What is Systemic Lupus Erythematosus (SLE)?

From the Guidelines

Systemic Lupus Erythematosus (SLE) is a chronic, potentially lethal autoimmune disorder characterized by immune complex deposition and vasculopathy, affecting multiple organs and tissues, with a complex interplay of genetic susceptibility and environmental factors. According to the most recent evidence 1, SLE is a multisystem disease that can affect various organs, including the cardiovascular, dermatologic, gastrointestinal, immune-hematologic, musculoskeletal, neuropsychiatric, pulmonary, and renal systems. The exact cause of SLE remains unknown, but it is believed to involve a combination of genetic predisposition and environmental triggers, such as infections, certain medications, or sunlight exposure 1.

Some key characteristics of SLE include:

• Clinical heterogeneity, with symptoms varying widely among patients
• Higher prevalence among women, particularly those of childbearing age
• Estimated prevalence of 20 to 150 cases per 100,000 population
• Immune complex deposition and vasculopathy, leading to inflammation and damage to various body parts
• Increased risk of infections, particularly bacterial infections, due to immunosuppressant therapy and disease-related immune dysregulation

The diagnosis and management of SLE are complex and require a multidisciplinary approach, with treatment goals including long-term patient survival, prevention of organ damage, and optimization of health-related quality of life 1. The European League Against Rheumatism (EULAR) recommends that treatment in SLE aims at remission or low disease activity and prevention of flares, with hydroxychloroquine recommended in all patients with lupus, at a dose not exceeding 5 mg/kg real body weight 1.

In summary, SLE is a complex autoimmune disease that requires prompt diagnosis, multidisciplinary management, and individualized treatment to prevent organ damage and optimize quality of life. With proper treatment, patients with SLE can experience significant improvements in symptoms and quality of life, and reduce their risk of morbidity and mortality 1.

From the Research

Definition and Overview of Systemic Lupus Erythematosus (SLE)

• Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect various parts of the body, including the skin, joints, kidneys, heart, lungs, nervous system, blood, and other organs or systems 2, 3, 4, 5.
• It is characterized by inflammation and immune-mediated injury to multiple organ systems, with approximately 3.4 million people worldwide having received a diagnosis of SLE 3.
• SLE is a highly complex and heterogeneous autoimmune disease that most often afflicts women in their child-bearing years, with approximately 90% of people with SLE being female 3.

Clinical Manifestations and Diagnosis

• The disease can cause a range of symptoms, from mild rashes and arthritis to debilitating fever, fatigue, joint pain, and severe organ and/or life-threatening disease 2, 3, 5.
• There are no uniformly accepted diagnostic criteria for SLE, but the 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology classification criteria are an estimated 96.1% sensitive and 93.4% specific for SLE 3.
• The classification criteria include both clinical factors, such as fever, cytopenia, rash, arthritis, and proteinuria, and immunologic measures, such as SLE-specific autoantibodies and low complement levels 3.

Treatment and Management

• The primary goal of treatment is to achieve disease remission or quiescence, defined by minimal symptoms, low levels of autoimmune inflammatory markers, and minimal systemic glucocorticoid requirement while the patient is treated with maintenance doses of immunomodulatory or immunosuppressive medications 3.
• Hydroxychloroquine is the standard of care for SLE and has been associated with a significant reduction in mortality 3, 6.
• Treatments in addition to hydroxychloroquine are individualized, with immunosuppressive agents, such as azathioprine, mycophenolate mofetil, and cyclophosphamide, typically used for treating moderate to severe disease 3.
• Recent approvals by the Food and Drug Administration include belimumab, voclosporin, and anifrolumab for the treatment of SLE 3.