Lupus is a common autoimmune disorder affecting at least 5 million people worldwide, most commonly women of childbearing age. Lupus causes inflammation and can affect almost every tissue in the body including the skin, joints, and organs.

What is Lupus?

Lupus is a chronic inflammatory autoimmune disorder that appears in several forms. The disease is chronic and causes inflammation and pain which affects the skin, joints, and internal organs. Lupus can affect many different parts of the body which causes a variety of different symptoms that vary from person to person. The most common early signs and symptoms of lupus are extreme fatigue, joint pain, and a butterfly-shaped rash on the face. There is no cure for lupus, but with proper treatment many people can experience remission of symptoms.

Types of Lupus

There are several different types of lupus with different presentations and degrees of organ system involvement.

Systemic Lupus Erythematosus (SLE): this is the classic and most common form of lupus. SLE accounts for around 70% of all cases of lupus and is what this article focuses on.
Discoid lupus erythematosus (DLE): this is also known as cutaneous lupus and affects only the skin. Rashes that are coin-like in appearance appear mostly on the face, neck, and scalp.
Neonatal lupus: this is a rare condition that affects infants of women with lupus. At birth, the baby may present with a skin rash, liver problems, or low blood cell counts, but these symptoms usually disappear within 6 months without any lasting effects. With proper testing, physicians can identify mothers who are at risk and the infant can be treated either before or at birth. Most infants of mothers with lupus are entirely healthy.
Drug-induced lupus: signs and symptoms of lupus can appear only while the person is taking a specific drug. The drugs that most commonly cause drug-induced lupus are hydralazine used to treat hypertension and procainamide used to treat arrhythmia (4). The symptoms ease when the drug is stopped.
Latent lupus: latent lupus describes a constellation of features suggestive of SLE but does not qualify as classic SLE. Many people with latent lupus persist with their clinical presentation of signs and symptoms over many years without ever developing classic SLE.
Antiphospholipid antibody syndrome: Antiphospholipid antibody syndrome can occur as part of the clinical manifestations seen in SLE or it may occur as a primary form without other clinical features of lupus. Symptoms include arterial and venous thrombosis, recurrent fetal loss, and immune thrombocytopenia with a variety of antibodies directed against cellular phospholipids (fats in cell membranes that contain phosphorous).
Late-stage lupus: this presentation is defined as chronic disease duration of longer than 5 years. Usually additional symptoms present due to long-term complications of SLE or that result either from the disease itself or as a consequence of its therapy. These late complications may include end-stage renal disease, atherosclerosis, pulmonary emboli, and avascular necrosis. In late-stage lupus, cognitive disabilities can be a common manifestation, even if there is no other evidence of active disease.

Summary: There are several different presentations of lupus including symptoms that occur only while taking certain medications and late-stage lupus which occurs when symptoms last longer than 5 years, causing more complications than typical lupus.

Signs and Symptoms

Generally, SLE is more severe in presentation than discoid lupus, though no two people with SLE will have identical symptoms. For some people, only the skin and joints will be involved. For others, the joints, kidneys, blood, or other organs and tissues may be affected.

The most common signs and symptoms of lupus include:

Extreme fatigue, 76% of lupus patients say fatigue caused by lupus has forced them to cut back on social activities (1)
A butterfly-shaped rash on the cheeks and nose
Pain or swelling in the joints, 65% of people with lupus say chronic pain is the most difficult part of having lupus (2)
Swelling in the hands, feet, or around the eyes
Headaches
Low fevers
Sensitivity to sunlight or fluorescent light
Chest pain when breathing deeply
Hair loss
Sores in the mouth or nose

Lupus affects many different body systems and causes a variety of other symptoms. These systems include musculoskeletal, integumentary, cardiopulmonary, central nervous, and renal among others.

Musculoskeletal System

Joint pain is the most common symptom of SLE. Acute joint pain can involve any joint but typically affects the small joints of the hands, wrists, and knees. Symptoms at the joints may be chronic in one area or can migrate to different locations. Most cases of joint pain in SLE are symmetrical, meaning if one joint is affected the same joint on the opposite side of the body will also be symptomatic. Though some people with SLE do report asymmetrical presentations.

Unlike other types of inflammatory arthritis, the joint pain in SLE does not cause erosion or destruction to the bone. Symptoms are not usually severe enough to cause joint deformities, but there may be temporary functional impairments due to joint pain. When deformities do occur, the most common are ulnar deviation of the wrist, swan-neck deformities of the fingers, fixed subluxations of the fingers, and tendon abnormalities such as tenosynovitis or rupture.

Lupus often causes myalgia, or aches and pains in the muscles. Rarely, lupus may also cause myositis which is an inflammation in the muscle. This most commonly occurs in the hips, thighs, shoulders, and arms. When the muscles are inflamed, they also become weak making it hard to perform normal daily activities such as standing up out of a chair or raising your arms above your head.

Many patients with lupus also develop osteoporosis which means low bone density. Osteoporosis makes your bones weak and more likely to break. Osteoporosis has no symptoms so the only way to know is to get a bone density test called a DEXA scan. This will measure how strong your bones are. If you have osteoporosis your doctor can recommend medications to improve your bone density.

Integumentary System

The most common manifestation of lupus in the integumentary system is the classic butterfly rash over the nose and cheeks. Rashes occur most commonly in areas that are exposed to sunlight and may be exacerbated by the use of cosmetic products containing alpha hydroxy acids.

Discoid lesions associated with DLE are raised, red, scaling plaques with follicular plugging and central atrophy. This raised edging and sunken center gives them a coin-like appearance.

Vasculitis (inflammation of cutaneous blood vessels) involving small and medium size vessels may cause other skin lesions, such as ulcers or gangrene at the finger tips (death of body tissue due to lack of blood flow).

Raynaud’s phenomenon occurs in about 20% of people with lupus (3). Blood vessels go into temporary spasm when exposed to cold air causing a temporary lack of blood flow. The most affected areas are the fingers, toes, ears, and nose. The affected areas turn white, then then blue, then red as the blood flow returns. Symptoms include the affected areas feeling numb and cool in response to cold temperatures or stress.

Diffuse or patchy alopecia (hair loss) may be a temporary symptom of SLE. Hair usually regrows once the disease activity of SLE is under control. However, permanent hair loss can occur if extensive scarring on the scalp happens with discoid lesions.

Painless ulcers of the mucous membranes are also a common symptoms of SLE involving the mouth, nose, and vagina.

Cardiopulmonary System

Signs of cardiopulmonary abnormalities may develop, such as pleuritis (inflammation in the chest wall), pericarditis (inflammation surrounding the heart), and dyspnea (shortness of breath). Other more serious presentations include inflammation of the heart muscle and/or lung tissue.

Pulmonary hypertension and congestive heart failure are less common with people diagnosed with SLE and are usually secondary to a combination of factors. Though if antiphospholipid antibody syndrome is present, there is a high risk of thrombosis (blood clot).

Central Nervous System

A significant number of people with SLE will have central nervous system involvement at some point in their illness, sometimes referred to as neuropsychiatric manifestations. Symptoms may be related to specific autoantibodies that react with nervous system antigens or cytokine-mediated brain inflammation and include headaches, irritability, and depression.

The pattern of cognitive dysfunction in those with SLE is varied and intensity can vary within the same person. Cognitive symptoms can also be affected by mood. SLE may cause difficulties with verbal memory, attention, language skills, and psychomotor speed. Progressive cognitive impairment, sometimes subtle and sometimes obvious, may develop even in the absence of clinically diagnosed episodes of neuropsychiatric disease. People with SLE may or may not have other signs of lupus when they experience neurologic symptoms.

Emotional instability, psychosis, seizures, cerebrovascular accidents (stroke), cranial neuropathy, peripheral neuropathy, and organic brain syndrome can also occur. Organic brain syndrome is a state of diffuse cerebral dysfunction associated with a disturbance in consciousness, cognition, mood, affect, and behavior. Return to the previous level of cognitive function may follow remission of the neuropsychiatric flare, or permanent cognitive impairment may occur.

Renal System

Pathologic changes may also occur in the kidneys which include hematuria (blood in the urine) and proteinuria (high levels of protein in the urine). Renal effects may progress to kidney failure and if severe, may be life-threatening.

Other Systems

Common early signs of SLE include fever, weight loss, a general feeling of being unwell, and fatigue. Anemia is another common finding in those with SLE which is associated with absent menstruation in women. Sometimes the spleen and lymph nodes can become enlarged. Nausea, vomiting, diarrhea, and abdominal pain may occur with GI involvement. Hepatitis may also develop.

All symptoms mentioned in this section can occur at the onset or at any time during the course of lupus. Nearly all people with SLE experience fluctuations in disease activity with exacerbations and periods of remission, without disease activity.

Summary: Lupus can affect almost any tissue in the body. Joint pain, swelling, extreme fatigue, and a butterfly-shaped rash across the cheeks and nose are hallmark symptoms. Lupus can also affect the heart, kidneys, and central nervous system.

Causes

The cause of SLE remains unknown, but evidence points to a combination of immune, environmental, hormonal, and genetic factors. The primary causative factor is thought to be immune system dysregulation causing autoimmune disease. The body produces antibodies against in own cells and in SLE the antibodies are produced against many different tissues such as red blood cells, immune cells, or almost any other organ or tissue in the body. This wide range of targets has classified lupus as being a disease of generalized autoimmunity.

SLE also shows a strong familial link with a much higher frequency among first-degree relatives. There is large degree of evidence in support of genetic influence for developing lupus (5). There are dozens of known genetic variants linked to lupus and these genes impact both who gets diagnosed as well as how severe the disease activity is. Around 20% of people with lupus will have a parent or sibling who already has lupus or may develop lupus. Around 5% of the children born to individuals with lupus will develop the illness.

Summary: The main causes of lupus are immune system dysregulation and genetics. Many people diagnosed with lupus will have a parent or sibling with the disease.

Risk Factors

SLE is primarily a disease of young women and it is rarely found in older people. 9 out of 10 people with lupus are women. It usually develops in women of childbearing years, but many men and children also develop lupus. Other risk factors include:

Certain ethnicities have a 2-3 times higher risk of developing lupus (African American, Hispanic/Latina, Asian American, Native American, Alaska Native, Native Hawaiian, and other Pacific Islanders) (6)
Having a first-degree relative who is diagnosed with lupus
High physical or mental stress levels
History of streptococcal or viral infection
Excessive exposure to sunlight or ultraviolet light
Abnormal estrogen metabolism
History of Epstein-Barr viral infection
Taking certain drugs (hydralazine, anticonvulsants, penicillins, sulfa drugs, and oral contraceptives)

Summary: There are several risk factors for lupus including being female in childbearing years and having a family member with lupus. Certain ethnicities such as African American and Hispanic, and Pacific Islanders are more likely to be diagnosed with lupus.

Diagnoses

Diagnosis of lupus is difficult because it often mimics other diseases and the symptoms are often vague and vary greatly from individual to individual. Lupus symptoms can come and go and can change which contributes to the difficulty in diagnosis. On average, it takes nearly six years for people with lupus to be diagnosed, from the time they first notice their lupus symptoms. Around 63% of people with lupus surveyed report being incorrectly diagnosed and more than half of those people report seeing four or more different healthcare providers for their symptoms before being accurately diagnosed (7).

The American Rheumatism Association has issued a list of criteria for classifying SLE. Usually four or more of these signs are present at some time during the course of disease:

Abnormal titer of anti-nuclear antibodies (blood test): present in all cases of lupus
Butterfly rash over the cheeks and nose
Discoid rash (raised, red, scaling plaques with sunken centers)
Hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia (low blood cell counts)
Neurological disorder: seizures or psychosis
Nonerosive joint pain of two or more peripheral joints characterized by swelling, tenderness, or effusion
Oral or nasopharyngeal ulcerations
Photosensitivity (skin becomes sensitive to light)
Pleuritis or pericarditis (lung or heart inflammation)
Positive lupus erthematosus cell preparation, anti-DNA, or anti-splice sosomal test or chronic false-positive serologic test for syphilis (specialized tests done by your doctor)
Renal disorder: proteinuria (protein in the urine >.5g/day) or excessive cellular casts in urine

During your initial examination with your doctor, they will ask you questions about your symptoms such as:

What symptoms are you having?
How often do you have these symptoms?
When did your symptoms start?
Does anything make your symptoms better or worse?
Are your symptoms constant or do they come and go?
Do your symptoms get worse at a certain time of day?
Do your symptoms get in the way of your daily routine?
Does anyone in your family have lupus or other autoimmune disease?

Your doctor will also conduct a thorough physical exam including looking at your skin, range of motion, strength, and movement patterns.

In addition to the routine history and physical examination done by your doctor, laboratory tests are an important part of the diagnosis of SLE and are used for subsequent monitoring of disease activity. Your doctor will check for changes in your body, like inflammation, that could be caused by lupus. Inflammation usually happens when your immune system is fighting and infection or an injury. When lupus makes your immune system attack healthy tissue, it can cause inflammation in lots of different body parts. This leads to symptoms including swelling, pain, and stiffness.

Blood tests can help doctors see things like how the immune system is working or if there are signs of inflammation in the body. Blood tests that your doctor might order include:

A complete blood count (CBC): measures the number of red and white blood cells and platelets (cells that help your blood clot) in your blood
Antibody tests: help reveal if your immune system is attacking healthy tissue (97% of people with lupus test positive for antinuclear antibodies)
Blood clotting time tests: looks at how well your blood is clotting
Complement tests: check for signs of inflammation

Urine tests can help doctors see if there are issues with your kidneys. Your doctor may test your urine once or test it many times to check for changes. The most common urine tests look for cell casts which are bits of cells that normally get removed when your blood is filtered through your kidneys. Your doctor will also be looking for protein being spilled into your urine because the kidneys are not filtering the waste properly, which is called proteinuria.

Your doctor may also order a biopsy where a small piece of tissue from different parts of your body are removed and then examined under a microscope. Almost any tissue can be biopsied, but the most common sites are the skin and kidney in people who may have lupus. The doctor can then check that tissue to see if there are any signs of inflammation or damage.

Imaging can also be useful in diagnosis and MRI scans of the head are usually ordered for all people experiencing new episodes of neurological symptoms such as seizures, altered consciousness, or psychosis. Neurophysiological assessment may be helpful for identifying subtle consequences of central nervous system events such as stroke, and in the monitoring of response to drug treatment.

Lab work alone cannot usually diagnose lupus. Your doctor will take a positive antibody test accompanied by several other clues to come to a diagnosis. It is positive for positive lab tests to come and go over time so it makes it less likely to receive a lupus diagnosis. It’s also possible to get somewhat different results at different labs. If your doctor rules out lupus but you continue to have signs and symptoms, talk to your doctor or seek additional medical help. Whether or not its lupus, it is important to address signs of inflammation and pain.

Summary: Lupus is diagnosed with a combination of laboratory tests including blood tests, urine tests, and biopsies, imaging, medical history and physical exam.

Treatment Options

While there is no cure for lupus right now, having the right treatment plan can help control symptoms. Treating lupus is a team effort and will likely include several health professionals. The goals of treatment are to control symptoms like joint pain, swelling, and feeling tired, keeping the immune system from attacking tissues in the body, and protecting organs from damage. There are several different treatment options for the medical management of lupus. These options include medication, physical therapy, diet, lifestyle and activity modifications.

Medication

The objectives of medical intervention for lupus are to reverse the autoimmune and inflammatory processes and prevent exacerbations and complications of the disease. At the present time, medication management is the primary means of accomplishing these goals. The following are drug treatment options for managing lupus:

Non-steroidal anti-inflammatory drugs (NSAIDs): mild symptoms can be managed with over-the-counter NSAIDs such as Ibuprofen or Naproxen. These drugs work to relieve muscle and joint pain while reducing inflammation. These drugs are taken orally.
Corticosteroid-sparing agents: this class of medication is used earlier to preserve bone and offer protection from premature cardiovascular disease. Examples include Methotrexate. These drugs can be given orally or by injection.
Anti-coagulants: these drugs are used for individuals who have antiphospholipid antibody syndrome and reduce the risk of blood clots. They are taken orally.
Antimalarial agents: this class of drugs is useful against skin, joint, and renal symptoms of lupus. They can help to protect skin from rashes and UV light. Examples include chloroquine (Aralen) and hydroxychloroquine (Plaquenil). They are taken orally.
Immunomodulating drugs: these drugs suppress the immune system to reduce disease activity and lower inflammation levels. They are used only with active disease and caution needs to be taken if there is kidney involvement due to side effects of the drugs. They are taken orally or by injection.
Corticosteroids and cytotoxic drugs are given in more severe disease that has not responded to other types of drug therapy. Steroids help control inflammation and cytotoxic drugs suppress the immune system to reduce disease activity. These drugs have more severe side effects than other medication management so are not usually the first line of treatment.

Lifestyle Modifications

It is important to protect your joints, muscles, and bones when you have lupus. When you have joint pain, it can be hard to be active, but when you are less active, your muscles get weaker, making your joint pain worse. It is important to have some form of regular physical activity to maintain muscle strength and cardiovascular fitness. Working with a physical therapist can help you set up an activity plan that’s right for you.

Protecting your bones is also important to lower your risk of osteoporosis. Lifestyle modifications can help including:

Making a plan to quit smoking
Drinking alcohol only in moderation
Eating foods with plenty of calcium and vitamin D such as milk and leafy greens
Getting regular physical activity
Getting a bone density test every 1 to 2 years

Physical Therapy

Physical therapists can be instrumental in those with lupus by helping people modify their activities, avoid excessive fatigue, improve endurance and strength, and protect their joints. Disease activity can be managed with carefully controlled and sometimes restricted activities, as programmed by your physical therapist. Physical therapists can teach clients with lupus how to pace activities and conserve energy.

Energy conservation is an organized procedure for finding ways to reduce the amount of effort and energy needed to accomplish a given task. It is especially important for those with lupus that deal with generalized fatigue. By reducing the amount of energy needed to accomplish a task, more energy is available. Physical therapists can help people with lupus make the following changes:

Schedule the most strenuous activities during periods of highest energy
Before starting any activity, analyze the task and answer the following questions
- Is the task necessary?
- Can it be eliminated or combined?
- Am I doing this out of habit?
- Can it be simplified by combining or eliminating steps?
- Can a larger job be divided into smaller tasks?
- Are there any assistive devices or tools that could make the task easier?
- Can this be done by someone else?
Alternate more strenuous tasks with easier ones
Plan frequent rest periods, sit down, or take naps as needed
Cluster activities so that it is not necessary to make frequent trips or walk long distances at home, school, or work
Avoid or keep to a minimum repetitive climbing of stairs
Keep certain tasks, such as housekeeping, to a minimum
Sit down to perform activities of daily living such as tooth brushing or hair combing
Avoid sitting on low or soft furniture that requires more energy expenditure to get up

Physical therapists can also help those with lupus follow a prescribed exercise plan to help improve strength and endurance. Excessive best rest can worsen fatigue, promote muscle disuse and atrophy, and promote osteoporosis, so it is important to have some form of regular exercise. Physical therapists can help prescribe exercises that will strengthen muscles while avoiding undue stress on inflamed joints and balancing activity with rest to avoid excessive fatigue.

Whether you choose primarily medication management, alternative treatment, or a combination of options, there are things that you can do to help make your treatment plan work well. Playing an active role in your health care with these tips can help make your treatment plan the most effective:

Use a journal to keep track or your medicines with the dosage and any side effects you notice
Let your treatment team know if you have any side effects or if your symptoms change after starting a new medication, whether they improve or get worse
Keep everyone on your team updated about changes in your treatment plan, for example, if you start a new medication or stop taking one
Write down questions about your treatment for the doctor ahead of time and take them to your appointments
Ask a family member or friend to go with your to appointments for support and to help keep track of questions and information about your treatment

Summary: The treatment for lupus involves a combination of medication, activity and lifestyle modifications. There are several different types of medications used to treat lupus with the goal of reducing inflammation and symptoms and preventing complications from the disease.

Prognosis

The prognosis today for lupus is better than in the past. With close follow-up and treatment, 80-90% of people with lupus can expect to live a normal lifespan. The prognosis for lupus improves with early detection and intervention that prevents organ damage and can improve life expectancy.

Though people with lupus do have an increased prevalence of valvular and atherosclerotic heart disease, caused by the disease activity of lupus as well as the drug therapy necessary in severe cases. In those with symptomatic large vessel occlusive disease, poor short-term outcomes are expected. Prognosis is also less favorable for those who develop cardiovascular, renal, or neurologic complications or severe bacterial infections. High stress, poor social support, and psychological distress are also associated with poor outcomes in those with SLE (8).

African American lupus patients are more likely to have organ system involvement, more active disease, and lower levels of social support compared to Caucasians with lupus. People from racial and ethnic minority groups also tend to develop lupus at a younger age, experience more serious complications, and have higher mortality rates.

In those that do not have severe organ involvement, a normal lifespan is expected if they:

Follow the instructions of their physician
Take their medication(s) as prescribed
Know when to seek help for unexpected side effects of a medication or a new manifestation of lupus

Summary: 80-90% of people with lupus will live a normal lifespan. Prognosis improves with early diagnosis and treatment and frequent medical checkups.

Prevention

There is no known way to prevent lupus, but preventative measures can reduce the risk of flare-ups. For photosensitive people, avoidance of excessive sun exposure and/or the regular application of sunscreen usually prevents rashes. Regular exercise helps prevent muscle weakness and fatigue. Immunization can protect against specific infections that increase the risk of lupus.

Support groups, counseling, and reliance on family members, friends, and healthcare professionals can help alleviate the effects of stress. Lifestyle choices and personal behavior are very important for people with lupus as well. These include avoiding smoking, excessive alcohol consumption, taking medication not as prescribed, and not attending regular medical checkups.

Complications

There are several complications from SLE and the medication management used to control symptoms. Its important to be aware of the following complications:

Skin scarring from discoid lesions
Kidney failure (9)
Stroke
Heart attack
Pregnancy complications
Avascular necrosis, temporary or permanent blood loss to an area of bone, most common in the hip
Cataracts
Bone fractures
Sjogren’s syndrome, as many as 10% of people have this syndrome characterized by dry eyes and mouth
Thyroid disease, both hypothyroidism and hyperthyroidism is more common in people with lupus

Cardiovascular disease, not lupus itself, is the number one cause of death in people with lupus. The number two cause of mortality in people with lupus is infection.

Summary: Serious complications can happen with lupus including stroke, kidney failure, and infections.

Economic Impact

The average annual direct health care costs of a person with lupus was over $33,000. The average annual total lost hours of economic productivity due to lupus was between $1,200 and $20,000. Combining direct and indirect costs the annual costs can be as high as $50,000 for those with lupus. These estimates may be even higher for those with more severe or active forms of lupus (10).

Around 55% of people with lupus reported a complete or partial loss of their income because they are no longer able to work full-time due to lupus symptoms. One in three have been temporarily disabled by the disease and one in four currently receive disability payments. One in four also receive their health care through government-sponsored programs such as Medicare or Medicaid.

Summary: The economic impacts of lupus are high with around 55% of people with lupus reporting complete or partial loss of income due to symptoms of the disease. The financial impact can be up to $50,000 annually for people diagnosed with lupus.

Conclusion

Lupus is an autoimmune condition that affects a variety of tissue in the body, most commonly the skin and joints. Extreme fatigue, pain and swelling in the joints, and a butterfly-shaped rash over the cheeks and nose are the most frequent symptoms. Lupus is caused by a combination of genetics and immune, environmental, and hormonal factors. Risk factors for lupus include being female in childbearing years, having certain ethnicities, and having a family member with lupus. Treatment typically involves a combination of medication, activity, and lifestyle modifications. Most people with lupus can live a normal lifespan, but serious complications do exist. The economic impacts of lupus are high due to inability to work and lower productivity. If you have symptoms or risk factors for lupus, talk to your doctor today to get screened and begin treatment to avoid compliations.

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