What is Lupus?

Lupus is an autoimmune condition which can affect many different parts of the body. An autoimmune disease is a condition where the body’s own immune system attacks itself, instead of protecting itself and keeping it healthy.

Here when we use the term Lupus we refer to ‘Systemic’ Lupus and not Cutaneous Lupus, which affects only the skin.

Lupus comes from the latin Lupus meaning wolf. The reason being that first description of Lupus referred to erosive skin lesions that resembled the bite of a wolf! (I imagine that not many people get bitten by wolves these days……!) It is now interpreted as being that the disease is cunning and can masquerade as many things (although I was not aware that wolves could masquerade as anything other than a wolf….!)

In fact, medicine does not completely understand this condition which consists of a wide variety of different symptoms and problems for many people.

The history of the understanding of Lupus is quite interesting. In fact, originally, Lupus was thought to be an infection and only later on did it come to be understood to be an autoimmune disease.

I note here that Rheumatologists treat Systemic Lupus, but not Cutaneous Lupus. Cutaneous Lupus is treated by Dermatologists.

Are Cutaneous and Systemic Lupus the same thing?

No. Cutaneous Lupus and Systemic Lupus appear to be different. Cutaneous Lupus is a disease affecting the skin.

There may be no antibody associated with Cutaneous Lupus, but rather the diagnosis made on biopsy.

Its important to know the difference. I see many clients concerned that they have “Lupus’ because they have Cutaneous Lupus.

The outcomes and treatments needed are quite different for Cutaneous Lupus than Systemic Lupus.

That said, Systemic Lupus can also involve the skin, but

Cutaneous Lupus doesn’t involve other organs and doesn’t carry the same risk of complications and impact on life as Systemic Lupus can do.

How can Systemic Lupus affect the body?

Lupus can affect literally any part of the body. Not everyone gets all of these things.

The way that Lupus affects the body depends on which part of the body is affected.

The most common things that people get are:

Muscle pains
Joint pains and/or arthritis

Fatigue occurs in 80-100% of people with Lupus and is the most common complaint in people with Lupus.

Other common features are:

Raynaud’s disease
Arthritis and/or Arthralgias (joint pains without swelling)
Mouth ulcers (usually painless)
Hair loss with bald spots ( known as alopecia)
Face Rash (aka ‘butterfly rash’)
Photosensitivity (rashes in response to sunlight)

The disease can change in the course of a persons’ lifetime.

Various different things can develop according to the disease process.

I will go through some of these possibilities here, but please note that most people do not get all of these things!

Lupus can involve many different organs and the symptoms depend on which organs are involved.

Lymph nodes and spleen

  • Its quite common to have enlarged lymph nodes and an enlarged spleen in active disease.
  • Enlarged lymph nodes can be part of what is known as ‘Kikuchi’ syndrome.


Skin involvement in lupus is very common. The types of skin problems that can occur are:

  • Butterfly rash
    • this is the classic ‘lupus’ rash where there is redness and thickening across the bridge of the nose and cheeks, with no involvement of the groove between the nose and the cheeks
  • Photosensitivity – this is where people get rashes on exposure to sun, it does not mean people who get sunburn very easily!
  • Red circular lesions, known as ‘plaques’
  • Discoid skin lesions – this is scarring from skin lesions
  • Hair loss, known as ‘alopecia’

Immune system

  • Specific auto- antibodies can be developed. Antibodies are naturally produced by the immune system usually to things that do not belong in the body. Antibodies are an important part of signalling to the body that there is a problem and directing the focus of inflammation. Auto-antibodies are when the immune system forms antibodies against parts of the body it is in. There are different auto-antibodies for different autoimmune conditions. The specific auto-antibodies for Lupus are:
    • ANA, anti Smith antibodies, Ds DNA antibodies.


There can be:

  • Hemolytic Anemia (where there is immune destruction of the red cells)
  • Low white cells and in particular low lymphocytes.

Vascular system

There can be:

  • Raynaud’s disease –  50% of people with Lupus get Raynaud’s disease
  • Vasculitis – the most common type of vasculitis in lupus is inflammation of very small vessels, usually causing various skin rashes. (Vasculitis is inflammation of the blood vessels).
    • It is reported that between 11-36% of people with Lupus have this sort of vasculitis.
    • It tends to affect skin, gut and kidneys.
  • Thrombo-embolic disease – ie increased risk of blood clots.
    • This is associated with particular antibodies:
      • Anti-phospholipid antibodies, lupus anticoagulant and anti-cardiolipin antibodies.


  • This affects 50% of people with Lupus.
  • Kidney involvement in Lupus is a major cause of poor quality of life and death.

Gut (Gastrointestinal Tract known as the GIT for short)

  • 40% of people with Lupus have involvement of the gut.
    • Most of it is due to medication side effects
  • But, Lupus can affect nearly any organ in the GIT.
    • From the oesophagus, with reflux, to mesenteric ischemia where the blood supply to the intestines is affected.
  • Mouth ulcers are very common, usually painless


  • Pleurisy, shrinking lungs, and pneumonitis (inflammation of the lungs)
  • Pulmonary hemorrhage (bleeding in the lungs)
  • Pulmonary hypertension (high blood pressure in the lungs)


  • There is an increased risk of ischemic heart disease
  • But also primary myocarditis can occur.
    • (Myocarditis is inflammation of the heart muscle where ‘myo’ means muscle in Greek).
  • Inflammation of the valves of the heart known as ‘Libman Sacks endocarditis’ can occur
    • (endocardium is the tissue the valves are made of)
  • Pericarditis is the most common cardiac problem occurring in 25% of people with Lupus
    •  Pericarditis is inflammation of the outer lining of the heart, which is known as the ‘pericardium’ because it is ‘around the heart’.

Brain and nerves

  • Seizures and psychosis are the 2 things that form part of the ACR criteria for diagnosing people with Lupus
  • But it can cause even mild cognitive problems as well as delirium.
  • It can also cause:
    • Peripheral nerve problems
    • Headaches
  • Depression, Anxiety and Mania can also be associated with Lupus


  • Literally any part of the eye can be affected,
    • although it is not common for the eyes to be so affected in lupus
  • Dry eyes is the most common thing to affect the eyes in Lupus.
Is Lupus a serious disease?

This is a common question and a lot of people are concerned when they hear the term ‘Lupus’ as it has a reputation of being a serious dangerous and disabling condition.

However, most people with Lupus have very mild disease:  with tiredness, joint pains and an occasional rash.

That said, it can be quite a serious condition affecting many organs and needing serious treatment.

If you think you have, or you do have Lupus its important to see a good Rheumatologist to support and guide you in the treatment of your condition.

Cutaneous Lupus is treated by Dermatologists and whilst it can be disfiguring and nobody likes their skin being affected, it is not life threatening.

Who gets Lupus?

Lupus is far more common in women than in men.

In fact 90% of people with Lupus are women!

Lupus can also occur in children but most people diagnosed with lupus for the first time are between the ages of 16-55.

Lupus is more common in people of African and Asian genes, than people from Caucasian genetics.

Why do people get Lupus?

We don’t understand why people get Lupus. There are various theories about it, the most common one being that it is an ‘Auto-immune’ disease.

Why people get an autoimmune disease like Lupus is not yet clear as there are many different types of autoimmune diseases! In fact over 80!

It is thought that possibly there is a viral or another infective trigger, a potential role for ‘genes’ but nothing conclusive.

How do we make a diagnosis of Lupus?

This is an interesting question. When we think of something being diagnosed, we automatically think that there must be some ‘test’ that definitely makes the diagnosis! However in medicine this is not the case.

Most diagnoses are made on the observation of what a person is experiencing in combination with test results, but there it not necessarily ‘one test’ which makes ‘the diagnosis’. This particularly applies to Lupus which covers a diverse range of symptoms and problems that a person can experience.

Lupus is primarily a clinical diagnosis, which means it is one based on the clinical experience of the Rheumatologist.

Over the years groups of Rheumatologists have got together to try and better understand this disease and how a diagnosis might be made.

To this end, several lists have been made of the common ‘symptoms’ and ‘signs’ of the disease.

One of these is known as the ACR criteria. (ACR stands for American College of Rheumatology) There are 11 criteria and if you have 4 of the features then a diagnosis of Lupus can be made. If you do not, a diagnosis of lupus can be made if your Rheumatologists considers that your symptoms are characteristic of Lupus. Another more recent one is known as the SLICC criteria, which includes biopsy proven lupus nephritis even if there are no other symptoms.

Its interesting to note that things in the body change with time and new symptoms can develop with time.

So if a diagnosis of Lupus cannot be made at one point in time, it may be made at a later point in time if new symptoms occur.

The common symptoms of lupus assessed as part of diagnosis are in the table below.

Please note none of this replaces the clinical skill of your Rheumatologist which comes first in making the diagnosis. Machines cannot diagnose Lupus!

Malar RashFixed erythma [meaning 'redness'] over the cheeks, sparing the groove between the nose and lip area. This is known as the nasolabial groove. Often said to look like a 'butterfly' rash
Discoid RashPatches of raised redness with thickening, scaling and then scarring from older areas
PhotosensitivitySkin rash in response to sunlight. Not sunburn!
Mouth UlcersPainless. Can also be in the nasopharynx, ie the nose and the back of the throat.
Arthritis. Non eroding.2 or more joints. Tender, swollen or fluid in the joint.
Pleuritis or PericarditisInflammation of the lining of the lungs or the heart
Kidney involvementSignificant protein in the urine or other cells such as red cells
Neurological InvolvementSeizures or Psychosis not due to other causes.
Hematological involvementLow platelets or low white cells – low lymphocytes on at least 2 occasions, and/or low total white cell count on at least 2 occasions, Hemolytic Anemia
Immunological InvolvementHigh DsDNA, positive anti Smith antibody or positive antiphospholipid antibodies
ANA positiveANA is the anti nuclear antibody strongly associated with Lupus.
What is 'the ANA'?

ANA is short for ‘Anti Nuclear Antibody’.

It is an immune antibody that is associated particularly with Lupus, but also with other autoimmune conditions such as immune thyroid disease.

Antibodies are naturally produced by the immune system usually to things that do not belong in the body. Antibodies are an important part of signalling to the body that there is a problem and directing the focus of inflammation.

Auto-antibodies are when the immune system forms antibodies against parts of the body it is in.

An ANA is an antibody that is directed against proteins that occur in the nucleus of the cell.

ANA can occur in the presence of autoimmune disease, but it can also occur with some viral infections.

Not everyone with an ANA has an autoimmune disease, and having an ANA does not mean that you have an autoimmune disease.

I have an ANA – does that mean I have Lupus?

The good news is, that just because you have an ANA  (which is an immune antibody associated with Lupus) does not mean that you have Lupus!

A diagnosis of Lupus is based on a lot of factors and can’t be diagnosed on the basis of a blood test.

You need at least 4 features typical of Lupus in order to be diagnosed with Lupus, or a kidney biopsy positive for Lupus if there are signs of kidney disease.

See the section of how Lupus is diagnosed to understand this further!

About 20% of the normal population (ie people without any disease) have a positive ANA. It does not mean that you will necessarily develop Lupus or any other autoimmune disease.


What tests are done to help diagnose Lupus?

The tests that are done are different according to the symptoms that each person has.

Baseline tests for lupus are blood tests such as immune markers, ANA, ds DNA, C3 C4, and other immune markers to rule out other disease conditions.

If joints are involved tests will be done for Rheumatoid Arthritis, such as RF and anti CCP antibodies.

Other broader tests are done to see if other organs are affected, such as FBC, looking at red and white cell counts, liver function testing, kidney and other biochemistry testing. Urine will be tested to see if there is any kidney damage or inflammation.

If there are breathing or heart problems or abnormalities are found on examination, tests may be done to look at the lungs or the heart as needed such as CT Chest, or an Echocardiogram, which looks at the function of the heart and for fluid around the heart.

Your Rheumatologist will guide you and explain to you what tests are needed and why. They will be tailored to your particular situation.

How do we treat Lupus?

Not all features of Lupus will necessarily need treating. Lupus can be quite mild, and in that case, sometimes anti inflammatories and/or paracetamol as needed may be all that is required for more minor features.

If there are more significant features of Lupus, such as persistent rash, arthritis, or involvement of other organs, more significant medications will be needed to control the disease.

Medically, we treat Lupus with medications known as the DMARDs.

Different types of DMARDs are used depending on which part of the body is affected with Lupus and how severe the Lupus is.

Your Rheumatologist will discuss this with you and guide you with your treatment options.

As a brief overview:

Joint disease and skin disease is known to respond well to Plaquenil.

Kidney disease is known to respond well to Mycophenolate.

If people do not respond to traditional DMARD therapies, there are now new biological agents which can be used to treat Lupus such as Belimumab.

Are there things apart from drugs that can be used to treat Lupus?

Many patients come and see me seeking other options apart from medications to treat their Lupus. I’m definitely not opposed to other things, and whilst there are many different things we can do to support our health and well-being, its important to consider the severity of your Lupus with respect to the choice to take medications.

The biggest thing people ask me about is food and diet, and whether that can make a difference.

Food definitely impacts us and there is a lot of information these days available about food on the internet. There is no one Lupus diet, and it appears to be something that each person needs to tailor for themselves.

I am not aware of a diet that cures Lupus however.

I support people making changes to their diet, as its important to optimise the health of our bodies.

It is known that the way that we eat can either promote or reduce inflammation in the body, so if there is a way of eating that can lessen the strain on the body then it makes sense to explore this.

That said, I’m not aware of a diet that cures Lupus, so I would encourage the consideration of medications when and if they are recommended if your disease requires them, in addition to lifestyle modifications.

People often seek complementary therapies for their Lupus instead of medications.

I am not aware of any complementary modality that cures Lupus and whilst I am not opposed to people seeking complementary health care, given that Lupus can be potentially severe I would suggest that these things be considered complementary, ie in addition to, and not instead of medical treatment.

When approaching health its important to consider things as part of an overall picture.

There are many aspects of life that we can adjust to better support ourselves. Health does not just come from medications, nor just from food, but looking at things as a whole can be supportive for many people.

Lupus can be severe and in my experience medications form a solid platform of treatment.

Many people are interested in exploring ways they can support their health and well-being through lifestyle and I would absolutely encourage this. However, given the potential severity of Lupus its important to discuss this with your treating Rheumatologist to make sure that you are not missing out on other important needed parts of your best treatment such as medications.

What happens to people with Lupus?

For most people, Lupus is a mild disease with some joint aches and pains, occasionally a malar rash and fatigue. Most people lead a normal life.

Often these flares appear to be precipitated by stress, but sometimes not.

In some people Lupus is a very serious condition with multiple organs involved requiring intensive treatment.

If multiple organs are involved then the outcome for Lupus is quite serious and can result in an earlier death than the general population.

If people have long term Lupus they are also at a higher risk of cardiovascular disease and death from cardiovascular events.

This makes sense given that we now know that chronic inflammation and a marker called ‘CRP’ (which is a measure of inflammation) is associated with cardiovascular disease.