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Systemic Lupus Erythematosus

Last updated Jan. 5, 2019

Approved by: Maulik P. Purohit MD MPH

Nephron

Very high magnification of histomorphologic changes in a lymph node due to systemic lupus erythematosus. H&E stain.


What are the other Names for this Condition? (Also known as/Synonyms)

  • Disseminated Lupus Erythematosus
  • Libman-Sacks Disease
  • Lupus

What is Systemic Lupus Erythematosus? (Definition/Background Information)

  • Systemic Lupus Erythematosus (SLE or Lupus) is an autoimmune disorder that may affect any area of the body such as the skin, joints, blood cells, kidneys, brain, heart, and lungs. Women are more commonly affected by SLE than men
  • Autoimmune disorders cause the body to mistake normal healthy tissues and organs for dangerous foreign material and attack them leading to long-term chronic inflammation. Such an attack by the body’s immune cells against its own tissue can be severe, resulting in significant signs and symptoms
  • SLE may be drug-induced or hereditary, but the exact cause is not yet known. Systemic Lupus Erythematosus caused by certain drugs is called Drug-Induced Lupus
  • The key symptoms depend upon the part of the body that is affected. Most commonly, the part of the body that is affected is the skin. The classical symptoms may include a rash on the face in the shape of a “butterfly” and sensitivity to sunlight. The condition may be diagnosed through physical examination, complete medical history, and specialized blood tests
  • There is no cure for SLE, but there are many forms of treatment, which may control flare-ups well. There is no effective preventive method for SLE onset that is caused by genetic predisposition. Drug-Induced Lupus can be prevented by avoiding known drugs that have been implicated to cause Lupus-like symptoms
  • SLE can range in severity from mild to very severe; the prognosis depends on the severity of the disorder and also its response to therapy. Those who respond well to treatment have a better prognosis than individuals who do not respond well to treatment

There are several different types of Lupus and these include:

  • Discoid Lupus Erythematosus: In this condition the main signs and symptoms include a persistent skin rash
  • Subacute Cutaneous Lupus Erythematosus: In this condition, there are multiple sores on the skin that occur when exposed to sunlight
  • Drug-Induced Lupus Erythematosus: In this condition, signs and symptoms similar to Lupus appear after taking certain medications. In many individuals, these symptoms go away after the medication is stopped
  • Neonatal Lupus: In this condition, the signs and symptoms of Lupus are observed in newborns

Who gets Systemic Lupus Erythematosus? (Age and Sex Distribution)

  • Individuals of any age may be affected by Systemic Lupus Erythematosus; but it is more commonly seen between the ages of 10-50 years
  • Women are more prone to be affected by SLE than men
  • The disease is more prevalent among many people of African American and Asian descent

What are the Risk Factors for Systemic Lupus Erythematosus? (Predisposing Factors)

 Risk factors associated with Systemic Lupus Erythematosus are:

  • Female gender
  • Individuals of African American and Asian race
  • Genetic predisposition
  • Medication that has been known to cause Drug-Induced Lupus 

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Systemic Lupus Erythematosus? (Etiology)

The exact cause of Systemic Lupus Erythematosus is not fully known or understood.

  • The use of some drugs may cause the onset of Drug-Induced Lupus Erythematosus
  • Some individuals may have a genetic predisposition for the condition; which may then be triggered by infections, certain drugs, or on exposure to the sun

What are the Signs and Symptoms of Systemic Lupus Erythematosus?

The signs and symptoms of Systemic Lupus Erythematosus include:

  • A rash on the face in the shape of a “butterfly”
  • Fatigue
  • Low-grade fever
  • Loss of appetite
  • Muscle pain
  • Hair loss (alopecia)
  • Arthritis; joint pain and inflammation
  • Oral and nasal ulcers
  • Sensitivity to sunlight
  • Inflammation of the lining of the lungs and heart
  • Poor blood circulation in the fingers and toes (causing coldness in these areas)
  • Swollen lymph nodes
  • Additional symptoms may arise depending on the organ involved
  • Chest pain while taking deep breaths

The signs and symptoms of SLE depend on the severity of the condition.

How is Systemic Lupus Erythematosus Diagnosed?

The American College of Rheumatology (ACR) has established a set of 11 criteria to make (or rule-out) the diagnosis of Systemic Lupus Erythematosus. These criteria are closely related to the symptoms of Lupus which include:

  • Skin signs and symptoms of Lupus: Malar rash (butterfly-shaped rash stretching across the cheeks and nose); photosensitivity rash, due to sunlight; discoid (red and raised) rashes on the skin
  • Mouth and nose signs and symptoms of Lupus that include oral and nasal ulcers
  • Joints signs and symptoms of Lupus such as joint pain or arthralgia
  • Blood changes in lupus: Decreased red blood cells, white blood cells, and platelets
  • Renal signs and symptoms of Lupus that may range from proteinuria (the presence of proteins in urine) to end-stage kidney disease
  • Brain signs and symptoms of Lupus such as seizures, stroke, meningitis (infection of the protective coverings of the brain), and psychosis
  • Heart and lung signs and symptoms of Lupus that include chest pain, pulmonary hypertension, pleuritis, myocarditis (inflammation of heart muscle) or pericarditis
  • Serology test: Antinuclear antibody (ANA) positive
  • Serological blood test: Anti- double stranded DNA (ds-DNA) positive

Out of these 11, 4 criteria are needed to make a diagnosis of SLE.

In addition to the above 11 criteria, there are other tests that may help determine the involvement of various organs. The tests are chosen by the physician depending upon the patient’s presentation (signs and symptoms and medical history).

  • Routine blood testing (sedimentation rate/C-reactive protein)
  • Blood chemistry panels
  • Body fluid analysis such as pleural (lung cavity) fluid and pericardial (heart cavity) fluid analysis
  • Tissue biopsies of the affected organs such as skin biopsy and kidney biopsy 

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Systemic Lupus Erythematosus?

The complications that might occur due to Systemic Lupus Erythematosus include:

  • Blood clots in the legs and lungs (thrombosis and thromboembolism)
  • Kidney failure
  • Hemolytic anemia (destruction of red blood cells)
  • Inflammation of the heart (pericarditis and myocarditis) and inflammation of the blood vessels (vasculitis)
  • Fluid around the heart and lungs (pericardial effusion and pulmonary effusion respectively)
  • Brain stroke
  • Low platelet count in blood resulting in clotting complications (thrombocytopenia)
  • Women with SLE may be at an increased risk for heart disease (SLE induced heart damage)
  • Increased risk of developing cancers such as leukemia and lymphoma (SLE induced lymphoma and leukemia)
  • There is also an increased risk of breast cancer
  • Complications with pregnancy such as early termination and stillbirth

How is Systemic Lupus Erythematosus Treated?

There is currently no cure for Systemic Lupus Erythematosus. The treatment undertaken consists of decreasing inflammation and autoimmune activity in the body and minimizing the occurrence of flare-ups.

The following drugs have been found to be helpful in the treatment of SLE:

  • Non-steroidal anti-inflammatory drugs (NSAIDs)
  • Corticosteroids
  • Antimalarial medications
  • Immunosuppressive drugs (cytotoxic drugs)
  • A procedure termed plasmapheresis may be used to remove unwanted substances from blood, to help in suppressing the immune system.

How can Systemic Lupus Erythematosus be Prevented?

  • There is no effective preventive method for the onset of Systemic Lupus Erythematosus
  • Flare-ups can be prevented by managing medications, avoiding sun exposure, utilizing suitable sunscreens and clothing (to cover-up), and close monitoring of the symptoms

What is the Prognosis of Systemic Lupus Erythematosus? (Outcomes/Resolutions)

  • Systemic Lupus Erythematosus is an autoimmune disorder that can range in severity from mild to severe to fatal; the outcome of the disorder depends on the severity of the disease. Through proper treatment and monitoring of the condition, many people with SLE are able to lead normal, healthy lives, while managing flares
  • SLE tends to be more active in younger individuals under the age of 40 years, and in people who are newly diagnosed with SLE
  • Pregnant women are often able to deliver at full term safely. It is important that  treatment during pregnancy is adequate with close monitoring of both the mother and the developing baby
  • Drug-Induced SLE is infrequent (less than 5%) and most often resolved upon discontinuation of the medication

Additional and Relevant Useful Information for Systemic Lupus Erythematosus:

According to the Lupus Foundation of America, approximately 5 million people worldwide suffer from a form of Lupus.

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 11, 2015
Last updated: Jan. 5, 2019