Overview

Systemic lupus erythematosus (SLE), commonly referred to as lupus, is a chronic autoimmune disease that can affect multiple organ systems in the body. In SLE, the immune system mistakenly attacks healthy tissues, leading to widespread inflammation and tissue damage. The disease can impact the skin, joints, kidneys, heart, lungs, blood cells, and nervous system. SLE is known for its unpredictable course, with periods of remission and flare-ups. It primarily affects women of childbearing age, though it can occur in men and children as well. The condition varies greatly in severity, ranging from mild cases affecting the skin and joints to severe cases involving vital organs.

Causes

The exact cause of systemic lupus erythematosus remains unknown, but it is believed to result from a combination of genetic, environmental, hormonal, and immune system factors. Genetic predisposition plays a significant role, as certain genes involved in immune regulation increase the risk of developing lupus. Environmental triggers such as ultraviolet (UV) light exposure, infections, certain medications, and stress can initiate or exacerbate the disease in susceptible individuals. Hormonal factors, particularly estrogen, may also contribute to the higher prevalence of SLE in women. The autoimmune response in SLE leads to the production of autoantibodies that attack the body’s own tissues, causing inflammation and damage.

Symptoms

SLE is known for its diverse range of symptoms, which can vary from person to person and fluctuate over time. Common symptoms include:

• Fatigue: Persistent tiredness is one of the most common and disabling symptoms.
• Joint pain and swelling: Non-erosive arthritis typically affects the small joints of the hands, wrists, and knees.
• Skin rashes: The characteristic butterfly-shaped rash (malar rash) appears over the cheeks and nose in many cases.
• Photosensitivity: Sensitivity to sunlight leading to skin eruptions or worsening of symptoms.
• Fever: Low-grade fevers are common during disease flares.
• Hair loss: Thinning or patchy hair loss can occur.
• Oral ulcers: Painless mouth or nasal sores.
• Raynaud’s phenomenon: Fingers and toes turning white or blue in response to cold or stress.
• Kidney involvement: Lupus nephritis, causing proteinuria, hematuria, and potential kidney failure in severe cases.
• Cardiopulmonary involvement: Inflammation of the heart (pericarditis), lungs (pleuritis), or blood vessels (vasculitis).
• Neuropsychiatric symptoms: Seizures, headaches, mood disturbances, and cognitive dysfunction may be present.

Diagnosis

Diagnosing systemic lupus erythematosus is complex due to its varied presentation and overlapping symptoms with other conditions. There is no single definitive test for SLE, so diagnosis is based on clinical criteria and laboratory findings. The process includes:

• Medical history and physical examination: Assessment of characteristic symptoms, such as joint pain, skin rashes, and systemic involvement.
• Antinuclear antibody (ANA) test: A positive ANA is present in nearly all individuals with lupus but can also be found in other autoimmune diseases.
• Specific autoantibodies: Testing for anti-double-stranded DNA (anti-dsDNA), anti-Smith (anti-Sm), antiphospholipid antibodies, and others help confirm SLE diagnosis.
• Blood tests: Complete blood count (CBC) may show anemia, leukopenia, or thrombocytopenia. Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) indicate inflammation.
• Urinalysis: Detects protein or blood in urine, indicating kidney involvement.
• Imaging studies: X-rays, echocardiograms, or other imaging may be used to assess organ damage.
• Classification criteria: The American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria assist in diagnosing SLE based on a combination of clinical and laboratory features.

Treatment

Treatment for systemic lupus erythematosus is tailored to the severity and type of organ involvement, aiming to reduce symptoms, control inflammation, prevent flare-ups, and minimize long-term complications. Common treatment approaches include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs): Used to manage mild joint pain and inflammation.
• Antimalarial drugs: Hydroxychloroquine is widely used to treat skin and joint symptoms and reduce disease flares.
• Corticosteroids: Low to high doses are used depending on disease severity, especially during flare-ups, but long-term use is minimized due to side effects.
• Immunosuppressive agents: Drugs like azathioprine, methotrexate, or mycophenolate mofetil are used to control moderate to severe disease and reduce reliance on steroids.
• Biologic therapies: Targeted treatments like belimumab (a monoclonal antibody) are used in certain cases to control immune activity.
• Lifestyle modifications: Patients are advised to avoid excessive sun exposure, maintain a balanced diet, engage in regular physical activity, and quit smoking.
• Regular monitoring: Ongoing assessment by a rheumatologist is crucial to adjust treatment plans based on disease activity and side effects.

Prognosis

The prognosis for systemic lupus erythematosus has improved significantly over the past few decades, thanks to early diagnosis, better treatment options, and comprehensive disease management. Many individuals with SLE lead productive and fulfilling lives, especially with appropriate medical care. However, lupus remains a chronic condition characterized by unpredictable flares and remissions. The long-term outlook depends on the severity of organ involvement, particularly in the kidneys and cardiovascular system. Early and aggressive treatment of active disease, regular follow-ups, and lifestyle adjustments can help reduce the risk of complications, improve quality of life, and extend life expectancy for patients with lupus.