Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder that, while challenging to diagnose due to its wide range of symptoms, is now better understood and more effectively managed than ever before. Advances in medical research and treatment have transformed SLE from a once-daunting condition into one that can be successfully controlled, allowing individuals to lead full and active lives. Though SLE affects multiple organs and systems, early recognition and proactive management can help prevent long-term complications, offering hope and improved outcomes for those living with the condition.
What is Systemic Lupus Erythematosus?
SLE is an autoimmune condition where the body’s immune system mistakenly attacks healthy tissue. In healthy individuals, the immune system defends the body against invaders like bacteria and viruses. However, in people with SLE, this defence mechanism goes awry, leading to inflammation and damage to the body’s own tissues and organs.
A key characteristic of SLE is the overactivity of B-cells, a type of white blood cell that produces antibodies. In SLE patients, these B-cells produce abnormal antibodies that target the body’s own cells, leading to the formation of immune complexes. These immune complexes can accumulate in tissues and organs, causing chronic inflammation and tissue damage.
Who is Affected by SLE?
SLE is more common in women than men, with a female-to-male ratio of about 9:1. The condition often begins between the ages of 15 and 45. Though rare, SLE can be more severe in certain demographics. Indigenous Australians and individuals of Southeast Asian ancestry, for example, are at higher risk of developing more severe forms of the disease. Socioeconomic factors, such as access to healthcare, also play a role in disease outcomes, with poorer outcomes reported in lower socioeconomic groups.
What Causes SLE?
The exact cause of SLE remains unclear, but both genetic and environmental factors contribute to its development. There is no single gene responsible for SLE; instead, multiple genetic predispositions may increase the risk of developing the disease. Environmental triggers such as sunlight, infections, stress, and certain medications are thought to play a role in initiating or worsening the disease.
Symptoms of SLE
SLE is notorious for its diverse range of symptoms, making diagnosis a complex task. Common symptoms include:
- Fatigue: This is one of the most common complaints, affecting 53-80% of patients. Fatigue can be caused by disease activity, medications, or psychological factors.
- Joint Pain: Up to 95% of people with SLE experience arthritis or joint pain, typically affecting the hands, knees, and other joints. Unlike other forms of arthritis, SLE does not usually cause permanent joint damage.
- Skin Issues: Many patients experience skin rashes, the most recognizable being the butterfly-shaped malar rash, which spreads across the cheeks and nose. SLE can also cause other skin conditions like discoid lupus, where scaly patches appear on the scalp and face, often leaving scars.
- Kidney Problems: Lupus nephritis, an inflammation of the kidneys, occurs in approximately 50% of patients and can lead to severe complications if untreated.
- Neuropsychiatric Symptoms: SLE can affect the brain and nervous system, leading to symptoms such as headaches, confusion, memory problems, and in severe cases, seizures or psychosis.
- Heart and Lung Involvement: Patients with SLE may experience inflammation of the heart (pericarditis) or lungs (pleuritis), which can cause chest pain and shortness of breath.
Because of the variety of symptoms, SLE can mimic other conditions, making early diagnosis difficult. It is essential for patients experiencing unexplained symptoms affecting multiple organ systems to seek medical evaluation for a potential autoimmune disorder like SLE.
How is SLE Diagnosed?
Diagnosing SLE is complex and relies on both clinical evaluation and laboratory tests. There is no single definitive test for SLE, but doctors typically begin with an antinuclear antibody (ANA) test. ANAs are found in 98% of people with SLE, but they can also be present in healthy individuals, so further testing is needed to confirm the diagnosis.
Additional blood tests can measure the presence of other antibodies specific to SLE, such as anti-double-stranded DNA and anti-Smith antibodies, which are highly indicative of the disease. Tests may also measure levels of complement proteins (C3 and C4), which tend to be low during periods of active disease.
Treatment Options for SLE
The goal of treatment for SLE is to control the disease, reduce flares, and prevent organ damage. SLE treatment is highly individualized, depending on the severity of the disease and the organs affected. Common treatments include:
- Hydroxychloroquine: This is a cornerstone of SLE treatment and is recommended for all patients unless contraindicated. Hydroxychloroquine has disease-modifying properties and can help reduce inflammation, prevent flares, and improve long-term outcomes.
- Glucocorticoids: These powerful anti-inflammatory drugs are used to control severe flares, but long-term use is associated with side effects like weight gain, high blood pressure, and osteoporosis. Physicians aim to taper glucocorticoid doses as quickly as possible.
- Immunosuppressants: Medications such as methotrexate and mycophenolate help reduce immune system activity and are often used in patients with more severe disease or when organs like the kidneys are affected.
- Biologic Therapies: Newer treatments such as belimumab and anifrolumab target specific pathways in the immune system and are used in patients who do not respond to standard treatments.
Living with SLE
SLE is a lifelong condition that requires careful management to prevent complications. Regular monitoring by healthcare providers is essential to assess disease activity and adjust treatment plans. Patients are also encouraged to take steps to reduce their risk of flares, such as avoiding sun exposure, quitting smoking, and engaging in regular exercise. Since cardiovascular disease is a major cause of morbidity and mortality in SLE patients, managing blood pressure and cholesterol levels is crucial.
Patients with SLE can live full, active lives with proper management and care. Early diagnosis and treatment significantly improve outcomes, helping to prevent irreversible organ damage and improve quality of life.
Conclusion
Systemic Lupus Erythematosus is a complex and sometimes unpredictable disease that affects many aspects of a person’s health. Although challenging to diagnose and manage, advancements in research and treatment have dramatically improved the prognosis for people living with SLE. With ongoing medical care and lifestyle adjustments, many patients can successfully manage their symptoms and lead fulfilling lives.
About the Author:
A/Prof Alberta Hoi is the Head of the Lupus+ Clinical Research Hub at the School of Clinical Sciences Monash University. She is also an internationally renowned clinician, and serves as the Head of Monash Lupus Clinic, while also holding senior rheumatology positions at Monash Health and Austin Health. With over 25 years of clinical experience, A/Prof Alberta Hoi specialises in managing complex cases of systemic lupus erythematosus (SLE), Sjogren and Dermatomyositis.
