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Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE) is a long-term autoimmune disorder in which, for reasons not clearly understood, the immune system (the body's natural defense against illness and infection) starts to attack healthy cells, tissues and organs. This in turn leads to chronic inflammation. As with other autoimmune conditions, such as rheumatoid arthritis, it is believed that a combination of genetic and environmental factors is responsible for triggering SLE in certain people.

Other types of Lupus

SLE is what most people mean when they use the term 'lupus'. But there are several other types of lupus to consider. In brief, the two other main examples are:

Discoid Lupus Erythematosus (DLE)

DLE is a mild form of lupus affecting the skin and in a few cases it progresses to the body's tissues and organs. DLE symptoms might include:

  • Red, circular, scaly marks on the skin that can thicken and scar
  • Hair loss and/or permanent bald patches

DLE can usually be successfully controlled using medication and by avoiding exposure to direct sunlight.

Drug-Induced Lupus

Lupus caused by medication is known as drug-induced lupus and over one hundred medications are known to cause lupus-like side effects in certain people. Stopping the course of medication will usually help resolve symptoms under the guidance of a GP.

Who is affected by SLE?

SLE is an uncommon condition. 90% of cases occur in women, the majority of whom are of childbearing age (between 15 and 50 years) when their symptoms arise.

Ethnicity is a significant factor affecting SLE. In the UK 1/5,000 white women develop SLE, 1/1,000 women of Chinese origin develop SLE and 1/625 Afro-Caribbean women develop SLE.

Symptoms

SLE can affect all body tissues and organs and symptoms range from mild to severe. Many people will experience long periods of time with few or no symptoms before experiencing a sudden flare-up where their symptoms are particularly severe.

All symptoms of SLE can have a considerable impact on a person's quality of life, particularly pain and chronic fatigue, leading to distress, depression and/or anxiety.

Almost all SLE sufferers have joint pain and swelling. Frequently affected joints are the fingers, hands, wrists and knees. Arthritis might develop as a consequence of SLE.

Other common symptoms include:

  • Chest pain when taking a deep breath;
  • Fatigue;
  • Fever with no other cause;
  • General discomfort, uneasiness, or malaise;
  • Hair loss;
  • Mouth sores;
  • Sensitivity to sunlight;
  • A "butterfly" (malar) rash over the cheeks and bridge of the nose, which gets worse in sunlight and might also be widespread;
  • Swollen lymph nodes.

Other symptoms depend on which part of the body is affected by SLE:

  • Brain and nervous system: headaches, numbness, tingling, seizures, visual disturbances, personality changes;
  • Digestive system: abdominal pain, nausea and vomiting;
  • Cardiovascular system: abnormal heart rhythms (arrhythmias);
  • Respiratory system: coughing up blood and breathing difficulties;
  • Skin: patchy skin colour, Raynaud's phenomenon.

Diagnosis

SLE can be difficult to diagnose as there are many other common conditions with similar symptoms. To be diagnosed with SLE, 4/11 typical signs of the disease must be recognised.

Tests used to diagnose SLE might include:

  • Antibody tests, including antinuclear antibody (ANA) panel
  • CBC
  • Chest x-ray
  • Kidney biopsy
  • Urinalysis

SLE might also alter the results of a number of other tests, including but not limited to:

  • Antithyroglobulin antibody
  • Antithyroid microsomal antibody
  • Complement components (C3 / C4)
  • Coombs' test
  • Cryoglobulins
  • ESR
  • Kidney / liver function blood tests
  • Rheumatoid factor

Complications

Some people with SLE have abnormal deposits in their kidney cells, leading to a condition called lupus nephritis. Patients with this condition may eventually develop kidney failure and need dialysis or a kidney transplant.

SLE might also lead to damage in many different parts of the body, including:

  • Blood clots in the legs (deep vein thrombosis) or lungs (pulmonary embolism);
  • Destruction of red blood cells (hemolytic anemia) or anemia of chronic disease;
  • Fluid around the heart (pericarditis), endocarditis or inflammation of the heart (myocarditis);
  • Fluid around the lungs (pleural effusions) and damage to lung tissue;
  • Pregnancy complications, including miscarriage;
  • Stroke;
  • Severely low blood platelets (thrombocytopenia);
  • Inflammation of the blood vessels.

Treatment

There is no cure for SLE and the goal of any treatment is to control symptoms.

Mild SLE might be treated with:

  • Non-steroidal anti-inflammatories(NSAIDs) for arthritis and pleurisy;
  • Corticosteroid creams for skin rashes;
  • Hydroxychloroquine (an anti-malaria drug) and low-dose corticosteroids for skin and arthritis symptoms.
  • Protective clothing, sunglasses and sunscreen for sun exposure;
  • Manual therapies (chiropractic, osteopathy, physiotherapy) for arthritis and muscle pain.

Severe SLE treatment might involve:

  • High-dose corticosteroids to decrease the immune system response;
  • Cytotoxic drugs (blocking cell growth) following a poor response to corticosteroid medications;
  • Preventative heart care;
  • Immunisations;
  • Bone density scans;
  • Support groups / counselling for depression and mood changes.

Prognosis

Prognosis depends on the severity of the disease, but the outcome for SLE patients has improved in recent years. SLE tends to be more active in the first years after diagnosis and in younger patients (less than 40 years old).

Women with SLE who become pregnant are often able to carry safely to term and deliver a healthy infant, as long as they do not have severe kidney or heart disease and the SLE is being treated appropriately. However, the presence of SLE antibodies may increase the risk of pregnancy loss.

Contributing Author:
Nikki Harris MOst. MA APNT Dip. Cert Acup. 

References

Hahn, B.H. & Tsao, B.P. (2008). Pathogenesis of systemic lupus erythematosus. In: Firestein GS, Budd RC, Harris ED Jr., et al., eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008: chap 74.

Ruiz-Irastorza, G., Ramos-Casals, M., Brito-Zeron, P. & Khamashta, M.A. (2010). Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann Rheum Dis. 2010;69: pp.20-28.

http://www.nhs.uk/Conditions/lupus/Pages/Introduction.aspx

http://www.nlm.nih.gov/medlineplus/ency/article/000435.htm