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Content Update

March 25, 2022

Drug Updates in Lupus Anifrolumab (Saphnelo™) is a type I interferon (IFN) receptor antagonist, immunoglobulin G1 kappa (IgG1κ) monoclonal antibody approved in 2021 for adults with moderate to severe systemic lupus erythematosus (SLE). Voclosporin (Lupkynis™) is a calcineurin-inhibitor immunosuppressant approved in 2021 for adults with active lupus nephritis (LN) in combination with a background immunosuppressive therapy regimen.



Upon completion of the chapter, the reader should be able to:

  1. Discuss the incidence and risk factors for systemic lupus erythematosus (SLE).

  2. Describe key events in the pathogenesis of SLE including contributions of genetics, hormonal, and environmental factors to the immune dysfunction.

  3. Explain symptoms and signs of the disease and tissue damage associated with SLE.

  4. List diagnostic criteria for SLE.

  5. Recommend nonpharmacologic therapies including cognitive behavioral therapy, sun protection and smoking cessation.

  6. Compare and contrast pharmacologic treatment options for SLE by their mechanisms of action, side effects, contraindications, and effectiveness.

  7. Discuss the clinical manifestations and appropriate therapy used to treat the corresponding disease manifestations associated with SLE.

  8. Provide a counseling plan for adjunct therapies used to treat clinical manifestations of SLE.


Often difficult to diagnose, systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease in which the immune system attacks the body’s own tissue, causing inflammation and tissue damage. A delay in definitive diagnosis can lead to delays in organ-saving treatment. SLE often involves multiple organs and is characterized by flares and periods of quiescence.


The incidence and prevalence of SLE is highest in North America, with estimates at 23.2 per 100,000 person-years, and 241 cases per 100,000 people.1 Known risk factors for SLE include demographics, genetics, and interactions with environmental triggers. image The strongest risk factor for SLE is gender. Nine of 10 people living with a lupus diagnosis are women,2 and women of color are disproportionately affected. On average, women of color are two to three times more likely to have SLE and more severe disease.2 Women of childbearing age are also most likely to be affected, with most diagnoses occurring between 15 and 44 years of age.2 Genetics may predispose some individuals to the development of SLE after interactions with certain triggers. Normal variations in a variety of genes can increase the risk of developing SLE, and in most cases, multiple genes are involved. Environmental triggers include exposure to sunlight, infections, or exposure to certain medications (see Table 56–1).3-5

Table 56–1Medications Associated with Drug-Induced Lupus3-5

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