What is lupus?

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Lupus is an autoimmune connective tissue disease. This means that the immune system turns against the parts of the body known as the connective tissue. The connective tissue is the tissue that 'connects' different parts of the body. There are many different types of connective tissue in different parts of the body and the type of connective tissue that is targeted is what causes each person with lupus to have different symptoms.

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The lupus that we treat in Rheumatology is the systemic lupus, where many different parts of the body can be involved. There is another type of lupus known as 'cutaneous lupus' (cutaneous refers to skin). This is managed by dermatologists and its treatment and outcome is quite different.

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Systemic lupus is also known as SLE, which is short for the long name of Systemic Lupus Erythematosis. It can affect many different parts of the body and cause many symptoms including rash, joint pains and swellings, kidney inflammation, lung and heart inflammation, tiredness, mouth ulcers, dry eyes and dry mouth, Raynaud's disease

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What are the symptoms of systemic lupus?

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The symptoms of systemic lupus depend on which part of the body is involved and this can be different with each person.

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Most people with systemic lupus have mild disease, with joint aches and pains, a bit of tiredness, and perhaps an occasional butterfly rash. Other people have more severe disease with the involvement of many different organs including the kidneys.

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Systemic lupus typically affects joints causing aches pains and sometimes swelling in the joints. The most common joints involved are the fingers.

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The most common symptoms in systemic lupus are:

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• Arthritis/Arthralgia.

  • More than 90%.

  • Symmetrical, migratory, non erosive.

• Fatigue.

  • In 80-100%

• Fever.

  • More than 50%

• Mouth or nose ulcers. 

  • Usually painless​

• Raynauds disease.

  • In up to 50%

• Kidney involvement.

  • 50%.

  • Can be associated with significant impact on quality of life and increase risk of dying.

• Skin rash.

  • Butterfly rash on the face is the most common.

  • Most people develop some sort of skin and or mucous membrane involvement at some stage of the disease.

• Gut symptoms occur in up to 40%.

  • Usually due to drug side effects or infections

• Lymph node enlargement is common usually in the neck, arm pits or groins

• Anemia and low platelets

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Systemic lupus can also cause other symptoms depending on which organs are involved. Cognitive dysfunction is quite common, the heart and lungs can be affected causing palpitations and/or chest pain and a feeling of not being able to breathe properly.

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What causes systemic lupus?

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We don't understand completely what causes lupus. We know that it is an autoimmune disease associated with certain autoimmune antibodies but what causes or triggers them is not known. There appear to be many defects in the immune pathway with people with lupus that appear to be different with different people.

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It is thought that there is a genetic contribution.

• People with a close relation, first degree, who has lupus are more than 17 times likely to get lupus.

• If you have a sibling with systemic lupus you are 29 times more likely to get systemic lupus.

• It is not known why or how this genetic factor works and it is clear that there are many other factors at play.

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Who gets systemic lupus?

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Systemic lupus is a disease which more women appear to get than men.

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It is noted to be more common in Asians, African Americans and Hispanic Americans. It is not common in Africa.

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It is not a common disease. Anywhere between 20 -150 people in every 100,000 people having the disease. That means in a small city of 200,000 no more than 300 people will have the condition. Contrast that to the incidence of Rheumatoid arthritis which is 1% of the population meaning that in a town of 200,000 people 2000 people will have the condition.

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65% of people are diagnosed between the ages of 16 and 55.

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Systemic lupus can also affect children but it is far less common than in adults. The disease if it occurs in children tends to be far more severe than in adults.

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How is systemic lupus diagnosed?

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The diagnosis of lupus is made on clinical features, ie the sorts of symptoms that are occurring in conjunction with blood tests and other x-rays investigations if needed. A diagnosis of lupus is not always straightforward and the best person to confirm or deny a diagnosis of lupus is a Rheumatologist.

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Auto-immune antibody testings are done including ANA, ENA, DS DNA and C3 and C4. If clinically indicated your Rheumatologist may order some specific blood test investigations for immune antibodies that increase the risk of clotting in association with Lupus.

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Other blood testings will be done looking at liver kidney and immune and blood functioning.

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Xrays and scans may be ordered depending on any other symptoms.

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ANA is the typical antibody that is said to diagnose systemic lupus. However, just because this is positive in the blood it doesn't mean that a person has lupus, and this is why it is important to see a Rheumatologist to investigate your symptoms if there is any question that you might have Lupus.

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How is systemic lupus treated?

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Treatment of systemic lupus is always tailored by your Rheumatologist according to the severity of your disease.

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Many people with systemic lupus have very mild disease and required very little treatment. Other people have severe and at times life threatening disease depending on which organs are involved and the treatment that they require is far more intense in keeping with their disease intensity.

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The mainstay of treatment for systemic lupus is DMARD therapy, which are the common group of immune drugs that we use in Rheumatology. To read more about DMARDs click here.

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The common DMARDs that we use to treat lupus are

 

• Hydroxychloroquine (aka Plaquenil)

• Sulfasalazine

• Methotrexate

• Mycophenolate

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And if the disease is very severe:

• Cyclophosphamide or Rituximab

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To control symptoms such as aches and pains, anti inflammatories can be used, or prednisolone if required.

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Many people like to explore the impact of dietary and lifestyle changes on their systemic lupus. Whilst these measures are not a cure for lupus some people find they can improve their quality of life in addition to taking medications.

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Please note that the most important thing is to have a good relationship with your Rheumatologist. If you have a diagnosis of lupus this is usually a lifelong diagnosis and your treatment will need to be tailored in partnership with your Rheumatologist over the years.

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What is the long term outcome with systemic lupus?

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Each person has their own individual course with systemic lupus. Typically the way that the disease starts tends to be the way that it continues. Thus, if it starts with aches and pains and a rash this is most likely how it will be over the years. However, if it starts with the involvement of many organs early on such as the lungs and the kidneys then this is a sign that the course of systemic lupus is going to be complicated and potentially life threatening.

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In addition, diseases such as chronic systemic lupus are associated with an increased risk of typical cardiovascular disease. Thus its important to liase with your GP as well as your Rheumatologist to make sure that all your risk factors for heart disease such as high blood pressure and cholesterol and diabetes are well controlled.

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We do know that men are more likely to have more organ involvement with systemic lupus than women and thus have a worse outcome than women tend to.

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Also people of Asian descent seem to have more severe systemic lupus as well.

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Those of lower levels of education tend to have a worse outcome and it is suggested that this may be due to poor compliance with treatment.