Lupus (systemic lupus erythematosus, or SLE) is a chronic autoimmune disease in which the immune system attacks the body’s own tissues, causing inflammation across multiple organ systems. It affects around 50,000 people in the UK, with women accounting for approximately 90% of cases. Lupus is most commonly diagnosed between the ages of 15 and 45 and is significantly more prevalent in women of Black African, Caribbean, and South Asian heritage.
Types of Lupus
- Systemic lupus erythematosus (SLE) — The most common and serious form; can affect any organ
- Cutaneous (discoid) lupus — Affects skin only; causes scarring, coin-shaped rashes; does not typically affect internal organs
- Drug-induced lupus — Caused by certain medications (hydralazine, isoniazid, procainamide); resolves when drug is stopped
- Neonatal lupus — Rare; affects newborns of mothers with certain lupus antibodies; usually resolves by 6 months
Symptoms of Lupus
Lupus is often called “the great imitator” because its symptoms overlap with many other conditions, making diagnosis challenging. Symptoms vary widely between individuals and often flare and remit:
Common Symptoms
- Extreme fatigue — The most universal symptom; often debilitating
- Butterfly (malar) rash — Red rash across the cheeks and bridge of the nose; present in ~50% of people
- Joint pain, swelling, and stiffness — Often migratory; similar to RA but usually does not cause joint destruction
- Skin sensitivity to sunlight (photosensitivity) — Rashes or flares triggered by UV exposure
- Hair loss (alopecia)
- Mouth or nose ulcers
- Raynaud’s phenomenon — Fingers/toes turning white or blue in cold
- Fever without obvious cause
- Chest pain — From pleuritis (inflammation around lungs) or pericarditis (around heart)
- Swollen lymph nodes
- Headaches, memory problems (“lupus fog”)
Organ Involvement in Severe Lupus
- Kidneys (lupus nephritis) — Affects up to 60% of SLE patients; can cause proteinuria, haematuria, hypertension, kidney failure if untreated
- Brain and nervous system (neuropsychiatric lupus) — Seizures, psychosis, stroke, depression
- Heart — Pericarditis, Libman-Sacks endocarditis, accelerated atherosclerosis
- Lungs — Pleuritis, pulmonary hypertension, pneumonitis
- Blood — Anaemia, low white cells (leucopenia), low platelets (thrombocytopenia)
Diagnosis
There is no single test for lupus. Diagnosis is based on clinical criteria (the 2019 EULAR/ACR classification) using a combination of symptoms and investigations:
Blood Tests
- ANA (antinuclear antibody) — Positive in >95% of SLE patients but not specific (also positive in other conditions and healthy people)
- Anti-dsDNA antibodies — Highly specific to lupus; correlates with disease activity and lupus nephritis
- Anti-Sm (Smith) antibodies — Highly specific to SLE
- Anti-Ro and anti-La antibodies — Associated with neonatal lupus and skin manifestations
- Anti-phospholipid antibodies — Raise risk of clots and miscarriage (antiphospholipid syndrome)
- Complement levels (C3, C4) — Low during flares
- FBC — May show anaemia, leucopenia, thrombocytopenia
- CRP and ESR — ESR elevated in lupus, CRP typically normal unless infection
Urine Tests
Urine dipstick and protein:creatinine ratio (PCR) or albumin:creatinine ratio (ACR) performed regularly to screen for lupus nephritis — a serious complication requiring prompt treatment.
Other Investigations
- Skin or kidney biopsy (for diagnosis and classification of lupus nephritis)
- Echocardiogram, ECG (for cardiac involvement)
- Chest X-ray or CT (for lung involvement)
- MRI brain (for neuropsychiatric lupus)
Treatment
Hydroxychloroquine (Plaquenil)
Hydroxychloroquine is the backbone of lupus treatment, recommended for virtually all SLE patients regardless of disease severity. It reduces flare frequency, organ damage accumulation, and mortality. Regular eye screening (annual OCT scan) is required as there is a small risk of retinal toxicity with long-term use.
NSAIDs and Steroids
- NSAIDs (ibuprofen, naproxen) — For joint pain and fever; avoid if kidney disease present
- Corticosteroids (prednisolone) — For active flares; the goal is to minimise long-term use due to side effects (osteoporosis, weight gain, infection risk, cataracts)
Immunosuppressants
- Azathioprine — Maintenance therapy; used for organ-threatening and non-renal lupus
- Mycophenolate mofetil (MMF) — First-line induction and maintenance for lupus nephritis; also used for other manifestations
- Cyclophosphamide — IV pulses for severe lupus nephritis or neuropsychiatric lupus (the Euro-Lupus regimen)
- Methotrexate — Used for skin and joint manifestations
Biologic Therapies
- Belimumab (Benlysta) — Anti-BLyS biologic; NICE-approved for adults with active SLE who are inadequately controlled on standard therapy; now also NICE-approved for lupus nephritis
- Anifrolumab (Saphnelo) — Anti-interferon type I receptor; approved by MHRA and NICE (2023) for moderate-to-severe SLE; a significant new addition to the treatment landscape
- Rituximab — Used off-label for refractory cases; particularly for severe haematological manifestations
- Voclosporin (Lupkynis) — Calcineurin inhibitor approved with MMF for lupus nephritis
Lupus and Pregnancy
Lupus is compatible with pregnancy, but requires careful planning and specialist management. Key points:
- Disease should ideally be in remission for at least 6 months before conception
- Hydroxychloroquine is safe in pregnancy and protects both mother and baby — should be continued
- Anti-Ro/La antibodies can cause neonatal lupus and fetal heart block — requires fetal cardiac monitoring
- Antiphospholipid syndrome raises risk of miscarriage and clots — treated with aspirin and/or heparin
- Lupus nephritis in pregnancy requires specialist obstetric nephrology care
- Methotrexate, cyclophosphamide, and MMF are contraindicated in pregnancy
Monitoring and Follow-Up
People with lupus require regular monitoring, typically every 3–6 months in stable disease:
- Blood pressure (hypertension is common and accelerates organ damage)
- Urine dipstick and PCR (renal screen)
- Blood tests: FBC, renal profile, LFTs, complement, anti-dsDNA
- Annual eye review (hydroxychloroquine screening)
- Cardiovascular risk assessment (lupus is a major cardiovascular risk factor)
- DEXA scan (bone density) in those on long-term steroids
- Skin cancer awareness (especially in discoid lupus)
Living with Lupus
- Sun protection — High-factor sunscreen (SPF 50+) and protective clothing are essential; UV triggers flares
- Pacing and rest — Managing fatigue requires balancing activity with adequate rest
- Vaccinations — Annual flu vaccine, COVID-19 booster, and pneumococcal vaccine are recommended; live vaccines should be discussed with your specialist
- Smoking cessation — Smoking worsens lupus activity and cardiovascular risk
- Mental health — Anxiety and depression are common; psychological support should be part of multidisciplinary care
Support and Resources
- Lupus UK — lupusuk.org.uk — Helpline: 01708 731251; has local groups across the UK
- LUPUS Europe — lupus-europe.org
- NHS Lupus information — nhs.uk/conditions/lupus
- Versus Arthritis — versusarthritis.org — includes lupus resources
This page is for general information only. If you think you may have lupus, see your GP. A rheumatologist specialist will manage your care.
Related Health Guides on YourHealthXpert
Explore these related NHS-aligned health guides:
- Rheumatoid Arthritis Guide — Both lupus and RA are autoimmune conditions causing joint inflammation; understand the diagnostic differences and NHS treatment pathways.
- Chronic Kidney Disease Guide — Lupus nephritis affects around one in three lupus patients; learn about NHS kidney monitoring and treatment in lupus.
- Osteoporosis Guide — Corticosteroids used in lupus treatment significantly increase osteoporosis risk; understand NHS bone protection strategies.
- Anxiety & Depression Guide — Living with a chronic, unpredictable condition like lupus significantly impacts mental health; learn about NHS psychological support.
- High Blood Pressure Guide — Lupus causes vascular inflammation that raises cardiovascular risk including hypertension; understand the NHS monitoring approach.
- Menopause Guide — Lupus predominantly affects women of reproductive age and interacts with hormonal changes; learn about managing lupus through the menopause.