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This eBook has been developed by Idorsia Pharmaceuticals Ltd as a resource to improve understanding of lupus. It provides a useful summary of current information about the disease, the diagnosis procedure, and available treatments. It highlights the impact lupus can have on those living with the condition and those around them. It also outlines the strategies people with lupus disease can use to manage their condition as effectively as possible.
The book is intended for the use of a general audience.
About this eBook
“The symptoms are completely unpredictable – one day you feel well and the next day you feel terrible. Before my diagnosis, I would make an appointment with the doctor, but by the time I got there, the symptoms would have gone.”
— Patient
Introduction
Systemic lupus erythematosus – known more simply as lupus – is an autoimmune disease, which means that the body’s immune system malfunctions and attacks the body's own tissues. Some autoimmune diseases affect just one organ, but in the case of lupus, all parts of the body can
be affected.
Understanding lupus
Diagnosis and management of lupus
Burden of lupus
Practical
guidance on managing lupus
Introduction
Disclaimer
This resource has been developed by Idorsia Pharmaceuticals Ltd in order to provide an environment to deliver a better understanding of lupus and to pool information regarding the disease, the diagnosis procedure, available treatments, and the impact it could have on the life of someone diagnosed with lupus. The resource is for use by a general audience.
The information in this resource is not intended as a substitute for advice/treatment by a physician, whose instructions should always be followed. Neither does the information provided constitute an alternative to advice from a doctor or a pharmacist and should not be used on its own to produce a diagnosis or to commence or cease a particular treatment.
The links provided are for informational purposes only; they do not constitute an endorsement or an approval from Idorsia of the services or opinions of the corporation, organization or individual. Idorsia bears no responsibility for the accuracy, legality, or content of the external sites or subsequent links.
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Introduction
Understanding BED
Diagnosis and treatment of BED
The burden of BED
Practical Guidance on Managing BED
Understanding
Binge-Eating Disorder
About this eBook
About this eBook
This eBook has been developed by Idorsia Pharmaceuticals Ltd as a resource to improve understanding of lupus. It provides a useful summary of current information about the disease, the diagnosis procedure, and available treatments. It highlights the impact lupus can have on those living with the condition and those around them. It also outlines the strategies people with lupus disease can use to manage their condition as effectively as possible.
The book is intended for the use of a general audience.
The disease can range from mild to life-threatening and can randomly become worse (so-called 'flare-ups') and then better again, which can make living with lupus unpredictable and its impact on day-to-day life wide ranging.
As a result, its symptoms vary widely and are often similar to lots of other conditions, which need to be ruled out before a diagnosis is made. Lupus therefore often goes undetected or misdiagnosed for long periods of time. Yet early diagnosis is important to manage the symptoms of lupus, introduce treatment to reduce the risk of long-term complications of the condition, and to enable those affected to access wider support such as that of local patient groups.
Despite several high-profile celebrities sharing their diagnosis of lupus publicly, awareness of the condition remains low and misperceptions and stigma continue to affect people living with it.
“There is a stigma attached to lupus.
I always talk about my illness and explain
it to people first; I tell them it’s an autoimmune disease but it’s not contagious and I can’t pass it on to them.”
— Physician
Lupus is an autoimmune disease, which means that the body’s immune system malfunctions and attacks the body's own tissues, causing inflammation and damage.
Understanding lupus
As this immune response can occur anywhere in the body, any organ can be affected: most commonly the skin, joints, blood cells and lungs, as well as the brain, heart and kidneys. It is important to remember that just because lupus can affect any part of the body, it doesn’t mean that it will and the number of organs affected and corresponding symptoms can vary dramatically from person to person.
What is lupus?
How is the immune system involved?
Types of lupus
Introduction
Understanding lupus
Diagnosis and management
of lupus
Burden of lupus
Practical
guidance on managing lupus
Types of lupus
Calcium-channel blockers reduce electrical conduction within the heart by blocking the entry of calcium into the muscle cells. This decreases the force of contraction of the muscle cells and dilates arteries, thus reducing blood pressure.
ARBs (angiotensin II receptor blockers) block the action – rather than the formation – of angiotensin II. Both ACEs and ARBs are often part of the treatment for people with chronic kidney disease.
ACE (angiotensin-converting enzyme) inhibitors block the formation of angiotensin II, a substance that narrows blood vessels, which, in turn, lowers blood pressure. People with chronic kidney disease may benefit from having an ACE
inhibitor as one of their medications.
Beta blockers work by blocking the effects of epinephrine, a hormone also known as adrenaline. This leads to the heart beating more slowly and with less force and to improved blood flow, which reduces blood pressure.
Alpha blockers keep the hormone norepinephrine (noradrenaline) from tightening the muscles in the walls
of smaller blood vessels, which improves blood flow and lowers blood pressure.
There are a few different types of lupus, although the most common one is systemic lupus erythematosus, to which people refer to simply as ‘lupus’.
Systemic lupus erythematosus (SLE) is so called because it affects many different systems in the body. As it can affect all parts of the body it is usually considered more serious than the other types.
Types of lupus
There are a few different types of lupus, although the most common one is systemic lupus erythematosus, to which people refer to simply as ‘lupus’.
Cutaneous lupus is a form of lupus that only affects the skin. It causes many types of rashes and lesions that are typically found on the face, neck, and scalp. The most common, called a ‘discoid rash’, is raised, red, and scaly, but not itchy. While most people with cutaneous lupus will never experience symptoms elsewhere, a small minority of people will go on to develop SLE.
Drug-induced lupus occurs as a side effect of some medications. The lupus-like symptoms usually disappear once the person stops taking the medication.
Any bleeding on the brain is very dangerous as the blood pools together and places pressure on the surrounding brain tissue. This, coupled with the interrupted blood supply due to the burst blood vessel, reduces much-needed blood flow to the brain and can cause severe damage.
While it’s not fully understood why some people develop aneurysms, there are certain factors such as genetics, high blood pressure and smoking, that increase the risk of an aneurysm developing and of it rupturing.
Unlike other types of brain hemorrhage, which can be caused by severe head injuries, aSAH is specifically caused by the rupture of an aneurysm – a weak, bulging spot on the wall of a brain artery – which allows blood to escape and accumulate in the space around the brain.
What causes aSAH?
Childhood lupus is the name given to SLE in children. Compared to the adult form, childhood lupus tends to affect certain organs such as the kidneys to a greater degree and often requires more aggressive treatment.
Neonatal lupus is not a true form of lupus as it is caused by antibodies from the mother acting on the baby in the womb. It typically causes skin rashes that disappear within a few months, often without the need for any treatment. Some babies with neonatal lupus can also be born with
a serious heart defect, which requires separate treatment.
The immune system is incredibly complex, involving many different defense mechanisms that each work together to protect the body from germs and ‘bad’ cells that might turn into cancer.
Sometimes, as in the case of lupus, something goes wrong in the body and the immune system starts to attack its own, healthy cells – this is called an autoimmune response. During this response, the body produces antibodies – substances carried in the blood to destroy ‘foreign’ threats – which mistakenly attack the body’s own tissues, causing damage and inflammation. These antibodies remain within the body, leading to further tissue and organ damage.
How and why lupus affects the immune system’s defense mechanisms is still not fully understood. However, a combination of genetic, hormonal, and environmental factors is thought to play a role.
How is the immune system involved?
Immune system involvement
Normal immune response
Autoimmune response
Foreign threat invades
Antibodies attack and remove invading threat
Antibodies continue to protect body
Immune system forms antibodies against its own body cells
Antibodies attack the body’s own cells
Antibodies remain in the body causing inflamation and tissue damage
How common is lupus and who gets it?
It is believed that 5 million people worldwide have a form of lupus. Although it affects people of all nationalities, races, ethnicities, genders, and ages, there are certain groups with a much higher risk of developing the condition.
Given its genetic disposition, a female first-degree relative (a sister, daughter or mother) of someone with lupus may be considered six times more likely to develop the disease themselves compared to those with no family history of lupus.
As well as affecting more women, lupus is also more common in people of Afro-Caribbean and Asian origin compared to Caucasians and is likely to affect these ethnic groups more severely as well.
As much as ninety per cent of diagnosed cases are in women. Lupus particularly affects females during their childbearing years, with its occurrence increasing after puberty and decreasing after menopause.
What causes lupus?
It is not fully understood what causes lupus, but scientists agree that it is likely to be the result of a combination of contributors, including genetic, hormonal, and environmental factors.
Research suggests that some viruses may be linked to the development of lupus. These include the Epstein-Barr virus (EBV, which causes mononucleosis or “mono”), herpes zoster virus (which commonly causes shingles), cytomegalovirus, and HIV (human immunodeficiency virus). Further research is needed to understand how these infections may contribute to the risk of the disease.
Lupus is much more common in women than men, a difference that has been shown to be related, at least in part, to the female hormone estrogen.
While lupus is not directly ‘inherited’ in the same way as other genetic diseases that are passed from parent to child, the existence of certain genes that run in some families, genes that affect how the immune system works, puts some people at an increased risk of lupus.
Although most causes of lupus are entirely out of an individual’s control, cigarette smoking has been linked to the development of multiple autoimmune diseases, including lupus. For this reason (and many other health reasons), stopping smoking is advisable.
Signs and symptoms of lupus
Some of the symptoms of lupus are non-specific to the disease and, depending on which part of the body is affected, can be mistaken for other conditions, leading to misdiagnosis.
Some people may experience many different symptoms, some only one. This can also change over time, with symptoms flaring up and improving for periods of time.
“During a flare, fatigue can prevent us from doing the most simple, everyday things such as taking a shower, brushing our hair, brushing our teeth, getting dressed or getting out of bed.”
— Patient
Symptoms of lupus
Relapse or ‘flare ups’
As any part of the body can be affected by lupus, the condition can manifest itself in a multitude of ways.
Symptoms of lupus
Lupus
Skin: reshes, sensivity to light
Fatigue
Joint and muscles: aches and pains
Nervous system: headaches, depression, and seizures
Fever and/or night sweats
Kidney: problems
Weight: changes
Head: hair loss, oral/nasal ulcers
Blood and circulation: swollen glands, poor circulation in the fingers and toes, anemia
Liver and spleen: enlarged
When symptoms worsen, or flare up, this is known as a relapse and can last for a few weeks or sometimes longer.
Relapse or 'flare up'
What is the outlook for people with lupus?
While there is no cure for lupus, treatment is available to manage the symptoms and reduce the risk of more serious complications.
It is hard to predict how lupus will affect each individual. Early diagnosis is important so that patients and their families can access support as soon as possible and manage the risk of more serious complications affecting vital organs.
“My initial reaction when I was told I had lupus was relief – I finally had an answer that made sense and now I could fight it.
It scared me because there’s no cure, but
I could figure out what medication may help me with it, I could join a support group, I could talk to people who have it
to really figure out what it was and what
it was doing to my body.”
— Patient
Diagnosis and management of lupus
There is no single test for lupus and, depending on the individual’s symptoms and circumstances (for example, where they have relatively isolated and not specific symptoms and where no
family history of the condition is known), a diagnosis can be
very challenging.
Lupus is often described as ‘the great imitator’ as its symptoms can appear like other diseases, which often have to be ruled out first before a diagnosis of lupus is made.
For example, someone who goes to their doctor because they are experiencing fatigue and joint pain would likely be considered to have arthritis, and it is only when non-arthritic symptoms start to appear, such as a rash on the face or kidney problems, that an underlying cause of lupus may be suspected.
What tests are used to diagnose lupus?
Assessing the severity of lupus
Family history
Lupus can range from mild to severe depending on how it affects the body.
Assessing the severity of lupus
Mild: joint and skin problems, tiredness.
Moderate: inflammation of other parts of the skin and body, including the lungs, heart, and kidneys.
Severe: inflammation causing severe damage to the heart, lungs, brain, or kidneys, which can be life threatening.
Mild
Moderate
Serve
An assessment for lupus requires a careful history and physical examination, along with specific laboratory tests to identify signs that are typical of lupus or that suggest a different disease altogether. Patients will therefore have a combination of the following tests to reach a diagnosis of lupus:
What tests are used to diagnose lupus?
Physical examination: to check for signals, such as rashes or arthritis.
Blood test: a number of different blood tests are used to check for blood cell count, different antibodies and protein levels that may be higher in some forms of lupus.
Urine test: to detect blood or proteins in urine that may
indicate signs of kidney problems.
Depending on what symptoms a person is experiencing or which organs a doctor might think are involved, people may also undergo other tests. Examples include:
X-ray: to examine the inside of the body, mostly bones and joints, although it can also be used to detect problems affecting soft tissue such as internal organs.
Chest scan and echocardiography: to check for an enlarged heart or any other heart or vascular abnormalities.
Chest scan and echocardiography: a scan that uses high-frequency sound waves to look inside the body.
MRI scan: to detect changes in the brain or other
internal organs.
Family history
Although genes certainly play a role in lupus, they do not determine who develops the condition and there is no single gene that is associated with lupus.
As such, it is not considered worthwhile for all family members to be screened for the condition if a relative
is diagnosed with lupus.
However, if someone with a known family member with lupus starts showing symptoms themselves, then they should speak
to their doctor.
This is proven by the fact that even if one identical twin has lupus, the chance of the other having lupus is only 25%.
What treatments are available for lupus?
There is no cure for lupus, however most people with lupus are prescribed a combination of different medications to manage their symptoms, improve their quality of life, and reduce the risk of more serious complications.
The choice of treatment is dependent on the preferences of the person with lupus, which part of their body is affected, and the severity of the condition at the time. Given the changing nature
of lupus, regular monitoring by a rheumatologist (and often
a wider multidisciplinary healthcare team) is needed to review
the best treatment course for the individual.
Which healthcare professionals
are involved
in the treatment of lupus?
Research into new treatments for lupus itself is ongoing and may lead to new options becoming available for people with lupus
in the future.
Which healthcare professionals are involved in the treatment of lupus?
The main physician for someone with lupus will typically be
a rheumatologist, who treats diseases that cause inflammation and swelling. Depending on other symptoms,
a range of healthcare professionals may be involved in the care of patients with lupus, including:
Hepatologists (liver specialists)
Nephrologists (kidney specialists)
Dermatologists (skin specialists)
Cardiologists (heart specialists)
Hematologists (blood specialists)
Gastroenterologists (specialists in digestive disorders)
Neurologists (specialists in the nervous system)
Endocrinologists (hormone specialists)
Pediatricians (specialists in the care of children – if lupus is diagnosed in childhood)
Nurse specialists
Psychiatrists
Psychologists
“I advise other patients to keep a diary of their symptoms to discuss with their healthcare professional. I only remember what has happened with my lupus in the last couple of days, not what has happened in the last 6 months, so I write it down.”
— Patient
Burden of lupus
The burden of lupus can differ drastically from person to person, from the actual physical effects of the disease, to the wider emotional and social impact of the condition on an individual and their loved ones. It is important to know that this is to be expected and there are many forms of support available.
“From the outside we are completely healthy, so patients need to decide whether or not to tell people they are ill. Some employees may be at risk of losing their jobs or being discriminated against.
If they decide not to tell anyone at work, then they need to act as though they are completely healthy, which is not always possible, because they may need to rest during the day. It is not easy to find
a compromise.”
— Patient
Work and employment
Given the unpredictability of its symptoms and the impact of severe fatigue on productivity, Lupus can impact a person’s ability to work either full time or at all. Many people benefit from talking about their condition with their employers who may be able to offer more flexible working arrangements to suit their changing needs.
While lupus in childhood is uncommon, for those who are affected the condition can cause problems with memory and concentration, as well as physical symptoms. Time off for illness and hospital appointments may also be needed, all of
which can affect a child’s performance in school.
Studying
During flare-ups, students may need to rest or be excused from certain classes, such as physical education. Making teachers and school staff
aware of the condition as an underlying issue
is helpful to make this process as simple as possible.
The emotional impact of diagnosis
and symptoms
There is a higher incidence of depression and anxiety in those affected with lupus compared to the general population. It is thought that the uncertainty of
a lupus diagnosis, combined with fatigue, pain and
its treatment, all contribute. Seeking professional psychological help can be an important step for
overall wellbeing.
Many people struggle with the ‘invisibility’ of the disease. While others who have visible symptoms such as marked rashes on the face or weight-gain from steroid treatment experience self-esteem issues.
While flare-ups can be linked to periods of distress, the unpredictability of the condition, even when symptoms are not present, plus the impact of disability on work and the family dynamic, can all contribute to the long-term psychological impact of lupus.
Contraception and pregnancy
Certain hormones may be linked with flare-ups of lupus symptoms, so women wanting to take contraceptive pills should speak to their doctor about the options that are most appropriate for them.
Pregnancy in women with lupus carries a higher risk for the mother and baby compared with pregnancy
in women without lupus. Women with lupus should talk to their doctor before trying to get pregnant to discuss these risks and any necessary changes
to medication.
“You have to find a way to cope and make changes to your lifestyle – you learn how
to live life the best way you can with lupus. You have to get as much rest as you can, but on the other hand you have to try not to let it stop you doing anything.”
— Patient
Practical guidance on managing lupus
In addition to treatments to manage the symptoms of lupus, there are many things people living with lupus can do to help control their condition more effectively, from being open with family and friends about what it feels like to live with lupus and how they can help,
to learning more about the condition to take a more active role
in its management.
Managing pain and fatigue
Making positive lifestyle changes
Avoiding infection
Managing photosensitivity
Making positive lifestyle changes
Many of the problems caused by lupus can be made worse by smoking, so giving up smoking is recommended for anyone living with or at high risk of the condition.
A healthy, well-balanced diet can benefit most people, including those with lupus.
Finding the right balance of exercise for an individual is important to help improve fatigue and overall health, without causing exertion. Walking and swimming are typically recommended as exercises for people with lupus because they can improve fatigue, fitness and stamina without putting too much strain on the joints.
Managing pain and fatigue
Pain and fatigue can be two of the most difficult symptoms of lupus to manage day-to-day and are ‘invisible’, so it’s important to let people around you know how you’re feeling and if they can help in any way.
Avoid certain strenuous activities and plan time to rest before
and after more demanding tasks.
In addition to pain medication, it can be helpful to:
Take regular hot showers or baths to ease joint or muscle pain.
Increase your intake of water or other liquids.
Managing photosensitivity
Sensitivity to light – or photosensitivity as it is medically known – is a common symptom of lupus and can cause
the hallmark rashes or even flare-ups.
When outside, people with lupus should wear high-factor sunscreen (factor 50+) to block out damaging ultraviolet (UV) rays. In many countries, sunscreen is available on prescription for people with lupus.
It is advisable to keep out of the sun when it is most intense, wear a hat, and cover up as much as is comfortable to protect the skin.
Indoor lighting and appliances can also emit harmful UV rays, which can cause cumulative skin damage in people with lupus. Choosing bulbs and appliances with the lowest irradiance can
be helpful to reduce the risk.
Avoiding infection
If someone with lupus is being treated by medications
that dampen their body’s immune response, it may
make them more susceptible to infection.
Washing hands often with warm soapy water, carrying and using antibacterial gel, and avoiding individuals who have any contagious conditions are all simple ways of reducing the risk of infection in day-to-day interactions.
Joining a patient association
“It would be a very positive step if, every time a lupus diagnosis is made, physicians tell the patient that there is a group of other patients that they can communicate with to help them to understand and cope with their disease.”
— Patient
There are many patient associations that are dedicated to helping patients and their families to live well with lupus, through providing detailed information, practical advice, and valuable support.
A list of organizations around the world can be found on the World Lupus Federation website at worldlupusfederation.org/lupus-groups-around-the-world
Introduction
Understanding lupus
Diagnosis and management
of lupus
Burden of lupus
Practical guidance on managing lupus
Introduction
Understanding lupus
Diagnosis and management
of lupus
Burden of lupus
Practical guidance on managing lupus
Introduction
Systemic lupus erythematosus – known more simply as lupus – is an autoimmune disease, which means that the body’s immune system malfunctions and attacks the body's own tissues. Some autoimmune diseases affect just one organ, but in the case of lupus, all parts of the body can be affected.
“The symptoms are completely unpredictable – one day you feel well and the next day you feel terrible. Before my diagnosis, I would make an appointment with the doctor, but by the time I got there, the symptoms would have gone.”
— Patient
Introduction
Introduction
Understanding lupus
Diagnosis and management
of cerebral vasospasm
Burden of lupus
Practical guidance on managing lupus
As a result, its symptoms vary widely and are often similar to lots of other conditions, which need to be ruled out before a diagnosis is made. Lupus therefore often goes undetected or misdiagnosed for long periods of time. Yet early diagnosis is important to manage the symptoms of lupus, introduce treatment to reduce the risk of long-term complications of the condition, and to enable those affected to access wider support such as that of local patient groups.
The disease can range from mild to life-threatening and can randomly become worse (so-called 'flare-ups') and then better again, which can make living with lupus unpredictable and its impact on day-to-day life wide ranging.
Despite several high-profile celebrities sharing their diagnosis of lupus publicly, awareness of the condition remains low and misperceptions and stigma continue to affect people living with it.
“There is a stigma attached to lupus. I always talk about my illness and explain it to people first; I tell them it’s an autoimmune disease but it’s not contagious and I can’t pass it on to them.”
— Patient
Understanding lupus
Understanding lupus
Lupus is an autoimmune disease, which means
that the body’s immune system malfunctions
and attacks the body's own tissues, causing inflammation and damage.
As this immune response can occur anywhere in the body, any organ can be affected: most commonly the skin, joints, blood cells and lungs, as well as the brain, heart and kidneys. It is important to remember that just because lupus can affect any part of the body, it doesn’t mean that it will and the number of organs affected and corresponding symptoms can vary dramatically from person to person.
Types of lupus
How is the immune system involved?
What is lupus?
How is the immune
system involved?
The immune system is incredibly complex, involving many different defense mechanisms that each work together to protect the body from germs and ‘bad’ cells that might turn into cancer.
CLOSE
Sometimes, as in the case of lupus, something goes wrong in the body and the immune system starts to attack its own, healthy cells – this is called an autoimmune response. During this response, the body produces antibodies – substances carried in the blood to destroy ‘foreign’ threats – which mistakenly attack the body’s own tissues, causing damage and inflammation. These antibodies remain within the body, leading to further tissue and organ damage.
How and why lupus affects the immune system’s defense mechanisms is still not fully understood. However, a combination of genetic, hormonal, and environmental factors is thought to play a role.
Types of lupus
There are a few different types of lupus, although the most common one is systemic lupus erythematosus, to which people refer
to simply as ‘lupus’.
CLOSE
Systemic lupus erythematosus (SLE) is so called because it affects many different systems in the body. As it can affect all parts of the body it is usually considered more serious than the other types.
Cutaneous lupus is a form of lupus that only affects the skin. It causes many types of rashes and lesions that are typically found on the face, neck, and scalp. The most common, called a ‘discoid rash’, is raised, red, and scaly, but not itchy. While most people with cutaneous lupus will never experience symptoms elsewhere, a small minority of people will go on to develop SLE.
Drug-induced lupus occurs as a side effect of some medications. The lupus-like symptoms usually disappear once the person stops taking the medication.
Childhood lupus is the name given to SLE in children. Compared to the adult form, childhood lupus tends to affect certain organs such as the kidneys to a greater degree and often requires more aggressive treatment.
Neonatal lupus is not a true form of lupus as it is caused by antibodies from the mother acting on the baby in the womb. It typically causes skin rashes that disappear within a few months, often without the need for any treatment. Some babies with neonatal lupus can also be born with
a serious heart defect, which requires separate treatment.
How common is lupus and who gets it?
It is believed that 5 million people worldwide have a form of lupus. Although it affects people of all nationalities, races, ethnicities, genders, and ages, there are certain groups with a much higher risk of developing the condition.
Given its genetic disposition, a female first-degree relative (a sister, daughter or mother) of someone with lupus may be considered six times more likely to develop the disease themselves compared to those with no family history of lupus.
As much as ninety per cent of diagnosed cases are in women. Lupus particularly affects females during their childbearing years, with its occurrence increasing after puberty and decreasing after menopause.
As well as affecting more women, lupus is also more common in people of Afro-Caribbean and Asian origin compared to Caucasians and is likely to affect these ethnic groups more severely as well.
What causes lupus?
It is not fully understood what causes lupus, but scientists agree that it is likely to be the result of
a combination of contributors, including genetic, hormonal, and environmental factors.
Lupus is much more common in women than men,
a difference that has been shown to be related, at least in part, to the female hormone estrogen.
While lupus is not directly ‘inherited’ in the same
way as other genetic diseases that are passed from parent to child, the existence of certain genes that run in some families, genes that affect how the immune system works, puts some people at an increased risk of lupus.
Research suggests that some viruses may be linked to the development of lupus. These include the Epstein-Barr virus (EBV, which causes mononucleosis or “mono”), herpes zoster virus (which commonly causes shingles), cytomegalovirus, and HIV (human immunodeficiency virus). Further research is needed to understand how these infections may contribute to the risk of the disease.
Although most causes of lupus are entirely out of
an individual’s control, cigarette smoking has been linked to the development of multiple autoimmune diseases, including lupus. For this reason (and many other health reasons), stopping smoking is advisable.
Signs and symptoms of lupus
Some of the symptoms of lupus are non-specific to the disease and, depending on which part of the body is affected, can be mistaken for other conditions, leading to misdiagnosis.
Some people may experience many different symptoms, some only one. This can also change over time, with symptoms flaring up and improving for periods of time.
“During a flare, fatigue can prevent us from doing the most simple, everyday things such as taking a shower, brushing our hair, brushing our teeth, getting dressed or getting out of bed.”
- Patient
Relapse or 'flare up'
Symptoms
of lupus
Symptoms of lupus
As any part of the body can be affected by lupus, the condition can manifest itself in
a multitude of ways.
CLOSE
Relapse or 'flare up'
When symptoms worsen, or flare up, this is known as a relapse and can last for a few weeks or sometimes longer
CLOSE
What is the outlook for
people with lupus?
While there is no cure for lupus, treatment is available to manage the symptoms and reduce the risk of more serious complications.
It is hard to predict how lupus will affect each individual. Early diagnosis is important so that patients and their families can access support as soon as possible and manage the risk of more serious complications affecting vital organs.
“My initial reaction when I was told I had lupus was relief – I finally had an answer that made sense and now I could fight it. It scared me because there’s no cure, but I could figure out what medication may help me with it, I could join a support group, I could talk to people who have it to really figure out what it was and what
it was doing to my body.”
— Patient
Diagnosis and management of lupus
There is no single test for lupus and, depending on the individual’s symptoms and circumstances (for example, where they have relatively isolated and not specific symptoms and where no family history of the condition is known), a diagnosis can be very challenging.
Lupus is often described as ‘the great imitator’ as its symptoms can appear like other diseases, which often have to be ruled out first before a diagnosis of lupus is made.
For example, someone who goes to their doctor because they are experiencing fatigue and joint pain would likely be considered to have arthritis, and it is only when non-arthritic symptoms start to appear, such as a rash on the face or kidney problems, that an underlying cause of lupus may be suspected.
Diagnosis and management of lupus
Family history
Assessing the severity of lupus
What tests are used to diagnose lupus?
Assessing the severity
of lupus
Lupus can range from mild to severe depending on how it affects the body.
Mild: joint and skin problems, tiredness.
CLOSE
Moderate: inflammation of other parts of the skin and body, including the lungs, heart, and kidneys.
Severe: inflammation causing severe damage to the heart, lungs, brain, or kidneys, which can be life threatening.
What tests are used to diagnose lupus?
An assessment for lupus requires a careful history and physical examination, along with specific laboratory tests to identify signs that are typical of lupus or that suggest a different disease altogether. Patients will therefore have a combination of the following tests to reach a diagnosis of lupus:
Physical examination: to check for signals, such as rashes or arthritis.
CLOSE
Blood test: a number of different blood tests are used to check for blood cell count, different antibodies and protein levels that may be higher in some forms of lupus.
Urine test: to detect blood or proteins in urine that may indicate signs of kidney problems.
Depending on what symptoms a person is experiencing or which organs a doctor might think are involved, people may also undergo other tests. Examples include:
X-ray: to examine the inside of the body, mostly bones and joints, although it can also be used to detect problems affecting soft tissue such as internal organs.
Chest scan and echocardiography:
to check for an enlarged heart or any other heart or vascular abnormalities.
Chest scan and echocardiography:
a scan that uses high-frequency sound waves to look inside the body.
MRI scan: to detect changes in the brain or other internal organs.
Family history
Although genes certainly play a role in lupus, they do not determine who develops the condition and there is no single gene that is associated with lupus.
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This is proven by the fact that even if one identical twin has lupus, the chance of the other having lupus is only 25%.
As such, it is not considered worthwhile for all family members to be screened for the condition if a relative is diagnosed with lupus.
However, if someone with a known family member with lupus starts showing symptoms themselves, then they should speak to their doctor.
What treatments are available for lupus?
There is no cure for lupus, however most people with lupus are prescribed a combination of different medications to manage their symptoms, improve their quality of life, and reduce the risk of more serious complications.
The choice of treatment is dependent on the preferences of the person with lupus, which part of their body is affected, and the severity of the condition at the time. Given the changing nature
of lupus, regular monitoring by a rheumatologist (and often a wider multidisciplinary healthcare team) is needed to review the best treatment course for the individual.
Research into new treatments for lupus itself is ongoing and may lead to new options becoming available for people with lupus in the future.
Which healthcare professionals are involved in the treatment of lupus?
Which healthcare professionals are involved in the treatment of lupus?
The main physician for someone with lupus will typically be a rheumatologist, who treats diseases that cause inflammation and swelling. Depending on other symptoms,
a range of healthcare professionals may be involved in the care of patients with lupus, including:
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Nephrologists (kidney specialists)
Dermatologists (skin specialists)
Cardiologists (heart specialists)
Hematologists (blood specialists)
Hepatologists (liver specialists)
Gastroenterologists (specialists in digestive disorders)
Neurologists (specialists in the
nervous system)
Endocrinologists (hormone specialists)
Pediatricians (specialists in the care of children – if lupus is diagnosed in childhood)
Nurse specialists
Psychiatrists
Psychologists
“I advise other patients to keep a diary of their symptoms to discuss with their healthcare professional. I only remember what has happened with my lupus in the last couple of days,
not what has happened in the last
6 months, so I write it down.”
— Patient
Burden of lupus
The impact of insomnia is often underestimated.
In reality, it can be a distressing condition that can reduce quality of life. It may affect many aspects
of daily life, from studying and employment to social activities and relationships.
Insomnia can have a significant economic impact and increases the risk of accident and injury on the road or in the workplace.
Burden of lupus
“From the outside we are completely healthy, so patients need to decide whether or not to tell people they are ill. Some employees may be at risk of losing their jobs or being discriminated against. If they decide not to tell anyone at work, then they need to act as though they are completely healthy, which is not always possible, because they may need to rest during the day. It is not easy to find a compromise.”
- Patient
Work and employment
Given the unpredictability of its symptoms and the impact of severe fatigue on productivity, Lupus can impact a person’s ability to work either full time or at all. Many people benefit from talking about their condition with their employers who may be able to offer more flexible working arrangements to suit their changing needs.
While lupus in childhood is uncommon, for those who are affected the condition can cause problems with memory and concentration, as well as physical symptoms. Time off for illness and hospital appointments may also be needed, all of which can affect a child’s performance in school.
Studying
During flare-ups, students may need to rest or be excused from certain classes, such as physical education. Making teachers and school staff aware of the condition as an underlying issue is helpful to make this process as simple as possible.
There is a higher incidence of depression and anxiety in those affected with lupus compared to the general population. It is thought that the uncertainty of a lupus diagnosis, combined with fatigue, pain and its treatment, all contribute. Seeking professional psychological help can be an important step for overall wellbeing.
The emotional impact of diagnosis
and symptoms
Many people struggle with the ‘invisibility’ of the disease. While others who have visible symptoms such as marked rashes on the face or weight-gain from steroid treatment experience self-esteem issues.
While flare-ups can be linked to periods of distress, the unpredictability of the condition, even when symptoms are not present, plus the impact of disability on work and the family dynamic, can all contribute to the long-term psychological impact of lupus.
Contraception and pregnancy
Certain hormones may be linked with flare-ups of lupus symptoms, so women wanting to take contraceptive pills should speak to their doctor about the options that are most appropriate for them.
Pregnancy in women with lupus carries
a higher risk for the mother and baby compared with pregnancy in women without lupus. Women with lupus should talk to their doctor before trying to get pregnant to discuss these risks and any necessary changes to medication.
Joining a patient association
“It would be a very positive step if, every time a lupus diagnosis is made, physicians tell the patient that there is a group of other patients that they can communicate with to help them to understand and cope with their disease.”
Practical guidance
on managing lupus
In addition to treatments to manage the symptoms of lupus, there are many things people living with lupus can do to help control their condition more effectively, from being open with family and friends about what it feels like to live with lupus and how they can help, to learning more about the condition to take a more active role in its management.
“Some mornings you get up and wonder how on earth you are going to get through the day. Somehow you do, but it can be a massive struggle.”
— Patient
Practical guidance
on managing lupus
Managing photosensitivity
Avoiding
infection
Managing pain
and fatigue
Making positive
lifestyle changes
Making positive
lifestyle changes
Many of the problems caused by lupus can be made worse by smoking, so giving up smoking is recommended for anyone living with or at high risk of the condition.
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A healthy, well-balanced diet can benefit most people, including those with lupus.
Finding the right balance of exercise for an individual is important to help improve fatigue and overall health, without causing exertion. Walking and swimming are typically recommended as exercises for people with lupus because they can improve fatigue, fitness and stamina without putting too much strain on the joints.
Managing pain and fatigue
Pain and fatigue can be two of the most difficult symptoms of lupus to manage day-to-day and are ‘invisible’, so it’s important to let people around you know how you’re feeling and if they can help in any way.
In addition to pain medication, it can be helpful to:
Avoid certain strenuous activities and plan time to rest before and after more demanding tasks.
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Take regular hot showers or baths to ease joint or muscle pain.
Increase your intake of water or other liquids.
Managing photosensitivity
Sensitivity to light – or photosensitivity
as it is medically known – is a common symptom of lupus and can cause
the hallmark rashes or even flare-ups.
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When outside, people with lupus should wear high-factor sunscreen (factor 50+) to block out damaging ultraviolet (UV) rays. In many countries, sunscreen is available on prescription for people with lupus.
It is advisable to keep out of the sun when it is most intense, wear a hat, and cover up as much as is comfortable to protect the skin.
Indoor lighting and appliances can also emit harmful UV rays, which can cause cumulative skin damage in people with lupus. Choosing bulbs and appliances with the lowest irradiance can be helpful to reduce the risk.
Avoiding infection
If someone with lupus is being treated by medications that dampen their body’s immune response, it may make them more susceptible to infection.
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Washing hands often with warm soapy water, carrying and using antibacterial gel, and avoiding individuals who have any contagious conditions are all simple ways of reducing the risk of infection in day-to-day interactions.
- Patient
There are many patient associations that are dedicated to helping patients and their families to live well with lupus, through providing detailed information, practical advice, and valuable support.
A list of organizations around the world can be found on the World Lupus Federation website at worldlupusfederation.org/lupus-groups-around-the-world
Disclaimer
This resource has been developed by Idorsia Pharmaceuticals Ltd in order to provide an environment to deliver a better understanding of lupus and to pool information regarding the disease, the diagnosis procedure, available treatments, and the impact it could have on the life of someone diagnosed with lupus. The resource is for use by a general audience.
The information in this resource is not intended as a substitute for advice/treatment by a physician, whose instructions should always be followed. Neither does the information provided constitute an alternative to advice from a doctor or a pharmacist and should not be used on its own to produce a diagnosis or to commence or cease a particular treatment.
The links provided are for informational purposes only; they do not constitute an endorsement or an approval from Idorsia of the services or opinions of the corporation, organization or individual. Idorsia bears no responsibility for the accuracy, legality, or content of the external sites or subsequent links.
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Idorsia does not give any warranty or representation, whether expressly stated or implied, of any kind related to the comprehensiveness, usefulness, reliability or timelines of the content of this resource. Idorsia cannot be held liable for any direct or indirect damage of a material or non-material nature that might be caused by the use or non-use of the information presented.
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