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Systemic Lupus Erythematosus

Overview

Lupus erythematosus is an autoimmune inflammatory disease that occurs when the body&rsquo;s immune system attacks its own tissues and organs. The inflammation caused as a result of the immune reaction can affect various organs and tissues, like kidney, skin, joints, blood cells, heart, lungs, and brain.&nbsp;

A facial rash that looks like a butterfly unfolding across both cheeks is one of the most striking and distinctive features of systemic lupus erythematosus (SLE). However, it does not occur in some individuals. As a result, the diagnosis of SLE is delayed because the signs and symptoms are often confused with other ailments.&nbsp;

Some people are born with a tendency towards developing SLE that is exacerbated with some drugs, sunlight and infections, etc. The treatment of lupus is mainly symptomatic because there is no permanent cure to this disease<a href="#accordion">[1]</a>.

<div>&nbsp;</div>

House wasn&rsquo;t wrong to be frustrated every time someone said &ldquo;It may be Lupus&rdquo;. Apart from the fact that diagnosing it as Lupus was the easy way out, he had to feed that ego too, right?&nbsp;

Systemic lupus erythematosus is an extremely hard-to-diagnose disease because it is a systemic disease, i.e., it can show a wide array of signs and symptoms. These symptoms often overlap with other diseases too, so even though it may be easy to claim it&rsquo;s Lupus, remember what House said: &ldquo;It&rsquo;s not Lupus&rdquo;!&nbsp;&nbsp;

The United States has 20-150 cases of SLE in every 100,000 individuals. According to research, it presents 164 white females compared to 406 cases of African Americans. In the late 20th century, there was an increase in positive cases because of improvised detection. The numbers almost tripled from what was observed at the beginning of the 20th century because of the advanced technology and a patient-oriented approach that was used to diagnose SLE.

In Asia, South America, North America, and Europe, the incidence rate is 1-25 in 100,000 individuals. A closer inspection reveals that the greater majority of cases contributing to the prevalence of SLE in Europe are individuals of African or Asian ethnicity. The United States has the highest incidence rate of SLE among African American, Hispanic American, African Caribbean, and Asian individuals as compared to white people<a href="#accordion">[3]</a>.

<div>&nbsp;</div>

SLE is not the only variant of lupus. It&rsquo;s relatively common, but there are other types of lupus as well, including<a href="#accordion">[1]</a>:

&nbsp; &nbsp; &nbsp;

Cutaneous Lupus Erythematosus: This lupus affects the skin. Individuals who develop cutaneous lupus erythematosus experience skin issues, such as sensitivity to the sun, rashes, and hair loss.

&nbsp; &nbsp;

Drug-Induced Lupus: In such patients, it&rsquo;s usually a reaction to certain medications. People with drug-induced lupus may have symptoms similar to SLE, however, it is temporary. The condition subsides once the causative medication is stopped.

&nbsp;&nbsp;

Neonatal Lupus: This is a rare variant. Neonatal lupus, as the name suggests, is a condition found in newborns. Such children have antibodies that are passed to them from their mothers who had SLE during pregnancy.

<div>&nbsp;</div>

Signs And Symptoms

A few signs and symptoms commonly seen in SLE patients are<a href="#accordion">[2]</a>:

&nbsp;

<ul>
	<li>Sensitivity to sunlight</li>
	<li>Confusion</li>
	<li>Shortness of breath</li>
	<li>Stomach pain</li>
	<li>Fatigue</li>
	<li>Hair loss</li>
	<li>Mouth sores&nbsp;&nbsp;</li>
	<li>Dry eyes</li>
	<li>Fatigue&nbsp; &nbsp; &nbsp;</li>
	<li>Chest pain</li>
	<li>Swollen glands</li>
	<li>Headaches</li>
	<li><a href="https://www.merisehat.pk/disease/depression" target="_blank">Depression</a></li>
</ul>

&nbsp;

Raynaud&#39;s phenomenon is one of the common symptoms of SLE in which the fingers turn white or blue when cold. The patient also has a tingling sensation on the fingertips.&nbsp;The rash on the cheeks and nose (also known as the butterfly rash) should not be ignored either.

<div>&nbsp;</div>

Causes

Lupus is a disease with various manifestations. Not only is it complicated, but it&rsquo;s even more difficult to track its cause and etiology. However, some factors are consistent with the incidence of this disease. These include<a href="#accordion">[1]</a>:

&nbsp;

<ul>
	<li>Hormonal Changes</li>
</ul>

The hormone estrogen is one of the reasons contributing to SLE. This implies that the female gender is prone to this disease. Another reason for estrogen being considered a major factor is that the women who are 15-44 years old (reproductive age) are more likely to have SLE.

&nbsp;

<ul>
	<li>Environmental Factors</li>
</ul>

Environmental factors also play a major role in the prevalence of SLE. Mostly, it&rsquo;s sun exposure, but other things like daily consumption of synthesized drugs, viral infections, and compromised environmental conditions can contribute as well. Stress can also be a reason for SLE in some patients.

&nbsp;

<ul>
	<li>Family History</li>
</ul>

Having a family history of SLE is one of the strongest reasons for developing the disease. If the genetic makeup predisposes to developing lupus, all the family members will be at equal risk.

<div>&nbsp;</div>

The recommended first line modality used for screening purposes is an antinuclear antibody (ANA) test. But it should be noted that ANA is not specific for SLE and can be used in a wide array of various connective tissue diseases, infections, and malignant (cancerous) conditions.

&nbsp;

Diagnosis

Diagnosing SLE is difficult because the signs and symptoms are non-specific and vary from person to person. There is no single testing modality that can diagnose SLE, and therefore, a combination of blood and urine tests, observation of signs and symptoms, and physical examination is necessary to reach a diagnosis<a href="#accordion">[5]</a>.

&nbsp;

<h5>Blood And Urine Tests&nbsp;</h5>

These include:

&nbsp;

<ul>
	<li>Complete Blood Count (CBC): A CBC informs the doctor about the number of red blood cells, white blood cells, and platelets along with the amount of hemoglobin, which is essentially a protein in your red blood cells. Anemia is a common finding in the case of SLE. Moreover, a low white blood cell or platelet count may be observed in lupus as well.</li>
</ul>

&nbsp;

<ul>
	<li>Erythrocyte Sedimentation Rate (ESR): It is a blood test that determines the rate at which the red blood cells settle to the bottom of a test tube in an hour. If it is faster than the physiological value, it may indicate lupus.</li>
</ul>

&nbsp;

<ul>
	<li>Kidney And Liver Tests: Blood tests can also be used to assess how efficiently the patient&rsquo;s kidneys and liver are functioning. These tests are important because lupus can affect these organs.</li>
</ul>

&nbsp;

<ul>
	<li>Urinalysis: It is a detailed assessment of the urine sample that may show an increased level of protein or red blood cells in the urine. This usually happens when lupus has affected the kidneys.</li>
</ul>

&nbsp;

<ul>
	<li>Antinuclear Antibody (ANA) Test: A positive test for the presence of these antibodies indicates that the immune system is activated. Most people with lupus have a positive ANA test, but a lot of people with a positive ANA do not have lupus.</li>
</ul>

&nbsp;

<h5>Imaging Tests</h5>

These include:&nbsp;

&nbsp;

<ul>
	<li>Chest X-ray: A chest X-ray may exhibit abnormal shadows, which indicates fluid or inflammation in the lungs.</li>
</ul>

&nbsp;

<ul>
	<li>Echocardiogram: This imaging modality uses sound waves to produce real-time images of the heart while it is pumping blood. It helps to review problems with the valves and other parts of the heart.</li>
</ul>

&nbsp;

<h5>Biopsy</h5>

Lupus tends to harm the kidneys in various ways and, thus, treatment plans depend upon the type of damage that has occurred. The sample is obtained with the help of a needle or through a small incision. Skin biopsy is also prescribed to confirm a diagnosis of the cutaneous variant.

<div>&nbsp;</div>

<h4>"Did you know that autoimmune diseases e.g. SLE can have an effect on your heart rate. Head on over to the <a href="https://www.merisehat.pk/page/sehat-scan/" target="_blank">sehat scan</a> and check your heart rate today!" </h4>

Treatment

There is no exclusive treatment for lupus but the major goal of the doctor is to design a management plan that relieves the symptoms and allows the patient to lead a normal life. The treatment may also depend upon the degree of the disease and the body part(s) involved<a href="#accordion">[4]</a>.

&nbsp;

It may include:

&nbsp;

<ul>
	<li>Topical steroids in the form of ointments and creams for rashes.</li>
</ul>

&nbsp;

<ul>
	<li>Antimalarial drugs to relieve joint pain, stiffness, skin rashes, pericarditis (inflammation of the lining of the heart), pleuritis (inflammation of the lining of the lung), fatigue and fever. These medications may also prevent its spread&nbsp; to certain organs, such as the kidney and central nervous system, and may help to reduce flares by as much as 50%.</li>
</ul>

&nbsp;

<ul>
	<li>Anti-inflammatory medication to relieve pain, swelling, and fever associated with lupus.</li>
</ul>

&nbsp;

<ul>
	<li>Corticosteroids to minimize the immune response.&nbsp;</li>
</ul>

&nbsp;

<ul>
	<li>Targeted immune system agents along with disease modifying drugs (DMARDs) to reduce the immune response in severe cases.</li>
</ul>

<div>&nbsp;</div>

Along with serious complications to the major organs, lupus can affect numerous other parts of the body causing major pain and inflammation. As it is an autoimmune disease, it eventually leads to organ failure. It can slowly involve multiple organs, like the brain, kidney, skin, blood, joints, and lungs, leading to widespread destruction throughout the body.

&nbsp;

The following are the complications of SLE<a href="#accordion">[1]</a>:

&nbsp;

<ul>
	<li>Skin Scarring&nbsp;</li>
</ul>

The name of the disease lupus has been derived from the word &ldquo;wolf&rdquo; in Latin. It is because the skin manifestation of this disease resembles the markings of a wolf. Often, there is a red facial rash over the patient&rsquo;s cheek and the bridge of the nose. Some patients may have red-colored, circular rashes called plaques. The plaques might develop into a scar (known as discoid lupus) and get worse with exposure to sunlight. Other common manifestations in the skin are mouth sores and extensive hair loss.

&nbsp;

<ul>
	<li>Blood</li>
</ul>

There&rsquo;s a major decline in the number of white blood cells, platelets, and red blood cells in patients with SLE that compromises regular coagulation and respiration. These symptoms appear in some patients while they don&rsquo;t manifest at all in others. The involvement of red blood cells indicates that the individual has to face a great deal of fatigue, and the involvement of white blood cells means that the immunity is compromised and there&rsquo;s a greater possibility of contracting more infections. Many patients also face serious bruising due to lower blood platelet count, which is responsible for the clotting of the blood. It&#39;s important to keep a check on the blood abnormalities due to lupus so periodic blood tests are advised as they help monitor the level of different blood cells.&nbsp;

&nbsp;

Moreover, SLE patients frequently present with blood clots. The clotting is majorly seen in the legs (causing <a href="https://merisehat.pk/disease/deep-vein-thrombosis" target="_blank">deep venous thrombosis</a>), lungs (causing pulmonary embolism), and in the brain (causing brain <a href="https://www.merisehat.pk/disease/stroke" target="_blank">stroke</a>). The clotting is majorly due to the production of antiphospholipid antibodies that are abnormally synthesized and lead to a higher tendency of blood clotting.

&nbsp;

<ul>
	<li>Joints</li>
</ul>

Arthritis is inflammation of joints and it is a common feature in patients of lupus. The joints may be inflamed with or without swelling and cause severe pain. This pain and stiffness may be more pronounced in the morning than at any other time of the day. Although it is not a permanent feature of the disease, it may last anywhere from a few days to a few weeks but it is not severe enough to compromise the mobility of the patient.

&nbsp;

<ul>
	<li>Kidneys</li>
</ul>

Almost half of the patients with SLE present with potentially life-threatening kidney involvement. Symptoms include feeling fatigued or developing arthritis, rash, or high fever, or experiencing weight loss. Moreover, the patient appears quite pale. Kidney problems often start at the same time or shortly after lupus symptoms appear. Early diagnosis and prompt treatment of the kidney can lead to the betterment of the symptoms with time. The kidney can be monitored by a frequently performed urine test called a urinalysis.

&nbsp;

<ul>
	<li>Brain</li>
</ul>

Although brain involvement in lupus erythematosus is quite rare, it is a serious issue. Whenever SLE affects the brain, the patient experiences confusion, depression, seizures, and, in severe cases, strokes.

&nbsp;

<ul>
	<li>Heart And Lungs</li>
</ul>

The heart and lungs are involved due to inflammation in the pericardium and pleura. These membranes are coverings of the heart and the lungs respectively; therefore, the patient may experience severe chest pain, irregular heartbeat, and collection of fluid around the lungs and the heart when the inflammation is extensive.

<div>&nbsp;</div>

A risk factor is essentially something which has the tendency to increase the individual&rsquo;s probability of getting a particular disease. Following are the risk factors for SLE:

&nbsp;

<ul>
	<li>Gender</li>
</ul>

SLE is more common in women aged between 15-45 than men.

&nbsp;

<ul>
	<li>Genes</li>
</ul>

The probability of getting SLE is higher if there are other family members who have SLE.

&nbsp;

<ul>
	<li>Ethnicity</li>
</ul>

People of Black, Native American, Asian, or Hispanic ethnicity have a higher risk of getting SLE.

&nbsp;

<ul>
	<li>Workplace</li>
</ul>

The risk is great if the work requires being around smoke, being exposed to excessive sunlight or being exposed to chemicals.

&nbsp;

<ul>
	<li>Other Diseases</li>
</ul>

Bacterial and viral infections, specifically Epstein-Barr virus, are known to be associated with SLE.

<div>&nbsp;</div>

Currently, there is no assured way to prevent SLE because the exact cause is unknown. The only known risk factors are non-modifiable, i.e., that they cannot be changed.

&nbsp;

Obinutuzumab is a type II humanized anti-CD20 monoclonal antibody (mAb) that depletes B cells. It has been tested in patients suffering from lupus nephritis and has presented promising results. More than 100 patients with Class III or Class IV lupus nephritis were randomly given obinutuzumab or placebo given with corticosteroids and mycophenolate mofetil (MMF). Complete renal response was achieved in 40% of the patients by the end of 52 weeks in the obinutuzumab group and in 18% of the patients in the placebo group at the end of week 76. Moreover, this response was constant in 41% of the patients in the obinutuzumab group and in 23% of the patients in the placebo group by week 104. Because the occurrence of serious infections was 8% in the obinutuzumab group and in 18% in the placebo group, it made obinutuzumab not only an efficacious drug but also a preference in the management of lupus nephritis<a href="#accordion">[6]</a>.

&nbsp;

Key Points

<ul>
	<li>The United States has 20-150 cases of Lupus Erythematosus in every 100,000 individuals. Women are at higher risk as compared to men; there are reportedly 164 cases in white females and 406 cases in African American females.</li>
	<li>Lupus erythematosus is an autoimmune inflammatory disease that is caused when the body&rsquo;s immune system starts attacking its own tissues and organs.</li>
	<li>The inflammation caused as a result of the immune reaction can affect various organs and tissues, like kidney, skin, joints, blood cells, heart, lungs, and brain. The diagnosis of lupus erythematosus is usually delayed because the signs and symptoms are often confused with other ailments.</li>
	<li>Some of the common symptoms include sensitivity to sunlight, confusion, shortness of breath, stomach pain, fatigue, hair loss, mouth sores, dry eyes, fatigue, chest pain, swollen glands, headaches, and <a href="https://www.merisehat.pk/disease/depression" target="_blank">depression</a>.</li>
	<li>Raynaud&#39;s phenomenon is one of the common manifestations in which the fingers turn white or blue when cold. The patient also has a tingling sensation on the fingertips. Furthermore, the rash on the cheeks and nose (called the butterfly rash) should not be ignored.</li>
	<li>Some factors that are consistent with the incidence of this disease include hormonal changes, environmental effects, certain drugs, and hereditary contribution.</li>
	<li>There is no exclusive treatment for lupus but the major goal of the doctor is to design a management plan that relieves the symptoms and allows the patient to have a normal life. This includes the use of corticosteroids, DMARDs, analgesics, antimalarial drugs, and anti-inflammatory agents.</li>
	<li>The recommended first line modality used for screening purposes is an antinuclear antibody (ANA) test.</li>
	<li>There is no single testing modality that can diagnose SLE therefore a combination of blood and urine tests, signs and symptoms, and physical examination is necessary to make a diagnosis.&nbsp;</li>
	<li>Obinutuzumab is a type II humanized anti-CD20 monoclonal antibody (mAb) which depletes B cells. It has been tested in patients suffering from lupus nephritis and has presented promising results.</li>
</ul>

<div>&nbsp;</div>