Systemic Lupus Erythematosus

Systemic lupus erythematosus or systemic lupus erythematodes (SLE) is an autoimmune disease associated with damage of many organs and tissues. Some of its forms have very severe symptoms and poor prognosis. Like the majority of other autoimmune disorders, SLE is more common in women.

Causes

The causes are unknown. Like other autoimmune diseases, systemic lupus erythematosus develops due to combination of a genetic predisposition and some outer factor. It is possible that the disease is triggered by a banal viral infection. The immune system easily overcomes the infection but it gets somehow “reprogrammed” and starts to attack our own tissues. There occur special autoantibodies attacking and damaging the tissues (such as ANA autoantibodies and anti-dsDNA autoantibodies).

Symptoms

The symptoms are extremely variable. Some cases may be almost asymptomatic, other may be fatal. The current symptoms depend on affection of organ systems.

Skin

Skin affection is characteristic but not always present. There occurs the classic butterfly erythema, a reddening of both cheeks that look like butterfly wings. Other areas of skin may be affected by flushed annular lesions known as erythema nodosum and skin of people with SLE is usually more sensitive to sunlight. The patients may also suffer from excessive hair loss.

Butterfly rash

 

Schematic drawing - Butterfly rash

Lungs

They can be affected by gradual deposition of fibrotic tissue (lung fibrosis) resulting in shortness of breath during physical exercise. The lining covering the lung, so-called pleura, may be affected as well causing pleurisy with chest pain during inspiration.

Brain

Brain affection may be very inconspicuous. The symptoms can manifest as a change of behavior, dementia, memory loss and movement disorders.

Joints

Systemic lupus may cause inflammation of many joints with their pain, swelling and deformations. This is a very common form of SLE.

Kidneys

Kidneys are not affected as frequent as skin or joints but their disorders are especially dangerous. The disease usually has a form of glomerulonephritis with blood and protein in urine. The condition may progress into chronic renal failure with the need of lifelong dialysis.

Heart

All layers of the heart may be damaged by lupus inflammation, for example the muscle layer (myocarditis) and the pericardium (pericarditis). The most common symptoms are arrhythmias, chest pain and signs of heart failure.

 

Note: There is a special risk in pregnant women with SLE. The autoantibodies of a sick mother can cross the placenta and harm the fetus. The newborn suffers from symptoms of so-called “lupus neonatorum” (newborn lupus). Skin and heart are the most typically affected systems.

Diagnosis

In addition to clinical symptoms, it is important to confirm any of the autoantibodies from a blood sample. However, it may be very challenging to state the right diagnosis of SLE.

Prevention

Unfortunately, there is no effective way how to prevent SLE.

Treatment

The treatment is difficult. We use a wide range of medications that suppress the aggressive immune system. However, these drugs usually have some side effects. Our target is to suppress the immunity as selectively as possible without causing serious immunodeficiency.

 

Corticosteroids are probably the most frequently used medications. The strongly suppress the inflammation and often bring a quick relief. However, they also have strong side-effects when used in chronic medication - obesity, stretch marks, hypertension, behavioral changes, osteoporosis, frequent infections and peptic ulcers (when combined with other anti-inflammatory drugs). Unfortunately, lupus may be such a serious disease that it is often necessary to deploy corticosteroids regardless their side-effects.

 

In addition, we use special immunosuppressive chemotherapeutic agents such as cyclophosphamide, cyclosporine and azathioprine that also have a wide range of adverse effects.