Characteristics of lupus

Lupus is a complex, chronic, multisystem autoimmune disease in which many patients experience irreversible organ damage.1-3

Lupus is characterized by:

Icon: antibodies

Pathological production of autoantibodies such as ANA1,4

Icon: disease activity

Waxing and waning disease activity1

Icon: Inflammation in multiple organ systems

Inflammation in multiple organ systems5

Icon: B cells

Abnormal activation of B and T cells6,7

ANA = antinuclear antibodies.

Mechanism of disease

Learn how lupus affects the body.

video transcript

NARRATOR:
Systemic lupus erythematosus, commonly referred to lupus or SLE, is the most common form of lupus.

REFERENCE: 1. Maidhof W, Hilas O. Lupus: An overview of the disease and management options. Pharm Ther. 2012;37(4):240.

NARRATOR:
Patients suffer from a range of debilitating effects of this autoimmune disease . . . each and every day.

REFERENCES: 1. Anders HJ, Saxena R, Zhao MH, et al. Lupus nephritis. Nat Rev Dis Primers. 2020;6(1):1-25. 2. Cancro MP, D’Cruz DP, Khamashta MA. The role of B lymphocyte stimulator (BLyS) in systemic lupus erythematosus. J Clin Investig. 2009;119(5):1066-1073. 3. Maidhof W, Hilas O. Lupus: An overview of the disease and management options. Pharm Ther. 2012;37(4):240.

NARRATOR:
Lupus may manifest in 1 or multiple body systems, . . . such as the skin, . . . cardiovascular, pulmonary, . . . musculoskeletal, and renal systems, to name a few. Permanent organ damage can occur if lupus is not treated appropriately, which can have serious consequences for patients.

ON-SCREEN TEXT:
Skin
Cardiovascular
Pulmonary
Musculoskeletal
Renal

REFERENCES: 1. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Guidelines for referral and management of systemic lupus erythematosus in adults. Arthritis Rheum. 1999;42(9):1785-1796. 2. Anders HJ, Saxena R, Zhao MH, et al. Lupus nephritis. Nat Rev Dis Primers. 2020;6(1):1-25. 3. Cancro MP, D’Cruz DP, Khamashta MA. The role of B lymphocyte stimulator (BLyS) in systemic lupus erythematosus. J Clin Investig. 2009;119(5):1066-1073. 4. Heinlen LD, McClain MT, Merrill J, et al. Clinical criteria for systemic lupus erythematosus precede diagnosis, an associated autoantibodies are present before clinical symptoms. Arthritis Rheum. 2007;56(7):2344-2351. 5. Lopez R, Davidson JE, Beeby MD, et al. Lupus disease activity and the risk of subsequent organ damage and mortality in a large lupus cohort. Rheumatol. 2012;51(3):491-498.

NARRATOR:
Inside the body, . . . B cells produce antibodies to help fight off infection. In lupus, autoreactive B cells produce autoantibodies that attack and destroy healthy tissues and organs, causing widespread inflammation.

ON-SCREEN TEXT:
Pathogen
Antibodies
B cell
Inflammation
Autoantibodies
Autoreactive B Cell

REFERENCES: 1. Cancro MP, D’Cruz DP, Khamashta MA. The role of B lymphocyte stimulator (BLyS) in systemic lupus erythematosus. J Clin Investig. 2009;119(5):1066-1073. 2. Maidhof W, Hilas O. Lupus: An overview of the disease and management options. Pharm Ther. 2012;37(4):240. 3. Marieb EN, Hoehn K, eds. Human Anatomy & Physiology. 11th ed. Pearson Education, Inc; 2019. 4. Mok CC, Lau CS. Pathogenesis of systemic lupus erythematosus. J Clin Pathol. 2003;56(7):481-490. 5. Murphy K, Weaver C. Janeway's Immunobiology. 9th ed. Garland Science; 2008.

NARRATOR:
In lupus nephritis, the immune system attacks and damages nephrons. Let’s take a closer look at how this happens.

ON-SCREEN TEXT:
Nephrons

REFERENCE: 1. Anders HJ, Saxena R, Zhao MH, et al. Lupus nephritis. Nat Rev Dis Primers. 2020;6(1):1-25.

NARRATOR:
In the blood, autoantibodies bind to anti-dsDNA and form immune complexes. One mechanism of tissue damage in lupus nephritis is the formation of immune complexes that accumulate and deposit in the filtration membrane formed by podocytes in the glomeruli, which impairs kidney function. The resulting inflammation leads to a buildup of waste in the blood and protein excreted in the urine (or proteinuria), which is a defining feature of lupus nephritis. This may ultimately lead to end-stage kidney disease that requires dialysis or kidney transplant. Low levels of complement proteins in lupus nephritis make it difficult to clear immune complex deposits.

ON-SCREEN TEXT:
Autoantibodies
Plasma cells
Immune complexes
Podocytes
Immune complex deposit
Damage
Complement

REFERENCES: 1. Almaani S, Meara A, Rovin BH. Update on lupus nephritis. Clin J Am Soc Nephrol. 2017;12(5):825-835. 2. Anders HJ, Saxena R, Zhao MH, et al. Lupus nephritis. Nat Rev Dis Primers. 2020;6(1):1-25. 3. Crow MK. Etiology and pathogenesis of systemic lupus erythematosus. Kelley and Firestein's Textbook of Rheumatology. Elsevier; 2017;1329-1344. 4. Davidson A, Berthier C, Kretzler M. Pathogenetic mechanisms in lupus nephritis. In: Dubois' Lupus Erythematosus and Related Syndromes. WB Saunders; 2013: 237-255. 5. Lech M, Anders HJ. The pathogenesis of lupus nephritis. J Am Soc Nephrol. 2013;24(9):1357-1366. 6. Marieb EN, Hoehn K, eds. Human Anatomy & Physiology. 11th ed. Pearson Education, Inc; 2019. 7. Song K, Liu L, Zhang X, Chen X. An update on genetic susceptibility in lupus nephritis. Clin Immunol. 2020;210:108272. 8. Toong C, Adelstein S, Phan TG. Clearing the complexity: immune complexes and their treatment in lupus nephritis. Int J Nephrol Renovasc Dis. 2011;4:17.

NARRATOR:
In treating lupus, with or without lupus nephritis, steroids can help control symptoms, but long-term use may also contribute to further organ damage. As a result, guideline and treatment recommendations suggest limiting their use or minimizing their dosage, leaving patients with few treatment options.

ON-SCREEN TEXT:
Continued damage

REFERENCES: 1. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Guidelines for referral and management of systemic lupus erythematosus in adults. Arthritis Rheum. 1999;42(9):1785-1796. 2. Anders HJ, Saxena R, Zhao MH, et al. Lupus nephritis. Nat Rev Dis Primers. 2020;6(1):1-25. 3. Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736-745.

ON-SCREEN TEXT:
BENLYSTA (belimumab)
Intravenous Use 120 mg/via
Subcutaneous Use 200 mg/mL

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Persistent disease activity, including flares, can contribute to organ damage8-10

Graph displaying persistent disease activity can contribute to organ damage
Graph displaying persistent disease activity can contribute to organ damage

Representation of disease for illustrative purposes only.

Icon: 1 in 2 patients

Did you know?

Approximately 1 in 2 patients experience irreversible organ damage within 5 years of diagnosis2,3*†‡

Organ damage in patients with lupus can affect multiple organ systems2,11-13

Icon: Musculoskeletal

Musculoskeletal

Icon: Pulmonary

Pulmonary

Icon: Renal

Renal

Icon: Cardiovascular

Cardiovascular

Icon: Ocular

Ocular

List of organ systems is not all inclusive.

Increased difference in the occurrence of organ damage, as defined by SDI, between steroid-exposed (n=173) vs steroid-naïve (n=86) patients with lupus14§

Increased difference in the occurrence of organ damage, as defined by SDI, between steroid-exposed (n=173) vs steroid-naïve (n=86) patients with lupus
Increased difference in the occurrence of organ damage, as defined by SDI, between steroid-exposed (n=173) vs steroid-naïve (n=86) patients with lupus

* Damage in SLE is defined as an irreversible tissue injury occurring after diagnosis of SLE and lasting at least 6 months. SLICC/ACR Damage Index (SDI) is the internationally agreed and validated measure of organ damage.8,11

† Cohort analysis of 298 patients followed for a minimum of 5 years by the SLICC International Research Network, comprising 27 centers from 11 countries. Year 0 represents time of enrollment. Mean age at enrollment was 35.3 years. Fifty percent of patients acquired organ damage at Year 5.3

‡ Retrospective analysis of records from 401 patients (232 patients with ≥10 years of consistent follow-up) attending the University College London Hospital SLE clinic between 1978–2004. Year 0 represents time of diagnosis. Mean age at diagnosis was 31.2 years. Thirty-three percent of patients acquired organ damage at Year 5.2

§ A study of 259 patients (glucocorticosteroid-naïve group [86] and glucocorticosteroid-exposed group [173]) with SLE enrolled in the University of Toronto Lupus Clinic. Organ damage was measured using the SDI at 3, 5, and 8 years. The average baseline steroid dose was 34.6 mg/day in the steroid-exposed group. The average cumulative steroids in the first 3, 5, and 8 years (baseline SDI = 0) were 16.11 g, 22.61 g, and 36.71 g, respectively.14

SDI = SLICC/ACR Damage Index; SLE = systemic lupus erythematosus; SLICC/ACR = Systemic Lupus International Collaborating Clinics/American College of Rheumatology.

2023 EULAR recommendations

Early lupus diagnosis, prompt treatment initiation aiming for remission (or low disease activity if not possible), and strict adherence to treatment are essential to prevent flares and organ damage, improve prognosis, and enhance quality of life.15

See the data

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Did you know?

Lupus can be fatal. It is the 10th leading cause of death in women aged 15–24, and the 6th for African-American and Hispanic women aged 25–34.16

Lupus disease activity

Disease activity includes all signs and symptoms or laboratory abnormalities due to lupus-related pathophysiology.5,8

Clinical and/or serological disease activity may be present. Selected features may include8:

  • Inflammatory/non-inflammatory symptoms in any system
  • Autoantibodies (eg, ANA)
  • Low complement C3 and/or C4
  • Increased gamma globulin serum levels

Flares

A flare is a measurable increase in disease activity and includes new or worse clinical symptoms and/or laboratory abnormalities.5

Lupus flares, persistent disease activity, and the prolonged use of corticosteroids in the long-term management of lupus can all contribute to organ damage.3,8,9,10,17

Various tools, including SELENA-SLEDAI, can be used to measure disease activity.

Download the SELENA-SLEDAI tool

SELENA-SLEDAI = Safety of Estrogens in Lupus Erythematosus: National Assessment version of the Systemic Lupus Erythematosus Disease Activity Index.

Lupus nephritis is a real risk for many patients with lupus

Icon: 5 of 10 patients

Up to 5 of the next 10 patients with lupus a doctor sees may develop lupus nephritis18

31%–48% of patients will develop lupus nephritis at some point after their initial lupus diagnosis18*

Icon: 20% of patients

Approximately 20% of patients with lupus nephritis will progress to ESKD within 10 years of diagnosis19

* Data from a pragmatic review of 26 publications involving patients with lupus nephritis with or without a proven biopsy.

ESKD = end-stage kidney disease.

Just one renal flare could shorten a kidney’s life span by decades20-22*

With each renal flare, there is irreversible nephron loss – shortening the kidney’s life span and increasing the risk of ESKD

Potential impact of lupus nephritis on kidney life span

Potential impact of lupus nephritis on kidney lifespan chart
Potential impact of lupus nephritis on kidney lifespan chart

Adapted with permission from Anders HJ, et al., 2020.

45% of patients with lupus nephritis experience renal flares despite receiving immunosuppressive therapy20,23*

It is critical to reduce the number of renal flares to prevent progression to ESKD and the need for dialysis20,22

* Renal flares are defined as a rise in serum creatine level and/or proteinuria, abnormal urinary sediment, or reduction in creatine clearance.20,22

CKD = chronic kidney disease; GFR = glomerular filtration rate.

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Efficacy for lupus

BENLYSTA helped appropriate patients with lupus.

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How BENLYSTA works

Discover the mechanism of action of BENLYSTA, designed to target BLyS, an underlying cause of lupus.

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The risk of organ damage is real.
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