormones — clustering within families is described, but lupus is not a hereditary condition; the preponderance of women points to an influence of the sex hormones.
• UV light — the most important avoidable trigger; sun can set off a full systemic flare.
• Infections — can set off flares and are at the same time more dangerous under immunosuppression.
• Smoking — worsens the course and, on current evidence, weakens the effect of antimalarials.
• Stopping the baseline therapy on your own — one of the most common and at the same time most avoidable triggers of a flare.

5. Diagnosis: what ANA results tell you

The diagnosis never comes from a single laboratory value, but from the overall picture: complaints, examination, laboratory tests, urine and, where needed, a tissue sample. Professional societies use classification criteria for this.^³

• History and examination: how long have the complaints been there, and do they come in flares? Reactions to the sun? Miscarriages? Which medicines are you taking?
• Antinuclear antibodies (ANA): the screening test — a negative result makes SLE very unlikely.
• Specific antibodies: anti-dsDNA and anti-Sm point clearly towards lupus, anti-Ro/SSA and anti-La/SSB matter for planning a pregnancy, antiphospholipid antibodies for the risk of thrombosis. Complement C3 and C4 often fall during a flare.
• Blood count, kidney and liver values: the basis for the diagnosis and for monitoring treatment — more on this under Understanding blood values.
• Urine testing: protein and red blood cells are the most important early sign of kidney involvement; where the finding is abnormal, a kidney biopsy follows.

6. Treatment: principles and steps

According to current knowledge lupus cannot be cured, but in many cases it can be controlled well. Treatment follows three aims: keep activity lastingly low, prevent flares, and keep the long-term consequences of the treatment small. Which step fits is decided by the rheumatology practice treating you.^⁴

7. Medicines for lupus

The medicines have different jobs: some keep the condition quiet in the long run, others are meant for an acute flare.^⁴,⁵

Antimalarials: the backbone of treatment

Hydroxychloroquine comes from malaria treatment and dampens certain immune cells. The effect builds up over weeks to months — which is exactly why it is so often stopped for the wrong reason, because people “do not notice anything”. Taking it without gaps is one of the most important factors for the long-term course.

Steroids: indispensable, but with a use-by date

Glucocorticoids such as prednisolone often cannot be replaced during a flare. What is problematic is long-term use: it favours osteoporosis, a rise in blood sugar, high blood pressure and susceptibility to infection — more in the cortisone guide.

Immunosuppressants and biologics

Methotrexate is used above all where joints and skin are involved and is taken once a week — that weekly dosing is a classic stumbling block. Azathioprine and mycophenolate mofetil are used more often where organs are involved. What they all have in common: they dampen the immune system and make infections more of an issue. Up-to-date vaccination cover should be sorted out before treatment starts, and where several products come together it is worth a look at interactions.

8. Sun protection is treatment, not cosmetics

UV light damages skin cells, which releases components of the cell nucleus — precisely the structures the autoantibodies are directed against. That can set off a full systemic flare, sometimes only days later.^⁵

• High sun protection every day: broad-spectrum protection against UVA and UVB with a very high sun protection factor — also when it is cloudy and in winter, because UVA radiation passes through cloud and window glass. It only works if you use enough of it.
• Textile protection first: closely woven clothing, a wide-brimmed hat and sunglasses are more reliable than any cream. Avoid the midday sun, and no sunbeds.
• Keep an eye on vitamin D: anyone who consistently avoids UV has a raised risk of deficiency. Whether vitamin D makes sense is decided by the blood level — not by a blanket rule.

Often overlooked: some medicines make the skin additionally sensitive to light — which groups are affected is explained in the guide Medications and sun.

9. Kidney involvement

Lupus nephritis — inflammation of the filtering units of the kidney caused by deposited immune complexes — shapes the long-term course more than almost any other organ involvement. Its catch: it does not hurt. The first pointers come from the urine: protein and red blood cells appear long before blood values change. Later, water retention, foaming urine or new high blood pressure can be added.^⁴

Even where the kidneys are involved, the course today is usually considerably more favourable than it used to be — provided it is picked up early and treated consistently.

10. Planning a pregnancy and contraception

With stable lupus, pregnancy is usually possible. What is decisive is the planning — early, not first at the positive test.^⁴

• Wait for a stable phase: a stretch of quiet disease over a longer period is recommended; during an active flare there are considerably more complications.
• Switch medicines in good time: some immunosuppressants — among them methotrexate and mycophenolate — must not be used in pregnancy and need lead time. Others are regarded as compatible. Background is given in the guide Medications during pregnancy.
• Clarify the antibody status: anti-Ro/SSA, anti-La/SSB and antiphospholipid antibodies are determined beforehand; rheumatology and obstetrics look after you together.

Contraception: not every method fits

The central issue is the risk of thrombosis: if antiphospholipid antibodies are detectable or disease activity is high, oestrogen-containing products such as the combined pill are usually avoided. Progestogen-only methods or coils are then the better choice — more on this in the guide Medications and contraception. The decision is taken by the practice treating you together with you.

11. Everyday life with lupus

• See the baseline therapy through — and especially when you are feeling well. Being free of symptoms is the result of the treatment, not proof that it is unnecessary.
• Meet fatigue with pacing — portion out tasks and plan breaks before the exhaustion arrives. Moderate movement tends to work against it; pointers are given in the guide Medications and exercise.
• Take infections seriously — under immunosuppression, fever, feeling severely unwell or breathlessness need assessing promptly. Inflammation and steroids also raise the cardiovascular risk.
• Document your flares — date, complaints, triggers, changes. The guide Living with a chronic illness helps with structuring this.

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