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Lupus

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Last updated: 16th Jul 2021

Lupus

What is lupus?

Lupus is an inflammatory autoimmune disease that can cause damage to the joints, skin, organs (including the lungs, heart, brain, and kidney), and blood vessels1,2. Approximately 5 million people worldwide have systemic lupus erythematosus (SLE), the most common and serious type of lupus which can be life-threatening1,2. Less serious types of lupus include lupus dermatitis, neonatal lupus, and drug-induced lupus.  

What are the symptoms of lupus?

Lupus symptoms and severity vary widely, and often occur as lupus flares3. Early signs of lupus include joint pain, fever and tiredness3. Other symptoms of lupus include sensitivity to sun, arthritis, loss of appetite, muscle aches, facial rash (also known as a butterfly rash), chest pain and vasculitis3.

Why is lupus life-threatening?

For systemic lupus erythematosus (SLE), the worst affected patients have refractory lupus, and the accrual of life-threatening damage to vital organs, including the kidneys (lupus nephritis), heart, blood vessels, and brain4. Lupus-associated organ damage can be triggered either through autoimmune-induced inflammation to organ tissue, or through long-term corticosteroid use4,5. Indeed, mortality in SLE is consistently associated with incremental organ damage6.

How is lupus treated?

Lupus management varies depending on lupus severity and prior response to available lupus treatments. A key lupus treatment goal is to improve long-term outcomes and prevent long-term organ damage accrual, particularly for SLE7. Pharmacologic treatments for lupus and SLE include hydroxychloroquine, glucocorticoids, immunosuppressive drugs, and more recently, biologic agents7.

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References

  1. Klavdianou K, Lazarini A, Fanouriakis A. Targeted Biologic Therapy for Systemic Lupus Erythematosus: Emerging Pathways and Drug Pipeline. BioDrugs. 2020;34(2):133–147.
  2. Touma Z, Gladman DD. Current and future therapies for SLE: obstacles and recommendations for the development of novel treatments. Lupus Sci Med. 2017;4(1):p.e000239.
  3. The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults. https://academic.oup.com/rheumatology/article/57/1/e1/4318863. Accessed 25 February, 2021.
  4. Pons-Estel GJ, Alarcón GS, Scofield L, Reinlib L, Cooper GS. Understanding the epidemiology and progression of systemic lupus erythematosus. Sem Arth Rheum. 2010;39(4):257–268.
  5. Murimi-Worstell IB, Lin DH, Nab H, Kan HJ, Onasanya O, Tierce JC et al. Association between organ damage and mortality in systemic lupus erythematosus: a systematic review and meta-analysis. BMJ Open. 2020;10:e031850.
  6. Chambers SA, Allen E, Rahman A, Isenberg D. Damage and mortality in a group of British patients with systemic lupus erythematosus followed up for over 10 years. Rheum. 2009;48:673–675.
  7. Fanouriakis A, Kostopoulou M, Alunno A, Aringer M, Bajema I, Boletis JN et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.