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This lesson will discuss the complex pathophysiology behind systemic lupus erythematosus. You will learn why it occurs, what may trigger it, and how it can be properly diagnosed, treated, and managed.

Vengeance Upon The Body

I spent part of my childhood romping in the spinneys engulfing part of my family summer home. But one day, as a young lad, I was attacked by a hornet that left a welt on me as large as my kneecap! I would never forget that moment or my newfound disdain of hornets not associated with Charlotte. If I ever found a hornet’s nest, I promised myself I, small as I may be, will crush it! And like little angry old me, sometimes the immune system promises to do the same exact thing to our own body, resulting in serious consequences we’ll get to in a just a little bit.

What Is Systemic Lupus Erythematosus?

The disease I want to describe to you that involves an immune system gone wild is called systemic lupus erythematosus, or SLE for short. This is an incurable autoimmune condition that can affect any part of the body, including the skin, heart, lungs, kidneys, and nervous system.It’s got the word ‘systemic’ in the name because that word implies a body-wide problem as opposed to one affecting only a local area.

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‘Lupus’ is the Latin name for ‘wolf,’ and some people claim that the facial rash some people have in this disease causes them to mimic the facial coloration patterns some wolves have. Whether that’s actually where the term came from is not all that relevant, but the final word ‘erythematosus’ is important to know, as its Greek prefix ‘erythro’ references the ‘red’ rash some people with SLE have. SLE is more common in women, can affect a person of any age, and is more likely to occur in people of African descent.

Why Does Systemic Lupus Erythematosus Occur?

With that out of the way, we can now turn our attention to what I consider to be the most exciting part of this lesson, where we find out about one theory as to why this problem occurs and how the environment plays a role. The exact underlying cause of what causes SLE is not known, but changes to many different genes are a big part of the problem. These genes encode for many important things in our body that end up going wrong in this disease process.Just think about a computer’s code.

If a computer code is programmed incorrectly or changed improperly, it will cause the computer to malfunction, just like genes that code for the immune system may cause it to malfunction if improperly coded for.Under normal conditions, your body’s immune system, composed of white blood cells, such as T-cells, and proteins, such as antibodies and complements, is there to help fight off infectious agents of disease. These components trigger or use the inflammatory process to help fight off disease.

Inflammation is what causes all the redness, swelling, and pain associated with everything from hornet stings to cuts to diseases. Inflammation is good in the short-term fight against disease, but dangerous and harmful to our body in the long run, regardless of the cause.Another way by which our body protects itself is by having its own cells commit suicide through a process known as apoptosis. This is an orderly and clean process that results in a cell shriveling up and being cleared away by the body from the area where it died as a result of some sort of damage.

An Immune System Gone Wild

And here is where the trouble in people with SLE begins. People with SLE have an apoptotic process that goes a bit haywire. Everything from sun exposure to infection to drugs abnormally increases an abnormal process of apoptosis to occur.

The cells that commit suicide not only increase in amount, but are also not readily cleared away by your body’s garbage collection cells. This causes innards of the un-cleared cells, namely things like DNA and proteins, to be released – something that shouldn’t occur in a normal apoptotic process.As the dying cells do this, an equally aberrant immune response is triggered. The content of the dying cell that is expelled into the outside world is identified, erroneously, as being equivalent to that of a dangerous bacterium.

Antibodies are thereby produced against and latch onto these proteins to form antibody-protein complexes. These antibodies, coupled with an over-reactive subsets of things like T-cells, also trigger inflammation or begin to attack our own body’s cells, tissues, and organs that harbor these proteins.Furthermore, the antibody-protein complexes that form as a result of what I just mentioned, circulate around the body and lodge themselves in other places, such as the basement membrane of the skin (which helps to support the skin layers) or the glomeruli of the kidneys (which help to filter blood), triggering serious inflammation in those organs as well. Meaning, the trouble may only begin in one area, but it will eventually spread to other parts of the body.So, in essence, our immune system promises vengeance upon its own body. The proteins and DNA released by the apoptotic cells are like the hornets that fly out of their nest, the cell. The hornets are identified as a threat by our body, even if they don’t sting anyone at all, and the body produces weapons to kill anything, hive, nest, or otherwise, that harbors these hornets.

As collateral damage, the body also kills the hives of wasps, bees, and anything else that looks like a nest of any sorts, like a beautiful bird’s nest, all as a consequence of the immune system’s misplaced anger and paranoia toward its own body.

Clinical Signs, Symptoms, and Diagnostics

This erroneous response may lead to a combination of any of the following:

  • A malar rash (a butterfly-shaped rash on the face of some people who have SLE)
  • Arthritis
  • Kidney damage
  • Heart disease
  • Seizures

And a whole lot more. Diagnosing this condition isn’t as easy as you’d think, since so many variations of it exist. What doctors must do is conduct a physical exam to assess any obviously affected areas; run blood tests that look for things like the antibodies that attack your own cellular DNA; take a sample of kidney tissue for examination under the microscope; and conduct imaging studies, such as an echocardiogram, to assess any issues pertaining to the heart.

Treatment of Systemic Lupus Erythematosus

Now that you can appreciate the complexity of this disease and its diagnostics, it’s time we end this lesson on the sobering note that this terrible malady cannot be cured, only managed through a wide variety of treatments. Let’s tie back our treatment options to the pathophysiological process I described. First, we can use corticosteroids.

These guys can help to suppress some of the inflammation that is occurring by stopping the production of inflammatory molecules.We can also use immune suppressants that will suppress immune system cells, such as T-cells, involved in this disease. For instance, the drug mycophenolate can cause T-cells to commit suicide, thereby minimizing their role in SLE.

Finally, lifestyle changes, namely the avoidance of sunlight that can exacerbate the entire reaction I described before, is critical to making SLE more manageable for your future patients.

Lesson Summary

There was a lot here, I know, but it’s an important disease and process to understand, whether you end up treating humans, dogs, or cats. Therefore, it’s important you look over the material I outlined another time if you need to.

In summary, we discussed systemic lupus erythematosus, or SLE for short. This is an incurable autoimmune condition that can affect any part of the body, including the skin, heart, lungs, kidneys, and nervous system.This disease, namely exacerbated by U.V.

radiation in the form of sunlight, causes your body’s cells to undergo apoptosis in increasing amounts. This is coupled with improper clearance of apoptotic cells, which allows for the buildup of intracellular antigens in the body. These antigens, such as proteins and DNA, trigger an aberrant immune response against the cells harboring such molecules.Furthermore, immune complexes made of antibodies and these proteins float around in your body and lodge themselves in faraway places, such as the skin and kidneys, triggering inflammation and destruction in the areas where they get stuck. This process may lead to a combination of any of the following:

  • A malar rash (a butterfly-shaped rash on the face of some people who have SLE)
  • Arthritis
  • Kidney damage
  • Heart disease
  • Seizures

And a whole lot more.

Diagnosing this condition via blood tests, imaging tests, and even biopsies leads to treatment via anti-inflammatory and immune suppressive medication, in addition to important lifestyle changes.

Learning Outcomes

Upon completing this lesson, you could be able to:

  • Highlight the characteristics of systemic lupus erythematosus (SLE) and discuss the causes
  • Relate some theories about the reason the disease occurs
  • List the signs and symptoms of SLE
  • Indicate some diagnosis methods and treatments for SLE
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