The case for change: the debilitating burden of lupus1
We understand more about the burden of lupus than ever before, with many projects underway across the world looking to define the barriers faced by people living with lupus. A complex, chronic autoimmune disease, lupus causes the immune system to attack heathy tissue and affects an estimated 5 million people worldwide.2 Most people living with lupus have systemic lupus erythematosus (SLE), and experience systemic inflammation of multiple organs,3.4 which can cause many health problems including joint pain, skin rashes or lesions and debilitating fatigue.4-7
Most people living with SLE experience periods of worsening symptoms, known as flares, which are associated with increased risk of long-term damage to organs.8 Every flare a patient experiences regardless of severity, leads to increased risk of organ damage.9 In particular for those with moderate to severe disease, this significantly impacts use of health care services, leading to increased emergency hospital visits and health care costs.10 Up to 80 percent of people living with lupus are exposed to corticosteroids within five years of treatment, and will also use a median of five different medications to manage their symptoms. Corticosteroids can provide rapid symptom relief.11 However, long-term use of corticosteroids used in high doses, has been associated with irreversible organ damage, poor quality of life and other side effects.12.13 Despite this, there have been few new treatments over the past 60 years and patients are in urgent need of access to all available options.14
Lupus Europe’s 2020 Living with Lupus survey revealed the huge impact that SLE symptoms have on quality of life. Approximately half of respondents felt SLE had impacted their studies or employment status, as well as their ability to perform normal daily activities, with 58 percent of people reporting that lupus has an impact on their career.15
Because of the work of the patient advocacy and clinician community, including the Lupus Foundation of America’s ALPHA Project, the World Lupus Federation and Lupus Europe and others, we know that the challenges facing people living with SLE extend across a patient’s lifetime. People with lupus can wait an average of three-and-a-half years from the time they notice symptoms until they receive a formal diagnosis.16 Lupus can be challenging to diagnose because many of its main symptoms can be mistaken for symptoms of other diseases, making it difficult and time-consuming for patients to access the care they need.17-19
In the last decade, our understanding of the science and care of lupus has improved dramatically. As a result, new guidelines for how patients can be managed and treated have been developed, through the British Society of Rheumatology guideline (2017)20 and the European League Against Rheumatism (2019).21 However, due to the complex nature of lupus, there is variation in clinical practices and therefore in the care and treatment from patient to patient, around the world.22
Despite differences in care worldwide, the core challenges facing people living with lupus remain the same. There is exciting work ongoing to address this — from the ALPHA Project to the European Reference Network (ERN) ReCONNET, which aim to identify and address the barriers to accessing treatment and create a network of best practice care through the development of data registries and patient pathways, respectively.23.24 The recognition of SLE within the ReCONNET project demonstrates how Europe is helping lead the way in tackling some of the barriers to high-quality care.
This presents us with a unique opportunity. As we mark Lupus Awareness Month, and World Lupus Day, we want the global lupus community to join us in advocating for a better future for people living with lupus. We need to define the standard of care that people living with lupus deserve, which tackles the challenges across the patient pathway.
What quality care should look like
We need to reduce the time to diagnosis, educating patients and clinicians alike on the signs and symptoms of lupus, and minimizing delays that can cause increased organ damage and significantly impact people’s quality of life. Once they have received a diagnosis, we need to ensure that lupus patients have access to multidisciplinary and, where appropriate, specialist care, improving referrals to lupus specialists and taking advantage of advances in digital and telehealth. This care needs to consider patient preference, addressing their specific requests to improve their quality of life, and all lupus patients should receive a care plan that works for them. Following a historical lack of innovation in developing new treatment options, we now need to ensure that guidelines and health systems enable clinicians to prescribe all available treatments and those most appropriate for their patients to reduce disease activity, as well as minimize the use of corticosteroids.
There is a lot we can learn from other disease areas. The management of HIV has been transformed by access to multidisciplinary care.25 Thanks to treatment innovation and improvements in care pathways, the use of corticosteroids in rheumatoid arthritis has reduced significantly.26.27 To achieve optimal care in lupus, health systems should implement new care pathways that minimize the gaps in access to care and treatment, reducing the variation and fragmentation that lupus patients currently experience.
We want to support and collect insights from people living with lupus around the world to create a standard for high-quality lupus care. In doing so, we can empower the millions of people living with lupus to demand the care they deserve. It is time to set the bar for what high-quality care looks like for lupus. We stand ready to listen and collaborate with the global lupus community to chart the best path forward.
Note on development process
The above ‘Call to Action’ article was initiated by AstraZeneca to start a debate around what high-quality lupus care looks like from the patient’s perspective and invite the wider advocacy community to support our activity. These principles were debated and refined during a virtual discussion held on March 21 2022, organized and funded by AstraZeneca. Four academic, patient organization and professional group experts discussed the value of establishing a ‘Call to Action’, as a potential starting point for discussions on how to improve lupus care.
- Kent, T et al. “Burden of illness in systemic lupus erythematosus: results from a UK patient and carer online survey.” Lupus flight. 26.10 (2017): 1095-1100. doi:10.1177/0961203317698594
- The Lupus Foundation of America. What is Lupus? Available at: https://www.lupus.org/resources/what-is-lupus. Accessed April 2022.
- Centers for Disease Prevention and Control. Systemic Lupus Erythematosus. Available at: https://www.cdc.gov/lupus/facts/detailed.html#:~:text=doing%20about%20SLE%3F-,What%20is%20SLE%3F,%2C%20kidneys%2C% 20and%20blood%20vessels. Accessed April 2022.
- Santos, Liliana Ribeiro et al. “Recognition and management of systemic lupus erythematosus.” Prescriber 30 (2019)
- Al Sawah, Sarah et al. “Effect of corticosteroid use by dose on the risk of developing organ damage over time in systemic lupus erythematosus-the Hopkins Lupus Cohort.” Lupus science & medicine vol. 2.1e000066. 11 Mar. 2015, doi:10.1136/lupus-2014-000066
- McCauliffe, D P. “Cutaneous lupus erythematosus.” Seminars in cutaneous medicine and surgery vol. 20.1 (2001): 14-26. doi:10.1053/sder.2001.23091
- Uva, Luis et al. “Cutaneous manifestations of systemic lupus erythematosus.” Autoimmune diseases vol. 2012 (2012): 834291. doi:10.1155/2012/834291
- Langham, Julia, et al. “Disease Severity, Flares and Treatment Patterns in Adults with Systemic Lupus Erythematosus in the UK: A Real-World Observational Retrospective Cohort Analysis.” Rheumatology Advances in Practice, vol. 5, no. 3, 2021, https://doi.org/10.1093/rap/rkab061.
- Ugarte-Gil, Manuel F et al. “The number of flares patients experience impacts on damage accrued in systemic lupus erythematosus: data from a multiethnic Latin American cohort.” Annals of the rheumatic diseases flight. 74.6 (2015): 1019-23. doi:10.1136/annrheumdis-2013-204620
- Murimi-Worstell, Irene B et al. “Healthcare Utilization and Costs of Systemic Lupus Erythematosus by Disease Severity in the United States.” The Journal of Rheumatology flight. 48.3 (2021): 385-393. doi:10.3899/jrheum.191187
- Mosca, M et al. “Glucocorticoids in systemic lupus erythematosus.” Clinical and experimental rheumatology vol. 29.5 Suppl 68 (2011): S126-9.
- Bexelius, C et al. “Drivers of cost and health-related quality of life in patients with systemic lupus erythematosus (SLE): a Swedish nationwide study based on patient reports.” Lupus. 2013;22:793–801. doi: 10.1177/0961203313491849.
- Ruiz-Arruza, Ioana et al. “Glucocorticoids and irreversible damage in patients with systemic lupus erythematosus.” Rheumatology (Oxford, England) flight. 53.8 (2014): 1470-6. doi:10.1093/rheumatology/keu148
- Mahieu, et al. 2016. A critical review of clinical trials in systemic lupus erythematosus. Lupus, 25(10), 1122–1140. https://doi.org/10.1177/0961203316652492
- Cornet, Alain et al. “Living with systemic lupus erythematosus in 2020: a European patient survey.” Lupus science & medicine vol. 8.1 (2021): e000469. doi:10.1136/lupus-2020-000469
- Al Sawah S et al. “SAT0423 Understanding Delay in Diagnosis, Access to Care and Satisfaction with Care in Lupus: Findings from a Cross-Sectional Online Survey in the United States.” Presented at the European League Against Rheumatism (EULAR) 2015 Annual Conference. 2015.
- World Lupus Foundation. New Research Shows Top Barriers to Lupus Care and Diagnosis, Including Delayed Access to Specialists and High/Increased Health Care Costs. 2021. Available at: https://www.lupus.org/news/new-research-shows-top-barriers-to-lupus-care-and-diagnosis. [Last accessed: April 2022].
- Lupus Foundation of America. Access to Treatments. Available at: https://www.lupus.org/blog/access-to-treatments. [Last accessed: April 2022].
- Kernder, A et al. “Delayed diagnosis adversely affects outcome in systemic lupus erythematosus: Cross sectional analysis of the LuLa cohort.” Lupus. 2021 Mar; 30(3): 431–438. doi: 10.1177/0961203320983445
- Gordon C et al. The British Society for Rheumatology guideline for the management of systemic lupus erythematosus in adults: Executive Summary, Rheumatology, Volume 57, Issue 1, January 2018, Pages 14–18, https://doi.org/10.1093/rheumatology/kex291
- Aringer M et al. “2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol.” 2019;71(9):1400-1412. doi:10.1002/art.40930
- Tunnicliffe, David J et al. “Diagnosis, Monitoring, and Treatment of Systemic Lupus Erythematosus: A Systematic Review of Clinical Practice Guidelines.” Arthritis care & research flight. 67.10 (2015): 1440-52. doi:10.1002/acr.22591
- Lupus Foundation of America. Addressing Lupus Pillars for Health Advancement. Available at: https://www.lupus.org/partnerships-and-collaborations/the-alpha-project. Accessed April 2022
- European Commission. Health Program: DataBase, INTEGRATIONS/PROJECTS. Available at: https://webgate.acceptance.ec.europa.eu/chafea_pdb/health/projects/keyword/738/. Accessed March 2022
- Elgalib et al. AIDSCare. 2018;30(9):1114-1119 Elgalib, Ali et al. “Multidisciplinary care model for HIV improves treatment outcome: a single-centre experience from the Middle East.” AIDS-care flight. 30.9 (2018): 1114-1119. doi:10.1080/09540121.2018.1479028
- Dennison, Elaine M., and Cyrus Cooper. “Corticosteroids in Rheumatoid Arthritis: Effective Anti-Inflammatory Agents but Doubts about Safety Remain.” BMJ: British Medical Journal, vol. 316, no. 7134, 1998, p. 789–90, http://www.jstor.org/stable/25178546. Accessed 27 Apr. 2022.
- Clinical Immunotherapies. Corticosteroids in Rheumatoid Arthritis. Available at: https://link.springer.com/article/10.1007/BF03259279/ Accessed April 21 2022