Lupus affecting the brain (lupus cerebritis): symptoms, tests and management

Lupus is a long-term autoimmune condition. The NHS says that in lupus the body's natural defence system attacks healthy parts of the body, and that it ranges from mild to severe. In its severe form the NHS says inflammation can cause severe damage to the heart, lungs, brain or kidneys, which can be life-threatening.

Addenbrooke's Hospital explains that 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 the kidneys, brain, nervous system, lungs, heart, gastrointestinal tract and the linings around internal organs, and patients may have varied types and severity of symptoms over the course of their disease.

The terms used for brain involvement vary. Doctors speak of neuropsychiatric lupus or lupus affecting the central nervous system, and the older, informal word cerebritis suggests inflammation of the brain. The information below follows the sources used and describes the symptoms and what specialists do rather than any single label.

Addenbrooke's says that cognition means the ability to think, understand and process information, solve problems, remember, communicate and function in day-to-day life, and that cognitive dysfunction is an umbrella term for changes in any of these. Other phrases you may have heard are brain fog and cognitive impairment.

Every patient with lupus is different, but common themes include loss of concentration and attention, loss of memory or forgetfulness, inability to follow a conversation, losing the train of thought when speaking, and being unable to problem solve, plan or be organised. Patients often report that symptoms vary in severity and may not be linked to the activity of lupus.

The leaflet describes more worrying symptoms as disorientation, repetitive behaviour, getting lost in familiar places or visual hallucinations. The NHS lists headaches, depression and anxiety among the possible symptoms of lupus more generally.

• Poor concentration or attention.

• Forgetfulness or memory loss.

• Difficulty following conversations or finding words.

• Trouble planning, organising or problem solving.

• Low mood or anxiety.

• More worrying: disorientation, getting lost in familiar places, hallucinations.

Addenbrooke's lists several symptoms, which it calls red flags, that are rare but that the medical team want to know about. Blackouts may be simple faints but need an ECG and consideration of seizures. A very sudden onset of cognitive symptoms could be due to a stroke and it is important to have this assessed rapidly. Disorientation, hallucinations and severe language symptoms, such as sudden impaired speech or inability to find words, usually require urgent medical assessment.

It also asks people to mention episodes of losing memory of a significant event, such as a trip away, and concerns raised by family and friends, who may notice changes that you do not. It says limb weakness and visual problems require urgent review by neurologists or ophthalmologists. In an emergency such as sudden weakness, speech difficulty or collapse, call 999.

The leaflet says the causes of cognitive dysfunction are varied. They can be linked to lupus itself, particularly in people with antiphospholipid syndrome (APS) or other brain-directed antibodies, and activity of lupus elsewhere in the body may be enough to cause mild symptoms. Medicines, fatigue, stress, poor sleep (including sleep apnoea), depression or anxiety can contribute.

It specifically mentions that steroids have many side effects, with cognitive dysfunction reported as one, more pronounced with long-term use and higher doses, and that some pain relief containing opiates can contribute too. It advises not stopping steroids suddenly because this can cause serious medical problems related to the adrenal glands, blood pressure and infections, and to speak to your doctor if you have concerns.

Addenbrooke's says your lupus doctor may refer you to a neurologist or neuropsychologist if they have concerns, who will take a full history and may arrange tests to rule out other causes that require treatment. A brief neurological examination and cognitive assessment or neuropsychological assessment can take from 10 minutes to three hours.

Blood tests can include antiphospholipid antibodies (anti-cardiolipin, anti-beta 2-glycoprotein-1 and lupus anticoagulant), which can be associated with strokes, mini-strokes and antiphospholipid syndrome, and antibodies associated with autoimmune encephalitis. Other blood tests check for anaemia, vitamin B12, thyroid function and vitamin D, and lupus activity markers such as anti-dsDNA, complement and inflammatory markers.

Specialist procedures can include MRI or CT scans, an EEG, which records activity of the brain, and sometimes a small sample of cerebrospinal fluid, to rule out other causes and look for inflammation or blood vessel abnormalities. The same leaflet notes that the clinician may also assess mood, because depression or anxiety can contribute.

The NHS says that treatment for lupus can include hydroxychloroquine, steroids and immunosuppressant or biological medicines for severe disease, and that lupus is better managed when found and treated early. Which treatment is right for brain involvement is decided by the lupus team, sometimes with neurology and psychiatry, and depends on the cause found.

For cognitive symptoms in the absence of a serious underlying cause, Addenbrooke's suggests practical steps: talking to family, friends and employers; good sleep; pacing activity across the day and week; regular suitable exercise; relaxation or mindfulness; reviewing medicines with your doctor; and using memory aids such as a diary, calendar, whiteboard, phone alarms, pill dispensers and a single place for keys and medicines.

On the NHS, lupus is usually managed by a rheumatology service, sometimes a specialist lupus clinic with a dedicated nurse, which can refer on to neurology, neuropsychology, psychiatry or a memory clinic. Urgent symptoms are assessed through A&E or acute neurology.

Private rheumatology and neurology are options for faster appointments or second opinions, but investigations such as MRI, EEG and specialist antibody tests are the same. It helps if one team coordinates results and the lupus consultant stays informed.