Systemic lupus erythematosus

Lupus - a disease with a thousand faces

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect various organs, including the skin, joints, central nervous system and the kidneys.1 SLE is characterised by the production of pathogenic auto-antibodies and the loss of tolerance to nuclear self-antigens.2 Clinical heterogeneity, an unpredictable course and the occurrence of relapses are further typical features of SLE.3

Worldwide, more than 3.4 million people are affected by SLE4, of whom around 90% are women.5 It is estimated that around 1,200-4,000 patients in Switzerland suffer from SLE.5

In most cases, the development of SLE is multifactorial.5 Genetic interactions with environmental factors, in particular exposure to UV light, Epstein-Barr virus infection and hormonal factors, can trigger the disease and lead to dysregulation of the immune system.1

The physical and emotional burden for people suffering from SLE is substantial.7 The high morbidity, chronic course of the disease and dependence on corticosteroid therapy contribute to long-term organ damage, which can even lead to life-threatening systemic organ damage.4 However, new drugs and a better understanding of the disease have significantly improved the life expectancy and quality of life for those affected in recent years.5

Characteristics

 SLE is a chronic autoimmune disease that can affect all organs and tissues6

Unequal gender distribution

9 out of 10 of those affected are women, predominantly of childbearing age5

Prevalence

The prevalence of SLE is 20-50 cases per 100,000 inhabitants5

Symptoms

The clinical manifestations and the pattern of organ involvement are very heterogeneous in SLE.6 About 70% of patients have a relapsing-remitting course, the remaining patients divided equally between a prolonged remission and a persistently active disease.3

Symptoms that may be present at diagnosis:8

  • Joint pain (arthralgia/arthritis) (75%)
  • Photosensitive skin rash (55%)
  • Tiredness (fatigue) (48%)
  • Hair loss (alopecia) (41%)
  • Butterfly-shaped rash (butterfly erythema) (38%)
  • Raynaud’s syndrome (31%)
  • Blood count changes (haematological abnormalities) (31%)
  • Mucosal changes in the mouth (aphthae) (26%)
  • Fever (22%)
  • Inflammation of the costal pleura/pericardium
    (pleurisy/pericarditis) (12%)
  • Sjögren’s syndrome (8%)
  • Urine abnormalities (6%)
  • Neuropsychiatric symptoms (6%)
  • Weight loss/anorexia (3%)

If the disease is not recognised in time, the costal pleura, pleura, pericardium or heart valves can become inflamed. Also threatening is kidney inflammation (lupus nephritis), which can lead to kidney failure and dialysis.5

Diagnosis

The diagnosis of SLE is usually delayed due to the often diffuse symptoms. In the USA, it is estimated that it takes around 6 years from the onset of the first symptoms to diagnosis.9

The diagnosis of SLE is made clinically and is supported by laboratory tests that indicate immune reactivity or inflammation in various organs.3

Since 2019, EULAR and ACR have established new classification criteria to better recognise SLE.10

The following features are considered:10

  • Constitutional: fever
  • Haematological: leukocytes <4000, thrombocytes <100,000, haemolysis
  • Neuropsychiatric: delirium, psychosis or epilepsy
  • Mucocutaneous: acute cutaneous lupus (butterfly-shaped), subacute cutaneous or discoid lupus, alopecia or oral ulcers
  • Serosal: pleural or pericardial effusion or acute pericarditis
  • Muscle/skeleton: joint involvement (synovitis, pain on palpation, morning stiffness)
  • Kidneys: lupus nephritis, proteinuria >0.5 g/day
  • Immunological: antiphospholipid antibodies, low complement proteins (C3 and/or C4), SLE-specific antibodies (anti-dsDNA or anti-Smith antibodies)

Treatment

SLE is not curable.5 Early diagnosis, regular screening for organ involvement (especially nephritis), immediate initiation of treatment and strict adherence to treatment are essential to prevent relapses and organ damage, improve the prognosis and enhance the quality of life.11

Non-pharmacological measures, such as sun protection, not smoking, a healthy, balanced diet and regular exercise, are important to improve the long-term outcomes of treatment.11 The pharmacological therapies depend on the type and severity of organ involvement, concomitant diseases and the risk of progressive organ damage.11

According to the 2023 EULAR recommendations, hydroxychloroquine is recommended for all patients unless it is contraindicated. Glucocorticoids (GC) can be used as “bridging therapy” during phases of disease activity. For maintenance therapy, these should be minimised to equal to or less than 5 mg/day (prednisone equivalent) and, if possible, discontinued.11 If disease control is inadequate and to facilitate tapering off/stopping GC therapy, early initiation of immunosuppressive drugs (ISDs), such as methotrexate, azathioprine, mycophenolate, and/or biologic agents, such as anifrolumab, belimumab, should be considered.11

ACR: American College of Rheumatology; dsDNA: double-stranded DNA; EULAR: European Alliance of Associations for Rheumatology; SLE: systemic lupus erythematosus.

References:

  1. Kaul A, et al. Systemic lupus erythematosus. Nat Rev Dis Primers. 2016 Jun 16;2:16039. 
  2. Liu Z, Davidson A. Taming lupus-a new understanding of pathogenesis is leading to clinical advances. Nat Med. 2012 Jun 6;18(6):871-82. 
  3. Fanouriakis A, et al. Update on the diagnosis and management of systemic lupus erythematosus. Ann Rheum Dis. 2021 Jan;80(1):14-25. 
  4. Tian J, et al. Global epidemiology of systemic lupus erythematosus: a comprehensive systematic analysis and modelling study. Ann Rheum Dis. 2023 Mar;82(3):351-356. 
  5. University Hospital Zurich, Diseases and treatments, Systemic lupus erythematosus. Available online at: https://www.usz.ch/en/disease/systemic-lupus-erythematosus/. Last accessed on: 16/05/2024. 
  6. Fava A, Petri M. Systemic lupus erythematosus: Diagnosis and clinical management. J Autoimmun. 2019 Jan;96:1-13. 
  7. Rheumatism League Switzerland (2019): Systemic lupus erythematosus. Available online at: https://www.rheumaliga.ch/assets/doc/ZH_Dokumente/Broschueren-Merkblaetter/Krankheitsbilder/Lupus.pdf. Last accessed on: 16/05/2024. 
  8. Kapsala NN, et al. From first symptoms to diagnosis of systemic lupus erythematosus: mapping the journey of patients in an observational study. Clin Exp Rheumatol. 2023 Jan;41(1):74-81. 
  9. Al Sawah S et al. Understanding Delay in Diagnosis, Access to Care and Satisfaction with Care in Lupus: Findings from a Cross-Sectional Online Survey in the United States. Annals of the Rheumatic Diseases. 74. 812.3-812. 10.1136/annrheumdis-2015-eular.1159. 
  10. Aringer M. EULAR/ACR classification criteria for SLE. Semin Arthritis Rheum. 2019 Dec;49(3S):S14-S17. 
  11. Fanouriakis A, et al. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update. Ann Rheum Dis. 2024 Jan 2;83(1):15-29.

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