Systemic Lupus Erythematosus Symptoms and Immune Disorders

Identifying butterfly rashes, joint pain, and fatigue.

Clinical Immunology focuses on the immune system’s health. Learn about the diagnosis and treatment of allergies, autoimmune diseases, and immunodeficiencies.

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Systemic Lupus Erythematosus: Symptoms and Risk Factors

Systemic Lupus Erythematosus can look very different from one person to another. No two patients have exactly the same symptoms. The disease may affect just one organ or many at the same time. Symptoms can appear suddenly or develop slowly over time. Knowing the many possible symptoms and risk factors is important for early diagnosis and treatment.

Mucocutaneous Manifestations

The Malar Rash

The most iconic symbol of lupus is the malar rash, also known as the butterfly rash. This is a red or purplish rash that extends across the cheeks and the bridge of the nose, sparing the nasolabial folds. It is often photosensitive, meaning it appears or worsens after exposure to sunlight.

Photosensitivity and Discoid Lesions

Many lupus patients experience a pronounced reaction to ultraviolet light. Exposure to the sun can trigger not only skin rashes but also systemic flares, including fatigue and joint pain. Discoid lesions are distinct from the malar rash. They are thick, scaly patches that can cause scarring and permanent hair loss if they occur on the scalp. Patients may also develop ulcers in the mouth or nose, which are usually painless but recurrent.

The Three Pillars of Clinical Immunology

Inflammatory Arthritis

Joint pain and stiffness are among the most common early symptoms of lupus. The arthritis is typically polyarticular, affecting multiple joints such as the wrists, small joints of the hands, and knees. Unlike rheumatoid arthritis, lupus arthritis is generally non-erosive, meaning it does not destroy the bone. However, it can cause tendon laxity and deformities over time, a condition known as Jaccoud’s arthropathy.

Myalgia and Muscle Weakness

Muscle pain or myalgia is a frequent complaint during flares. In some cases, true myositis or inflammation of the muscle tissue occurs, leading to weakness and elevated muscle enzymes. This can contribute significantly to the profound physical fatigue experienced by patients.

Renal Involvement: Lupus Nephritis

The Silent Threat

Kidney involvement is one of the most serious manifestations of SLE. It occurs when immune complexes deposit in the filtering units of the kidneys, called glomeruli. Lupus nephritis may be asymptomatic in its early stages, detectable only through urine tests showing protein or blood.

Proteinuria: The leakage of protein into the urine, which can cause foamy urine and swelling in the legs and eyelids.
Hematuria: The presence of blood in the urine, which may be microscopic.

Hypertension: High blood pressure often develops as kidney function declines.
If left untreated, lupus nephritis can progress to end-stage renal disease requiring dialysis or transplantation

Neuropsychiatric Lupus

Central Nervous System

Lupus can affect the brain and spinal cord, leading to a wide range of neurological symptoms.

Headaches: Severe and intractable migraines are common.
Cognitive Dysfunction: Often described as lupus fog, this involves difficulties with memory, concentration, and information processing.
Seizures and Psychosis: In severe cases, inflammation can cause seizures or acute psychosis, requiring aggressive immunosuppression.

Peripheral Nervous System

Peripheral neuropathy can cause numbness, tingling, or burning sensations in the hands and feet. Mononeuritis multiplex, a condition affecting specific individual nerves, is a less common but serious presentation.

Hematological Abnormalities

Cytopenias

The blood counts are frequently affected in lupus due to autoantibodies attacking blood cells.

Leukopenia: Low white blood cell count, increasing susceptibility to infection.
Anemia: Hemolytic anemia can occur when antibodies destroy red blood cells. Anemia of chronic disease is also common.
Thrombocytopenia: Low platelet count, which can lead to easy bruising and bleeding.

Antiphospholipid Syndrome

Many lupus patients develop antiphospholipid antibodies. These increase the risk of blood clots in arteries and veins, which can lead to deep vein thrombosis, pulmonary embolism, or stroke. In pregnant women, these antibodies are associated with recurrent miscarriages.

Cardiovascular and Pulmonary Symptoms

Serositis

Inflammation of the lining tissues is a hallmark of lupus.

Pleurisy: Inflammation of the lining of the lungs, causing sharp chest pain when breathing deeply.
Pericarditis: Inflammation of the sac surrounding the heart, causing chest pain that improves when leaning forward.

Atherosclerosis risk

Chronic inflammation accelerates the hardening of the arteries. Lupus patients are at a significantly higher risk of heart attacks at a younger age compared to the general population.

Risk Factors

Genetic Susceptibility

Genetics plays a major role, but it is not the sole determinant.

Gene Loci: Variants in genes involved in the immune system, such as HLA DR2 and HLA DR3, are associated with lupus.
Family History: Having a sibling or parent with lupus or another autoimmune disease increases risk.

Hormonal Factors

The prevalence of lupus in women during their reproductive years points to estrogen as a driving factor.

Puberty and Pregnancy: Disease onset or flares often occur during periods of high hormonal activity.
Estrogen Therapy: Use of birth control pills containing high doses of estrogen or hormone replacement therapy may increase the risk of flares in susceptible individuals.

Environmental Triggers

Ultraviolet Light: UV radiation causes cell death in the skin, releasing nuclear antigens that trigger the immune system.
Infections: Viruses like the Epstein-Barr virus have been linked to the onset of lupus.
Silica Dust: Occupational exposure to silica is a known risk factor.
Smoking: Cigarette smoking is strongly associated with the development of lupus and can reduce the effectiveness of certain treatments like hydroxychloroquine.

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FREQUENTLY ASKED QUESTIONS

What is the butterfly rash?

It is a classic sign of lupus, presenting as a red rash covering the cheeks and bridge of the nose, resembling the shape of a butterfly.

Does lupus affect the kidneys?

Yes. Lupus nephritis is a common and serious complication where the immune system attacks the kidney filters, potentially leading to kidney failure if untreated.

Why does the sun make lupus worse?

UV light damages skin cells, causing them to die and release internal cell materials that the overactive immune system mistakes for foreign invaders, triggering a flare.

What is lupus fog?

It refers to cognitive difficulties experienced by patients, including confusion, memory loss, and difficulty concentrating.

Can stress trigger lupus?

Yes. Physical and emotional stress are known triggers that can precipitate a lupus flare or worsen existing symptoms.