What is Lupus - Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disorder that may affect many organ systems within the body. In particular it is associated with a distinctive rash and joint pain, however the most potentially serious problems are due to effects on the brain and kidneys.

Who gets Lupus - Systemic Lupus Erythematosus?

SLE occurs worldwide, but racial differences are seen. It is estimated to affect 1 in every 250 African-American women. There is a female predominance (9:1), and peak age of onset is between 20-40 years. The disease affects 0.1-0.2% of the general population. The disease affects 0.1-0.2% of the population, and is much more common in Africans.

Predisposing Factors

Cause unknown but probably multi-factorial. Factors thought to be involved:

* Genetic: 70% concordance between identical twins and increased incidence of HLA-B8 and HLA-DR3.
* Immunological: antinuclear antibodies are present which are thought to result from polyclonal activation of B cells by an antigenic stimulus (possibly viral). May be associated with impaired T cell regulation and deficiencies in complement.
* Drugs: hydralazine, isoniazid, anticonvulsants and procainamide may cause a mild lupus like syndrome which often resolves with withdrawal of the drug.
* Infection: viral infections may be responsible.
* Hormonal factors: high incidence in women suggest female hormones may modify the immune system.
* Sunlight is an exacerbating factor for the cutaneous (affecting the skin) manifestations in most cases.

Progression

SLE usually occurs in episodes, with flares and remissions that may last for long periods of time. In some patients, it may persist as a chronic illness. Generally speaking, if serious complications have not developed by the first few years, they are unlikely to do so.

Probable Outcomes

With current treatments, prognosis is good. Survival rates have increased from 50% at five years during the 1950s, to approximately 90% at 10 years presently.

How Will Lupus - Systemic Lupus Erythematosus Affect Me?

Features typical of SLE include:

* fever
* joint pains
* fatigue
* rash that worsens with sun exposure
* mouth ulcers
* hair loss
* urticaria (hives)

There may have been previous problems with pregnancy (multiple miscarriages), or a family history of autoimmune disease such as rheumatoid arthritis.

Other features may include:

* raised blood pressure.
* mouth ulcers.
* dry eyes
* hair loss.
* joint tenderness or swelling.
* signs of fluid in the lungs.
* signs of inflammation of the fibrous sac surrounding the heart (pericarditis).
* protein in the urine.
* scaly red rash in a ‘butterfly’ distribution over the face and other sun exposed areas.

How is Lupus - Systemic Lupus Erythematosus Diagnosed?

A combination of clinical and physical findings, plus appropriate tests are needed to make a diagnosis of SLE.

Tests may include:

* Blood tests
* Immunological tests
* Urine testing (protein in the urine suggests kidney involvement)
* Biopsy (taking a sample of the skin or kidney to look for abnormalities)
* MRI scan to look for involvement of the brain.

How is Lupus - Systemic Lupus Erythematosus treated?

Treatment depends on symptoms and severity of the disease.

* NSAIDs - useful for mild disease with arthralgia
* Hydroxychloroquine - mild disease when symptoms cannot be controlled with NSAIDS or for cutaneous disease.
* Corticosteroids - mainstay of treatment. Particularly for moderate to severe disease. Aim is to control disease activity before gradually reducing the dose.
* Immunosuppressives (azathioprine, cyclophosphamide) - usually in combination with corticosteroids for severe disease (e.g. renal or cerebral disease).
* Topical steroids - for discoid lupus. Should also avoid excessive sunlight (suncream and clothing).

Lupus - Systemic Lupus Erythematosus References

1. Braunwald, Fauci, Kasper, Hauser, Longo, Jameson. Harrison’s Principles of Internal Medicine. 15th Edition. McGraw-Hill. 2001
2. Cotran, Kumar, Collins 6th edition. Robbins Pathologic Basis of Disease. WB Saunders Company. 1999.
3. Hurst JW (Editor-in-chief). Medicine for the practicing physician. 4th edition Appleton and Lange 1996.
4. Kumar P, Clark M. CLINICAL MEDICINE. WB Saunders 2002 Pg 427-430.
5. Longmore M, Wilkinson I, Torok E. OXFORD HANDBOOK OF CLINICAL MEDICINE. Oxford Universtiy Press. 2001

Symptoms of This Disease:

* Inflammation
* Joint pain (Arthralgia)
* Raynaud’s Phenomenon Raynaud’s disease

Drugs/Products Used in the Treatment of This Disease:

* Methotrexate Injection and Tablets (DBL)
(Methotrexate)

* Azahexal
(Azathioprine)

* Endoxan
(Cyclophosphamide)

* Plaquenil
(Hydroxychloroquine)

* Solone
(Prednisolone 5mg, 25mg)