Lupus: Systemic Lupus Erythematosus

Lupus is a complex disease that affects at least 5 million people worldwide. It is an autoimmune condition; the body’s immune system, which usually helps fight infections, attacks healthy tissues and organs instead, causing inflammation and pain. Lupus mostly affects women, with the disease commonly appearing during the childbearing ages of 15 to 44. 

Types of lupus

There are many 4 different types of lupus: 

• Systemic lupus erythematosus (SLE): the most common form which affects various body systems
• Cutaneous lupus: a form that is limited to dermatological manifestations e.g. photosensitivity, hair loss, rashes
• Drug-induced lupus: caused by certain prescription drugs, present with similar symptoms as SLE, but usually temporary
• Neonatal lupus: a rare condition that affects infants of women who have lupus, though not all babies born to lupus sufferers will have the disease.

When people talk about lupus, they are usually referring to Systemic Lupus Erythematosus (SLE).  It is not a contagious disease and cannot be transmitted through sexual contact. 

Causes of SLE

There is no clear known cause. It may be caused by a combination of many underlying risk factors: 

Risk Factors 

• Hormones: Research has shown that there could be a possible link between oestrogen (female reproductive hormone) and lupus, which could explain why most sufferers are women as women produce more of it. 
• Race: Lupus is more common in Native Americans, African Americans, Hispanics, and Asians than Caucasians
• Genetics: Lupus may occur with family history of lupus or other autoimmune diseases such as haemolytic anaemia
• Environmental exposure
  • Smoking, infections, stress
  • Drugs e.g. long term use of hydralazine, tetracycline, TNF (tumour necrosis factor) blockers for conditions like rheumatoid arthritis

Symptoms of SLE

There is great dissimilarity in symptoms from patient to patient, and patients usually experience bouts of flares and periods of remission. Additionally, not all symptoms are specific to lupus e.g. some symptoms are similar to diabetes or arthritis.

Patients usually present with symptoms involving joint and mucosal inflammation. The most common symptoms include:

• Extreme fatigue 
• Pain or swelling in the joints
• Headaches
• Low-grade fevers
• Photosensitivity – exposure to sunlight may trigger rashes, fever, fatigue, joint pain
• Skin lesions or rashes, typically a butterfly-shaped rash on the cheeks and nose
• Hair loss; bald spots may be common.
• Mouth ulcers

More serious complications and manifestations may include:

• Chest pain when breathing deeply, which could be due to inflammation of the lining of the lungs (pleuritis) or heart 
• Weight gain, swollen ankles, high blood pressure, and decreased kidney function due to inflammation of the kidneys (lupus nephritis)
• Stroke, heart attack, repeated miscarriages, deep vein thrombosis (blood clot in legs) or pulmonary embolism (blood clot in lungs) due to higher risk of blood clotting

Diagnosis of SLE 

There is no one test that can diagnose SLE. Diagnosis is attained with a combination of clinical signs and symptoms, review of medical and family history, as well as a series of laboratory investigations. 

Blood tests

• Antinuclear antibodies (ANA) test
  • Antinuclear antibodies attack healthy cells (nuclei) and 97% of people with lupus have them in their blood. 
  • Positive results are often also seen in people without lupus but with other autoimmune disorders. Positive ANA by itself is not enough for a lupus diagnosis. 

• Antiphospholipid Antibodies (APLs) test
  • APLs bind to phospholipids which are structural components of cellular membranes as well as critical to platelet function; they increase the risk of blood clotting
  • APLs are present in up to 60% of people with lupus. A positive test can help confirm a diagnosis of lupus and also identify women who require preventive treatment and monitoring due to certain risks e.g. blood clots, miscarriage, or preterm eclampsia, premature labour.

• Anti-double stranded DNA (anti-dsDNA) test 
  • Anti-dsDNA is an antibody directed against double-stranded DNA. 
  • Presence of anti-dsDNA is primarily associated with lupus i.e. more specific test
  • A high titre (level) of anti-dsDNA is an indication of ongoing inflammation and damage to the kidneys (lupus nephritis)

• Complete blood count (CBC)
  • Measures levels of different blood cells
  • Abnormalities in blood cell counts may be related to lupus, lupus treatments, or infection. For example, leukopenia, a decrease in the number of white blood cells, is found in about 50% of people with lupus. Thrombocytopenia, or a low platelet count, occurs in about 50% of people with lupus.
  • Non-specific for lupus

• Chemistry Panel
  • Assessment of kidney and liver function as lupus can affect these organs

• C-Reactive Protein, Erythrocyte Sedimentation Rate (ESR)
  • Markers of inflammation
  • Not specific to lupus, many other causes for elevated result e.g. infection 
  • Useful to monitor changes in disease activity or response to treatment. 

Urine Tests

These can aid in diagnosis of lupus and monitor the effects of lupus on the kidneys:

• Urinalysis: The presence of protein, red blood cells, white blood cells, and cellular casts may all indicate kidney disease.

• Urine Protein/Microalbuminuria: Measures the amount of protein (or albumin) in urine. Even a small amount may indicate a risk for kidney disease.

• Creatinine Clearance: Measures how effective the kidneys are at filtering the blood to eliminate waste products. It is conducted on urine collected over a 24-hour period.

Skin or kidney biopsy

A biopsy is a minor surgery to remove a sample of tissue for viewing under a microscope. Biopsies of skin and kidney tissue can show signs of an autoimmune disease.

Imaging tests

• Chest X-ray: An image of your chest may reveal abnormal shadows which suggest inflammation in your lungs e.g. pleuritis
• Echocardiogram (ECG): This test uses sound waves to produce real-time images of your beating heart. It can check how well it is working and detect problems with the valves, fluid accumulation or inflammation of the pericardium.

Diagnosis in SLE is complex and difficult as the same symptoms can present in other conditions. Your doctor may use any or all the above tests to make your diagnosis or rule out other diseases that can be confused with lupus.

Treatment of SLE

There is no cure for SLE. The main aim of treatment is to relieve symptoms, prevent flare-ups and end-organ damage. This can be achieved with medications and through lifestyle changes.

Medications commonly used to treat lupus include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • NSAIDs such as naproxen sodium (Aleve), ibuprofen (Nurofen), and etoricoxib (Arcoxia) may be used to treat pain, inflammation and fever associated with lupus.
  • Side effects may include stomach bleeding, kidney problems and an increased risk of heart problems.

• Antimalarial drugs
  • Medicines that prevent or treat malaria e.g. hydroxychloroquine (Plaquenil) and chloroquine phosphate (Aralen) also treat joint pain, skin rashes, fatigue, lung inflammation and reduce flares. 
  • Side effects can include stomach upset and, very rarely, damage to the retina of the eye. 

• Corticosteroids
  • May help reduce inflammation, tenderness, and pain e.g. oral prednisolone for joint/skin involvement, IV methylprednisolone for aggressive states of the disease or for neuropsychiatric manifestations
  • Side effects include weight gain, thinning bones, high blood pressure, diabetes and increased risk of infection. This usually occurs with higher doses and long term therapy.

• B-lymphocyte stimulator (BLyS) protein inhibitor
  • E.g. Intravenous belimumab blocks the action of a specific protein in the body that is important in immune response.
  • Side effects include nausea, diarrhoea and infections.

• Immunosuppressants
  • Drugs that suppress the immune system may be helpful in severe cases refractory to other forms of treatment. Examples include azathioprine (Imuran, Azasan), mycophenolate (Cellcept), methotrexate (Trexall, Xatmep), cyclosporine (Sandimmune, Neoral, Gengraf) and leflunomide (Arava). 
  • Potential side effects may include an increased risk of infection, liver damage, decreased fertility and an increased risk of cancer. 

Lifestyle measures to reduce lupus flares and complications

• Use adequate sun protection when going out.
  • Ultraviolet light can trigger flares
  • Apply sunscreen with a sun protection factor (SPF) ≥ 30 liberally and put on protective clothing such as a hat, long-sleeved shirt and long pants when heading out.

• Get regular exercise.
  • Exercise can help keep bones strong, reduce risk of heart attack and promote general well-being.

• Avoid smoking. 
  • Smoking increases risk of cardiovascular disease and can worsen the effects of lupus on the heart and blood vessels.

• Learn relaxation techniques to manage stress
  • Stress can exacerbate symptoms

• Eat a healthy diet. 
  • Eat a balanced diet, with more fruits, vegetables and whole grains. 
  • Adhere to dietary restrictions e.g. low sodium, if you have kidney damage or high blood pressure.

• Ask your doctor if you need supplements. 
  • E.g. Calcium and Vitamin D supplement for healthy bones; some studies have suggested Fish oil to be beneficial in controlling symptoms of lupus as well as improving cardiovascular health

Conclusion

Although there is no cure for lupus, proper treatment and a good management plan can help alleviate symptoms and prevent complications in patients.