Did you know that Systemic Lupus Erythematosus (SLE) affects at least 5 MILLION people worldwide?
This is only a conservative number as we might only be scrapping the surface of the iceberg.
The word “Lupus” means wolf in Latin, which was first described in the 18th century. The disease was named Lupus due to the skin rash that resembles a wolf bite.
In the past, Lupus was only known as a dermatological condition. As of today, the understanding of Lupus has evolved.
It is now divided into four broad categories:
1.) Systemic Lupus Erythematosus
2.) Discoid Lupus Erythematosus
3.) Cutaneous Lupus Erythematosus
4.) Drug-Induced Lupus Erythematosus
In this post I will only focus on talking about the most prevalent type, which is Systemic Lupus Erythematosus.(SLE)
The reason it is being called systemic is because it can affect any organs in your body.
If you have been diagnosed with SLE, chances are you’re a young fertile female asian. Later I will explain why you are one of the more susceptible groups of individual to have this disease.
What is Systemic Lupus Erythematosus?
Basically, you can think of it as a disease triggered by your own immune system.
Yes, you are allergic to yourself.
Your body produces antibodies that target or sit on your organ tissues.
As a result, your body starts secreting a series of chemical substances called cytokines which are responsible for triggering inflammation at where the antibodies reside.
How can I be allergic to myself?
As with many other diseases, the underlying causes for SLE are not really well known, considering how far we’ve come in the realm of medicine.
What we do know though, is that genetics and environment play an important role in the disease origin.
To put things in perspective, let me give you an analogy…
For example you’re a woman with SLE susceptible gene embedded in your body, as someone who runs errand regularly, you’re frequently exposed under the sunlight..
UV rays then penetrate through your skin and damage your body cells causing DNA mutation.
The damaged tissues then undergo a programmed cell death known as apoptosis.
Nuclear contents that leak out from the cell are exposed to be targeted by your white blood cells.
They bind to these nuclear debris to form an antigen-antibodies complex. This complex is known as antinuclear antibodies.
The complex can either be deposited in the blood vessels or tissue organs, causing you to scream in agony.
The reason why I mentioned above that you are likely to be a woman of childbearing age was because the use of exogenous hormones has been linked to lupus onset and flares, suggesting hormonal factors as one of the triggers for the disease.
As a matter fact, the female to male ratio of SLE is at 9:1 during the childbearing years.
What are the symptoms?
SLE is known for being the “monkey of all disease”, meaning the range of symptoms varies so much from one person to another that it mimics many other diseases.
To illustrate the symptoms of SLE clearly, I will categorize them based on our body’s functioning system:
• Constitutional symptoms
Generally, the constitutional symptoms include vague complaints that can’t differentiate diseases from one another.
But you should know that SLE patients are easily tired even without doing much physical work. So your doctor might anticipate to find anaemia in your blood test.
You might also complain of having on and off fever and weight loss issues. This is seen when SLE is in active and infection occurs during the autoimmune state.
• Musculoskeletal symptoms
Up to 90% of SLE patients have joint pains and swelling of the hand, wrist and knee joints. It’s almost as if the immune system has a preference for attacking joints.
You should also take notice that the joint pain may be asymmetrical and the pain is disproportionate to the degree of swelling.
Apart from that, there won’t be any permanent joint deformity.
• Skin manifestation
Almost all SLE patients will experience some form of skin changes. The malar rash is classically seen in SLE. The pattern of malar rash resembles a butterfly spreading its wings as shown in the image below. It is also described as a reddish rash over the cheeks and nasal bridge). It usually occurs after sun exposure.
Apart from the malar rash, you may also experience hair loss or another type of skin rash called livedo reticularis. This form of rash is not specific to SLE but it can be suggestive of the disease.
It is also common for SLE patients to have mouth ulcer during the active phase of the disease.
If you have seen someone with SLE, you might probably notice that they put on gloves or socks frequently. This is because their skin changes colour when exposed to coldness.
The changes would lead to numbness and pain in the extremities. In medicine, we called this Raynaud’s phenomenon.
Did you happen to also experience one of the following events?
Interestingly, SLE has been closely associated with antiphospholipid syndrome. However, I will get into that in another post.
Basically, the body of SLE patients might contain a type of antibody called antiphospholipid antibody that can lead to the occurrence of deep vein thrombosis or repetitive miscarriages.
That being said, having one of these events in your past medical history makes it more suggestive of SLE.
Ultimately, I should highlight again that the symptoms of SLE are progressive and multiple organs can be affected.
Therefore it is not unusual for SLE patients to be admitted to the hospital for reasons that are not directly relatable to SLE.
In fact, this is also what makes the disease hard to be diagnosed.
Some of the clinical complications that SLE can cause include a new onset of hypertension. This is due mainly to renal insufficiency whereby renal vessels get occluded. This confuses the kidney into thinking that the bloody pressure is low.
It then attempts to compensate by releasing a hormone called renin to bring the blood pressure back to baseline.
More importantly, the lungs and heart can potentially be affected. You may experience some chest pain related to breathing or find it difficulty to breath.
Neuropsychiatric features such as history of seizures, memory or mood changes can also occur in SLE.
How do doctors diagnose SLE?
Well, usually your doctor will make the diagnosis only after considering the combination of the aforementioned symptoms together with a series of blood work results.
For the ease of your understanding, you just have to know that on top of your symptoms, your blood results are suggestive of SLE only if there is:
- Low haemoglobin level ( indicates anaemia)
- Leukopenia, Lymphocytopenia, Thrombocytopenia. In short, low white blood cells/platelet levels
- Low C3 & C4 complement level
- Abnormal renal profile (protein or casts in urine)
- **Inflammatory markers** such as ESR & CRP level increased
- **Antinuclear antibodies** increased (over 95% sensitivity)
- **Anti-dsDNA antibodies** (high specificity, but only 70% sensitivity)
- **Anti-sm antibodies** (highest specificity, but only 30-40% sensitivity)
** = IMPORTANT
For a doctor to confirm an SLE diagnosis, there is a list of up to 11 criteria needed to be fulfilled. If you are interested in checking out all the criteria, I’ll leave a link for you to check out here.
Viable options of treatment
If you are diagnosed with SLE, do not panic because your doctor will familiarize you with the symptoms and natural course of the disease as well as how you are going to manage it.
On the other hand, you can also consider joining a support group. Knowing that thousands of people in the world are going through the same experience will provide you a sense of relieve.
You can visit the link here if you are interested in joining an SLE support group on Facebook.
If the SLE happens to target your joints and cause you to have severe joint pain, you can try doing some SLE designated physical therapy to maintain posture and proper range of motion.
Besides that, you should also avoid over-exposing yourself under the sunlight because it can trigger the disease. If necessary, you can apply sunblock before going outdoor.
The aim of using pharmacological treatment is to relieve your pain symptom and prevent lupus flares from occurring.
Interestingly, Hydroxychloroquine is an effective anti-malarial drug that is used also for treating SLE.
The idea here is that the drug mediates a mild immune response in your body without causing your immune system to be suppressed.
Hydroxychloroquine is proven for its safety and efficacy. Hence, it is effective in treating and preventing lupus skin rashes, joint pain and joint inflammation.
More importantly, it has been associated with reduced morbidity and mortality in SLE patients in observational trials.
Similar to many diseases that can cause you to experience excruciating pain, your doctor will most probably prescribe you with pain killers such as NSAID’s to numb the pain.
For more severe cases where organs are involved, you might need to take systemic steroid therapy and be put under close monitoring.
The way steroids work in treating SLE is that it suppresses your overly active immune system to a modest level so it stops attacking your own tissue organs.
If the kidney or central nervous system is affected, cyclophosphamide and mycophenolate may also confer some benefits in improving the condition.
Do you know of anyone who suffers from SLE or that you might have SLE?
Leave a comment below and share your experience with us.
Thank you for reading the blog post.