Systemic Lupus Erythematosus (SLE)- Rheumatology

An autoimmune condition, SLE, or systemic lupus erythematosus, causes the immune system to unintentionally attack healthy tissues in the body. As a result, the skin, kidneys, joints, brain, and several other organs may be affected.

Women are more likely to develop lupus, usually between the ages of 15 and 45. Because its symptoms and signs often resemble those of other diseases, it can be challenging to diagnose SLE. Lupus currently has no known cure and requires life-long treatment.

Understanding SLE

A chronic autoimmune condition, systemic lupus erythematosus (SLE) can cause inflammation and pain across your entire body. Normally, the immune system defends the body against harmful microorganisms and diseases. However, when the immune system attacks healthy cells, mistaking them as foreign, the condition is known as an autoimmune disease. SLE is just one of the numerous autoimmune illnesses.

SLE is the most prevalent type of lupus. Though the word has been used to refer to a range of other immunological illnesses with comparable clinical manifestations and laboratory characteristics, when people mention lupus, they frequently mean SLE.

This condition can vary between stages when symptoms get worse and better. For example, symptoms of lupus include internal organ problems (such as those with the brain, lungs, kidneys, and heart), rashes, skin sensitivity, and joint pain. Several of your symptoms may flare up and then disappear in waves.

At times symptoms of lupus may be mild or unnoticeable. This means the disease is in remission. Other times, the condition's severe symptoms could make your daily life quite difficult. However, with proper medical care, most people with SLE can lead normal lives.

Causes Of SLE

Women experience SLE ten times more frequently than men. Though it can appear at any age, it primarily affects women between the ages of 15 and 44. In the US, the disease is more common in African Americans, African Caribbeans, Asian Americans, and Hispanic Americans.

Although the exact causes of SLE remain uncertain, a variety of factors have been connected to the illness.

Environmental factors

Environmental triggers can include:

• Viruses
• Trauma
• Certain medications
• Sunlight or Ultraviolet rays
• Physical or emotional stress
• Smoking

Genetics

Although no specific gene is associated with the condition, people with lupus frequently have relatives who also have other autoimmune diseases. So, you are more likely to develop the disease if your family members have it.

Hormones and Gender

SLE affects more women than men. Women may also experience severe symptoms during pregnancy and menstrual cycles. Some medical experts now think that the female hormone estrogen may contribute to the development of SLE due to both of these observations. However, further research is required to support this notion.

Risk Factors of SLE

The following factors may raise your risk of developing lupus:

1. Your Gender. Over 90% of identified cases are attributable to women of reproductive age. Thus, women are significantly at higher risk than men.
2. Age. Even though lupus can affect persons of any age, it is typically discovered between the ages of 15 and 45.
3. Race. African-American, Native American, Hispanic, and Asian women are more prone than Caucasian women to develop the disorder.

How Does Lupus Affect The Body?

Lupus can affect several body parts. It may result in aches and pains as well as severe complications in the organs. In addition, as an autoimmune condition, lupus makes your body attack itself, which may cause organ damage over time.

Skin, joints, blood, kidneys, heart, brain, and lungs are among the body parts that lupus might affect.

Let us take a closer look at each organ involved in SLE.

Skin

Lupus frequently causes skin problems. Some people living with lupus develop a red rash across their cheeks and nose bridge. The disease was named "lupus" (which means wolf in Latin) due to the position of the rash, which is the same as the common markings of a wolf. Large, circular, red rashes (plaques) that may leave scars (called discoid lupus) are among the other skin issues that could develop due to SLE. Sunlight typically makes skin rashes worse. Mouth sores and hair loss are also common.

Joints

People with lupus frequently develop arthritis. Pain may be present either with or without swelling. Mornings can be a tough time due to joint stiffness and pain.

Arthritis may be an issue for a few days to weeks or a chronic aspect of the illness. Luckily, arthritis rarely causes total disability.

Blood

Involvement of the blood can occur with or without additional symptoms. For example, people with lupus may experience dangerous drops in their platelets (cells that help clot the blood), white blood cells, or red blood cells.

Changes in blood counts can occasionally cause anemia (low red blood cell counts), severe infection (due to low white blood cell counts), as well as easy bruising (as a result of low platelet count). Yet, since many people do not exhibit symptoms that point to blood abnormalities, blood testing is crucial to identify any issues routinely.

Blood clots are more often seen in people with lupus. Deep venous thrombosis (a clot in the leg veins), pulmonary embolism (a clot in the lung veins), and brain clots (which cause a stroke) are common.

Blood clots developing in SLE may be linked with the production of antiphospholipid antibodies. These antibodies are abnormal proteins that can cause blood clotting and also increase the risk of miscarriage in women.

Kidneys

Up to 50% of people with lupus might have kidney involvement, which can potentially be fatal. Those with lupus who experience joint pain, a rash, a fever, and weight loss may also have kidney problems. Kidney disease can occasionally occur without any other lupus symptoms.

Typically, symptoms of kidney disease do not appear until the disease has advanced. Therefore, proper diagnosis and treatment of renal diseases are crucial. Urinalysis, a quick urine test, can identify the early symptoms of kidney disease.

Lungs and heart

Inflammation of the protective coverings of the lungs (pleura) and heart (pericardium) is common in SLE. When these structures become inflamed, you may experience chest pain, an irregular pulse, and a buildup of fluid around the heart (pericarditis) and lungs (pleuritis or pleurisy).

Brain

Thankfully, people living with lupus seldom experience brain involvement. However, confusion, seizures, depression, and strokes are possible when SLE affects the brain.

Symptoms Of SLE

There are many different symptoms that lupus patients may experience. Each person has a unique set of symptoms. However, many of these symptoms are similar to other medical conditions, which is one of the main challenges in diagnosing the disease.

Lupus symptoms may take time to develop. With time, you may even experience new symptoms. Also, the severity of your symptoms could change with time. When symptoms are in remission, they may occasionally be scarcely noticeable, but sometimes they may also flare up.

Symptoms of lupus can include:

• Joint pain and swelling
• General discomfort, ill feeling (malaise), or uneasiness
• Severe fatigue
• Headaches
• Sensitivity to sunlight

A rash on the nose and cheeks is called a “butterfly rash.”

• Anemia
• Fever with no other cause
• Hair loss
• Weight loss
• Mouth sores
• Chest pain when taking a deep breath
• Blood-clotting problems
• Raynaud’s phenomenon (fingers turning blue or white and tingling when cold)
• Swollen lymph nodes

Diagnosis can be challenging because many other diseases also exhibit lupus symptoms. See your physician if you experience any of these symptoms. Your doctor can perform tests to gather the information required to make an accurate diagnosis.

How Is SLE Diagnosed?

Lupus diagnostic procedures can be time-consuming and challenging. The signs and symptoms of lupus can coexist with those of other illnesses, such as diabetes and arthritis. Lupus symptoms may take some time to manifest, making diagnosis more difficult.

To determine whether lupus runs in your family, your healthcare professional will usually start by asking about your family history. Then, your doctor will want to talk about any symptoms you have had. After discussing your symptoms, your doctor will perform a physical examination to look for the typical signs and symptoms of SLE, including:

• Mucous membrane ulcers that can develop in the nose or mouth
• Sun sensitivity rashes, such as butterfly rash or malar rash
• Arthritis in the small joints of the foot, hands, wrists, and knees
• Hair fall or thinning
• Signs of lung or heart involvement, such as rubs, murmurs, or irregular heartbeats

Depending on your symptoms, you might need to see a variety of experts, such as doctors who treat blood issues (hematologists), disorders of the neurological system (neurologists), or problems with the kidneys (nephrologists). Unfortunately, there is no single test that can diagnose SLE, but there are screenings that can assist your doctor in making an informed diagnosis, such as:

Complete Blood Count (CBC)

This test assesses the amount of hemoglobin, a protein found in red blood cells, as well as the number of white blood cells, platelets, and red blood cells. Findings can show you have anemia, which is a typical lupus complication. Lupus can also cause a low white blood cell or platelet count.

Erythrocyte sedimentation rate (ESR)

This blood test measures how quickly red blood cells drop to the bottom of a tube in one hour. A faster-than-usual speed may indicate a systemic illness like lupus. There is no disease-specific sedimentation rate. ESR might be increased if you have lupus, an infection, cancer, or other inflammatory diseases.

Urine tests

If lupus has affected your kidneys, an analysis of your urine sample may reveal an elevated level of protein or red blood cells in the urine.

Antinuclear antibody test

An antinuclear antibody (ANA) test checks for antibodies, which are proteins in the body that fight off illnesses. A positive ANA test may be a symptom of an autoimmune disease. Lupus patients frequently have positive ANA results.

However, a positive antinuclear antibody test does not necessarily indicate lupus. Most lupus patients who take the ANA test get positive results. However, many people without lupus also have positive results.

Because of this, a positive ANA result alone is insufficient to diagnose lupus. Therefore, before diagnosing lupus, your doctor will often confirm with at least three other clinical signs (such as symptoms and family history).

Chest x-ray

Unusual shadows on a chest scan could indicate fluid or lung inflammation.

Echocardiogram

In this test, sound waves produce visualizations of your heartbeat in real time. It can identify issues in your heart's valves and other areas.

Biopsy

Lupus can affect the organs in various ways. Depending on the type of damage, treatments can change.

Testing a small sample of kidney tissue is sometimes required to establish the best course of action. This can be performed using a tiny incision or a needle. In addition, sometimes, a skin biopsy is done to confirm a diagnosis of lupus of the skin.

Treatment Of SLE

The symptoms and complications you are dealing with may affect how your doctor approaches treating your lupus. Several other factors are also considered, such as:

• The severity of your condition
• Your age
• Your overall well-being
• The kind of drugs you might be using
• Your medical history

Lupus is a chronic (lifelong) disease that requires ongoing management. Treatment aims to reduce organ damage caused by the disease and put your symptoms into remission (where they are no longer present or active). Sadly, lupus can strike without warning, and its effects may fluctuate over time. Therefore, you will need to see your doctor regularly and modify your treatment strategy based on your symptoms.

Some people with minor symptoms of lupus may just need little care and treatment. These people may experience symptoms that are observed and monitored to ensure they do not worsen, but they do not currently require treatment.

Others may require a vigorous course of treatment. However, these people typically experience more severe problems (like lung, heart, or kidney complications). Your healthcare practitioner will discuss the optimal course of action with you in light of your symptoms, complications, and medical history.

Medications

The following medicines can be used to treat lupus:

Steroids (corticosteroids, including prednisone)

Rashes can be treated directly with steroid creams. Cream use is typically risk-free and effective, especially for minor rashes. For mild or moderate lupus symptoms, using steroid pills or creams in low doses can be helpful. When internal organs are in danger, steroids can also be administered in higher amounts. However, side effects are most likely to occur at high doses.

Azathioprine (Imuran®)

This drug, which was first developed to prevent organ transplant rejection, is frequently used to treat the more severe lupus symptoms.

Hydroxychloroquine (Plaquenil®)

This drug is frequently used to treat moderate lupus-related issues like skin and joint disease. Moreover, it is used to alleviate oral sores and fatigue.

Mycophenolate mofetil (CellCept®) and Cyclophosphamide (Cytoxan®)

These treatments, which are chemotherapy drugs, have potent effects on suppressing the activity of the immune system. They are used to treat more severe cases of lupus, particularly lupus that involves the kidney.

Methotrexate (Rheumatrex®)

This medicine suppresses the immune system and is another chemotherapy drug. Its use is growing in popularity as a treatment for rheumatoid arthritis, skin conditions, and other non-life-threatening illnesses that have not responded to medications like hydroxychloroquine or low doses of prednisone.

Belimumab (Benlysta®)

This medicine, a monoclonal antibody, lowers the activity of white blood cells or lymphocytes that produce autoantibodies. Autoantibodies are significant because they harm tissue. For example, lupus that does not affect the kidneys and has not responded to previous forms of treatment is treated with belimumab.

Rituximab (Rituxan®)

This drug is also a monoclonal antibody that inhibits the production of autoantibodies by white blood cells (lymphocytes). When other forms of treatment have failed to control lupus, it is occasionally used to treat it.

Other newer drugs

Voclosporin has been demonstrated to be efficient in treating lupus in clinical trials. In addition, abatacept (Orencia), anifrolumab, and other medicines are actively being researched as potential lupus treatments.

Alternative medicine

People with lupus occasionally look for complementary or alternative treatments. Alternative treatments may help with lupus symptoms, but none have been proven to change the course of the illness.

Before beginning any therapies on your own, talk to your doctor about them. They can help you weigh the risks and benefits and let you know if the treatments will negatively affect your current lupus drugs.

Alternative and complementary therapies for lupus include:

1. Acupuncture: Small needles are implanted just beneath the skin during an acupuncture session. It might decrease the muscle pain that is associated with lupus.
2. Dehydroepiandrosterone (DHEA): Along with standard therapy, taking pills containing this hormone may help decrease lupus flare-ups. However, women who take DHEA may suffer from acne.
3. Fish oil: Omega-3 fatty acids found in fish oil supplements may be helpful for people with systemic lupus erythematosus. Initial research has shown some promise, but more studies are necessary. In addition, fishy breath, nausea, and belching are some side effects of fish oil supplements.

Complications of SLE

Inflammation due to lupus can affect several areas of your body. The following are the complications of SLE.

Kidneys

Severe kidney disease can result from lupus, and renal failure is one of the main reasons why patients with lupus pass away.

Central nervous system and brain

You may experience headaches, behavioral changes, dizziness, vision issues, seizures, or strokes if your brain is affected by lupus. In addition, many lupus patients have memory issues and may struggle to verbalize their thoughts.

Blood and blood vessels

Anemia (low levels of healthy red blood cells) and an elevated risk of bleeding or blood clotting are two blood issues that lupus may cause. It can also result in the inflammation of the blood vessels.

Lungs

Having lupus raises your risk of developing inflammation of the chest cavity lining, which can make breathing difficult. Pneumonia and bleeding into the lungs are also possible.

Heart

Your heart muscle, arteries, or heart membrane may become inflamed due to lupus. Moreover, there is a marked increase in the risk of heart attacks and cardiovascular diseases.

Pregnancy complications

Miscarriage is more likely to occur in lupus-affected women. It also raises the risk of preterm birth and high blood pressure during pregnancy. Doctors frequently advise waiting until your disease has been under control for at least six months before getting pregnant to lower the risk of these complications.

Infection

Because lupus can weaken the immune system, both the disease and its therapies make lupus patients more susceptible to infection.

Cancer

Although the risk is minimal, having lupus increases your risk of developing cancer.

Death of bone tissue

This happens when the blood flow to a bone decreases, which frequently causes little breaks in the bone before the bone eventually collapses.

Lifestyle and home remedies

If you have lupus, take care of your body. You can prevent lupus flares and, should they happen, manage your symptoms by taking a few easy steps.

Try to-

1. Schedule frequent doctor visits: Regular checkups, as opposed to going to the doctor when your symptoms get worse, may help prevent flares and help your doctor address everyday health issues like stress, nutrition, and exercise that can help avoid problems of lupus.
2. Be sun-smart: Use protective clothes, such as a long-sleeved shirt, hat, and long pants. In addition, use sunscreen with an SPF of at least 55 whenever you are outside because UV light can provoke a flare.
3. Eat a healthy diet: Prioritize eating whole grains, vegetables, and fruits as part of a healthy diet. Sometimes your healthcare provider may advise dietary restrictions, especially if you have high blood pressure, kidney damage, or gastrointestinal issues.
4. Quit smoking: Smoking raises your risk of cardiovascular disease and can worsen lupus' effects on your heart and blood vessels.
5. Exercise regularly: Exercise can lower your chance of heart attack, keep your bones strong, and improve your overall health.

Find out from your doctor if you require calcium and vitamin D supplements. Some evidence suggests that taking vitamin D may help people with lupus. In addition, depending on your age, a calcium supplement can help you reach the recommended dietary allowance of 1,000–1,200 mg of calcium per day, which is advised for maintaining bone health.

Outlook

Lupus affects each person differently. Therapies work best when you begin them as soon as symptoms appear and when your doctor personalizes them. If you experience any concerning symptoms, you must schedule an appointment with your doctor.

Living with a chronic illness can present its own challenges. Ask your doctor about local support groups. You can control your condition, manage your stress, and maintain good mental health by working with a skilled counselor or support group.

When To Get Medical Help?

In case you experience SLE symptoms, speak with your doctor. Also, if you have this disease and your symptoms worsen or a new sign appears, you should contact your physician.

To learn more about Systemic Lupus Erythematosus (SLE), please check our blog on SYSTEMIC LUPUS ERYTHEMATOSUS IN CHILDREN.

Resources

• Kaul, Arvind et al. “Systemic lupus erythematosus.” Nature reviews. Disease primers vol. 2 16039. 16 Jun. 2016, doi:10.1038/nrdp.2016.39
• Justiz Vaillant AA, Goyal A, Varacallo M. Systemic Lupus Erythematosus. [Updated 2022 Sep 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK535405/
• Fava, Andrea, and Michelle Petri. “Systemic lupus erythematosus: Diagnosis and clinical management.” Journal of autoimmunity vol. 96 (2019): 1-13. doi:10.1016/j.jaut.2018.11.001

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