Systemic Lupus Erythematosus

​Lupus (Systemic Lupus Erythematosus) is a chronic autoimmune disorder that occurs when the body’s immune system attacks its own tissues and organs. Inflammation caused by lupus can affect many different body systems, including the joints, skin, kidneys, blood cells, brain, heart, lungs, intestines and blood vessels.

Under normal circumstances, the body’s immune system protects the body against “foreign” invaders like viruses, bacteria and parasites. In lupus, the immune system malfunctions and produces antibodies and cells which attack its own organs – hence lupus is known as an “autoimmune” disease. The reason why this happens is unknown, but are believed to be linked to environment (exposure to sunlight, stress or infection), genetic and hormonal factors.

Lupus occurs more frequently in women than in men. Asians and Afro-Americans are more prone to develop lupus than Caucasians and the disorder is more severe in these ethnic groups.

Four types of Lupus exist:

• Systemic Lupus Erythematosus (SLE) – the generalised and most common form
• Discoid Lupus Erythematosus – only affecting the skin
• Drug-induced Lupus – Lupus caused by drugs
• Neonatal Lupus – Lupus in babies born to mothers with SLE

The outlook for people with lupus was once grim, but diagnosis and treatment of the disorder has improved tremendously in recent years. With early diagnosis and treatment, most people with lupus can lead normal, active lives.

​No two cases of lupus are exactly alike. Signs and symptoms may come on suddenly or develop slowly, may be mild or severe and may be temporary or last for a long period of time.

Most people with lupus experience episodes called flares, or worsening signs and symptoms that eventually improve or even disappear completely for a period of time with treatment. The course of the disorder is unpredictable, hence long-term treatment and follow-up is essential.

The signs and symptoms of lupus will depend on which body systems are affected by the disorder.

In general, signs and symptoms of lupus include the following:

• Fever, fatigue and weight loss.
• Joint pain, stiffness, swelling and diffuse muscle aches.
• Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose which worsens with sun-exposure.
• Hair loss, mouth ulcers and easy bruising.
• Chest pain and shortness of breath.
• Swelling around the eye-lids, swelling of the feet and legs and decrease in urine output.
• Psychosis, seizures (fits), memory or behavioural changes, drowsiness or stroke.

​Lifestyle changes are needed to reduce the incidence of relapses:

• Get adequate rest and sleep
• Be sun smart – avoid sun-bathing and stay out of the sun entirely when it is the strongest i.e. between 8am to 6pm
• Use sunblock and wear protective clothing
• Get regular exercise but do not overstrain your body
• Maintain a healthy diet
• Do not smoke and avoid excessive alcohol intake

​Under normal circumstances, the body’s immune system protects the body against “foreign” invaders like viruses, bacteria and parasites. In lupus, the immune system malfunctions and produces antibodies and cells which attack its own organs – hence lupus is known as an “autoimmune” disease. The reason why this happens is unknown, but are believed to be linked to environment (exposure to sunlight, stress or infection), genetic and hormonal factors.

​Diagnosing lupus is difficult because the disease varies considerably from person to person and the signs and symptoms come and go unpredictably and overlap with many other diseases. Therefore, doctors may not initially consider lupus until the signs and symptoms become more definite. Your doctor will take a detailed medical history and conduct a physical examination on you.

Even then, diagnosis can often be challenging and a number of laboratory tests are necessary to confirm the diagnosis. These tests include the following:

• Full blood count - This test measures the levels of haemoglobin, number of red blood cells, white blood cells and platelets. Results may indicate the presence of anaemia, or low white blood cell or platelet count. An extremely low platelet count can result in spontaneous bleeding in the skin (bruises), in the stomach or even in the brain.
  
• Erythrocyte Sedimentation Rate (ESR) – the ESR is raised in many disorders including lupus. It is sometimes a good measure of disease activity and as your condition improves your ESR may drop.
  
• Urine examination - An examination of your urine may show an increase in red blood cells or protein level. This can occur if lupus has affected your kidneys.
  
• Kidney function tests - Blood tests can assess how well your kidneys are functioning.
  
• Anti-nuclear antibody (ANA) - A positive test for these antibodies indicates a stimulated immune system which is common in lupus and other autoimmune diseases. However, a positive ANA test is not always indicative of lupus since certain infections or drugs can lead to a positive test. In fact, a small proportion of normal individuals can have a slightly raised ANA antibody test. Therefore, this test needs to be interpreted in conjunction with a proper history and physical examination.
  
• Anti-dsDNA antibody test - This test is often done together with the ANA test. Patients with lupus and kidney involvement often have a raised anti-dsDNA antibody level.
  
  

​Specialised clinics may result in better outcomes. Treatment of lupus depends on the signs and symptoms and which organs are involved.

Determining what medications to use requires a careful discussion of the benefits and risks with a rheumatologist. For adequate control of lupus, a rheumatologist may recommend more powerful drugs in higher doses initially but as the disease flare subsides, the dosage can usually be tapered off slowly and carefully. More aggressive lupus usually requires more powerful and long term drugs.

In general, when first diagnosed with lupus, your doctor may recommend the following medications:

• Non-steroidal anti-inflammatory drugs (NSAIDs)
  These include diclofenac acid (Voltaren) and the COX 2 inhibitors (Celebrex and Arcoxia). They are effective in controlling fever, muscle aches, joint pains and swelling.
  
• Antimalarial drugs
  Medications that are used to treat malarial can also be used to treat individuals with lupus. The two commonly known antimalarials are hydroxychloroquine (Plaquenil) and chloroquine. These medications have proved useful especially in lupus patients with joint and skin involvement. It has also proven to reduce lupus flares and help people with lupus to live longer.
  
• Corticosteroids
  These drugs counter the inflammation of lupus and are highly effective. They can have serious long term side-effects including weight gain, easy bruising, high blood pressure, diabetes, thinning of the bones (i.e. osteoporosis) and an increased risk of infection. Very often, doctors need to prescribe corticosteroids (e.g. prednisolone) in order to prevent permanent organ damage such as kidney failure or even death. To help reduce the side-effects, your doctor will try to find the lowest dose that controls the disease and prescribe the medicine for the shortest possible period of time.
  
• Immunosuppressive drugs
  These drugs suppress the immune system and may be useful in serious cases of lupus including patients with severe kidney or brain involvement. The most often used immunosuppressive drugs are cyclophosphamide, mycophenolate mofetil (cellcept) or mycophenolic acid and azathioprine. Cyclophosphamide is often given by injection into the veins. Potential side-effects include hair loss, feeling tired, nausea, change in menstrual cycle, increase risk of infection, low white cell count, liver injury, blood in urine and infertility.
  
• Mycophenolate mofetil or mycophenolic acid has proven to be effective in lupus affecting the kidneys and other major organs. It has fewer side-effects than corticosteroids and is proving to be an effective alternative to cyclophosphamide. These drugs should only be used under close supervision by a rheumatologist or a nephrologist (kidney specialist).
  
  

​Surgery may present some particular challenges in people who have lupus, so proper preparation is important. Although we may not be able to adequately prepare for emergency surgeries, there are many things that can be done for elective surgeries to increase the possibility of a safe outcome.

Keep all your doctors in the loop. It is very important that the surgeon and the rheumatologist communicate with each other prior to the surgery. If this does not happen, it is recommended that patients initiate this communication by asking the surgeon to contact the rheumatologist, and vice versa.

This is important as surgery may induce a lupus flare or may cause complications if surgery is performed while having a flare. Therefore, it is important to know the status of your lupus before having a procedure. In addition, some medications need to be stopped, increased or decreased prior to surgery. For instance, blood thinners will need to be stopped prior to surgery and restarted later on. Your rheumatologist and other doctors can provide specific recommendations.

​Your rheumatologist will review you post-surgery to make sure your wound has healed and recommends your treatment appropriately.