Systemic Lupus Erythematosus: The Great Imitator

butterfly or malar rash in SLE

Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can affect any part of the body, hence the term systemic. Most commonly affected organs are the joints, skin, heart, lungs, kidneys, liver, blood vessels, and nervous system. In 2008, around 250.000 Americans have SLE. Women aged 20-30 years old are nine time more likely to be affected than men. African American and Asians are more commonly affected and the disease tend to be more severe in these ethnic groups.

Diagnosis

As in any autoimmune disorder, the immune system lost the ability to differentiate self and non self, producing auto antibody that attacks normal cells. Why our immune system turned against us is still unknown, but viral infection, sunlight and allergies may trigger the changes.

SLE is dubbed the great imitator because the symptoms can mimic other diseases, making the diagnosis a challenging one to make. The course of disease is completely unpredictable with flare-ups alternating with remissions. Severity ranges from mild to life threatening. Initially, most patients unspecific symptoms like fatigue, rashes, painful joints, and low grade fever.

Based on The American College of Rheumatology's criteria, a person is said to have SLE if he or she has at least four of the symptoms listed below.

the location of rashes in SLE

• Butterfly shaped rashes over the cheeks (malar rash), oval shaped rashes with raised borders (discoid rash), or rash over sun exposed skin
• Arthitis in two or more joints lasting more than a few weeks
• Chest pain when taking deep breaths, a sign of inflammation of the outer linings of the lung (pleuritis) or the heart (pericarditis)
• Kidney problems ranging from mild protein or blood in the urine to renal failure
• Nervous system disturbances like seizures, stroke, or delirium
• Sores in the mouth
• Abnormal lab results: 
• low red blood cells, low white blood cells, or low platelets
• positive auto antibodies like anti nuclear antibody (ANA) or anti double stranded DNA (anti-dsDNA), anti Smith (referred to as anti-Sm) or antiphospholipid antibodies, or a false positive test for syphilis

Treatment

Sadly there is no cure for SLE and its management can be rather complicated. The good news is that immunosuppresive therapy can greatly improve survival. Maintaining a good quality of life remains the greater challenge, however.

The medications commonly used for SLE includes:

• Nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce joint pain and inflammation.
• Antimalarial drugs such as hydroxychloroquine help with fatigue, rashes, joint pain, and mouth sores.
• Immunosuppressants such as azathioprine, cyclophosphamide, cyclosporin are used in patients with more severe and wide-spread symptoms.
• Corticosteroids is also used in the more severe form of SLE.
• Biological agents such as rituximab and abatacept modulate the faulty immune system.