- Also Known As:
- Systemic Lupus Erythematosus
- Disseminated Lupus Erythematosus
- Lupus Erythematosus
- Discoid Lupus
- Subacute Cutaneous Lupus
- Drug-induced Lupus
- Neonatal Lupus
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What is lupus?
Lupus is a chronic, inflammatory autoimmune disorder. It is a condition in which the immune system, which normally protects the body from infections, produces an inappropriate immune response against its own tissues. Lupus may affect the skin, joints, blood vessels, and internal organs, especially the kidneys, heart, lungs, and brain. There are several types of lupus; the most common is systemic lupus erythematosus (SLE), which affects many areas of the body.
Anyone can get lupus at any time, but it most commonly affects women of childbearing age. It is diagnosed in 10 times more women than men and is more common in persons of African, Asian, Hispanic, and Native American descent. SLE is most frequently seen in people between the ages of 15 and 44, although children, including newborns, and older adults can also have lupus.
The cause of lupus is not fully understood. It is thought to involve both an inherited component and a trigger that may be related to environmental factors, such as exposure to sunlight, and/or the use of certain medications and viral infections. Lupus may co-exist with other autoimmune disorders, such as Sjögren syndrome, some cases of hemolytic anemia, Hashimoto thyroiditis, and idiopathic thrombocytopenia purpura (ITP; see Bleeding Disorders – Platelet disorders).
There are many types of lupus. Some of these are listed below.
- Systemic lupus erythematosus (SLE)–according to the Centers for Disease Control and Prevention, the incidence is difficult to establish due to vague signs and symptoms and onset. According to the National Resource Center on Lupus, more than 16,000 new cases of SLE are reported in the U.S. each year, and at least 1.5 million Americans are currently living with lupus. Ninety percent of newly diagnosed patients are women of child-bearing age. It affects many parts of the body (systemic).
- Discoid lupus–this type is characterized by a chronic skin rash, such as on the face or scalp; about 15% to 20% of people with this condition will progress to SLE.
- Subacute cutaneous lupus–associated with skin lesions on parts of the body that are exposed to sunlight.
- Drug-induced lupus–a form of lupus that can be caused by certain medications, such as some anti-seizure, high blood pressure, and anti-thyroid medications. The most common medications known to cause drug-induced lupus are the antibiotic isoniazid (used to treat and prevent tuberculosis), hydralazine (used to treat hypertension or high blood pressure), and procainamide (used to treat abnormal heart rhythms). Symptoms tend to occur after taking the medication for several months and usually resolve once the medication is stopped.
- Neonatal lupus–a rare form of lupus that affects newborns and that is characterized by a skin rash, liver problems, and low blood counts at birth. These usually resolve over several months. Newborns who have neonatal lupus may be born to women who have SLE, Sjögren syndrome, or no particular disease, but it is thought that it may be triggered in part by certain autoantibodies in the mother’s blood (anti-SSA and anti-SSB), which cross the placenta into the fetus’s/infant’s bloodstream and can persist for several months. Women known to have these autoantibodies may be monitored more closely during their pregnancy.
Signs and Symptoms
Signs and symptoms of lupus vary from person to person and by the type of lupus.
People with lupus may develop a rash, such as:
- A rash resembling a butterfly that appears across the nose and cheeks (malar rash)
- A red rash consisting of round or oval-shaped patches (discoid rash)
- A rash on areas of the skin that are exposed to sunlight (typically the face and extremities)
A rash is typically the only symptom in discoid and subacute cutaneous lupus. People with other types of lupus may have a combination of the following additional signs and symptoms:
- Muscle pain
- Arthritis-like pain in one or more joints (but no or little joint damage)
- Persistent fatigue
- Swollen lymph nodes
- Sensitivity to sunlight
- Raynaud phenomenon (fingers change color after exposure to cold temperatures or with stress)
- Hair loss
- Chest pain
- Mouth ulcers
- Inflammation and damage to organs and tissues, including the kidneys, lungs, heart, lining of the heart, central nervous system, and blood vessels
Symptoms of lupus may come and go over time and vary from person to person. They may worsen abruptly and then die down. Flare-ups may be triggered by changes in someone’s health status, such as physical or emotional stressors, and/or by outside stimulants such as exposure to sunlight. Women may experience flare-ups during pregnancy or shortly after giving birth.
People with lupus are at increased risk of infections, such as urinary tract infections, respiratory infections, yeast infections, salmonella, herpes and shingles, due to their weakened immune system from both the condition and its treatments. They are also at increased risk of cancer, bone tissue death, and pregnancy complications, including miscarriage and pre-eclampsia.
Tests and Diagnosis
Diagnosis of lupus can be challenging, especially because of its wide range of symptoms that may come on slowly and change over time. It is usually made by the clinical evaluation of physical signs and symptoms in combination with tests that can help to confirm the diagnosis or rule out other causes of a person’s signs and symptoms.
In 1982, the American College of Rheumatology (ACR) developed classification criteria to aid healthcare practitioners in making a diagnosis, in particular, of SLE. The criteria were last updated in 1997. If someone has four or more of the 11 criteria listed, that person may be diagnosed with lupus.
- A rash resembling a butterfly that appears across the nose and cheeks (malar, relating to the cheek)
- A red rash consisting of round or oval-shaped patches (discoid rash)
- Photosensitivity – rash develops on areas of skin that have been exposed to sunlight
- Mouth sores or nose ulcers – usually painless
- Arthritis in two or more joints, along with tenderness, swelling, or accumulation of fluid that lasts for a few weeks – arthritis associated with SLE is non-erosive, meaning the bones near affected joints are not damaged
- Inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
- Neurologic disorder – seizures and/or psychosis without other identifiable causes
- Blood (hematologic) disorder – anemia, low white blood cell count, or low platelet count
- Kidney problems – such as a high amount of protein in the urine or cellular casts in the urine
- Positive blood test for antinuclear antibodies (ANA, see tests below) – high levels are generally more specific to SLE; must be in the absence of medications known to be associated with drug-induced lupus
- Positive blood test for anti-double stranded DNA (anti-dsDNA), anti-Sm (Smith) antibody, cardiolipin antibody or lupus anticoagulant (see below), or a false-positive test for syphilis (meaning the person tests positive but does not have the disease)
In 2012, the Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the ACR’s SLE classification criteria. According to the revision, a patient is classified as having SLE if s/he has biopsy-proven kidney involvement (lupus nephritis) with ANA or anti-dsDNA antibodies or if s/he satisfies 4 of the 11 diagnostic criteria, including the presence of at least one sign or symptom and a positive autoantibody test.
The following laboratory tests for autoantibodies may be useful in the diagnosis of lupus:
- Anti-nuclear antibody (ANA) – positive in almost all people with SLE, although can also be positive in those with other autoimmune diseases as it indicates a stimulated immune system
- Anti-Sm antibody (anti-Smith antibody) – usually seen only in those with SLE
- Anti-double stranded DNA (Anti-dsDNA) – high levels are characteristic of active SLE
- Anti-SSA and Anti-SSB – may also be positive
- Anti-RNP – may be positive
- Anti-chromatin antibodies – may be present in people with SLE who are positive for ANA but negative for anti-dsDNA
- Histone antibodies – for drug-induced lupus
- Antiphospholipid antibodies – such as lupus anticoagulant, anticardiolipin, anti-β2 glycoprotein I
Other general tests that may be useful for evaluating someone with or suspected of having lupus:
- Urinalysis – may show blood, urinary casts, or protein in the urine, which can indicate kidney involvement
- Complete blood count (CBC) – may reveal anemia and decreased numbers of white blood cells and platelets, which can occur with lupus
- Comprehensive metabolic panel (CMP) – indicates current status of the kidneys and liver as well as electrolyte and acid/base balance and levels of blood glucose and blood proteins
- Serum protein electrophoresis (SPEP) – gamma globulin protein levels are indicative of inflammatory diseases such as SLE
- Complement 3 (C-3), C4 and CH50 – often decreased; the complement system is part of the immune system. Amounts may be associated with lupus but also gram-negative septicemia, shock, and malaria.
- Erythrocyte sedimentation rate (ESR) – increased with inflammation, such as with lupus as well as other inflammatory conditions
- C-reactive protein (CRP) – another marker of inflammation that may be elevated with lupus
- Cryoglobulin – frequently positive; cryoglobulins are abnormal proteins in the blood that will precipitate when the body temperature drops below normal, causing blockage of the blood vessels.
X-rays or other imaging tests may be ordered to examine organs potentially affected by lupus.
In addition, a kidney biopsy may be performed. This involves taking a piece of kidney tissue for examination to detect any changes in the tissue that could indicate lupus and help guide treatment.
There is currently no cure for lupus, although many of those affected may experience remissions of symptoms between flare-ups. The goals of treatment are to alleviate symptoms, to minimize the occurrence of flare-ups, and to minimize and address the development of complications associated with lupus. Periodic physical exams and laboratory testing are important for monitoring a person’s response to treatment as well as to detect new organ system involvement.
To help decrease the number of flare-ups, people with lupus should get sufficient rest, exercise, and should minimize stress and avoid exposure to sunlight. If someone notices that a particular substance makes her symptoms worse, then she should avoid exposure to it.
Medications can be given to relieve pain, minimize inflammation, and address complications. The most common drugs offered are nonsteroidal anti-inflamatory drugs (NSAIDs) such as ibuprofen or naproxen, antimalarial drugs, which have been found to be useful in treating lupus, and corticosteroids. In more aggressive cases, other immunosuppressive drugs may be used.
Dietary supplements, including dehydroepiandrosterone (DHEA), vitamin D, and fish oil have shown some benefit as complementary treatments for people with lupus.
People should work closely with their healthcare practitioner and with specialists as needed, such as a rheumatologist (a specialist in autoimmune disorders), to develop a treatment plan that is effective for them. This plan is likely to change over time due to changes in a person’s symptoms, general state of health, and as new treatments become available. Women wanting to become pregnant should talk to their practitioner about their health and the medications they are taking. Some treatments are safer than others for the fetus during pregnancy.
Research is ongoing to better understand the disease process of lupus as well as the role of genetics and to identify potentially useful biomarkers for detecting lupus as well as new, effective treatments. To learn more about treatments, see the links in the Related Content section.
Sources Used in Current Review
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