The pancreas is very important, since it produces the enzymes that the body uses to breakdown proteins, sugars, fats, and starches into a form that the body’s cells can use for energy. It also produces hormones that regulate digestion, blood sugar levels, and whether we feel hungry or full. This makes it vital to maintaining a healthy weight and to a functional metabolism in general. The pancreas also holds channels that connect the liver to the rest of the digestive system. Bile from the liver drains through the pancreas into the first part of the small intestine, called the duodenum, where it begins to digest the broken-down food. 

The hormones that the pancreas produces include:

• Insulin – Insulin signals the body to store sugar in the cells instead of letting it freely flow in the bloodstream. 
• Glucagon – Glucagon signals the body to release stored sugars into the bloodstream to supply energy 
• Gastrin and Amylin – Gastrin and Amylin signals the stomach to produce gastric acid or continue digestion.

When lupus causes inflammation to the pancreas, these hormones might not be produced in adequate amounts.

What is the Digestive System?

Systemic lupus erythematosus (SLE) can have an effect on most organs in the body, including the organs of the digestive system. You can read more about the effects of lupus on the microbes that live in the digestive system here. This also includes the pancreas.

The digestive system is a series of organs that are responsible for taking in and processing food, extracting nutrients, absorbing water, and breaking down complicated molecules, including toxins. The digestive system also regulates the flow of nutrients, vitamins, minerals, and sugars in the bloodstream, keeping the levels balanced and the cells of the body healthy. The dietary intake of each individual person has a large effect on the digestive system. You can read more about important nutrients like Omega-3 fatty acids and lupus here. 

The digestive system works like a disassembly line, breaking down food into molecules that the body can use. 

First, food and water is taken in through the mouth, crushed into smaller pieces by teeth, moved around by the tongue, partially digested by saliva, before being swallowed to the esophagus. 

From there, it is broken down in the stomach by gastric acid and enzymes, before entering the small intestine where it is digested and nutrients are absorbed into the body through the membrane. 

In the large intestine, whatever material remains is collected, drained of any residual water, and finally excreted from the body as feces. 

The liver, pancreas, gallbladder, and other organs support this system with regulatory hormones, additional enzymes, and areas that process certain molecules. The kidneys are not considered part of the digestive system, but they do support it by filtering out waste products from the bloodstream.

Pancreatitis and Lupus

Pancreas-related problems are a rare symptom of autoimmune diseases such as lupus. Specifically, Lupus causes inflammation and swelling as it attacks the cells of the pancreas, a condition known as pancreatitis. Pancreatitis interferes with how the organ produces enzymes and hormones, and blocking the ducts that carry bile from the liver to the intestines. 

The symptoms of pancreatitis includes abdominal pain (including pain that feels like it’s at your back,) nausea, vomiting, and fever. 

Other causes of pancreatitis, including gallstones, alcohol use, genetic disorders, infections, or injuries to the abdomen, are much more common. It is estimated that only about 2-4% of cases of pancreatitis are caused by autoimmune disease, according to the National Library of Medicine. However, lupus medications such as immunosuppressants and the stress on the body from lupus can make the pancreas more vulnerable to infection and damage. You can read more about lupus medications here. 

Diabetes and Lupus

Diabetes is one of the major issues that can come about when the pancreas is damaged. Diabetes is a condition where the body doesn’t receive enough of a signal from the hormone insulin to properly modulate blood sugar levels in the body. When a carbohydrate or sugar-rich meal is digested, this lack of insulin causes a surge of sugar in the blood that can lead to problems in the kidney, heart, brain, and other organs. Diabetes can occur when the body stops reacting to insulin, but also if the pancreas is damaged and doesn’t produce enough insulin. 

For many reasons, including damage to the pancreas, people with SLE have a very high risk of developing diabetes. You can read more about diabetes and lupus here. Having both diabetes and SLE causes them to ‘feed into’ each other, one condition making the other worse and so on. It is important to treat both diabetes and SLE individually to get both under control.

Treating Pancreatic Issues and Lupus

People with lupus who have diabetes have to take regular insulin injections to maintain proper blood sugar. 

Treatment with lupus medications, such as corticosteroids or azathioprine, reduces inflammation and, as a consequence, reduces damage to the pancreas as well as other organs. 

However, some of the medications for lupus can have poor interactions with diabetes, with corticosteroids causing weight gain and prednisone leading to worse insulin resistance – and, as a consequence, worse diabetes! It is important to make sure that your medicine regimen is safe for both conditions.

A Lupus Warrior’s Takeaway

If you start feeling symptoms of pancreatitis, it is worth getting checked out as soon as possible. This is because not only can the issues have serious consequences, but the symptoms of pancreatitis can also be symptoms of more urgent and serious conditions. Leaving possible pancreatitis for too long can, potentially lead to complications like scarring as the damage gets worse.

In other words, get checked out if you can, and make sure that your treatment team is aware of any diabetes or potential pancreas issues.

What are the risks of in vitro fertilization for people with lupus?

IVF and lupus have a complicated relationship.

Hormonal changes in the body are notorious for causing problems for people with autoimmune diseases like systemic lupus (SLE.) This is why some people with lupus are careful about what contraceptives they use. Spikes in hormone levels from, say, a birth control pill may induce symptom flares. Thrombosis (life-threatening blood clots) has also been reported. You can read more about clot risks for people with lupus here.

IVF has been researched for the risk for causing disease flares because of its use of hormone therapy.

37 women with lupus (SLE) or antiphospholipid syndrome participated in a 2017 study published in the Journal of Rheumatology. 97 IVF procedures occurred during the study. The procedures resulted in 27 pregnancies. From those pregnancies, there were:

• 23 live births from 26 neonates (3 twin pregnancies)
• 2 miscarriages
• 2 terminations for trisomy 13 and 21

6 spontaneous pregnancies occurred during follow-up. In total, 26 of the 37 women delivered at least 1 healthy child.

 

Exploring the risks of IVF

The researchers noted that “complications occurred in or after 8 IVF cycles.” During the pregnancies, 4 people experienced lupus flares and 4 others experienced major thrombotic events. Also reported were 6 mild SLE and 1 deep-vein thrombosis. 

37% of the infants were pre-term (early) births, 15% had HELLP, and 7% had preeclampsia, a serious condition that can hurt both mother and child.

Rheumatology Advisor highlighted two challenges of this study:

• A relatively small sample size
• 15 procedures used Oocyte donation cycles. This type of procedure involved a milder stimulation protocol

A separate 2000 study found that neonatal lupus, a form of lupus that appears in newborns, was also sometimes reported.

IVF, pregnancy, & lupus

There is always a risk of complications with pregnancy, and lupus can add to those risks. Check out this article on pregnancy and lupus to learn more about those risks.

IVF success rates are good, but people with lupus have a slightly higher risk of complications according to the research. However, people with lupus can reduce this risk by sticking to their treatment plan.

In a study published in 2013, the researchers ruled that several symptom flares were not linked to IVF therapy at all. Failure to adhere to medication treatment plans and prescribed diets caused the symptom flares and thrombosis, according to the clinicians and researchers.

If you choose to undergo IVF, be sure to add all clinicians to your lupus and health treatment team. It will be important to continue consistently monitoring your health. You should also discuss any personal risks with your lupus treatment team.

How Does Vision Work?

Eyes are specialized organs that detect light and transmit that information to the brain. The brain then interprets the light signals into what we perceive as vision. Humans generally have two eyes, which is important to how we determine depth and dimension. While we can still see with one eye, depth and distance become harder to judge, which can make it dangerous to perform activities that require quick reactions and precision, including driving.

Step 1: Light Enters the Eye

The human eye is a complex organ that works a lot like an analog camera – most of the eye is opaque, protected from dirt and scratches by an outer layer called the cornea, which is kept clean and in good condition by constant moisturizing through tears. The opaqueness of the eye means that extra light is not let in. 

The sclera is the protective outer coating of the eye, which in humans is white and full of blood vessels, and inside the eye, there is a clear fluid called vitreous humor which helps to bend the light and allows the eye to maintain its shape through pressure. This is important, because if the eye shape is off, it can affect how well the eye can focus on details. This is a common cause of near- or far-sightedness.

The first step of vision starts with light travelling through the cornea, a clear, thick, curved area in the front of the eye that bends and focuses the light. The light then enters the eye through an opening called the pupil, and then passes through the lens. The pupil, cornea, and lens allow the eye to focus on details, adapt to different levels of light in the environment, and protect the sensitive cells at the back of the eye from damage. The amount of light let in depends on the environment – in dim light conditions, the pupil is widened (or dilates) to let in more light, while in bright light the pupil contracts to a tiny dot. It is also affected by stress, fear, and other emotions – energetic emotions such as excitement, fear, aggression, or arousal cause dilation, while calmer emotions are associated with constricted, smaller pupils. 

Step 2: Light Receptors in the Retina React

The cells at the back of the eye are special cells that react in response to certain wavelengths of light. There are millions of these cells in the back of the eye, arranged in a two-layered sheet called the retina. The retina consists of color-detecting receptors called cones and light detecting receptors called rods. There are three types of cones, detecting low energy red light, medium energy green light, and high energy blue light. These receptors are triggered in different amounts, which we then interpret as the rainbow of colors around us. Other receptors called ‘rods’ are very good at detecting light and dark. They are able to detect dim light and are responsible for allowing us to see in the dark – though night vision is generally without color.

Step 3: Information is Transferred to the Brain and Processed

These cells transmit their information through a nerve bundle called the optic nerve, the connection from the eye to the visual cortex in the brain. The optic nerve is not the longest nerve in the body, but it still has a long way to carry its information – all the way to a region called the “occipital lobe” which is all the way in the back of the head! There, the information is processed, and our brains categorize what we see into shapes, types of objects, and other classifications. This is both how we see, and how we understand what we see. 

This means that sight is a complicated teamwork between the structures of the eyes, the optic nerve, and the brain. This ‘teamwork’ can degrade with age but also with extreme exposure to powerful light (including long term use of bright screens) and head trauma.

Inflammation caused by lupus can affect any stage of the vision process. When serious, it can cause loss of vision or even blindness if not caught and treated early.

Other Eye Symptoms of Lupus

Although the results are the same – pain, deterioration of vision, or loss of vision – and inflammation is generally the cause, the actual source of the symptoms can vary. 

There are many ways that lupus effects the eyes. Lupus can cause inflammation and damage to the outer layer of the eye (scleritis), the muscles that move the eyes (including the iris itself,) and the iris. It can also affect the nerves that control these muscles. Cutaneous lupus around the skin near the eyes, the eyelid, or the face in general can also effect vision. Keratitis, or inflammation of the cornea, is more direct. It affects the cornea, which is the layer that protects the eye. Inflammation causes the area to become irritated and sensitive, blurring vision both from the inflammation and the increased production of tears.

Lupus retinopathy and dry eye the most common eye related symptoms of lupus.  Retinopathy is when the retina, the back of the eye with all of the receptors, is damaged. The receptor cells die, reducing the amount of light and detail that the eye can detect.

Dry eye is exactly what it sounds like. Lupus that affects the tear glands and tear ducts can also interfere with vision as the eye dries out. Dry eye is a common symptom of lupus and certain other autoimmune diseases such as Sjogren’s Disease. The lack of tears means that the eye can’t clean out dirt and debris as effectively, allowing the eye to be scratched and damaged more easily. The loss of the tear layer also exposes the eye to damage from drying out and to bacteria that are better able to get a foothold in the eye and cause damage of their own. Rarely, lupus affects the eyeball itself.

However, the eyes do have a level of protection from the immune system, much like the brain and reproductive organs. So, the most common cause of vision loss is restricted blood flow due to vasculitis. Vasculitis is when inflammation from lupus constricts the blood vessels providing various parts of the vision system with oxygen. This starves the organ of vital oxygen and makes them unable to do their jobs – even potentially permanently damaging them. The constriction of blood vessels due to lupus is called vasculitis. 

The optic nerve and brain can both also be affected by lupus inflammation or vasculitis, effecting how the information from the eye is transmitted or processed.

Lupus Medications and the Eye

Lupus medications, including steroids and antimalarials are a major part of lupus treatment, but also are known to cause damage to the retina. Steroid medications raise the pressure in the eye and can also lead to cataracts. People on steroid medications, especially hydroxychloroquine (HCQ) should get their eyes checked often.

Alternate treatment options are sometimes used when doctors feel that it’s appropriate.  If someone with lupus starts getting eye-related symptoms, then they should talk to their treatment team about changing their medications to try to avoid the damage.

Other lupus medications are effective though, as are treatments that reduce inflammation overall, such as diet, exercise, and alternative therapies. Other medications for lupus, including the chemotherapy drugs bevacizumab, rituximab, and belimumab, are also useful. Anticoagulants (such as blood thinners) can directly prevent the vasculitis from causing more damage.

A Lupus Warrior’s Takeaway

If you start noticing eye-related symptoms, including sensitivity to light, it’s a good idea to get checked as soon as possible. Case studies have shown that, the sooner these problems are caught, the more likely it is that you can avoid permanent vision loss. 

You can read more about the eye and lupus here.

What is Hidradenitis Suppurativa?

Hidradenitis Suppurativa (also known as HS) is an often-painful chronic skin condition that involves tender fluid-filled, blister- or boil-like lumps on the skin. Usually, HS occurs where skin is in contact with skin, such as the armpits, breasts, and groin, possibly due to irritation of the skin or moisture in those areas. Other symptoms include painful acne, itching, excessive sweating, and sensitive nodules in the skin. 

It can also evolve into blackheads, scarring, larger fluid-filled lumps, the lumps can break open like a blister and leak fluid and can heal improperly. 4.1% of people in the United States have HS. HS is not itself an autoimmune disease, and it can occur on its own, but often shows up alongside autoimmune diseases like lupus. It also might encourage inflammation, which increases the symptoms of inflammatory and autoimmune diseases.

However, at present, there is no direct link between HS and lupus. 

Hidradenitis Suppurativa Treatment and Lupus

HS is treated with medications like corticosteroids, medical procedures like deroofing, skin surgeries, and lancing, and even hair removal. You can read more about it, here. 

HS and SLE are treated with very different medications, and some of the medications for HS have been indicated in cases of Drug-induced lupus (DLE.) DLE is a form of lupus that occurs in response to the side effects of medications. DLE can sometimes be recovered from when medications are changed, but can also become a chronic illness. Treating lupus can sometimes reduce HS symptoms, which means that either the HS symptoms were actually lupus, or the reduced lupus-related stress on the body helped ease the symptoms of HS.

Much of the treatment for HS involves taking care of the open wounds that result from the lumps (or boils.) Antiseptic, vinegar, and wound dressing are key to avoiding infection.

Other treatments for HS include: Ice packs applied to the affected areas, or lidocaine, can reduce the pain and itchiness of that particular area of skin.

Calcineurin is a hardworking molecule found in everything from plants to insects to humans. Calcineurin gets its name from the metal calcium (Ca on the periodic table) which is one of the materials that is used to build this molecule. Calcineurin inhibitors prevent calcineurin from doing its job and are used as immunosuppressant medications to treat lupus. Calcineurin inhibitors are under investigation for people with kidney focused lupus (lupus nephritis) that does not respond to other treatments. 

Is this a good idea? The answer is complicated.

What is Calcineurin?

Calcineurin is a phosphatase, a molecule that breaks apart and deactivates certain molecules that contain phosphate. In this case, it affects serine and threonine, which are involved in many different functions throughout the body. The nervous system is one area where calcineurin is involved, the kidneys are another. However, one of calcineurin’s other functions is activating T-cells, the immune system cells in the body produced in the thymus. 

T-cells are one of the immune system cells involved in autoimmune diseases such as lupus, which makes controlling calcineurin an important avenue for treating lupus. In many autoimmune diseases, some research indicates that calcineurin might not be doing its job properly. People with lupus might benefit from calcineurin being less active.

What is a Calcineurin Inhibitor?

Calcineurin inhibitors are used to treat autoimmune disorders such as lupus. Calcineurin inhibitors, also known as CNIs, include cyclosporine, tacrolimus, pimecrolimus, and voclosporin. 

Calcineurin inhibitors are highly fat soluble, which makes them able to easily reach organs that have a high fat content or insulation, such as the lymph nodes and kidneys. In general, calcineurin inhibitors work by taking up space on receptors on cells that would usually accept calcineurin, blocking the molecule from the receptor. Fewer calcineurin molecules are able to signal to the cells that they need to produce cytokines and activate t-cells. Tacrolimus and pimecrolimus work this way. Voclosprin, also known as “Lupkynis” does the same thing, but is actually more like Cyclosporin:  

Cyclosporin actually does the opposite – it attaches itself to cyclosporin, holding it back from attaching to receptors.

Most CNIs are taken orally (by mouth,) but some can be given topically (on the skin.) Either way, this reduces inflammation and the symptoms of autoimmune diseases such as lupus. 

Generally, tacrolimus and cyclosporine are used for lupus, and CNIs are taken alongside other treatments. Tacrolimus is a calcineurin inhibitor that doesn’t constrict the blood vessels as much as the others and doesn’t encourage scarring in injured organs which makes Tacrolimus a potential alternative for people who already have kidney damage. Other CNIs can also be used for lupus: Voclosporin seems to be especially beneficial for patients who are already on immunosuppressants. 

CNIs are not excreted in breast milk, so they are sometimes used to manage LN during that time. However, overall, CNIs are not usually recommended for use in pregnancy. A modified version of cyclosporin is approved for use in children. Lower doses might be needed for older kidney transplant patients because of the possibility that it can exacerbate kidney damage. 

Calcineurin and the Kidneys

Calcineurin is also involved in proper function of the membranes in the kidneys that filter the bloodstream. These membranes are on special cells called podocytes which act as the barrier between the bloodstream and the kidneys. When there is too much calcineurin, or when it isn’t functioning correctly in the body, then the podocytes can start to weaken and collapse. This may potentially be one of the main ways that the kidneys are damaged in lupus, both SLE and LN.

Calcineurin Inhibitor Side Effects

Patients on CNIs generally start with a higher dose (0.1mg/kg per day) and are gradually reduced to a lower chronic dose (dependent on patient.) This lower dose is generally tolerated well, but CNIs are stored in fat and will stay in the system for a significant amount of time.

On higher doses, CNIs can exacerbate kidney damage – this is the main reason why doctors are cautious before prescribing CNIs for lupus. However, there is some evidence that implies that the reduced kidney functionality might be from lupus damage instead. Doctors are still cautious, however, but do use CNIs when there is both a lower risk of kidney damage in the patient and a need for less immunosuppression for some reason.

Neurological and sensory symptoms can be exacerbated, and hypertension can be a potential side effect. Diabetes is also a side effect, mainly of tacrolimus. Gastrointestinal symptoms have also been reported. Tacrolimus doesn’t appear to have effects on fertility or pregnancy, though, fortunately.

Additionally, a 2024 study using the Lupus Registry of Nationwide Institutions (LUNA)  found that calcineurin inhibitors did not increase the risk of cancer in people with lupus.

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. 

Humans in general are very visual – we pay a lot of attention to color, patterns, and movement. Most of society is designed around sight-based communication, including traffic signals, signboards, advertisements, books, and art. While there are accommodations for people who have their sight impaired, such as braille and screen readers, these accommodations are not always enough to navigate a world so targeted to the visual. When lupus interferes with sight, it has a huge effect on everyday life and can be psychologically frustrating for people with lupus. 

Vision problems are sometimes considered an early symptom Systemic Lupus Erythematosus (SLE) have eye-related symptoms. If patients have other telltale symptoms (for example, the malar rash and severe fatigue) that a doctor recognizes, then this can lead to tests such as the antinuclear antibody test. 

Vision loss is often painless, and if caught quickly can potentially be treated with medication. Usually, however, when vision problems appear alone, the priority is to adjust vision with glasses and other external fixes. If you have been diagnosed with lupus and start to have vision problems, however, this is recognized as a sign of increased, severe lupus activity. In fact, Eye disease is often associated with high levels of inflammation, so it’s important to have these issues diagnosed and treated as soon as possible.

What is Vision?

The sense that we call vision is actually two different processes – the detection of light by the eye and the heavy processing and categorization that happens in the brain. All senses require the brain to interpret those sensations to a degree, based on what you know about the world. However, there is so much visual information – including color and depth perception – that the brain has to take more shortcuts than with other senses to cut down on energy and processing speed. We actually don’t see exactly what is in front of us like a camera recording or a computer – we ‘see’ in categories and shapes, which speeds up our ability to perceive and react to the world. What this means is that, if lupus damages or compromises any part of the system – the eye, the brain, the nerve the connects them, or the blood vessels that feed these organs – it can have major effects on vision. 

How Does Vision Work?

Eyes are specialized organs that detect light and transmit that information to the brain. The brain then interprets the light signals into what we perceive as vision. Humans generally have two eyes, which is important to how we determine depth and dimension. While we can still see with one eye, depth and distance become harder to judge, which can make it dangerous to perform activities that require quick reactions and precision, including driving.

Step 1: Light Enters the Eye

The human eye is a complex organ that works a lot like an analog camera – most of the eye is opaque, protected from dirt and scratches by an outer layer called the cornea, which is kept clean and in good condition by constant moisturizing through tears. The opaqueness of the eye means that extra light is not let in. 

The sclera is the protective outer coating of the eye, which in humans is white and full of blood vessels, and inside the eye, there is a clear fluid called vitreous humor which helps to bend the light and allows the eye to maintain its shape through pressure. This is important, because if the eye shape is off, it can affect how well the eye can focus on details. This is a common cause of near- or far-sightedness.

The first step of vision starts with light travelling through the cornea, a clear, thick, curved area in the front of the eye that bends and focuses the light. The light then enters the eye through an opening called the pupil, and then passes through the lens. The pupil, cornea, and lens allow the eye to focus on details, adapt to different levels of light in the environment, and protect the sensitive cells at the back of the eye from damage. The amount of light let in depends on the environment – in dim light conditions, the pupil is widened (or dilates) to let in more light, while in bright light the pupil contracts to a tiny dot. It is also affected by stress, fear, and other emotions – energetic emotions such as excitement, fear, aggression, or arousal cause dilation, while calmer emotions are associated with constricted, smaller pupils.

Step 2: Light Receptors in the Retina React

The cells at the back of the eye are special cells that react in response to certain wavelengths of light. There are millions of these cells in the back of the eye, arranged in a two-layered sheet called the retina. The retina consists of color-detecting receptors called cones and light detecting receptors called rods. There are three types of cones, detecting low energy red light, medium energy green light, and high energy blue light. These receptors are triggered in different amounts, which we then interpret as the rainbow of colors around us. Other receptors called ‘rods’ are very good at detecting light and dark. They are able to detect dim light and are responsible for allowing us to see in the dark – though night vision is generally without color.

Step 3: Information is Transferred to the Brain and Processed

These cells transmit their information through a nerve bundle called the optic nerve, the connection from the eye to the visual cortex in the brain. The optic nerve is not the longest nerve in the body, but it still has a long way to carry its information – all the way to a region called the “occipital lobe” which is all the way in the back of the head! There, the information is processed, and our brains categorize what we see into shapes, types of objects, and other classifications. This is both how we see, and how we understand what we see. 

This means that sight is a complicated teamwork between the structures of the eyes, the optic nerve, and the brain. This ‘teamwork’ can degrade with age but also with extreme exposure to powerful light (including long term use of bright screens) and head trauma.

Inflammation caused by lupus can affect any stage of the vision process. When serious, it can cause loss of vision or even blindness if not caught and treated early.

Other Eye Symptoms of Lupus

Although the results are the same – pain, deterioration of vision, or loss of vision – and inflammation is generally the cause, the actual source of the symptoms can vary. 

There are many ways that lupus effects the eyes. Lupus can cause inflammation and damage to the outer layer of the eye (scleritis), the muscles that move the eyes (including the iris itself,) and the iris. It can also affect the nerves that control these muscles. Cutaneous lupus around the skin near the eyes, the eyelid, or the face in general can also effect vision. Keratitis, or inflammation of the cornea, is more direct. It affects the cornea, which is the layer that protects the eye. Inflammation causes the area to become irritated and sensitive, blurring vision both from the inflammation and the increased production of tears.

Lupus retinopathy and dry eye the most common eye related symptoms of lupus.  Retinopathy is when the retina, the back of the eye with all of the receptors, is damaged. The receptor cells die, reducing the amount of light and detail that the eye can detect.

Dry eye is exactly what it sounds like. Lupus that affects the tear glands and tear ducts can also interfere with vision as the eye dries out. Dry eye is a common symptom of lupus and certain other autoimmune diseases such as Sjogren’s Disease. The lack of tears means that the eye can’t clean out dirt and debris as effectively, allowing the eye to be scratched and damaged more easily. The loss of the tear layer also exposes the eye to damage from drying out and to bacteria that are better able to get a foothold in the eye and cause damage of their own. Rarely, lupus affects the eyeball itself.

However, the eyes do have a level of protection from the immune system, much like the brain and reproductive organs. So, the most common cause of vision loss is restricted blood flow due to vasculitis. Vasculitis is when inflammation from lupus constricts the blood vessels providing various parts of the vision system with oxygen. This starves the organ of vital oxygen and makes them unable to do their jobs – even potentially permanently damaging them. The constriction of blood vessels due to lupus is called vasculitis. 

The optic nerve and brain can both also be affected by lupus inflammation or vasculitis, effecting how the information from the eye is transmitted or processed.

Lupus Medications and the Eye

Lupus medications, including steroids and antimalarials are a major part of lupus treatment, but also are known to cause damage to the retina. Steroid medications raise the pressure in the eye and can also lead to cataracts. People on steroid medications, especially hydroxychloroquine (HCQ) should get their eyes checked often.

Alternate treatment options are sometimes used when doctors feel that it’s appropriate.  If someone with lupus starts getting eye-related symptoms, then they should talk to their treatment team about changing their medications to try to avoid the damage.

Other lupus medications are effective though, as are treatments that reduce inflammation overall, such as diet, exercise, and alternative therapies. Other medications for lupus, including the chemotherapy drugs bevacizumab, rituximab, and belimumab, are also useful. Anticoagulants (such as blood thinners) can directly prevent the vasculitis from causing more damage.

A Lupus Warrior’s Takeaway

If you start noticing eye-related symptoms, including sensitivity to light, it’s a good idea to get checked as soon as possible. Case studies have shown that, the sooner these problems are caught, the more likely it is that you can avoid permanent vision loss. 

You can read more about the eye and lupus here.

The signs of inflammation-related anemia, or “anemia of inflammation,” can show up throughout the entire body as the brain, organs, and other body tissues receive insufficient oxygen. The symptoms of anemia can range from uncomfortable to dangerous and even life-threatening.

Symptoms of Inflammation-Related Anemia

Anemia deprives the body of oxygen, which all of the cells in your body use in the process of turning carbohydrates and fat from fuel into the energy. When there are not enough healthy red blood cells carrying oxygen to your organs and tissues, your body is forced to work with less. This causes the following symptoms:

• Fatigue and lack of energy
• Headaches and dizziness
• Difficulty thinking clearly
• Lower stamina during exercise, more muscle pain, and longer recovery time after exercise
• Shortness of breath
• Pale skin

 

While treatment of inflammation-related anemia is possible, it is not always prioritized by lupus treatment teams. Clinicians often prioritize the health of organs, which is crucial but can lead to people living with anemia symptoms.

The symptoms of inflammation-related anemia in lupus can mask other symptoms of SLE and can get in the way of lupus treatment and a healthy lifestyle.

Anemia can also be an important sign of dangerous SLE symptoms, such as internal bleeding, or serious inflammation damage of the organs. Of particular note, anemia caused by kidney damage can inhibit the body’s ability to filter the blood properly. This can leave toxic waste products to float around in the blood stream.

In general, people with lupus-related anemia were found to be more sensitive to light and to have more kidney problems. Organs besides the kidneys can also be involved and may provide clues for the optimal lupus treatment methods.

Lupus and Anemia

A study published in the European Journal of Rheumatology found that of the people they tested, over 50% of people with systemic lupus experienced anemia as a symptom. Systemic lupus damages organs throughout the body, including the organs responsible for blood production. These organs can include the:

• Bone marrow
  • Produces red blood cells
• Spleen
  • Stores red blood cells
• Liver
  • Filters toxins from the blood
• Kidneys
  • Filter toxins from the blood
  • Produce erythropoietin, a hormone that encourages the production of red blood cells.
• Gut
  • Absorbs iron, a key component of red blood cells

Macrophages and Anemia

In addition to impacting specific organs, lupus alters the effectiveness of the immune system as a whole. One way it does this is by reducing the number of macrophages. Macrophages are white blood cells that “eat” & destroy bacteria and damaged red blood cells. Fewer macrophages can lead to an increase in unhealthy and damaged red blood cells circulating in the body.

On the other hand, during symptom flare-ups, the immune systems of people with SLE can become turbocharged and end up attacking healthy red blood cells. If they attack too many red blood cells, then there is not enough for the body to use, leading to anemia.

Lupus, Anemia, and You

Fortunately, anemia – regardless of cause – is very treatable on its own through:

Medications

If the cause is unknown or isn’t treatable, then synthetic erythropoietin, the hormone that causes the bone marrow to produce more red blood cells, may help. Injected directly into the body, medications that use synthetic erythropoietin (such as EPO and Abseamed) help spur the body into producing more red blood cells on its own. These medications reduce the anemia symptoms and may help with other lupus symptoms.

These medications do have some potential side effects, however, such as high blood pressure, swelling, fever, dizziness, nausea, and soreness. Synthetic erythropoietin also does not address the underlying issues, such as organ damage, and might cover up the problems. As with all medications, talk to your lupus treatment team about synthetic erythropoietin to see if it is the right choice for you.

Supplements

If medication is inappropriate, or if the cause of the anemia is the poor absorption of nutrients into the body, then injections of iron, vitamin b12, and folic acid may be helpful. These nutrients are key components to building and maintaining red blood cells in the body, and supplements of iron, vitamin b12, and folic acid increase blood production by ensuring that the body has enough raw materials.

A diet rich in these vitamins and nutrients, along with dietary supplements can also help. Talk to a nutritionist on your lupus treatment team to help figure out meals and vitamins/supplements, that may be beneficial.

Blood Transfusion

For very severe cases of anemia, the best way to take care of it may be not so much producing more of your own blood, but getting an infusion of someone else’s blood. A blood transfusion will give you all of the blood components that you need and will help you build up a healthy level of blood.

Side effects are generally minimal, but for many people with lupus this is only a short-term solution to keep them out of dangerous levels of anemia, give them time to find and treat the underlying cause of their anemia, or until they figure out the right treatment for their anemia.

Some people with lupus, however, may find that blood transfusions are their only, or primary, choice.

Want to know more about lupus and inflammation-related anemia?

Anemia might not be the only thing causing fatigue — find out how you can manage your lupus fatigue in this article.

Kidney damage is a major contributor to lupus-related anemia, and there is a type of lupus, lupus nephritis, that specifically attacks the kidneys. Learn more about it here.

Other types of anemia that can come with lupus: Hemolytic anemia is a lupus symptom that involves less-resilient or more easily damaged red blood cells. Read more about it in our article here!

Methotrexate, an immune-system suppressant and cancer chemotherapy drug used to treat leukemia and some types of arthritis, may lead to or exacerbate organ damage and anemia. Read more about it here.

Systemic lupus erythematosus (SLE) is an autoimmune disease that turns the body’s own immune system against itself. So, it shouldn’t seem too out-of-the-blue that many treatments for SLE target the immune system. Usually, these medications reduce inflammation, or by forcing the immune system to be less active. These medications have serious and unpleasant side effects, but for many people with lupus, reducing or even stopping their lupus symptoms is worth it. You can read more about lupus medications here. 

However, other therapies try to actually make the immune system work the way it’s supposed to.

CAR-T cell therapy is a type of therapy called “immunotherapy” which uses the body’s own immune system to treat patients. CAR – T therapy takes T-cells already produced by the body and modifies them. After being modified, the patient’s own immune system is better able to target and attack cancerous cells but there are a lot of potential benefits for people with autoimmune diseases. In fact, a recent study demonstrated that CAR-T cell therapy can suppress lupus symptoms into what is known as remission – without compromising the patient’s immune system!

What is Remission?

Remission in lupus is the long-term lack of symptoms or flares after treatment. Some people with well-managed lupus were able to go for up to 5 years, or even longer, without dealing with symptoms or flares. Usually, this is achieved with lupus medications, including immunosuppressants, taken over many years. Over time, these medications control inflammation in the body to a point where it can heal some of the damage and stop the rampaging symptoms of lupus, but they don’t cure it. Lupus is always there and can be triggered back into the disease later on.

There are several often-frustrating side effects to lupus medications, and one of them is that a suppressed immune system is more vulnerable to sickness and infection. When someone has a compromised and vulnerable immune system, it is known as being immunocompromised. It is also harder for a vaccine to ‘take’ in people who are immunocompromised – there just is less of an immune system for the vaccine to train. Because of this, researchers have been looking into treatment methods that can allow people with lupus to achieve remission without weakening their immune systems. Not everyone responds to typical lupus treatments either, a situation known as refractory lupus – they need alternative therapies. You can read more about refractory lupus here. CAR-T cell therapy is one such therapy under investigation.

What is CAR-T Therapy?

There are several different types of CAR – T therapies, but overall, CAR stands for “Chimeric Antigen Receptor.” Receptors are structures on cells that allow them to “receive” certain molecular structures on their surface. Each receptor only matches with a limited number of molecules that fit its shape. Once a receptor attaches to its matching molecule, or a closely related one, a switch is flipped in the cell, starting a chain reaction throughout the cell that allows it to respond. This is how cells throughout the body work, and in the case of an antigen receptor, immune system cells like T-cells use these receptors to sniff out pathogens and activate the correct response. With CAR, these antigen receptors are laboratory engineered structures that better recognize B cells.  Equipped with these upgraded receptors, T Cells are able to sniff out and destroy B cells.

B-Cells and Lupus

B cells, also known as b-lymphocytes, are a type of immune system cell that attacks viruses, bacteria, parasites, and cancer – anything that uses the body’s own cells to hide or reproduce. These cells usually detect special antigens that stressed or damaged cells produce and will attach to those antigens and trigger a self-destruct mechanism in the cell. However, in autoimmune disease, these cells ‘sniff out’ healthy cells too, attacking them. Not only does this cause damage to those cells and to the organs that use those cells, but the defective B cells signal to the body that there is a threat, leading to inflammation, or higher levels of immune system activity. Over time, the damage accumulates, and the body becomes more trained to attack these cells, leading to a vicious cycle of autoimmunity. In addition to also killing off sick cells, T cells are one of the ways that the body culls out the B cells that target healthy cells, but for yet unknown reasons, the T-Cells aren’t doing their jobs properly in people with autoimmune diseases such as lupus. CAR-T therapy puts T cells back on the job, reducing the amount of B cells in the body that can cause problems for a patient. You can read more about T Cells and their functions here. 

Because it improves the function of the immune system – working with what is already there without involving other organs – serious side effects are rare.

Chimeric? What does that mean?

“Chimeric” means that the CAR antigens were developed using the genetics of multiple species to get the receptor structure they were looking for. Since all life forms on the planet use the same general source code, DNA (or RNA for viruses,) the body has no issue using or replicating the new antigens. 

Several of the side effects of CAR tend to be reactions to the infusion process itself. CAR – T therapy is usually given intravenously as an infusion, and the white blood cells modified as part of the therapy are drawn from the patient’s own bloodstream.

Unfortunately, the body doesn’t always respond well to needles, having blood drawn, or having medication flowing directly into the bloodstream. Sometimes the body will go into a stressed state known as vasovagal synope. Essentially, the body panics, thinks that you are wounded, and lowers the heartbeat and breathing rate. This leads to a sudden drop in blood pressure and feelings of lightheadedness. Nausea, fever, fatigue, loose stools, and confusion can result as the body overreacts. A person can even fall unconscious for a short time. While vasovagal synope usually goes away on its own, it’s something that should be looked into further if it’s common. 

This is not a reaction to the antigens themselves, but the process of administering the medication. Since the cells come from the person’s own body, rejection or allergic reaction is unlikely.

The Research on CAR-T Cell Therapy

In the study, presented at the American College of Rheumatology’s annual meeting in 2023,  8 patients with lupus were treated with CAR-T therapy, 5 of which had follow ups a year or more post-CAR T therapy. All of these patients were able to achieve drug-free remission on this therapy, for 8 months or more. They were not taking immunosuppressants, and tested negative for autoantibodies – antibodies that marked the body’s own cells. Their immune systems were left intact and relatively robust, and the patients were able to be successfully vaccinated for measles, mumps, rubella, varicella zoster virus, Epstein-Barr, tetanus, and pneumococcus. This is because, after the autoimmune B cells were cleared from their system, they produced fresh, new B cells. 

These cells did not immediately attack the body, and instead behaved as normal and were able to be trained to the vaccines. This does not happen under immunosuppressants, so this was an exciting find. Patients tested negative for autoantibodies and remained in remission until the end of the study. 

Of course, this study was done on 5 people – a tiny sample size. None of them were of African descent, and only one was registered as Hispanic, which is not representative of the population. There are also side effects and cost limitations to CAR-T treatment, and it is not approved for lupus patients in the US. However, it is approved for several cancer treatments, which makes this study extremely promising. It is also not the only study of its kind:

In a 2020 study on 43 patients, the researchers saw 51% of their patients go into remission for more than 3 years after the therapy. Some of the patients had remissions as long as 9 years (and still going) by 2020. They noted few adverse effects from the treatment, as well. 

Side Effects of CAR-T

Aside from the effects of the infusions and blood draw, CAR-T has a few other notable side effects. In addition to potential allergic reactions, the depletion of certain vital minerals and cells in the bloodstream, and a weakening of the immune system as the T Cells clear out the B cells. 

Cytokine Release Syndrome

Cytokine release syndrome (CRS) also known as a “Cytokine Storm” CAR-T cells are modified, but living, cells and can replicate themselves in the body. As they begin to target B cells, they release cytokines into the blood stream. Cytokines are proteins made by the body to act as messengers, helping the immune system coordinate itself and the rest of the body into combatting a threat. Or, in some cases, sounding the all-clear. Cytokines have many functions, but the sudden rush of cytokines caused by the modified T cells can overwhelm the body, suddenly ramp up inflammation, and even potentially be life threatening. The symptoms of a cytokine storm are very similar to a flu, and this is because the cause is similar – the immune system is pushed into hyperactivity by a storm of cytokines. However, without a threat like the flu, the body is making itself sick for no benefit.

While possible, the cytokine storm complication of CAR-T seems to be uncommon. 

Nervous System Symptoms

It’s not clear why CAR-T can have several serious effects on the immune system, but it can cause:

• Headaches
• Unconsciousness
• confusion
• seizure
• tremors
• loss of balance
• trouble speaking and understanding language.

These seem to be temporary, however, and doctors only advise against avoiding dangerous activities and using heavy machinery for several weeks after the therapy.

In addition, while CAR-T seems to cause long-lasting remission, relapse is also still possible after the therapy, so it shouldn’t be thought of as a cure – like any other treatment for lupus.

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.