What is Lupus? An introduction to Lupus

What is Lupus?

The name Lupus is derived from the Latin for ‘wolf’. The condition was previously thought to be characterised by a pink facial rash that was thought to resemble a wolf’s mask. Later on the facial rash was described as a “butterfly rash”. Lupus is a chronic autoimmune disease which actually presents in a number of very different ways and often without the facial rash it has become synonymous with. The condition is often referred to as the greatest mimic. Lupus is difficult to diagnose as signs and symptoms can be non-specific with misdiagnoses made. It is often confused with conditions such as rheumatoid arthritis and multiple sclerosis. Lupus is now thought to be more common worldwide than leukaemia, multiple sclerosis, and many other well-known diseases. The incidence of the condition nearly tripled in the last 40 years of the 20th century. It affects women more than men with 90% of sufferers being female. The reported prevalence of systemic lupus erythematosus (SLE) in the population is 4.91/100 000 person-year.

Lupus is a long term health condition with no known cure and can be life-threatening. It is characterised by autoantibody production. This means your immune system produces antibodies to cells within the body. The normal function of the immune system is to protect and fight off viruses, bacteria and germs by producing proteins called antibodies. With lupus, the immune system malfunctions and cannot distinguish between foreign invaders and healthy tissue. Antibodies are then produced against the body’s healthy cells and tissues, causing inflammation, pain and damage in various parts of the body. 

Lupus has a prevalence of approximately 1 in 2,500 in European populations [3]. It is more frequent in those of non-European ancestry. It affects predominantly women (the female-to-male ratio is 9:1) of child-bearing age. It is characterised by variable clinical features, including malar rash, glomerulonephritis, arthritis, and neuropsychiatric disease [4]. Although the exact aetiology is not fully understood, a strong genetic link has been identified through the use of association and family studies. There are different types which are listed below. Systemic lupus erythematosus is the most common form of lupus.

 

1.Systemic Lupus Erythematosus (SLE)

Systemic Lupus Erythematosus (SLE) is typically the most common form of the condition and is generally considered more serious than the other three forms. SLE can affect many parts of a person’s body, including kidneys, heart, lungs, brain, blood and skin. Symptoms tend to vary among patients and can change often and suddenly

2. Cutaneous Lupus

Cutaneous lupus is a form that only affects the skin and causes rashes. These rashes may be anywhere but are usually found on the face, neck and scalp. This type does not affect any of the internal body organs although 1 in 10 people living with discoid lupus will develop SLE. Sometimes people living with the condition get a malar rash, which is often referred to as a butterfly rash. This rash commonly extends from one cheek over the nose to the other cheek. The rash was named lupus because early physicians believed the rash looked a lot like the bite of a wolf. Read more on Cutaneous Lupus.

3. Drug Induced Lupus

Drug Induced Lupus occurs after a person takes certain types of medication. The symptoms are similar to SLE, but they usually disappear when the medicine is stopped. Read our article on Drug Induced Lupus for a comprehensive list of drugs that cause Lupus.

4. Neonatal lupus

In rare cases, the newborn of a mother with lupus may have neonatal lupus. This condition can cause skin rashes, anaemia or liver problems. Symptoms usually go away after a few months and don’t cause permanent damage.

Symptoms of Lupus:

It is said that no two cases are alike and it mimics several other conditions which make diagnosis very difficult.

Common symptoms include the following:

Swollen, stiff, and painful joints
Fever
Fatigue
Rashes on the skin and/or sensitivity to the sun
Malar or butterfly rash
Swelling around the ankles
Chest pain with deep breaths
Unusual hair loss
Pale or purple fingers from cold or stress
Mouth ulcers, often painless

 

Prevalance

The majority of lupus patients are young women aged 16-55 but men and even young children can be affected. It is estimated that 1 in 750 women suffer from the condition in the UK with the ratio of women to men being 9:1. Lupus is a worldwide disease more common in some races than others. The incidence in white women is 1 in 1000 compared with that in black women of 1 in 250.

 

Treatment

There is no known cure but there are medications that help with relieving symptoms, and medications which can also prevent serious end organ damage. Drugs that are commonly use in the treatment of the condition include hydroxychloroquine (a drug that has historically been used to treat malaria), corticosteroids and immunosuppressants.

 

References:

1.Perl A: Pathogenic mechanisms in systemic lupus erythematosus.Autoimmunity 2010, 43:1-6.

2. Townsend MJ, Monroe JG, Chan AC: B-cell targeted therapies in human autoimmune diseases: an updated perspective.Immunol Rev 2010, 237:264-283.2.

3.Johnson AE, Gordon C, Palmer RG, Bacon PA: The prevalence and incidence of systemic lupus erythematosus in Birmingham, England. Relationship to ethnicity and country of birth.Arthritis Rheum 1995, 38:551-558. 

4.Lipsky PE: Systemic lupus erythematosus: an autoimmune disease of B cell hyperactivity.Nat Immunol 2001, 2:764-766.

5.Block SR, Winfield JB, Lockshin MD, D’Angelo WA, Christian CL: Studies of twins with systemic lupus erythematosus. A review of the literature and presentation of 12 additional sets.Am J Med 1975, 59:533-552.

6.Deapen D, Escalante A, Weinrib L, Horwitz D, Bachman B, Roy-Burman P, Walker A, Mack TM:A revised estimate of twin concordance in systemic lupus erythematosus.Arthritis Rheum 1992, 35:311-318.