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Lupus is an autoimmune disease, that is, it causes the body’s immune cells to attack its own tissues and organs, causing an inflammation. Also known as systemic lupus erythematosus, this inflammatory disease can affect many different parts of the body, such as joints, kidneys, liver, leading to severe pain. As the signs and symptoms of lupus are the same as those observed in various other ailments, it is difficult to diagnose the condition. However, one of the most distinguishable features of lupus is a facial rash that appears on both cheeks and looks like the unfolding of the wings of a butterfly.

This disease is hereditary, meaning that some people have the disease passed on to them by their parents before their birth. They may suffer from the disease only if it is triggered by external factors such as exposure to sunlight, certain drugs or an infection. Although there is no cure for the disease, with the help of early diagnosis and effective treatment options that are available, patients can control its symptoms and lead healthy lives.

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Types

There are four main types of lupus:

 

Systemic Lupus Erythematosus (SLE)

It is the most common type of lupus and affects many organs such as the skin, kidneys, joints and heart.

Lupus of the Skin

This form of lupus is limited to the skin. It is further divided into three types:


  • Acute Cutaneous Lupus: This generally affects women in their 30s. It can present locally as a ‘butterfly rash’ on the face. The generalised form of this lupus occurs above and below the neck.

  • Sub-acute Cutaneous Lupus Erythematous: This affects young to middle-aged women. People with this type of lupus are highly photosensitive.

  • Chronic Cutaneous Lupus Erythematous: Rashes that leave major scars are typical of this type of lupus. The rashes usually occur on the scalp, face and neck. It can also be present on the ears and upper torso. The prevalence of chronic cutaneous lupus is higher among smokers.


Drug-Induced Lupus Erythematosus

Certain prescription medications can also lead to the development of lupus. The clinical presentation of drug-induced lupus is similar to SLE. The medications commonly associated with the development of lupus are minocycline, isoniazid, hydralazine, procainamide and anti-TNF.

Neonatal Lupus Erythematosus

Women who have lupus may give birth to babies with a condition known as neonatal lupus erythematosus. However, this is a rare occurrence.

Symptoms

Characteristic symptoms of lupus are the development of a 'butterfly rash’ on the cheeks and sensitivity to sunlight. However, patients with lupus normally complain of:


  • Fatigue

  • Loss of appetite

  • Arthritis

  • Ulcers

  • Muscle pain

  • Hair fall

  • Mouth sores

  • Fever


 

Symptoms of lupus also depend on the affected organ system:

  • Heart and lungs: Inflammation of the heart and lung lining is expected when these organs get affected. Symptoms involve chest pain, irregular heartbeat, coughing up blood and fluid build-up in the lungs or around heart.

  • Brain: This organ is rarely affected by lupus. Seizures, headache, vision problems and depression are some of the symptoms that arise when the brain is affected.

  • Kidneys: Swelling in the legs is suggestive of kidney involvement. However, kidneys can be affected even without showing any symptoms. This requires regular check-ups, and timely treatment if lupus nephritis occurs.

  • Blood: Low levels of white blood cells, red blood cells, and platelets indicate blood involvement. Blood clots can develop in the legs, lungs, and brain.

Causes And Risk Factors

Causes

Lupus is an autoimmune disease that occurs when the immune system of your body starts attacking itself or becomes allergic to itself. It reacts to a foreign stimulus and generates a large number of antibodies which attack different tissues in the body. Sometimes, the autoimmune reaction is limited to the skin tissue, such as in discoid lupus erythematosus (DLE); in cases of SLE, the reaction is seen throughout the body. The actual cause is not known because there is no defined trigger that can been identified as the cause of lupus. There are many genes in our body which can increase the risk of developing lupus. Sometimes people whose family members have other autoimmune disease may develop lupus.

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Risk Factors

It mainly affects people in their 20s and 30s. Anybody can suffer from lupus but it more common in women than in men. Women of child-bearing age are mainly affected by lupus, though it may be seen in children and the elderly.

The following are some of the other risk factors for lupus:


  • Family history: People with a family history of lupus are at a higher risk of developing this autoimmune disorder.

  • Race: Women of African-American, Hispanic, Asian and Native American descent are more prone to develop lupus than women of other races.

Prevention

As it is an autoimmune disorder, there are no preventive measures for this disease. However, early diagnosis and identification of factors that trigger this disorder can help in the management of lupus.

Diagnosis

Lupus diagnosis is not difficult. But as the symptoms of lupus vary from patient to patient, often, it becomes too late before a patient reaches the right doctor. A rheumatologist looks after such patients. There are intermittent flare-ups which imitate a lot of other diseases.

 

Blood tests and ANA (antinuclear antibody) testing is done to confirm the disease.

The doctors use a combination of clinical symptoms, physical examinations, x-rays and lab tests for the diagnosis of lupus. Some of the laboratory investigations which aid the diagnosis of lupus are a blood test, urinalysis, serum creatinine test and chest x-ray. Additional tests which give an idea regarding the extent of different organ involvement include:


  • Rheumatoid factor

  • Kidney biopsy

  • Liver function test

  • Kidney function test

  • ESR and CRP

  • Cryoglobulins

  • Coomb’s test

  • Radiological imaging of the brain, heart, lungs, muscles or joints

Treatment

There is no cure for the disease as of now. But there are a lot of treatment options that can control the symptoms of lupus. For joint and muscle pain, pain killers like ibuprofen are given. Hydroxychloroquine in combination with quinacrine is especially effective in patients who experience fatigue, skin rashes and joint diseases. Immunosuppressants like methotrexate and cyclosporine are also prescribed to control the hyperactive immune system.

Nothing can be done to prevent lupus, but proper care and early diagnosis can prevent life threatening complications. There are several advancements in the treatment of lupus and many more research advances that can help patients in the near future.

Lupus affects the skin and patients suffering from lupus are more prone to suffer from allergies. There are many foods that tend to increase flare-ups in lupus. Patients should discuss dietary changes with their doctor to minimise allergic reactions.

Lifestyle Management

Patients can make lifestyle changes that can help in the management of lupus:


  • Avoid excessive exposure to sunlight

  • Always applying a sunscreen with at least 30 SPF before going out

  • Wear a hat and clothing that cover most of the body

  • Quit smoking as it can worsen the disease

  • As this disease weakens the immune system, stay protected from infections. This involves washing hands frequently, getting all the required vaccinations, and not being around sick people

  • Exercise regularly as this can help to lower the impact of SLE

  • Avoid stress as this can further weaken the immune system. Meditation or relaxation techniques can help to reduce stress

  • Have a healthy diet and include fruits, vegetables, and whole grains in all meals

Prognosis And Complications

Prognosis

Severe cases of lupus are difficult to treat and can be life-threatening. Patients who have lupus with no life-threatening organ involvement can expect to have a normal life provided they maintain a healthy lifestyle and follow their doctor’s instructions.

Complications

The following are some of the complications of lupus:


  • Lupus Nephritis: Lupus can cause damage to the kidney cells in some patients. This eventually leads to kidney failure. Patients with lupus nephritis will require dialysis or a kidney transplant.

  • Thyroid Disorders: Lupus can lead to the development of autoimmune thyroid disorders such as hypothyroidism and hyperthyroidism.

  • Sjögren’s Syndrome: Lupus can lead to Sjögren’s Syndrome in about 10% of the patients. It is characterised by dryness of the mouth and eyes.

  • Pregnancy Complications: Lupus can lead to miscarriages during pregnancy. Some of the medicines used for the treatment of lupus can also harm the baby.

References


  1. Types of lupus. Johns Hopkins Medicine. Available at: https://www.hopkinsmedicine.org/health/conditions-and-diseases/lupus/types-of-lupus. Accessed on: March 25, 2021.

  2.  Okon LG, et al. Best Practice & Research: Clinical Rheumatology. 2013 Jun;27(3):391-404

  3. Systemic lupus erythematosus [Updated on January 21, 2020]. Medline Plus. Available at: https://medlineplus.gov/ency/article/000435.htm. Accessed on: March 25, 2021.

  4. Lupus [Updated on October 11, 2014]. Cleveland Clinic. Available at: https://my.clevelandclinic.org/health/diseases/4875-lupus. Accessed on: March 25, 2021.

  5. Lupus risk factors. Johns Hopkins Medicine. Available at: https://www.hopkinsmedicine.org/health/conditions-and-diseases/lupus/lupus-risk-factors. Accessed on: March 25, 2021.

  6.  What causes lupus? [Updated on November 28, 2016]. Lupus Foundation of America.  Available at: https://www.lupus.org/resources/what-causes-lupus. Accessed on March 25, 2021.

  7. Lifestyle changes to manage systemic lupus erythematosus (SLE). Winchester Hospital. Available at: https://www.winchesterhospital.org/health-library/article?id=19779. Accessed on: March 25, 2021.

  8. Prognosis and life expectancy [Updated on July 18, 2013]. Lupus Foundation of America. Available at: https://www.lupus.org/resources/prognosis-and-life-expectancy#:~:text=The%20prognosis%20of%20lupus%20is,do%20die%20from%20the%20disease. Accessed on: March 25, 2021.

  9. Lupus complications and prognosis. Johns Hopkins Medicine. Available at: https://www.hopkinsmedicine.org/health/conditions-and-diseases/lupus/lupus-complications-and-prognosis. Accessed on: March 25, 2021.

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