Cognitive symptoms in systemic lupus erythematosus (SLE)

About lupus

Lupus is an autoimmune disease caused by various faults in the body’s immune system.

One way this occurs is that the immune system starts making autoantibodies (proteins) that, instead of defending the body from infection, attack its own healthy tissue. Other ways include the body’s innate defence mechanisms (the complement system) or B and T cells directly causing damage. Over activity of the immune system can lead to inflammation and potentially damage tissues in the body.

Lupus is a multi-system condition, which affects different people in different ways. It can affect the skin and joints or other major organs such as kidneys, brain, nervous system, lungs, heart, gastrointestinal tract and/or the linings around internal organs.

Lupus is a long-term condition and patients may have varied types and severity of symptoms over the course of their disease.

What is cognitive dysfunction and how can it affect me?

The term ‘cognition’ refers to your ability to think, to understand and process information, to solve problems, to remember, to communicate, and to function fully in your day-to-day life as a thinking person. The term ‘dysfunction’ refers to an impairment or abnormality. Many of us have day-to-day cognitive symptoms, but significant dysfunction due to an underlying medical cause is uncommon.

Cognitive dysfunction is an umbrella term to describe changes or problems in any aspect of cognition. Other phrases you may have heard used include brain fog and cognitive impairment.

The causes of cognitive dysfunction are varied and can be linked to SLE, particularly in those with the anti-phospholipid syndrome (APS) or other brain-directed antibodies. The activity of lupus in the rest of the body may be sufficient to cause mild cognitive dysfunction symptoms. Medications, fatigue, stress, poor sleep (including sleep apnoea, when you briefly stop breathing), depression or anxiety can also contribute to cognitive dysfunction.

Every patient with lupus is different and reports different symptoms but common themes include:

1. loss of concentration and attention
2. loss of memory or forgetfulness
3. inability to follow a conversation
4. loss of train of thought when speaking to someone or forgetting what you are saying
5. unable to problem solve, plan or be organised

More worrying symptoms include disorientation, repetitive behaviour, getting lost in familiar places, or visual hallucinations.

Cognitive symptoms are commonly reported by patients with lupus, and these can fluctuate from day-to-day. Patients often report that their symptoms also vary in severity and may not be linked to the activity of lupus.

Cognitive dysfunction in some instances may be the result of a direct attack of lupus on the brain; this may be visible on scans and accompanied by other features such as seizures (fits). More commonly though, the brain scan is normal and there are no associated features suggest that the brain is being affected by lupus.

How can I understand my symptoms?

Initial recognition of these symptoms may be by you or by your family and friends noticing a change. It can be scary to have episodes of forgetfulness or difficulties in carrying out a simple task and patients may worry they have something seriously wrong with them or are developing dementia.

These symptoms are usually not a sign of early dementia and an assessment by a neurology specialist with support from neuropsychologists can be arranged by your lupus team to rule this out.

Your lupus doctor may make a referral to a neurologist or a neuropsychologist if they have concerns about your symptoms. These specialists will talk to you about your concerns and take a full history of how your symptoms have developed. They may also arrange for you to undergo various tests to rule out any other causes of cognitive dysfunction that require treatment.

When should I tell my doctor about the cognitive problems I have?

We encourage you to talk to your lupus doctor about any changes you have noticed in your health at your clinic appointments. If you have concerns between appointments, you can contact your dedicated specialist nurse. It may be useful to keep a diary of your symptoms and bring this with you to clinic, along with a relative or friend who can give an independent account of your symptoms.

We advise you to discuss any of the following with your medical team as although these symptoms, commonly referred to as ‘red flags’, are rare we would like to know about them:

Your doctor may consider other factors, such as anxiety, depression and a review of your medication if you have been on long-term steroids (of a high dose), immunosuppression over ten years and/or strong painkillers, as these can have an effect on your cognitive ability.

If you are referred to a specialist, you may see:

Neurologist

A neurologist is a doctor who specialises in treating conditions of the nervous system. This includes the spinal cord and brain, and nerves in your arms, legs and muscles.

Psychiatrist

Psychiatrists also look after diseases of the brain and increasingly work alongside neurologists in diagnosing and managing cognitive symptoms.

Neuropsychologist

A neuropsychologist specialises in understanding the relationship between the brain and behaviour. The brain is very complex and any damage to it can alter behaviour, mood and cognition. A neuropsychologist will undertake assessments of how your brain is processing information and will then be able to establish whether any dysfunction exists, and if so, what the cause of it is.

Counsellor

A counsellor is trained to listen and be able to advise you with psychological worries. They enable you to gain better understanding of these, often through cognitive behavioural therapy and cognitive analytical therapies.

Memory clinic

You may be referred to a specialist in a memory clinic who will be able to assess your cognitive dysfunction in detail, particularly if you have concerns about your memory. The assessments that the doctors carry out in these clinics enable them to rule out other causes of memory loss and can give you an idea of what processes in your brain are not functioning correctly and how you can help yourself.

Specialist lupus nurse

Addenbrooke’s has a a dedicated specialist nurse for your lupus, and it is always advisable to discuss any concerns with them as they will be able to review your care with the team.

What tests will I need and why?

If you are referred to a specialist, you may need:

What can I do to help myself?

Here are some tips on how to manage the symptoms of cognitive dysfunction:

Emotional support

If you have been told there is no serious underlying cause of your cognitive dysfunction you may still feel worried about how to manage your symptoms. Having problems with cognitive function often affects your quality of life and everyone deals with these problems in different ways. There is no right or wrong way to manage your symptoms, but the following ideas may help.

Communication with others

Talking to family, friends and healthcare professionals about the issues you have can be very helpful and reassuring. You can feel isolated and frustrated when you are finding it difficult to complete a task or to socialise with friends. The people around you may not be aware you have these issues and could support you to manage them by being more aware.

Some people have found that their cognitive dysfunction has caused problems at work, as they are not able to function as well as before. You may feel you need more time to carry out certain tasks and that you tire more easily. If you feel it is appropriate, discussing your difficulties with your managers may be helpful. They may be able to change or adapt your role or provide you with more assistance.

Coping with fatigue

Ensuring you have a good night’s sleep will enable you to feel rested the next day. Being overtired can make your cognitive dysfunction worse because when you are tired your ability to concentrate diminishes and you may feel unable to function as well as normal.

Pacing is a way of planning and structuring your day or week. If you know you have a busy day ahead, try to have a more restful one beforehand. Plan tasks throughout your day and have regular breaks. For example, you don’t have to do all your housework in the morning. Spreading it out over the day with breaks will allow you to recharge your batteries and work more effectively.

Exercise

Regular exercise, at a level that is suitable for you, is good for all‑round wellbeing, whether this is walking, running, swimming or visiting the gym; whatever you enjoy is the best for you.

Techniques such as yoga, pilates or tai chi are recommended. These exercises focus on strength flexibility and breathing to boost your physical and mental wellbeing. All these techniques can relieve stress and tension, which in turn can have a beneficial impact on your cognitive function.

Relaxation

Meditation and relaxation techniques may also be beneficial as they allow you some ‘me time’ and provide a switch-off period.

Mindfulness relaxation is a form of meditation that can be done in 10-minute exercises. It uses breathing methods, guided imagery, and other practices to relax the body and mind and help reduce stress.

Including these exercises in your daily life is a good way of dealing with stress and anxiety, both of which can affect your cognitive dysfunction. When your brain is overloaded, mindfulness exercises can allow you to switch off and your brain to refocus.

Management of medications

Some analgesia (pain relief) contains opiates and side effects can contribute to cognitive dysfunction. It is best to discuss your pain relief with your GP or consultant and they can review your medications.

Steroids have many side effects; cognitive dysfunction being reported as one of them. The effects are more pronounced with long-term use and at higher doses. Your consultant will be aiming to reduce your steroids to a suitable lower dose as soon as clinically possible. If you have concerns, ensure your doctor is aware of them and your medication can be reviewed as appropriate. You should not stop steroids suddenly as this can cause serious medical problems related to the adrenal glands, blood pressure, and infections.

Memory aids

Memory aids are tools that assist you in remembering day-to-day tasks and appointments. Use of these can improve your independence and help you to feel more in control. Some memory aids are described below:

• A memory centre is an area in your home, for example a desk in the corner of a room where you keep your organisational aids such as calendar, diary, white board and any other device that holds information regarding yourself or others that you are worried about forgetting. It is also a good place to put easy-to-lose items such as keys and medicine dispensers. It is designed to reduce stress, so that if you are worried you have forgotten something there is only one place you need to check.
• White boards These boards come in different sizes and are re-useable with a dry marker pen. You can put important information on them, such as what is happening that day, the phone numbers of close family and friends and daily messages such as ‘John has gone to Sainsbury’s and will be back at 2pm’.
• A day/ date clock – this is best placed near a white board or calendar/ diary.
• Diary/ calendar – small diaries that fit into your pocket or handbag are useful for jotting down appointments and so on. Larger desk diaries or wall calendars can be used in the home in prominent places.
• Lists/ post-it notes/ notebook – post‑it notes are handy as they are often brightly coloured and stand out. If there is something you must not forget, you can write notes on one and put it in a prominent place. Make check lists and ‘tick off’ tasks as they are completed.
• Smart phone apps – mobile phones are a handy electronic tool to use as they often come with different functions, and some have applications that can be downloaded.
• Alarm clocks/ alarm settings on mobile phones – these can be used not just for getting you out of bed in the morning, but as a reminder to collect your children from school, for example.
• Seven-day pill dispensers – If you have difficulty remembering when to take medicines or recalling if you have taken them, a pill dispenser box may be useful. These can store a week’s worth of medication and when you use it you can see what time the tablets must be taken each day. Your pharmacist can set these up for you if you prefer.
• Visual cues – Using the same location for items such as car keys allows you to find them again when needed. Keeping a note beside the kettle may be a trigger to remember to take medication.

Family and friends can assist with the memory aids as they can make notes/ reminders of appointments and so on that you need to remember using the tools above. Building the tools into daily life makes them become a normal part of life. Some patients worry that they won’t remember to look at the tools, so family and friends can jog their memories to look at the diary for today’s list of tasks, for example.

Organisational tips

Patients often report that they struggle to multitask or be able to plan things. One tip for this is to make a list of what you need to do and tick off as you go. Try not to do everything at once as this can overwhelm you and make you feel more ‘muddled’. By pacing yourself you will find you will be able to work more effectively.

Fatigue will also play a part here as the more tired you are the more likely your brain will not function quite as well. Taking regular breaks and trying to switch your mind off from the previous task can be helpful to ‘recharge’ your mind. For example, undertake 30 minutes of housework, stop and perhaps go for a short walk, and then do another task on your list.

Clinic appointment tips

Hospital clinics can be very busy so being prepared with a short list of questions or issues you want to discuss prior to your appointment can be helpful. It’s very easy to forget something once you are in with the doctor so a notebook with your list is a good way to remember. Write down what the doctor tells you at the time as this will aid you once you are home. If possible, take someone into the consultation with you, as a second person will also remember what the doctor has said. There is a dedicated specialist nurse linked to the clinic. Ensure you have their contact numbers and telephone them if you are unsure about anything. Some hospitals will be able to post you a copy of your clinic letter. Request this if you can.