Clinical: life versus lupus

22 July 2020

Lupus is often associated with women, but a fifth of cases are diagnosed in childhood. It can make a huge impact on a young person’s life but, with support, they can thrive, says journalist Julie Penfold.

Lupus is a long-term autoimmune condition where the body’s immune system starts to attack healthy tissues, causing inflammation to the joints, skin and organs such as the kidneys, lungs, heart and the brain. The most common form of lupus is systemic lupus erythematosus (SLE). It’s a relatively rare condition that affects around 98 people in every 100,000 (Rees et al, 2014).

Around 90% of lupus cases are diagnosed in females and it’s most common in women of child-bearing age – but it can also affect people of other ages, including children (Lupus UK, 2020). People from a black and minority ethnic (BAME) background including those of African, Caribbean or Asian origin have an increased risk of developing lupus.

A murky history

It’s not yet fully understood what causes lupus, but genetic factors can be involved. Hormonal changes due to puberty, pregnancy and the menopause can also trigger the condition. Pregnancies in women with lupus are considered high-risk and they are advised to discuss the risks with their doctor before trying to get pregnant (NHS, 2018). Complications that can occur during pregnancy include pre-eclampsia, stillbirth, miscarriage, premature delivery and thrombosis. However, it is possible for these risks to be safely managed (Lupus Trust, 2020).

‘In terms of what causes lupus, we don’t really know,’ explains Dr Clare Pain, paediatric rheumatologist at Alder Hey Children’s Hospital. ‘In some cases, there are genetic links but that’s quite rare. There are specific gene defects that can cause lupus, but that is very unusual. It’s likely that it’s a combination of having a bit of a genetic predisposition and then getting a second hit – a trigger of some kind that sets the immune system into an overactive state that doesn’t switch off. There have been suggestions about viral infections and ultraviolet light, but we don’t really know why. It’s quite difficult for young people to get their heads around why it’s them that has lupus compared to someone else.’

Another type that’s less common is cutaneous lupus, where the skin is specifically affected and a variety of rashes can develop. ‘The most commonly known is discoid lupus, which causes circular rashes and can lead to scarring,’ explains Paul Howard, chief executive of Lupus UK. ‘This scarring can become especially noticeable when it affects parts of the head where there is hair, because it can lead to permanent hair loss. It’s a real concern for a lot of young women and it can have a significant impact on their confidence and self-image.’

What are the symptoms of lupus?

Signs of lupus can include:  

  • Joint pain and stiffness  
  • Extreme tiredness and fatigue  
  • Skin rashes – these often appear over the nose and cheeks  
  • Sensitivity to light – including ultraviolet light from the sun  
  • Mouth ulcers and swollen glands  
  • Poor circulation in the fingers and toes – symptoms of Raynaud’s.

NHS, 2018

A blight on young life

Lupus can affect children and young people differently and symptoms can range from mild, moderate to severe and potentially life-threatening. ‘Juvenile-onset lupus erythematosus or juvenile SLE is diagnosed in childhood and tends to be more severe,’ says Paul. ‘There is a higher likelihood of more severe symptoms developing, including lupus nephritis where the kidneys are affected.’

A young person affected by mild lupus might experience joint and skin problems, mouth ulcers and tiredness. When lupus is classed as moderate, inflammation has developed in other parts of the skin and body, including the lungs, heart and kidneys. When lupus is severe, inflammation causes severe damage to the heart, lungs, brain or kidneys and it can be life-threatening (NHS, 2018).

In teenagers who are affected by SLE, it varies enormously from one young person to the next and from one day to the next. Even when symptoms are mild, they can still have a debilitating impact on a person’s life.

Dr Eve Smith is a paediatric rheumatology grid trainee at Alder Hey Children’s Hospital and an academic clinical lecturer at the University of Liverpool’s Experimental Arthritis Treatment Centre for Children. Eve has a particular interest in children and young people and has received Wellcome Trust funding to carry out a Living with Lupus study to help inform treatment targets and outcome measures for the future.

‘One of the common issues that young people really struggle with is that lupus is an invisible illness,’ Eve says. ‘Patients with lupus might look well on the outside, but they could be struggling a lot of the time, particularly with fatigue and being able to concentrate and get their head around their school work. They can also find it difficult to remember things. It’s hard for young people because they don’t want to be different.’


Diagnosis frustration

Getting a diagnosis can be a highly frustrating experience, particularly if symptoms are mild. Diagnosis is based on the young person’s symptoms and there are blood tests that can help to identify the presence of certain antibodies. However, lupus can present with symptoms that are similar to various other conditions, and similar blood test results can also occur to further confuse matters.

Paul feels part of the issue with slow diagnosis is that lupus isn’t routinely considered early enough. ‘Lupus isn’t a condition that is particularly well recognised, even within the medical profession,’ he says. ‘This means GPs will [often] consider other options before they think of lupus. They won’t necessarily request the blood tests or make a referral to a specialist until they’ve put together the whole picture. People often present with what seems like unrelated symptoms over a few months, so it can be difficult to put those together. Research we’ve carried out in the past has shown that as many as 48% of people were initially misdiagnosed and it takes an average of about six and a half years to get the right diagnosis.’

Clare sympathises with GPs but says early referral is vital. ‘The longer it takes to get a diagnosis and treatment, the worse the outcome is,’ she says. ‘It’s really key to get early referrals to us. But it can be difficult for GPs who may never have seen a case of lupus in a child when they can present with such minor or more significant symptoms. I think it depends on what they present with, because sometimes we get really early referrals. If you just have mouth ulcers, a slight glow in your cheeks and you feel tired as a teenager, these are quite common symptoms, as are headaches, so it can be more difficult then for GPs to consider lupus.’

There is currently no cure for lupus, but medications can help to relieve many of the symptoms of the condition. These include hydroxychloroquine, anti-inflammatory medications such as corticosteroids and immunosuppressants. Hydroxychloroquine is used more in mild to moderate lupus cases. In moderate to severe lupus, additional treatments such as steroids can be used. ‘They can be life-saving for many people when they have serious kidney impairment, but they can cause side effects,’ adds Paul. Immunosuppressants prevent lupus from causing damage to the body, but they can increase the risk of infection and that’s been a particular concern during the Covid-19 pandemic.

‘Some of the treatments that we give can change your appearance and affect how young people are feeling,’ Clare explains. ‘They can put on weight, get spots and it can also affect fertility. We tend to try and get on top of inflammation quite quickly using steroid medicines. Sometimes these are given orally or via a drip. These are the medicines our young people probably hate the most. They’re really effective, but they do have a lot of side effects such as weight gain. We’ve also seen patients that have had fractures because their bones have become thin as a result of the long-term use of steroids.’

Lifestyle factors are important in managing lupus too. Exposure to sunlight is a common trigger and patients are often prescribed high factor sunscreen. Regular, gentle exercise and eating a healthy diet are recommended too.

Advocating for young people

School nurses can be real advocates for young people living with lupus, says Jane Kelly, rheumatology nurse specialist at Alder Hey Children’s Hospital. ‘Lupus can be a very unpredictable condition. It’s important to understand the condition and how it can affect young people. Lupus can affect their concentration and memory, and can cause changes in mood. This can have a detrimental effect on quality of life and wellbeing, including education, but many describe fatigue as their worst symptom.’

Jane advises: ‘Providing a safe environment where young people can just chill and take time out when they need to take a breather can help.’ When children go back to school, she recommends ‘introducing more breaks, developing a care plan for school and making special arrangements to have more time during exams, particularly if they need to take a break’. School nurses can also help by speaking to teachers to make them aware of how the condition affects them. Young people can develop confidence when they have the advocacy of their school nurse, and that relationship is key when they are struggling or are worried about their exams.

Clare adds that communication between school nurses and specialist teams can also be beneficial for young people. They can liaise about questions and concerns as they crop up. ‘I think all paediatric rheumatology teams in the UK would be very open to communication with school nurses.’ 



British Society for Rheumatology. (2020) Covid-19 guidance. See: rheumatology.org.uk/news-policy/details/Covid19-Coronavirus-update-members (accessed 11 June 2020).

Lupus Trust. (2019) Lupus and pregnancy. See: lupus.org.uk/lupus-and-pregnancy (accessed 11 June 2020).

Lupus UK. (2015a) Epidemiology of lupus. See: lupusuk.org.uk/medical/gp-guide/introduction-to-lupus/epidemiology-of-lupus (accessed 11 June 2020).

Lupus UK. (2015b) Juvenile-onset lupus. See: lupusuk.org.uk/medical/gp-guide/diagnosis-of-lupus/associated-illnesses/childhood (accessed 15 June 2020).

NHS. (2017) Lupus. See: nhs.uk/conditions/lupus (accessed 11 June 2020).

Rees F, Doherty M, Grainge M, Davenport G, Lanyon P, Zhang W. (2014) The incidence and prevalence of systemic lupus erythematosus. Annals of the Rheumatic Diseases 75(1): 136-41. 

Picture Credit | iStock | Science Picture Library


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