Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder with strikingly diverse clinical manifestations that can affect many organ systems, including the skin, joints, central nervous system, and kidneys.1

SLE is characterized by periods of increased disease activity, known as flares, and remission; these cycles vary in frequency and severity.2

SLE patients who continue to have flares and uncontrolled underlying disease activity experience substantial physical, social, and economic burden and diminished quality of life.3-9

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SLE prevalence varies by gender, ethnicity, region, and social economic status; prevalence is higher among Hispanics, African Americans, and American Indian/Alaskan Native populations relative to White North European population. Further, women are far more frequently affected than men; for each man, 8–9 women are affected.1,10-14

SLE is caused by an autoimmune reaction in which the innate and adaptive immune systems direct an inappropriate immune response against nucleic acid–containing cellular particles.1

ANAs are traditionally viewed as essential mediators of pathology in SLE, particularly when they form immune complexes. Virtually all patients with SLE are positive for ANAs or other characteristic SLE autoantibodies.1,15

The pathogenesis of SLE hinges on the breakdown of immune regulation leading to aberrant activity of autoreactive T- and B-cells, persistent autoantibody production, and cytokine release which drive organ damage.16-24

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Inflammation pathways in SLE

Pathogenesis of SLE involves the breakdown in both B-cell and T-cell tolerance.25,26

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SLE clinical manifestations are strikingly diverse1

SLE CLINICAL MANIFESTATIONS ARE STRIKINGLY DIVERSE1


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SLE diagnosis, classification, and disease activity measures


Diagnosis of SLE is made based on clinical manifestations and laboratory tests, including the detection of autoantibodies, functional tests, and imaging.1

The three main classification criteria that have been used are ACR (1997), SLICC (2012), and EULAR-ACR (2019).1,45

Assessment of disease activity is challenging because of the multifaceted complexity of the clinical presentations and their variation over time.1

To access overall systemic disease activity1,25,45,46

  • SLEDAI
  • SELENA-SLEDAI
  • Hybrid SLEDAI
  • SLEDAI-2K
  • BILAG-2004 Index
  • PGA
  • SRI*
  • BICLA*

To assess disease activity on specific organs1,25,45

Joint count - musculoskeletal disease activity

CLASI - mucocutaneous disease activity

*Commonly used composite indices to evaluate therapeutic response.25