What is lupus?
Lupus is a chronic, autoimmune disease that can damage any part of the body (skin, joints, and/or organs inside the body). Chronic means that the signs and symptoms tend to last longer than six weeks and often for many years.
In lupus, something goes wrong with your immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). Normally our immune system produces proteins called antibodies that protect the body from these invaders. Autoimmune means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”) and creates autoantibodies that attack and destroy healthy tissue. These autoantibodies cause inflammation, pain, and damage in various parts of the body.
Lupus is also a disease of flares (the symptoms worsen and you feel ill) and remissions (the symptoms improve and you feel better).
These are some additional facts about lupus that you should know:
- Lupus is not contagious, not even through sexual contact. You cannot “catch” lupus from someone or “give” lupus to someone.
- Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above.
- Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS the immune system is underactive; in lupus, the immune system is overactive.
- Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
- Our research estimates that at least 1.5 million Americans have lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus.
- More than 16,000 new cases of lupus are reported annually across the country.
- It is believed that 5 million people throughout the world have a form of lupus.
- Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too. Most people will develop lupus between the ages of 15-44.
- Women of color are two to three times more likely to develop lupus than Caucasians.
- People of all races and ethnic groups can develop lupus.
How is lupus diagnosed?
In lupus, something goes wrong with your immune system, the part of the body that fights off viruses, bacteria, and germs (“foreign invaders” like the flu). Normally, our immune system produces proteins called antibodies that protect the body from foreign invaders. When you have lupus, your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues, so autoantibodies (auto means self and anti means against: against self) are made that damage and destroy healthy tissue. These autoantibodies cause inflammation, pain, and damage in various parts of the body.
A doctor who is considering the possibility of lupus will look for signs of inflammation which include, pain, heat, redness, swelling, and loss of function at a particular place in the body. Inflammation can occur on the inside of your body (your kidneys or heart, for example), on the outside (your skin), or both.
There are many challenges to reaching a lupus diagnosis. Lupus is known as “the great imitator” because its symptoms mimic many other illnesses. Lupus symptoms can also be unclear, can come and go, and can change.
A physician will carefully review the following while evaluating a lupus diagnosis:
- your current symptoms
- your laboratory test results
- your medical history
- the medical history of your close family members (grandparents, parents, brothers and sisters, aunts, uncles, cousins)
All of this information may be necessary for a doctor to make a diagnosis of lupus.
A variety of laboratory tests are used to detect physical changes or conditions in your body that can occur with lupus. Each test result adds more information to the picture your doctor is forming of your illness. However, for a number of reasons listed below, laboratory tests alone cannot give a definite “yes” or “no” answer:
- No single laboratory test can determine whether a person has lupus.
- Test results that suggest lupus can be due to other illnesses or can even be seen in healthy people.
- A test result may be positive one time and negative another time.
- Different laboratories may produce different test results.
If multiple criteria are present simultaneously, a physician—a family practitioner, internist or pediatrician—may reach a lupus diagnosis. If, however, as is often the case, symptoms develop gradually over time, the diagnosis may not be as obvious, and consultation with a rheumatologist may be needed.
Are my dental problems a result of the medications I take for my lupus?
Many of the medications prescribed to manage lupus can cause complications, including oral health problems. For example, prescribed steroids suppress the immune system and can increase one’s risk of an oral fungal infection and poor wound healing. Drugs such as hydroxychloroquine (Plaquenil) and methotrexate may themselves cause oral sores or ulcerations. If you also have Sjögren’s syndrome, you may experience dryness of the mouth as a result of reduced flow of saliva. This can increase one’s risk of a variety of oral maladies, including oral infections (cavities, gum disease, fungal infection) and difficulty with speaking, eating and swallowing. It is important for everyone to take care of their oral health and see a dentist regularly, especially if you have lupus. If you are experiencing oral complications, consult with your dentist.
Can oral health problems be a result of my lupus?
There are no studies showing lupus directly causes oral health issues. However, it is very common for people with lupus to develop oral lesions or ulcerations in the mouth area. These are often not painful and they typically mimic numerous other conditions, such as lichen planus (common skin disorder) or recurrent aphthous ulcers (canker sores). Additionally, many of the medications prescribed to manage lupus can cause oral health complications, including cavities, gum disease, fungal infection or dry mouth. Talk to your healthcare provider regularly about your oral health and any complications you may be experiencing.
Is there a time period for developing additional autoimmune diseases after the first diagnosis?
There is no time limit on when a second (or even third) autoimmune disease may develop, although it is likely to happen shortly after the first diagnosis. Nevertheless, it is still possible to develop a second autoimmune disease more than ten years after the diagnosis of the first. This said, it is important to maintain ongoing communication with your doctors about any changes to your health and/or specific symptoms you may be experiencing.
What are common diseases that overlap with lupus?
There are many diseases that overlap with lupus including autoimmune thyroid disease , celiac disease, myasthenia gravis, antiphospholipid syndrome, rheumatoid arthritis, polymyositis, dermatomyositis, scleroderma and Sjögren’s syndrome. There are also many less common autoimmune diseases that can affect people with lupus. It is important to keep the lines of communication open with your healthcare provider to discuss all of the symptoms you are experiencing and ensure any potential overlapping diseases are recognized.
How can I tell the difference between a lupus flare and just being tired?
It is normal to be tired at the end of the day, especially if you have been physically active. If you have lupus and your work or home life requires high levels of energy, it is normal to feel exhausted. However, not every bout of fatigue is a lupus flare. The Lupus Foundation of America has specific parameters for defining a “flare” that may help you better evaluate what is causing the fatigue. The best way to determine whether or not you are having a flare is to learn about the symptoms, track them carefully, and keep the lines of communication open between you and your doctor.
Living well with lupus
How can a person tell if lupus is active?
When a lupus flare occurs, many people will notice a return of the symptoms they experienced previously. However, some people may also develop new symptoms. Active disease is caused by inflammation in an organ or organ system. Common symptoms that indicate a flare are:
- ongoing fever not due to an infection
- painful, swollen joints
- an increase in fatigue
- sores or ulcers in the mouth or nose
- general swelling in the legs
Some flares happen without symptoms. This is why it is important to see a trained lupus doctor who regularly monitors your health.
Lupus and your body
Can clinical depression be caused by the effects of lupus on the brain?
Yes, although depression can also be related to a number of other factors. Lupus can affect all organs of the body, including the brain, and when the brain is affected, one of the main symptoms can be depression. We do not know precisely how the neuropsychiatric issues (symptoms of lupus that affect the brain) lead to depression, but it’s probably due to a complex combination of biochemical abnormalities that affect different parts of the brain.
Clinical depression may also be brought on by the different medications used to treat lupus or by factors in a person’s life that are unrelated to lupus. Having any chronic disease, including lupus, can lead to feelings of helplessness, dejection and sadness. Whether depression is caused by lupus or not, it is an important part of your overall health. Keep your doctor informed about how you are feeling-good health is mental and emotional as well as physical. Learn more about lupus and depression.
Can lupus cause depression?
Life with lupus can be challenging. With symptoms that come and go, disease flares and remissions, and the uncertainty of what each day will bring, it’s normal to experience feelings of unhappiness, frustration, anger, or sadness. It’s also normal to grieve for the loss of the life you had before lupus.
As you learn more about lupus and how to adjust and adapt to necessary life changes, feelings of unhappiness, frustration, anger and sadness will lessen.
However, there is a difference between temporary negative feelings and negative feelings that become overwhelming and long-lasting, which may signal a serious but treatable illness called clinical depression.
Symptoms of Clinical Depression
People are considered clinically depressed when they have a depressed or irritable mood, decreased energy, and other symptoms in the list below that last for more than a few weeks and are severe enough to disrupt daily life. Probably the best single marker for clinical depression is loss of interest in activities and responsibilities that used to be important; for example, if you find yourself saying, “I used to enjoy gardening, cooking, and going to church. I don’t feel like doing any of those things anymore.” Clinical depression may be brought about by lupus, by the various medications used to treat lupus, and/or by any of the factors and forces in a person’s life that are not related to lupus. For reasons that are not entirely understood, people with chronic disease often experience this type of depression.
If recognized and properly treated, symptoms of clinical depression can improve.
These are among the most common psychological and physical symptoms of clinical depression:
- Feelings of helplessness or hopelessness
- Crying (often without reason)
- Insomnia or restless sleep, or sleeping too much
- Changes in appetite leading to weight loss or weight gain
- Feelings of uneasiness, anxiety, or irritability
- Feelings of guilt or regret
- Lowered self-esteem or feelings of worthlessness
- Inability to concentrate or difficulty thinking
- Diminished memory and recall
- Lack of interest in things formerly enjoyed
- Lack of energy
- General slowing and clouding of mental functions
- Diminished sexual interest and/or performance
- Recurrent thoughts of death or suicide
Clinical depression may not be recognized in people with lupus because its symptoms and the symptoms of active lupus can be so similar. For example, lack of energy, trouble sleeping, and diminished sexual interest can be attributed to the lupus itself. However, these are also symptoms of clinical depression.
A variety of factors can contribute to clinical depression in people with chronic illnesses. The most common cause is the emotional drain from the stress of coping with the complications of physical illness. Add to that economic, social, and workplace concerns. Moreover, various medications used to treat lupus—especially corticosteroids—may cause clinical depression. When certain organs or organ systems are affected by lupus (such as the brain, heart, or kidneys), clinical depression may occur. A lupus flare also can trigger clinical depression, both because you feel ill, and because it may seem as though you are never going to be free of lupus.
Facts About Clinical Depression and Lupus
- Between 15 and 60 percent of people with a chronic illness will experience clinical depression.
- Clinical depression may be a result of the ways in which lupus physically affects your body.
- Some of the medicines to treat lupus—especially corticosteroids such as prednisone (and at higher doses of 20 mg or more)—play a role in causing clinical depression.
- Clinical depression may be a result of the continuous series of emotional and psychological stressors associated with living with a chronic illness.
- Clinical depression may be a result of neurologic problems or experiences unrelated to lupus.
- Clinical depression also produces anxiety, which may aggravate physical symptoms (headache, stomach pain, etc.).
- Two common feelings associated with clinical depression are hopelessness and helplessness. People who feel hopeless believe that their distressing symptoms may never improve. People who feel helpless believe they are beyond help—that no one cares enough to help them or could succeed in helping, even if they tried.
What Can You Do?
Clinical depression generally improves with a combination of psychotherapy and medication.
Seek psychotherapy. You should not feel embarrassed or hesitant about asking your doctor for a referral to a psychiatrist, psychologist, or therapist. Psychotherapy, under the guidance of a trained professional, can help you learn to understand your feelings, your illness, and your relationships, and to cope more effectively with stress. Cognitive behavioral therapy—a special type of psychotherapy—can be very helpful when you are living with chronic illness. Support groups led by a therapist or trained counselor, such as those organized by the LFA national network of chapters and affiliates, also can be instrumental in helping you deal with symptoms of clinical depression. To find a group in your area, go to lupus.org/chapters or call toll-free 800-558-0121 begin_of_the_skype_highlighting 800-558-0121 FREE end_of_the_skype_highlighting .
Take antidepressant medications. Several types of prescribed drugs can help ease the effects of clinical depression. Anti-anxiety medicines are also available to reduce worry and fearful feelings. In some people, improvements can occur in a matter of weeks once medication is started.
Find ways to reduce pain. Chronic pain can be a factor in the development of clinical depression. Besides medication (which can also play a role in clinical depression), experts often recommend non-medication ways to conquer—or at least reduce—chronic pain, such as yoga, Tai Chi, Pilates, acupuncture, biofeedback, meditation, behavioral changes, play therapy, and chiropractic care.
You may consider over-the-counter treatments for your depression and/or pain. It is important to discuss all herbs and supplements with your rheumatologist or primary care provider before trying them as certain ingredients can cause reactions with your prescribed medications.
Get more exercise. If you are physically able, take part in some sort of physical activity every day. This can be as simple as walking the dog, yard work or gardening, or window shopping at the mall.
Improve your sleep habits. Not getting enough restful sleep can cause many health problems, including symptoms of clinical depression. To improve your sleep, and, in turn, your mental well-being, try to:
- Get seven to eight hours of sleep in a 24-hour period.
- Do aerobic exercise every day, such as brisk walking—or whatever you can manage.
- Avoid caffeine, nicotine, and alcohol several hours before bedtime.
- Know which medications keep you from sleeping and take those early in the day.
- Have a good mattress, comfortable bed linens, the right room temperature, and the right amount of darkness.
- Include rest periods throughout your day when needed.
If you still aren’t getting enough sleep, find a reputable sleep center and talk to your doctor about sleep medications and/or possible physical causes for your sleeplessness.
Build a support system. Stay in touch with family members, former work buddies, or long-time friends. Make phone calls, join Facebook, try videoconferencing, or consider adding an animal companion to your family.
Change your self-talk. Feelings of anger and self-pity can bring on unproductive thoughts; for example, “It’s not fair. I haven’t done anything wrong. Why me? Or I’m too weak even to fight off this illness.” Replace negative, self-defeating inner language with truthful, productive thoughts, such as: “I feel lousy, but I have many blessings.”
You can also list the people and things in your life for which you are grateful: A loving spouse or significant other; your children, and the children of your extended family; caring relatives; good friends; a beloved pet; work or hobbies you enjoy and are able to do; a home you love; volunteer activities; fellowship at school, at a place of worship, or at a community center. Try to add to this list every day!
Discover the values of volunteerism. Volunteerism can provide real emotional benefits. Helping with a charitable cause that is meaningful to you can create social, supportive connections. Helping others can have a positive impact on your sense of well-being.
Strive to accept the new “you.” Pace yourself, and don’t feel badly about delegating some of your responsibilities. Ask for help, and accept help graciously. Finally, focus on what you have and what you can do, rather than on what you don’t have and can’t do.
Just as clinical depression develops over time and not overnight, conquering clinical depression is a gradual process. However, most people with lupus find that, in time, their overall attitude and sense of well-being are greatly improved.
When lupus causes hair loss, will it grow back?
Dr. Werth is a professor of dermatology and medicine at the University of Pennsylvania School of Medicine in Philadelphia. She is also a member of the Lupus Foundation of America’s Medical-Scientific Advisory Council.
Whether or not hair grows back on the scalp depends on whether there is scarring, as well as how much scarring there might be. When the hair loss is widespread, but there is no scarring, the hair will often grow back. If there is too much damage to the skin, the hair may not be able to regrow. If you have lupus and find that you are experiencing hair loss, do not use over-the-counter hair loss treatments before speaking with your doctor. Talk to a dermatologist about your treatment options.
Does lupus cause hair loss?
Dr. Werth is a professor of dermatology and medicine at the University of Pennsylvania School of Medicine in Philadelphia. She is also a member of the Lupus Foundation of America’s Medical-Scientific Advisory Council.
A lupus diagnosis often brings many physical changes, including hair loss. Hair loss is a common side effect of lupus and the medications used for treatment. Recent severe illness, thyroid problems, certain nutritional deficiencies, genetics and specific skin diseases of the scalp can also cause hair loss.
If hair loss is caused by medication, you may have to wait until your lupus is under control to treat the hair loss. This type is “mostly reversible”. Hair loss associated with discoid lesions and scarring is generally permanent, so early treatment is key. In all cases, the most important way to control hair loss is to control disease activity.
It is important not to experiment with over-the-counter medications, like Rogaine, without your doctor’s approval. Rogaine is for treating male- and female-pattern alopecia, which is a completely different type of hair loss than we usually see in lupus. Brittle hair also is common, and many treatments-including steroids and immunosuppressives-cause hair to thin. It is important to work with your doctor to discover the cause and identify the best way to treat or manage the hair loss
What can be done to ensure that lupus is diagnosed earlier rather than later?
Dr. Cynthia Aranow is co-director of the Clinical Trials Unit of the Center for Autoimmune and Musculoskeletal Disease at The Feinstein Institute for Medical Research in Manhasset, NY. Dr. Aranow’s current research focuses on the brain’s involvement in lupus.
Lupus is a disease that is known for being difficult to diagnose because the symptoms are different from person to person, they mimic the symptoms of many other diseases, and they can come and go. It can sometimes take several years to receive an official diagnosis. To diagnose lupus as early as possible, there are three important things you can do:
Educate yourself about lupus. Learn as much as you can about the signs and symptoms of lupus. The Lupus Foundation of America has many online resources that can help. Our “I Might Have Lupus” section is a great place to look for answers to frequently asked questions about diagnosis.
Communicate with your doctor. Tell him or her about any symptoms you might be experiencing and any family history of lupus or other autoimmune diseases. Try keeping track of your symptoms so that your doctor can see how they change over time.
See a rheumatologist. If you have learned about lupus and talked to your primary care doctor, and you still think lupus is a possibility, make an appointment to see a rheumatologist. He or she can help determine whether you have lupus.
What is drug induced lupus?
Drug-induced lupus is a lupus-like disease caused by certain prescription drugs. The symptoms of drug-induced lupus are similar to those of systemic lupus, but only rarely will any major organs be affected. The drugs most commonly connected with drug-induced lupus are hydralazine (used to treat high blood pressure or hypertension), procainamide (used to treat irregular heart rhythms), and isoniazid (used to treat tuberculosis). Drug-induced lupus is more common in men because they are given these drugs more often; however, not everyone who takes these drugs will develop drug-induced lupus. The lupus-like symptoms usually disappear within six months after these medications are stopped.
What kinds of symptoms are common in children with lupus?
There are many symptoms of lupus, some invisible and some very visible. These symptoms can be of great concern to your child, especially those that cause a change in appearance, such as hair loss, a bloated look in the face and neck, skin rashes or lesions (sores), weight gain, and easy bruising. These effects may be caused by lupus or by the medications used to treat the disease. Some of these will diminish or go away entirely when lupus symptoms are controlled by the medication, and also as the dosages of medications are lowered and discontinued. When present, though, these visible symptoms of lupus can be very upsetting to your child.
Symptoms that are invisible — muscle aches, joint pain, severe fatigue, chest pain, memory loss, and difficulty processing information — can also be difficult to deal with. There may be times when people ask you or your child questions about why such a healthy-looking young person walks with a cane or does not participate in a school activity or needs a home tutor. Be patient as you help your child learn to cope both with these effects of the disease, and other people’s reactions and attitudes
How does photosensitivity affect people with lupus?
Each person with lupus is unique on how the exposure to the UV rays of the sun or fluorescent lights may affect them. Some individuals will have new or increased skin rashes or sores (lesions). Those with systemic lupus may have increased joint pain, fatigue, fever, and flu like symptoms.
Can my medications increase my photosensitivity?
Some medications increase the effects of the sun on a person’s body. People with lupus taking these drugs including tetracycline antibiotics and many others may also very occasionally develop “phototoxic” reactions. These will lead to easy sun burning, so if you are taking these “photosensitizing” medications, you will need extra protection against sunlight. Ask your doctor or pharmacist if any of your current or new medications might make you extra-sensitive to the sun.
Should I get my car windows tinted?
Generally, individuals that have a condition that precludes sun exposure can obtain a form from their local department of motor vehicles (or the state’s public safety department) to give to their physician to be filled out and signed by their physician. Standards and regulations may differ among states. You can contact your local department of motor vehicles for information in your state.
What is ultraviolet light?
Ultraviolet (UV) light is invisible radiation from the sun that has three different ranges of wavelengths, called UVA, UVB and UVC (which does not reach us because it is absorbed by the atmosphere). In general, UVA mostly ages the skin and UVB mostly burns the skin (“A Ages, B Burns”), although UVB also contributes considerably to skin aging and cancer. Of most concern to those with lupus is the short ultraviolet wave UVB, however, broad spectrum UVA is also capable of causing skin rashes (lesions) and flare symptoms in those with lupus but to a lesser extent than UVB
How can I decrease the UV exposure from fluorescent lights?
Ultraviolet rays are also given off by indoor fluorescent lighting commonly found in offices and stores. Light shields that cover fluorescent bulbs can be ordered from several different manufacturers. If you are affected by light sensitivity, look for light shields that have nanometer readings of 380 to 400. This ensures complete filtering of UVB and UVC (especially damaging to exposed skin cells), and almost all UVA. If you are extremely sensitive to light, you may want to consider wearing a broad-brimmed hat when visiting brightly lit stores or other public places.
What are the diagnostic criteria for lupus?
To help the doctors diagnose lupus, a list of 11 common criteria, or measures, was developed by the American College of Rheumatology (ACR). ACR is a professional association of rheumatologists. These are the doctors who specialize in treating diseases of the joints and muscles, like lupus. If you have at least four of the criteria on the list, either at the present time or at some time in the past, there is a strong chance that you have lupus.
- Malar rash – a rash over the cheeks and nose, often in the shape of a butterfly
- Discoid rash – a rash that appears as red, raised, disk-shaped patches
- Photosensitivity – a reaction to sun or light that causes a skin rash to appear or get worse
- Oral ulcers – sores appearing in the mouth
- Arthritis – joint pain and swelling of two or more joints in which the bones around the joints do not become destroyed
- Serositis – inflammation of the lining around the lungs (pleuritis) or inflammation of the lining around the heart that causes chest pain which is worse with deep breathing (pericarditis)
- Kidney disorder – persistent protein or cellular casts in the urine
- Neurological disorder – seizures or psychosis
- Blood disorder – anemia (low red blood cell count), leukopenia (low white blood cell count), lymphopenia (low level of specific white blood cells), or thrombocytopenia (low platelet count)
- Immunologic disorder –anti-DNA or anti-Sm or positive antiphospholipid antibodies
- Abnormal antinuclear antibody (ANA)
What does ‘borderline lupus’ mean?
Dr. Jinoos Yazdany is a Rheumatologist specializing in systemic lupus. She currently serves as the Associate Director of the Lupus Clinic and as an Assistant Professor in Residence at the University of California, San Francisco School of Medicine.
A doctor may use the phrase “”borderline lupus”” when symptoms or blood test results suggest lupus, but there is not enough information for a definite diagnosis. This occurs commonly because some of the blood tests used to evaluate lupus, such as the anti-nuclear antibody (ANA), can occur in people without lupus. In addition, some of the symptoms of lupus, such as joint pain, can occur from a variety of causes, and so are not specific to lupus. Doctors therefore must look for a combination of criteria from the blood tests, the medical history, and the physical examination to make an accurate diagnosis.
The most commonly used criteria were established by the American College of Rheumatology. If a person meets 4 or more of the 11 criteria, then a diagnosis of lupus is likely. If a person meets fewer than the required number, a doctor may use a term such as “”borderline lupus”” or “”incomplete lupus”” or “”undifferentiated”” disease. When this occurs, it is important to continue to follow up with your doctor for monitoring.
Studies suggest that a small percentage of people in this “”borderline”” group go on to develop additional symptoms or blood work findings and are eventually diagnosed with lupus or a related condition. This progression usually occurs in the first five years. However, most people never develop additional criteria.
How is drug-induced lupus related to systemic lupus?
Dr. Robert L. Rubin is a Professor in the Department of Molecular Genetics and Microbiology at the University of New Mexico School of Medicine in Albuquerque.
Drug-induced lupus (DIL) is a side-effect of long-term use of certain medications. Specific criteria for diagnosing drug-induced lupus have not been formally established. However, symptoms often overlap with those of systemic lupus erythematosus (SLE). These include: muscle and joint pain sometimes with swelling; flu-like symptoms of fatigue and fever; serositis (inflammation around the lungs or heart that causes pain or discomfort), and certain laboratory test abnormalities. Once the suspected medication is stopped, symptoms should begin to decline within days. Usually symptoms disappear within one or two weeks. Drug-induced lupus can be diagnosed with certainty only by resolution of symptoms and their failure to recur after stopping the medication.
Does drug-induced lupus resolve when the drug is stopped?
Dr. Robert L. Rubin is a Professor in the Department of Molecular Genetics and Microbiology at the University of New Mexico School of Medicine in Albuquerque.
Once the suspected medication is stopped, symptoms of drug-induced lupus should begin to decline within days. Usually symptoms disappear within one or two weeks.
What causes the overwhelming fatigue in lupus?
As many as 80 percent of people with lupus experience fatigue. For some people with lupus, fatigue is their main symptom and can be debilitating, even to the point of forcing them to stop working. It is unclear why extreme fatigue occurs in so many people with lupus, but disease activity, pain, age, and medicines being taken, as well as poor physical and mental health and lack of good social support, all appear to play a role.
In addition, poor coping strategies, feelings of helplessness, depression or anxiety, smoking, and lack of exercise have all been found to be related to lupus fatigue. Before the physician can conclude that fatigue is related to these factors, any “treatable” causes of fatigue (for example, anemia, kidney failure, or hypothyroidism) need to be ruled out.
Physicians experienced with lupus recognize the harmful and even destructive effects that extreme fatigue can have, and research is underway to learn more about how to treat the problem. Getting regular exercise and being part of a support group have been found to help. By making some adjustments, people with lupus-related fatigue will be less likely to push themselves to exhaustion
Do fluorescent lights produce harmful ultraviolet rays?
The issue of lupus photosensitivity is a complex one and one of the least scientifically studied clinical aspects of cutaneous and systemic lupus. It is my opinion that if a standard fluorescent tube lighting source is shielded by a standard acrylic plastic diffuser, there’s virtually no significant risk for people with systemic lupus. These plastic diffusers are available from a variety of companies. However, if a person with lupus is exposed to unshielded fluorescent lighting at close distances for prolonged periods of time, then the cumulative exposure to UVB and UVA could be a problem.
Living well with lupus
How does a person with lupus know when to call the doctor?
It is very important to inform your doctor of any new symptoms you experience, as these could be related to a flare, a medication side effect, or a new complication. You should call the doctor about any change in or worsening of symptoms as soon as possible. For example, if the doctor has put you on a new medication and you’ve been taking it as prescribed, and your symptoms are not getting better or have changed or gotten worse, you need to let the doctor know.
You should also be aware of symptoms that require that you see a doctor immediately. These include the following:
- severe abdominal pain
- chest pain or shortness of breath
- new onset of a fever or if your fever is much higher than usual
- excess bruising or bleeding anywhere on your body
- confusion or mood changes
- a combination of symptoms such as severe headache with neck stiffness and fever
What happens in autoimmune diseases like lupus?
In lupus, something goes wrong with the immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu).
Normally our immune system produces proteins called antibodies that protect the body from these invaders. Autoimmune means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”) and creates autoantibodies that attack and destroy healthy tissue. These autoantibodies cause inflammation, pain, and damage in various parts of the body.
Lupus is also a disease of flares (the symptoms worsen and you feel ill) and remissions (the symptoms improve and you feel better).
Is stress related to lupus?
Emotional stress, such as a divorce, illness, death in the family, or other life complications, and anything that causes stress to the body, such as surgery, physical harm, pregnancy, or giving birth, are examples of environmental triggers that can set off lupus or bring about a lupus flare.
What do I need to know about vasculitis and lupus?
Vasculitis is an inflammation of the blood vessels. Inflammation is a condition in which tissue is damaged by blood cells entering the tissues. In inflammatory diseases, these cells are mostly white blood cells. White blood cells circulate and serve as our major defense against infection. Ordinarily, white blood cells destroy bacteria and viruses. However, they can also damage normal tissue if they invade it.
Vasculitis can affect:
- very small blood vessels (capillaries),
- medium-size blood vessels (arterioles or venules)
- large blood vessels (arteries or veins).
How Does Vasculitis Damage the Body?
Several things can happen to an inflamed blood vessel.
- If it is a small vessel, it may break and produce tiny areas of bleeding in the tissue. These areas will appear as small red or purple dots on the skin.
- If a larger vessel is inflamed, it may swell and produce a nodule which may be felt if the blood vessel is close to the skin surface.
- The inside of the vessel tube may become narrowed so that blood flow is reduced.
- The inside of the vessel tube may become totally closed, usually by a blood clot which forms at the site of inflammation.
- If blood flow is reduced or stopped, the tissues which receive blood from that vessel begin to die. For example, a person with vasculitis of a medium-sized artery in the hand may develop a cold finger which hurts whenever it is used. If untreated, this can progress to gangrene.
What Causes Vasculitis?
Vasculitis can be caused by:
- Infection of the blood vessel walls. This is rare. When it occurs, bacteria, viruses, or fungi infect the blood vessel. White blood cells move in to destroy the infectious agents and damage the blood vessel in the process. This is a serious condition and requires prompt antibiotic treatment.
- Antibodies that react against cells in vessel walls. In some cases of vasculitis, an antibody against the cytoplasm of neutrophils (anti-neutrophil cytoplasm antibodies, or ANCA) is the probable cause of the inflammation and damage.
- An immune or “allergic” reaction in the vessel walls. This cause of vasculitis is more common. Substances that cause allergic reactions are called antigens (abbreviated from anti-genetic, meaning “against genetic material”). Antigens cause the body to make proteins called antibodies which attach (bind) to the antigen for the purpose of getting rid of it. Antigens and antibodies that bind together are called immune complexes. Two primary ways in which immune complexes destroy antigens are:
o by attracting white blood cells to digest the antigen
o by activating other body substances to help destroy the antigens
Unfortunately, some immune complexes do not serve their purpose of destroying antigens. Instead, they remain too long in the body and circulate in the blood and deposit in tissues. They commonly accumulate in blood vessel walls, where they cause inflammation.
It is likely that some white blood cells (cytotoxic cells) which kill infectious agents can also accidentally damage blood vessels and cause vasculitis. ANCA probably react against white blood cells in vessel walls and increase the injury to vessels.
Which Diseases Are Associated with Vasculitis?
Vasculitis can occur in many different illnesses. Vasculitis can also occur by itself without any obvious associated infection or other illness. Some of the illnesses that can cause vasculitis are:
- rheumatoid arthritis
- polymyalgia rheumatica
- granulomatosis with polyangiitis (Wegener’s granulomatosis)
- micropolyangiitis (occurs primarily in kidneys)
- temporal arteritis
- erythema nodosum
How is Vasculitis Caused by Lupus?
The antigens causing the immune complexes in lupus are often not known. In some cases, the complexes contain DNA and anti-DNA antigens, or Ro (also called SS-A) and anti-Ro antigens.
What Are Symptoms of Vasculitis?
Vasculitis can cause many different symptoms, depending upon what tissues are involved and the severity of the tissue damage.
Some individuals will not become ill but will notice spots on their skin. Others can be very ill with systemic symptoms and major organ damage.
The symptoms in this list are grouped based on the areas of tissue in which vasculitis occurs.
Systemic symptoms can occur in many illnesses and are not specific to vasculitis:
- generally feeling bad (malaise)
- muscle and joint pain
- poor appetite
- weight loss
Common vasculitis skin lesions are:
- red or purple dots (petechiae), usually most numerous on the legs
- larger spots, about the size of the end of a finger (purpura), some of which look like large bruises
- Less common vasculitis lesions are hives, an itchy lumpy rash and painful or tender lumps.
Areas of dead skin can appear as:
- ulcers (especially around the ankles)
- small black spots at the ends of the fingers or around the fingernails and toes (nail fold infarcts)
- gangrene of fingers or toes.
Aching, swelling, and heat in joints, and arthritis with pain. Deformities resulting from this arthritis are rare.
Vasculitis in the brain can cause many complications, from mild to severe, including:
- behavioral disturbances
Peripheral nerve symptoms of vasculitis may include:
- numbness and tingling (usually in an arm or a leg, or in fingers or toes),
- loss of sensation and loss of strength (especially in the feet or hands).
- Inadequate blood flow in the intestines can cause cramping, abdominal pain, and bloating.
- If areas in the wall of the intestine develop gangrene, blood will appear in the stool.
- If a hole (a perforation) develops in the intestinal wall, peritonitis with diffuse abdominal pain may occur; surgery may be required.
- Vasculitis in the coronary arteries is unusual in lupus.
- If it occurs, it can cause a feeling of heaviness in the chest during exertion (angina), which is relieved by rest.
- Heart attacks rarely occur as a result of vasculitis.
- Vasculitis in this tissue can cause pneumonia-like attacks, with chest X-ray changes that look like pneumonia and symptoms of fever and cough.
- Occasionally, inflammation can lead to scarring of lung tissue and chronic shortness of breath.
- Vasculitis is not common in kidneys of people with lupus, even those who have lupus nephritis.
- Vasculitis in the kidneys may not cause any symptoms, although most people with renal vasculitis have high blood pressure.
- The physician will find protein in the urine and red blood cells on microscopic examination of urine (these results will be reported to the physician by a laboratory performing urinalysis).
- The physician will also order a blood test for ANCA to see if that antibody is involved in inflaming the kidney.
- Sometimes, vasculitis of the eyes causes no symptoms. Usually, however, there is visual blurring which comes on suddenly and stays, and may result in some loss of vision
- Vasculitis involving the small blood vessels of the retina (at the back of the eye) can occur in lupus, affecting the retinas’ ability to form a visual image.
- In non-lupus types of vasculitis, such as temporal arteritis, there can be sudden loss of part or all of the vision in one eye, which is usually accompanied by severe headache.
Which Type of Physicians Know About Vasculitis?
If you suspect that you or a friend or relative has vasculitis, a physician should be consulted as soon as possible. Remember:
- vasculitis can be very mild and of little importance, needing no treatment OR
- very severe and life-threatening OR
- any degree in between.
Therefore, an expert should help you decide:
- if you have vasculitis
- if so, how serious it is
- if and how it should be treated.
Doctors trained in many different specialties are taught to recognize and treat vasculitis. These include:
- rheumatologists (joints and muscles)
- general internists (the adult body)
- dermatologists (skin)
- hematologists (blood)
- nephrologists (kidneys)
- gastroenterologists (the intestinal tracts, stomach, etc.)
- infectious disease experts
- pulmonologists (lungs)
- cardiologists (heart)
- geriatricians (seniors)
- neurologists (the nervous system)
- ophthalmologists (the eyes).
It may be important for your physician to consult with other medical specialists about your case. For example, if your physician is a rheumatologist and you have visual complaints which could be indicative of vasculitis, you may be referred to an ophthalmologist.
However, it is recommended that one physician is in charge of organizing your care for two reasons: to coordinate your care, and to help you with decisions.
How is Vasculitis Diagnosed?
The diagnosis of vasculitis is based on:
- a person’s medical history
- current symptoms
- a complete physical examination
- the results of specialized laboratory tests, including ANCA.
Blood abnormalities that often occur when vasculitis is present include:
- an elevated sedimentation rate
- a high white blood cell count
- a high platelet count.
- a positive ANCA test
Blood tests can also be used to identify immune complexes or antibodies that cause vasculitis in the circulation and to measure whether complement levels are abnormal.
If there are any symptoms that suggest heart involvement, tests that may be ordered include:
- EKG (electrocardiogram)
- ECHO cardiogram
- heart scans.
For lung symptoms, the physician may order:
- chest X-ray
- blood from an artery to measure the oxygen content
- pulmonary function test, which uses a specialized machine to measure how well the lungs handle air and oxygen.
- CT scan of the lungs.
If there are abdominal symptoms, the physician may order ultrasound or CAT scans of the organs in the abdomen, and/or special X-rays of the intestines.
If the brain is affected, the physician may order CAT scans and/or magnetic resonance images (MRI), and possibly an angiogram. MRI angiograms can be done as a combined test.
Blood vessel testing
The physician can use a procedure called angiography to see if there is inflammation in medium and large arteries or veins. This can be done in any area of the body by injecting dye into the arteries or veins and viewing the outlines of the blood vessels on X-ray. MRI techniques can be modified to visualize arterial and small vessel blood flow. Ultrasound can be used to see clotting in some of the larger arteries and veins that may be inflamed.
Tissue testing (biopsy)
The diagnosis of vasculitis is most firmly made by taking a biopsy of the involved tissue and examining that tissue under a microscope. Your physician may suggest this procedure.
Other laboratory tests
The physician may also order a urine analysis, or urinalysis.
How is Vasculitis Treated?
The choice of treatment for vasculitis depends on several factors:
- the severity of the vasculitis
- your general health
- your past reactions (positive and negative) to medications.
Many cases of vasculitis do not require treatment. For example, a few spots on the skin now and then (if not combined with other symptoms) may not require any medications.
Most physicians recommend corticosteroid medications as the initial treatment for vasculitis such as prednisone, prednisolone, or methylprednisolone (Medrol®).
Some people with severe vasculitis or vasculitis that does not respond well to steroid-type drugs will need to be treated with cytotoxic drugs or rituximab. Cytotoxic drugs kill the cells that cause inflammation in the blood vessels. The most frequently used are azathioprine (Imuran®), cyclophosphamide (Cytoxan®) and mycophenolate mofetil (Cellcept). Cytotoxic medications are usually used in combination with prednisone and are often effective in treating vasculitis. A biologic drug called rituximab (Rituxan®) that depletes B cells (which make autoantibodies such as ANCA) may be a good choice for treatment, often instead of cytotoxic drugs.
Experimental procedures that have been helpful in treating some cases of vasculitis include:
- intravenous gamma globulin
- cyclosporine (cyclosporin A, Neoral®, Sandimmune®) or tacrolimus (Protopic®), medications used to prevent organ rejection in transplant patients.
Experimental therapies change frequently. Your physician can provide you with current information.
What is the Prognosis for People with Vasculitis?
There are various outcomes for people with vasculitis.
For many people, vasculitis, especially if confined to the skin, may be annoying but never life-threatening. For those individuals, life can be normal — or close to it.
A small number of people have severe vasculitis involving major organ systems. In these cases, damage may occur so rapidly that treatment does not have time to work, or the condition may be resistant to treatment. An attack of vasculitis can be permanently disabling or even fatal.
For the vast majority of people with vasculitis, treatment is very effective. However:
- the symptoms of vasculitis may disappear, only to reoccur later and require treatment again OR
- the vasculitis may be suppressed but never really go away, so that some ongoing treatment is always required.
How does lupus affect my eyes?
Systemic lupus is a chronic autoimmune disease that can affect any part of the body, including the eyes. Lupus most often affects the heart, joints, skin, lungs, blood vessels, kidneys and central nervous system (CNS). The clinical course is unpredictable and is characterized by periods of remissions and flares, which may be acute or chronic.
The effects lupus may have in and around the eyes include2:
- Changes in the skin around the eyelids
- Dry eyes
- Inflammation of the white outer layer of the eyeball (scleritis)
- Blood vessel changes in the retina — the light-sensitive lining inside the eye
- Damage to nerves in the muscles controlling eye movement and the nerves affecting vision
Involvement of the Skin Around the Eyelids
- This is most often related to the discoid lupus erythematosus3 form of cutaneous lupus.
- The skin lesion is well-defined, slightly raised, scaly, and misformed (atrophic).
- There are typically no symptoms, but occasional burning and itching may occur.
- Scarring may result in deformities along the edge of the eyelids.
- Approximately 20 percent of people with lupus also have secondary Sjögren’s syndrome, a condition in which the tear glands do not produce sufficient tears to lubricate and nourish the eyes; the other moisture-producing glands are similarly affected. (Primary Sjögren’s syndrome is a systemic disease that, like lupus, can affect many parts of the body.)
- Typical symptoms are irritated, gritty, scratchy, or burning eyes, a feeling of something in the eyes, excess watering, and blurred vision.
- Advanced cases of dry eyes may result in damage to the front surface of the eye and impaired vision.
- The dry eye that is seen in lupus cannot be distinguished from other dry eye conditions.2,4
- This painful red eye condition is caused by inflammation in the white scleral (outer) layer of the eye.
- Scleritis occurs in approximately one percent of people with lupus and may be the first sign of the disease.
Retinal Vascular Lesions (blood vessel changes in the retina)
- This is the most common form of eye involvement in lupus.2
- The occurrence in lupus can vary depending on the population studied. The lowest incidence reported is three percent, seen in outpatient clinics, and the highest is 28 percent in those hospitalized for lupus-related complications.2
- The presence of these lesions seems to correlate with active disease.
- Retinal blood vessel changes are due to lack of adequate blood supply to this delicate tissue, and may cause decreased vision ranging from mild to severe. For individuals with severe retinal vascular disease, the prognosis for vision is poor.
- Retinal vein occlusions (blockages) and retinal artery occlusions have been reported, but these complications are rare and seem to be more related with CNS lupus.
- The choroidal layer of the eye — the nourishing tissue underneath the retina — can also be affected by lupus, but this is very uncommon. This involvement can appear as excess fluid between the retinal layers. There is an association between lupus choroidal disease and blood vessel disease in the rest of the body, which may be related to kidney disease and blood vessel disease complications seen in lupus.
Neuro-ophthalmic Involvement (nerve damage)
- Cranial nerve palsies can result in double vision, poor eye movement and alignment, poor pupil reflexes, and droopy eyelids.
- Lupus optic neuropathy occurs in one-two percent of people with lupus.2 Slow progressive vision loss also can result in more rapid loss of vision from lupus optic neuropathy.6
- Damage to the visual nerve fibers in the brain may cause hallucination and loss of peripheral vision and/or central vision.
Side-effects of Certain Lupus Medications
In addition, some of the medications used in the treatment of lupus may have ocular side effects. In particular, hydroxychloroquine (Plaquenil®) can cause retinal toxicity over time.
Annual comprehensive eye examinations are recommended by the American Optometric Association for people with lupus, especially for anyone taking Plaquenil.
What is photosensitivity?
Photosensitivity is the term used to describe sensitivity to the ultraviolet (UV) rays from sunlight and other light sources, such as indoor fluorescent light. Photosensitivity can cause rashes, fever, fatigue, joint pain, and other symptoms in people with both cutaneous and systemic lupus. UV rays are especially intense between the hours of 10 a.m. and 4 p.m., at higher altitudes, and in or around the snow and the water. Excess exposure to UV rays is a common trigger for increased disease activity (flare) of both cutaneous lupus and systemic lupus.
How can I manage my fatigue?
As many as 80 percent of people with lupus experience fatigue. For some people with lupus, fatigue is their main symptom. Fatigue can be debilitating, even to the point of forcing them to stop working.
It is unclear why extreme fatigue occurs in so many people with lupus, but the following appear to play a role:
- Disease activity
- Current medications
- Poor physical and mental health
- Lack of good social support
Poor coping strategies, feelings of helplessness, depression or anxiety, smoking, and lack of exercise can also be related to lupus fatigue. Before a physician can conclude that fatigue is related to these factors, he or she will rule out any “treatable” causes of fatigue such as anemia, kidney failure, or hypothyroidism.
Physicians experienced with lupus recognize the harmful and even destructive effects that extreme fatigue can have, and research is underway to learn more about how to treat the problem. Getting regular exercise and being part of a support group can help.
By making some adjustments, people with lupus-related fatigue will be less likely to push themselves to exhaustion.
Tips to Help You Manage Fatigue
- Alternate activities with periods of rest throughout the day.
- Establish good sleep patterns.
- Plan ahead; for example, shop for gifts throughout the year.
- Prepare meals in advance.
- Eat a healthy diet and exercise regularly.
- If you smoke, stop.
How does lupus affect the blood?
Dr. Michael Rosove is a Medical Oncologist in Los Angeles, CA and a Clinical Professor of Medicine at the University of California-Los Angeles, School of Medicine.
Blood is made up of many different parts, but those that are most often affected by lupus are the red blood cells, the white blood cells, and the platelets. Blood disorders are common in lupus.
The main issues having to do with lupus and the blood are:
- Anemia: low hemoglobin or red blood cells
- Thrombosis: excess blood clotting
- Blood transfusions
- Bone marrow testing
Hematologists, who are specialists in blood disorders, are often involved in the evaluation and treatment of individuals with lupus.
The Lupus Foundation of America would like to thank Michael Rosove, MD, for his input.
What are the common symptoms of lupus?
Because lupus can affect so many different organs, a wide range of symptoms can occur. These symptoms may come and go, and different symptoms may appear at different times during the course of the disease.
The most common symptoms of lupus, which are the same for females and males, are:
- Extreme fatigue (tiredness)
- Painful or swollen joints
- Anemia (low numbers of red blood cells or hemoglobin, or low total blood volume)
- Swelling (edema) in feet, legs, hands, and/or around eyes
- Pain in chest on deep breathing (pleurisy)
- Butterfly-shaped rash across cheeks and nose
- Sun- or light-sensitivity (photosensitivity)
- Hair loss
- Abnormal blood clotting
- Fingers turning white and/or blue when cold (Raynaud’s phenomenon)
- Mouth or nose ulcers
Many of these symptoms occur in other illnesses. In fact, lupus is sometimes called “the great imitator” because its symptoms are often like the symptoms of rheumatoid arthritis, blood disorders, fibromyalgia, diabetes, thyroid problems, Lyme disease, and a number of heart, lung, muscle, and bone diseases.
You play an important role in helping your doctor manage your disease. Listen to your body, ask questions and stay involved
Can I still work after my lupus diagnosis?
No matter the area of expertise, our achievements and accomplishments in the workplace contribute to our self-image and identity. It’s not surprising that people with lupus, especially soon after being diagnosed, often wonder if their illness will affect their ability to contribute in the workplace.
Many people with lupus are able to continue to work, although they may need to make changes in their work environment. Flexible work hours, job-sharing, and telecommuting may help you to keep working. It may be helpful to begin to make such arrangements soon after you have been diagnosed with lupus.
If you work in an office setting, changes may include:
- modifying your workstation to relieve physical stress factors
- placing light shields over fluorescent bulbs and anti-glare filters on computer screens
- using ergonomic keyboards and desk chairs
- having a couch available for periods of rest
If you work in outdoor occupations, changes may include:
- taking on tasks that are less physically demanding
- having more frequent rest periods
- avoiding the sun at midday
It’s understandable that you might not want to make your illness a matter of general knowledge among colleagues. You may be concerned that telling your employer about a lupus diagnosis might call into question your effectiveness in your job, or might somehow decrease your value as an employee.
In confronting these work-related issues, people with lupus have a valuable resource in the Americans with Disabilities Act (ADA). Passed in 1990, the ADA makes it against the law for an employer to discriminate against a qualified individual with a disability. Chronic illness, lupus included, is recognized as a disability for the purposes of administering the law. The law requires employers to make reasonable accommodations to enable a disabled employee to perform his or her job (modifications to work stations, assistive equipment, flexible work schedules, changes in job location, etc.). However, exactly what is “reasonable” can be a matter of interpretation, and sometimes a dispute can arise between employee and employer. The most important thing to know is that the ADA provisions apply only if the employer has been made aware of the employee’s disability.
Sometimes the physical and/or mental demands of your job may become too much for you to handle on top of the many physical and emotional changes that lupus can cause. You might benefit from changing to another job, or switching to part-time hours at your current job.