Cutaneous Lupus and Tumid Lupus – What is the Difference? 

Tumid lupus erythematosus (TLE)  is generally considered a rare subset of cutaneous lupus, similar to discoid lupus erythematosus (DLE)  and is diagnosed under that name, though some researchers think that it’s actually a different autoimmune disorder. In any case, TLE involves inflammation in the skin, leading to the formation of skin plaques on areas that have been exposed to light. It can happen on any exposed skin, so primarily on the face, scalp, and arms (though it can cover more areas.) 

Skin plaques are a type of skin lesion that appears as raised, thick, and flaking patches of dead skin, often over reddened and inflamed skin. It occurs when inflammation attacks the skin and causes the cells to overcompensate and divide more than usual, leading to more skin cells than usual reaching the surface in that patch of skin. The plaques can be large, encompassing whole parts of the body, or small, appearing like lumps with dry, flaking skin on top. The excess skin flaking can be small and dandruff-like or come off in large flakes.  

The plaques themselves are not always harmful, but they are uncomfortable and itchy, and can lead to serious comfort and appearance issues which can cause issues with mental health, social connections, and even work. If the skin is broken because of scratching, flaking, or blistering, there is a risk of infection, and when these plaques occur on the scalp, it can encourage hair loss.  

In TLE, these plaques appear in areas that have been exposed to light. TLE can also cause general issues like fatigue and brain fog, as the inflammation uses energy and disrupts other systems in the body. 

TLE doesn’t appear to be readily detected by antinuclear antibody tests, a common test to detect lupus. It is currently unknown why. 

Photosensitivity and Lupus 

People with lupus often experience photosensitivity. This means that, when exposed to ultraviolet light, many people with lupus will have a reaction – but not as rashes or other symptoms on their skin. You can read about the classic malar rash, here.  Sunlight exposure (which includes both heat and ultraviolet exposure) can cause all lupus symptoms to suddenly intensify, including the internal ones. This is called a flare.  

People with lupus are often advised to cover up and use sunscreen whenever they go outside. People who are photosensitive have their symptoms flare in summer and in situations that have water and snow, which reflect light. As a side effect, many people with lupus have vitamin D deficiencies and need supplements. You can read more about the weather and lupus here.  

Treating Tumid Lupus Erythematosus 

TLE is treated with topical corticosteroid creams that are put on the affected areas. Sunscreen and covering clothing are used to protect the skin from the sun, and the hope is that the combination of protection and medication will help reduce the symptoms.  

Usually, the rashes and lesions will clear up with treatment and sun protection within months. 

If topical creams do not work, then doctors will prescribe antimalarials such as hydroxychloroquine. Methotrexate and mycophenolate mofetil are also used if these do not work. The hope is that these drugs will reduce inflammation in general, which will reduce symptoms. 

A Lupus Warrior’s Takeaway 

People with lupus in general should be careful about sunlight exposure – use sunscreen, clothing, and indoor facilities when possible. This comes up often when travelling or going on vacation, and you can read more about travelling with lupus here.   

People with TLE have to be wary of sunlight but should also be vigilant for the appearance of other symptoms. Although TLE and CLE are mainly in the skin, both can potentially be a sign of systemic lupus erythematosus under the surface. 

Cutaneous lupus – including TLE – can occur alongside systemic lupus erythematosus (SLE), and about 75% of people with SLE have skin-related symptoms. According to some sources, about 10% of people with SLE will have symptoms that look like TLW.  

This means that it’s important for people with lupus to monitor their condition and update their treatment team about any new symptoms that occur. 

Hormones are chemicals that different cells in the body use to communicate with each other. By communicating, cells are able to work together to perform the many tasks that our bodies need to function. The body regulates everything from mood, to metabolism, to the immune system’s response with hormones.

One example of the important role hormones play in the body’s regulation is insulin. Insulin allows cells to absorb glucose and helps the body regulate blood sugar levels. When the body does not respond properly to it, it can result in diabetes.

Interestingly, there are also naturally occurring steroids in the body that are also a type of hormone. Based on the body’s receptors, they have been classified into five groups: glucocorticoids, mineralocorticoids, androgens, oestrogens, and progestogens. In the form of corticosteroids, are a major part of lupus treatment, and you can read more about it here.

Hormone Levels and Roles

Hormone levels are essentially consistent from one person to another. A person’s environment, mental state, and health can cause fluctuations

However, the family of hormones that control sexual function, reproduction, and development are different between the sexes. These hormones include:

• estradiol
• estrogen
• testosterone
• progesterone

The same hormone can fill many different roles, triggering different responses depending on the cell. The “sex hormones” are responsible for sex differentiation and reproductive behaviors. They also control a wide range of effects throughout the body. One of these effects is immune system regulation.

It is well-documented that sex-related hormones are related to systemic lupus erythematosus (SLE). Some researchers think that sex hormone levels, and in particular high levels of the hormone estrogen, might be the reason that females have higher rates of SLE and other autoimmune disease.

That said, hormones are not the only factor in SLE. The effects of hormones on the body are too complicated for treating immune system dysfunction with hormone therapy to be considered at this time. Genetics, and sex differences in chromosome expression, is thought to play a greater role than hormone levels in most cases of SLE. 

What is the Relationship Between Hormones and Lupus?

One potential explanation for sex differences and SLE is the varying levels of sex hormones in the body. In particular, the ratio of particular hormones to one another may affect how immune cells develop and mature. And, which immune cells the body gets rid of.

B cells are a class of immune system cell that targets and marks cells for destruction. This happens at “checkpoints” in the body, including before cells are released. This process ensures the eradication of intruders like bacteria and virus-infected cells.

Estrogen and prolactin (sex hormones associated with female reproduction and development) are also involved in protecting B cells from destruction. Higher levels of these hormones can allow more B cells to circulate in the body. Next, the increased level of B cells can lead to an immune response, and may trigger symptoms of lupus.

Estrogen Treatments

Estrogen is known to activate or encourage the activity of T cells. These cells seek out and destroy pathogens that use the body’s own cells to multiply, such as viruses, some bacteria, and tumors. T cells are a major component of lupus-related damage and symptoms, and you can read more about them, here.

When mice were treated with an estrogen antagonist (a type of medication which prevents estrogen from binding to receptors on cells) they had decreased rates of disease and milder symptoms.

One potential explanation for the reason women (females) develop SLE more frequently than men (males) is because they have more estrogen. This can be linked to two causes: 1) higher production levels of estrogen; and 2) higher levels of the natural conversion process of testosterone to estrogen (known as aromatase).

Before converting to estrogen, testosterone first becomes estradiol. A 2003 review on hormones in SLE noted estradiol levels are higher in people with SLE. This difference in estradiol levels was not found in males, though it should be noted that there are not as many large studies on SLE in men. This implies that there is a higher-than-normal conversion of testosterone to estrogen. Plus, it might amplify estrogen’s effect in the body further.

Testosterone Treatments

Testosterone, a male sex hormone, is known to suppress the immune system. Conversely, estrogen and other hormones in the same family push the immune system to produce and react to antibodies, leading to immune responses.

People with lupus may be more sensitive to the estrogen in their bodies due to genetics. This allows it to trigger responses more easily, and potentially allows it to accidentally target the body’s own cells. This is supported by the finding that, if a woman’s ovaries are working sub-optimally and producing low levels of estrogen, she also has a lowered risk of lupus.

Conversely, oral contraceptives, estrogen treatments, and ovulation induction as a part of fertility treatments have been known to trigger flares. When testosterone is administered, it is linked to reduced lupus symptoms.

However, estrogen also influences the development of T-cells in the organ called the thymus. The hormone is involved in activating CD3-T cells and producing cytokines. Notably, estrogen treatment can lower thymus activity, but low levels of estrogen can also lower thymus activity. This implies that normal estrogen activity is needed to keep the thymus and the immune system working at a healthy level. It is also necessary for the destruction and survival of cancer cells, a vital function of the immune system, as well.

What is Renal Replacement Therapy?

Kidneys are vital organs, and damage done to them affects the entire body. Lupus Nephritis (LN) is the term for when inflammation caused by lupus severely damages structures in the kidneys known as nephrons. This kidney damage, which occurs in about 40-60% people with lupus, can lead to a loss of kidney function. Lupus causes other damage to the kidneys as well, including urinary tract infections, but in all cases, kidney damage and potential kidney failure is the source of the most serious symptoms of lupus.

The symptoms of lupus nephritis include fluid retention and swelling, joint pain, muscle pain, fever, high blood pressure, abnormal urine, and even the well-known malar rash of lupus. You can read more about kidney disease and lupus here. 

Lupus nephritis is very serious, but the treatments for it are very effective. 10-30% of people with LN develop end-stage renal disease and kidney failure, which increases their risk of death. As long as they do not enter renal failure, however, people with lupus can live normal, relatively healthy lives.

Renal replacement therapies are used to maintain their health and offset the damage to the body caused by nonfunctional kidneys. Dialysis is a form of renal replacement therapy that helps the body remove waste, salt, and extra water in the bloodstream. It also helps balance potassium and sodium, important chemicals for the body, and helps maintain blood pressure.  You can read more about it here.

Do Flares Happen While on Dialysis?

Yes. Disease flares can occur even while on dialysis, a renal replacement therapy where treatments (including machines) stand in for failing kidneys and filter the blood. It does not appear to reduce flares, though. People who experienced flares were also likely to experience flares under renal replacement therapy. 

In a study on 121 patients with SLE on dialysis (both hemodialysis and peritoneal dialysis) 26.4% of them experienced an SLE flare. Most of these people were treated with cortiosteroids, and 34.3% of people took additional immunosuppressants to combat disease symptoms and flares. Flares included fevers, thrombocytopenia (low platelet count,) and leukopenia (abnormal levels of white blood cells in the bloodstream.) Both of these are clear markers of increased inflammation and are expected in cases of flares.

Flares can occur right after dialysis, possibly caused by the dialysis itself, which does stress the body. Fatigue is a known side effect of hemodialysis though researchers are not entirely sure why.

Reducing Flares on Dialysis

Renal replacement therapies take on the function of the kidneys, removing waste products and maintaining a balance of dissolved materials and fluid in the  bloodstream. Doctors try to minimize reactions to dialysis by having it done in several short, intermittent sessions. This reduces the stress on the body and potential for flare triggering events.  For more severe acute renal failure, continuous dialysis has to be used, which can be harder on the body, but is necessary.

Most people with lupus are not using dialysis to control flares, but to avoid the symptoms of renal failure. Instead, they use immunosuppressive therapy, a key treatment for lupus nephritis, to reduce inflammation and avoid symptom flares.

People with lupus are also advised to drink plenty of water and stay hydrated. This helps to maintain good kidney health and takes some of the pressure off of the kidneys. It also has many other health benefits.

Dialysis is not necessarily forever! 28% of people with lupus nephritis restore enough kidney function to come off of dialysis. Keeping up the treatment is key and will reduce flares. 

Lupus treatments have come to a point where long-term survival and even remission is possible. However, many people don’t respond or find their symptoms reduced by the “gold standard” of lupus treatments for lupus. You can read more about lupus medications here. This includes glucocorticoids  such as prednisone, hydroxychloroquine, immunosuppressives, antimalarials, and certain anticancer drugs. This condition is known as Refractory Lupus. 

While uncommon, there are still many people who struggle with refractory and resistant lupus. In a study looking at 257 patients with systemic lupus erythematosis, about 6.2% of people being treated for lupus had refractory lupus, defined as not having improvement of symptoms even after intensive treatment. 

Lupus nephritis and systemic lupus, and neuromyelitis optica can all develop into refractory lupus. A good diet, good exercise, and a healthy lifestyle that avoids tobacco and alcohol can help, but researchers are developing alternatives to the gold standard medical treatment to relieve the symptoms further. 

Currently, researchers are looking at biologics and other therapies, such as mesencymal stromal cell or hematopoietic therapy, as potential treatments for refractory lupus.

Biologics for Refractory Lupus

Biologics are relatively new therapies that target the specific parts of the immune system that go haywire in lupus. They are produced by laboratory-modified cells to help people take control of their immune system. They are made from modified forms of immune system particles, such as antibodies, that are found naturally in the body. Some biologics mark autoimmune cells for destruction, others block cytokines, the signals that set off the overactive immune system. B cell-targeted therapies in particular have a lot of potential for people with refractory lupus.

Biologics are very precise and effective but also require people to go to the hospital and get an infusion or find a trained medical caretaker to help them with the injections. That makes them something of a burden for the patient.

Rituximab for Refractory Lupus?

Rituximab is one such a promising therapy for reducing symptoms in refractory lupus. Rituximab is a biologic drug that is often perscribed as a chemotherapy to combat non-Hodgkin’s lymphoma (which is a cancer that affects the white blood cells,) and rheumatoid arthritis. 

Researchers looked at 26 papers and compared the results of a total of 300 patients with an average follow up time of 60 weeks. Those who took B-cell ablation with the chimeric anti-CD20 antibody rituximab (RTX) had reduced symptoms of refractory lupus nephritis, and a few even entered remission. 

The side effects are fairly mild and are mostly because the medication is delivered via infusion, which can be stressful for the body. However, these symptoms can include fever, shaking, chills, tiredness, headache, or nausea

A Lupus Warrior’s Takeaway

Most biologics are currently not commonly available, unfortunately: Rituximab, calcineurin inhibitors, and other biologics (such as belimumab) are presently in the midst of some very promising clinical trials. It will take a few years for these trials to conclude and the researchers to have enough data to ensure that the side effects are acceptable.

Nearly 50% of people with lupus experience some sort of brain or nervous system-related symptom related to their lupus. Despite how common it is, and despite the medical world being aware of these symptoms for over a century. Neurological symptoms are the least talked about and least understood product of lupus.

Neuropsychiatric symptoms can include:

• Headaches
• Depression
• Anxiety
• Brain Fog
• Fatigue
• Sensory issues (hearing, sight, smell)
• Balance issues (vertigo)
• Seizures

And even the severe breaks with reality known as psychosis. You can read more about the neuropsychiatric symptoms of lupus, here.

These neuropsychiatric symptoms, when present, usually show up along with other Systemic Lupus Erythematosus (SLE) symptoms at onset or diagnosis of the disease. They don’t generally have characteristics unique to SLE. Which makes it unclear whether the cause is SLE or if it is a comorbid or co-existing issue. Because of this ambiguity, it is difficult to properly diagnose and treat neuropsychiatric symptoms of lupus as a part of lupus. Plus, everyone’s lupus is different, and not everyone has these mental symptoms, or may only have a few. 

Although it is sometimes referred to as “Neuropsychiatric Lupus Erythematosus,” there do not seem to be patterns of symptoms that could indicate a specific neuropsychiatric form of lupus, like lupus nephritis or cutaneous lupus. However, neuropsychiatric involvement is clearly linked to SLE.

Although thinking about the neuropsychiatric symptoms of lupus can be frightening, it is important to know that they can happen. Frequently misdiagnosed or brushed aside in favor of the more physical symptoms of lupus. Many people with neuropsychiatric lupus feel like they are “crazy,” and have to endure this alone. Instead, there are many people experiencing the same or similar symptoms. And we know enough about it to work through it together.

What are the Risk Factors for Neuropsychiatric Lupus?

Although everyone’s lupus experience is different, people with more severe lupus – as measured by the SLEDAI scale – had a higher rate of neuropsychiatric symptoms. In a paper looking at 1121 registered patients, where 429 (38.3%) of these people had neuropsychiatric issues, researchers found that tests revealed a link between severe lupus symptoms, positive antiphospholipid antibody tests, and no anti-dsDNA antibodies at diagnosis. ANA tests were not very good at detecting neuropsychiatric lupus, however.

Interestingly, these people also had fewer years of education under their belt. Which is also linked to worse economic situations, higher levels of stress, and worse health overall.

How does Neuropsychiatric Lupus Happen?

Part of the reason for brain and nervous system symptoms in SLE is the effect of lupus on the circulatory system. Patients with primary neuropsychiatric lupus (very severe cases) were found via magnetic resonance imaging (MRI and DS_MRI) to have lower blood flow in their brains, compared to people with secondary (less severe) neuropsychiatric symptoms and people without reported brain symptoms. When blood flow is restricted or impeded by the cardiovascular symptoms of lupus. The brain doesn’t get enough oxygen to function at its full capacity. This can lead to fatigue, headaches, sensory issues, and even the seizures sometimes seen with lupus.

SLE can also affect the brain directly. Normally, the brain and spine are protected by a barrier that restricts the entry of immune system cells. For some people, this barrier – the blood brain barrier – is more permeable and lets these cells through.

In people with lupus who have permeable or damaged blood brain barriers. Autoimmune cells (the immune system cells involved with lupus) can enter the brain and cause damage or inflammation. Resulting in these neuropsychiatric symptoms.

Grey matter and white matter, types of neurons found in the brain, are, indeed, different in people with neuropsychiatric lupus. Other neurological structures in the brain show damage or weakening – atrophy. The amount of this damage is directly related to the severity of lupus symptoms and to how quickly the neuropsychiatric symptoms show up after diagnosis.

However, a review of the brain structures of mice modeling (imitating) neuropsychiatric lupus did not reveal damage to the blood brain barrier.  Many cases of neuropsychiatric lupus may be side effects of damage or inflammation throughout the body. Or, the symptoms may actually come from a combination of factors including other organ and brain issues caused by SLE – it isn’t easy to determine.

And, in the end, most people with lupus are, understandably, more concerned with managing the symptoms affecting their immediate lives, over determining the root causes.

Treating Neuropsychiatric Lupus

No therapies specific to neuropsychiatric lupus exist, and few therapies have been tested on people with severe neuropsychiatric symptoms specifically. In fact, they are often excluded from clinical trials due to complications.

Fortunately, there is evidence that controlling the symptoms through therapy and medications and reducing lupus activity through normal lupus medications is effective in treating the neuropsychiatric aspect of SLE. Reducing inflammation in particular helps the brain and the blood brain barrier to heal and become less permeable.

However, it should be noted that antimalarial medications, which are often used for lupus, seem to have an additional protective effect on the brain.

Overall, when caught early, typically due to good communication between patient and treatment team. The prognosis for neuropsychiatric SLE can be good.

T cells are a special type of white blood cell that come from the thymus known as a lymphocyte. White blood cells work to protect the body. They seek-out and destroy threats to the body. In particular, T cells specialize in attacking pathogens that use the body’s own cells to multiply.

Common targets of T cells include:

• viruses
• bacteria
• tumors

How do T cells properly identify, flag, and destroy the correct cells? Cells that have been damaged in some way have a distinct chemistry and a protein signal on the other membrane. T cells

However, T cells will sometimes make a mistake and attack the body’s own healthy cells. Usually, other immune system cells reign them in and destroy these faulty T cells before they cause problems. This process is known as regulation. 

But, for people with autoimmune diseases, including systemic lupus erythematosus (SLE) and lupus nephritis (LN), the regulatory cells fail. This allows T cells to go rogue and attack the body, causing the symptoms of lupus.

T cells vs B cells

The immune system actually has many different types of cells that work to protect the body. Each one specializes in a specific type of defense. Two types of cells called “Lymphocytes” have been linked directly to lupus:

B-Cells, are involved with investigating and recognizing invaders (known as antigens). When they identify a threat, they produce B-cell activating factor, a known sign of lupus.

T-Cells are more like the strike teams because they mark and kill problem cells. They also cause B cells to transform into plasma cells, which attack invaders with enzymes and toxins. Furthermore, they possess another important role: clean up. 

T cells come in several forms:

• Helper
• Regulatory
• Cytotoxic
• Memory

Helper T Cells

Helper T Cells are the scouts. They mark cells to be destroyed and trigger the immune responses of other cells. Helpers are incredibly important. When problems occur with these T cells, invaders slip through unnoticed and the body can’t fight disease effectively. 

As a safeguard against triggering the immune response unnecessarily and to keep the response targeted, Helpers need to receive two signals in order to activate the immune response.

1. The first signal comes from an attached antigen
2. The second comes from an activated cell immune system cell
   • Once activated, Helpers are part of the chain reaction known as the immune response

Once the antigen has been eliminated and the first signal goes away, the Helpers deactivate. This stops the chain reaction and the immune response dies down. 

When Helper T cells react to auto-antigens (proteins produced by your own cells), they are actually working properly. The problems that result in lupus occur elsewhere in the system, where Helpers that would react to auto antigens are usually destroyed. You can read more about ANA, a type of auto-antigen, here.

Regulatory T Cells

The thymus in most healthy individuals actually destroys autoimmune lymphocytes. This is extra important for T cells because there is a thin line between attacking rogue cells and healthy cells. T cells also have their own policing force: Regulatory T Cells or Tregs (or Regulators.)

Tregs are still somewhat of a mystery. But, most researchers believe that they act as an added level of security, limiting the immune response and also specifically preventing T cells from targeting the body’s own healthy cells. In fact, they specifically target and hold back T cells that are targeting the body’s antigens.

Or, rather, they are supposed to. Tor people with lupus and other autoimmune diseases, there is some evidence that their Tregs are faulty (either due to damage or due to genetic factors) and unable to do their job. Not only are they not able to stop T cells from targeting healthy cells, but they are also permitting a constant (or flaring) state of inflammation. That’s the bad news.

The good news is that T regs can be targeted with medications with promising effects. You can read more about genetic factors related to lupus here.

Cytoxic T Cells

Cytotoxic T Cells are also known as “Killer T Cells” and they do exactly that. They are extremely efficient assassins, directly killing infected or compromised cells. They do this through various means:

• Cytokine secretion
  • The killer cell spews poison at the infected or cancerous cell. It breaks down viruses and prevents the cell from multiplying or performing any of the functions that it needs to do to survive.
• Cytotoxic Granules
  • These tiny beads are made up of enzymes that break into the cell and break down the virus’s production factories in the cell. They also trigger the cell’s self-destruct sequence, also known as apoptosis or cell death. 
• Fas/FasL Interactions
  • Cytotoxic T cells have a protein called FasL on their surface. It binds to the Fas receptor on the target cell. This causes changes in the cell’s membrane that pulls several signaling proteins together, triggering apoptosis. 

Interestingly, the Killer T cells are very good at only attacking the cells that they are supposed to. All of these methods are targeted and minimize damage to cells nearby.

Killer T cells are also involved in cleanup. Once no more compromised cells remain, they attack each other (usually through the Fas/FasL method). All Killer and Helper cells that were involved in the infection are disposed of. This stops the inflammation response and prevents healthy cells from being attacked by rogue Killer T cells. 

Again, in autoimmune diseases, this isn’t the issue. The problem arises because the regulatory T cells don’t stop the Helper cells from flagging more targets.

There is one type of T cell that the cytotoxic T cells do not kill: the memory T cells. 

Memory T Cells

Memory T cells, as the name implies, are the immune systems “memory.” They allow the body to recognize antigens that it has encountered before.

They “remember” antigens they have encountered previously by having a specific receptor on their exterior. If they find an antigen they recognize, they set off the immune response faster than non-memory T cells (also known as “naive” T cells). Memory T cells let the body protect itself quickly from diseases that once caused it problems. In fact, memory T cells and other immune memory cells allow vaccines to work – vaccines work like target practice that let the body create the appropriate memory cells.

The problem arises when the Memory T cells remember antigens that are harmless (as in allergies) or auto antigens (as in autoimmune disease.)

T Cells and Lupus

Research has made it clear that there is a link between systemic lupus erythematosus and T cells, particularly faulty regulatory T cells. Poorly regulated T cells are known to attack organs and neurons throughout the body. This causes the many symptoms of lupus and other autoimmune diseases, (including brain fog).

 According to LupusResearch.org, the more we understand about how these T cells work, the closer we come to harnessing T cells in treating lupus and other autoimmune diseases. Studies have shown benefits of altering T cells through treatment with CAR (Chimeric Antigen Receptor) therapy.

This therapy involves T cells (called CAR-T cells) that have been genetically engineered to produce receptors for the antigens that self-targeting cells produce. In this case, the B cells responsible for sending the T cells into a frenzy against the body’s healthy cells. It holds a lot of promise. In studies in mice, CAR-T cells had a long lasting effect, and even made memory T cells after they were no longer in the body. This means that the T cells flagged and killed off the problematic cells on their own. The mice even appeared to enter disease remission! 

One of the advantages of this kind of approach is that it could be very specific to lupus. Plus, it does not have some of the other risks associated with immune system suppressing drugs.

Current treatment status

Recently, the main focus of clinical trials involving CAR-T cells is attacking cancers, particularly blood cancers. Use of CAR-T for lupus is fairly new, but researchers are very excited about it’s potential.

10% – 20% of cases of systemic lupus erythematosus (SLE) begin before age 20 and sometimes can start as early as age 5. Early symptoms are nearly identical to those experienced by adults with SLE, or drug-induced lupus (DIL). However childhood-onset lupus, also known as juvenile-onset lupus erythematosus (JSLE), often has more severe symptoms. The 5,000 – 1,000 children and teens fighting the disease are more likely to suffer from lasting kidney, brain, and damage to other organs and to have life-long consequences.

What are the symptoms of childhood lupus?

Childhood lupus is very similar to SLE and has many of the same symptoms, including:

• pain
• itching
• weight gain
• oral ulcers
• fatigue
• gastrointestinal symptoms including stomach pain
• cutaneous lupus
• lupus nephritis

In addition to these traditional indications of lupus, children are more prone to brain and nervous system-related symptoms than adults. These symptoms can include:

• seizures
• loss of vision
• higher risks of depression and anxiety
• hemiplegia (on one side) or diplegia (equally on both sides)
  • Sides of the body are paralyzed, stiff, or numb
  • Caused by lupus attacking the child’s growing bloodstream and restricting blood flow to the brain
• spasms
  • similar to cerebral palsy or a stroke

Fortunately, children with lupus do not typically suffer from intellectual disabilities, though they may require some level of special accommodations.

As with other forms of lupus, girls with a family history of lupus and people who are of African, Latin American, Asian, or Native American/First Nations descent are more likely to develop childhood lupus. While it is much less common for boys to develop childhood lupus, they experience more severe symptoms and a higher risk of organ damage.

What causes childhood lupus?

As with SLE, the causes of childhood lupus are not fully understood. It may come from problems with how the body produces and regulates certain immune system cells called cytokines.

Most researchers think that stress, hormone changes, pollutants, infections, and other outside factors may lead to childhood lupus. There is also a genetic component, and a family history of lupus does increase the risks.

Drug-induced lupus is also a possibility for children.

Why is childhood lupus more severe?

Researchers are not sure why childhood lupus presents with more severe symptomatology. It may be more aggressive with a younger immune system or have a larger effect on the small and still-developing organs of children. It may also react to changing hormones as these children go through puberty.

Childhood lupus may also appear more severe because it is typically caught only when the disease has progressed and caused notable damage. Because lupus is so rare in children, doctors will often attribute the symptoms to something more likely, including stress, puberty, sports-related injury, dehydration, poor nutrition or eating habits, or depression and anxiety. They will also typically treat these symptoms first. It is only when there is serious organ damage, especially kidney involvement, that doctors will look towards lupus as a possibility and perform the appropriate blood and urine tests.

Another factor in the damage that childhood lupus causes are the medications itself. Because the child’s lupus symptoms will often be very severe, they will generally be prescribed high doses of medications such as corticosteroids to bring their lupus back down. These high doses work well to bring lupus under control, but they come with serious side effects, can interfere with growth & development, and can leave lasting damage.

Though alternative treatments are in development, the current medication regimen will help stave off the kidney damage and vasculitis, which are what cause the most problems for children with lupus.

Treating childhood lupus

Medications for childhood lupus are the same as those for SLE, such as immunosuppressants. Initially, children with lupus are given higher doses to bring their lupus down. After their symptoms have subsided, they may be tapered back to a lower dose appropriate for their age and size.

Childhood lupus is often treated with corticosteroids. However, new treatments have also become available recently, including IV Benlysta and IV CYTX therapy. These are not in wide use but show promise for treating young Lupus Warriors.

Regular exercise and vitamin D supplements help children build strong bones and can help make up for lack of sun exposure related to photosensitivity.

It is important for children with lupus to have a sturdy support network of doctors, psychiatrists, and education professionals to help them deal with the challenges of lupus. This helps them both grow up resilient and be able to handle their disease. Transitioning them from childhood care to adult care is also vital. Their lupus treatment team will be able to help.

Outcomes of childhood lupus

Depending on the level of kidney damage, children with lupus may need dialysis for the rest of their lives. Other organ involvement can lead to a lot of hospital visits as well. Though they have the highest morbidity and mortality of all the forms of lupus, new advances in medicine mean that children with lupus can thrive and grow up to be happy, productive adults.

Sleep is very important for growth, learning, and healing, making it vital for children with lupus to get enough sleep. Find some strategies to do so here.

Here are a few tips from experienced Lupus Warriors that may be helpful for children with lupus and their parents.

Dermatologists identify three main types of Cutaneous lupus erythematosus (CLE):

1. Acute cutaneous lupus
   • Defining characteristic: butterfly rash across the nose and cheeks
2. Subacute cutaneous lupus
   • Defining characteristic: skin lesions which come in two types
     1. Papulosquamous lesions (Red, scaly patches that may resemble pimples)
     2. Annular lesions (Flat, pink circles with a red exterior. Rarely appear on the face)
3. Chronic cutaneous lupus (also known as discoid lupus)
   • Thick, raised patches that are often red and flakey
   • Commonly appear on the face, ears, neck, scalp, and hands

Increases in cutaneous lupus and SLE symptoms may be a reaction to medications, the result of a viral infection, or exposure to intense sunlight.

Fatigue and CLE

According to a recent NCBI study published in the British Journal of Dermatology, Lupus Warriors with CLE experience considerable amounts of fatigue. The researchers used a short survey of vitality to measure the energy levels of people with systemic lupus, cutaneous lupus, and other autoimmune skin and blistering diseases. All of the people in the study experienced increased levels of fatigue when compared to people that were not living with these conditions. This shows the impact these conditions have on the immune system, and the physical toll this places on the body.

Quality of Life with CLE

In healthcare, quality of life is an attempt to measure how a condition and treatment plan are impacting a person on an individual level. Because of this, two people with similar treatment plans can have dramatically different quality of life scores based on preferences, expectations, and needs.

For people with lupus, reporting a high quality of life often means that a person is able to partake in the activities that they enjoy. It may mean being able to go to work, go out with friends, pursue a hobby, or even a simple pleasure like cooking a meal for yourself. Fatigue makes every activity difficult — even stress management techniques that keep symptom flares at bay — and can affect your emotional state. Fatigue can make the management of other lupus symptoms more difficult.

Also, because cutaneous lupus lesions may be visible to other people, they can be a source of additional stress. While it’s easier said than done, try to ignore rude comments — both intentional and accidental.

Managing stress is an important part of life with cutaneous lupus. It may be helpful to speak with a mental health professional to learn strategies for managing stress, anger, or other powerful feelings.

How do you Deal with Fatigue from CLE?

Like other forms of lupus, there is no cure for cutaneous lupus. The symptoms can be treated with medical lotions and the usual lupus medications planned for you by your treatment team. The medications might help with your fatigue, but people with lupus will have bad days sometimes and will feel drained and lethargic.

Another useful strategy for minimizing lupus fatigue is to limit ultraviolet (UV) light exposure. UV light comes from many sources including the sun, lightbulbs, and even coals in a fire. In studies, up to 73% of people reported being photosensitive, meaning that UV light exacerbated lupus.

Fatigue can be managed, in great part, taking care of yourself and your general health. Rest, eat a balanced diet, take your medications, and pace out activities so as to not overexert yourself.

In our article on fatigue, we discuss lupus-related fatigue at-length and several other methods for reducing your fatigue and risks of flare-ups: check it out here.

Want more information about CLE?

LupusCorner has a few articles about the fatigue severity scale (FSS), a way for you to track your fatigue and figure out how you can overcome it. Tracking lupus fatigue may help identify factors that may help or exacerbate your symptoms — check out the article here and start tracking your fatigue symptoms.

Additionally, there are topical remedies (lotions, creams, etc.) for the management of cutaneous lupus. Learn more about several topical remedies.