The nightmare before lupus

6 minute read

There’s (still) no clear or common pattern, but clinicians and patients should be alert to neuropsychiatric symptoms as an early warning system.

“It’s never lupus,” says Gregory House, by way of explanation, as he retrieves a bottle of Vicodin stashed in a hollowed-out SLE textbook.

The autoimmune disease was a running joke on the diagnostic detective show thanks to its reputation as a master of disguise. Almost any unusual presentation could be it but generally wasn’t.

If SLE is a diagnostic nightmare, neuropsychiatric SLE – or NPSLE, in which symptoms are driven by disease activity on the brain and nervous system – is even harder because of the limitations of objective testing.

Symptoms are subjective and often under-reported, according to the authors of this study, published in the Lancet, which sought to establish whether neuropsychiatric features such as nightmares and hallucinations constitute a reliable prodrome for disease onset and subsequent disease flares.

Identifying a prodrome, they say, could help patients get a validated diagnosis and get their recurrent flares treated sooner. 

Part of the INSPIRE project, this study comprised a survey of more than 1000 lupus patients, mostly in the UK and Europe, and interviews with 119. The survey asked when they had first experienced each of a list of NP symptoms.

Interviewees were asked about disrupted sleep, nightmares and “daymares” – a less alarming and stigmatising term for hallucinations; timing of NP symptoms relative to disease onset; progression of flares and NP as an early warning system; and the impact of prodromal symptoms being unidentified or misattributed.

Clinicians were also surveyed and interviewed.

Patients who knew how their flares typically progressed commonly reported neuropsychiatric symptoms as preceding the more commonly recognised early symptoms of joint pain and rashes – symptoms that weren’t commonly recognised by clinicians and were absent from guidelines. These included sudden mood changes, mania, feelings of unreality, increased sensory symptoms and nightmares – possibly caused by increased brain inflammation.

One NPSLE patient described the stages of their flare progress:

  1. Feeling “over-excited” and “unable to settle”
  2. Insomnia
  3. Increasingly talkative
  4. Labile mood and uncontrollable emotions (description assessed by study psychiatrists as likely pseudobulbar affect)
  5. Cognitive dysfunction (worsening as flare progresses)
  6. Joint pain
  7. Severe fatigue
  8. Difficulty swallowing and nausea
  9. Joint swelling including visibly in the knees and hands

When untreated, this patient would progress to severe confusion and delusions such as: “There were people on the TV trying to kill me”.

Another patient with non-neuropsychiatric lupus said their hallucinations were mild and not unpleasant, “usually small animals”.

While other clinicians reported discussing sleep disruption with patients, only one of the rheumatologists did so and several others expressed scepticism.

For some patients, the interview was the first time they had considered the potential link between nightmares and flares, whereas for others the link was already clear.

“Several SLE patients recounted flares consistently involving the segueing of increasingly vivid and distressing nightmares into distorted reality and daytime hallucinations,” the authors say.

Nightmares “often involved being attacked, trapped, crushed, or falling”.

One patient described nightmares as “horrific, like murders, like skin coming off people, horrific”. Another said: “They are usually quite frightening, like there’s a serial killer after me … and he’s got my legs or something I can still feel something on my legs even when I’m then awake”. And another: “One of them was somebody attacking me and I ended up slitting their throat. Oh I mean, really nasty. I mean I’m not a violent person at all. I don’t even kill an insect … I’d be riding a horse, going around cutting people out with my sword, that kind of thing, which is bizarre.”

Why nightmares? The explanation could include “inherent effects on REM sleep stability and sleep architecture specific to the SLE or, less specifically, to generalised inflammation, or attributable to neurophysiological changes as a result of SLE CNS involvement”, the authors say.

“Initiating discussion of NP symptoms by first discussing disrupted dreaming sleep, and using more descriptive and less stigmatised language such as ‘daymares’ rather than ‘hallucinations,’ may encourage more patient openness in reporting these often feared and stigmatised symptoms,” they suggest.

The use of the term “daymare” resonated with some patients in the study, including one who said: “As soon as you said that it just made sense, it’s like not necessarily scary, it’s just like you’ve had a dream and yet you’re sitting awake in the garden … it’s like feeling really disorientated, the nearest thing I can think of is that I feel like I’m Alice in Wonderland”.

Many patients reported being (mis)diagnosed with a psychiatric or psychosomatic illness before their diagnosis with lupus, and several had been hospitalised in their late teens/early 20s with psychotic illness and/or suicidal ideation.

Clinicians described several cases “where patients with undiagnosed SLE had been involuntary psychiatric inpatients for many months before any autoimmune panels were requested”.

At the same time, while there was a consensus among rheumatologists that NP symptoms presented around the same time as disease onset and diagnosis, patient and psychiatrist accounts disagreed, with many or most patients experiencing their NP symptoms more than a year after their diagnosis.

This may contribute to delayed diagnosis, the authors say.

“[C]linicians and patients should remain vigilant for NP symptoms first presenting at any stage in the disease course,” the authors say. “Excluding symptoms occurring at various timescales before diagnosis as unrelated to SLE will also exclude symptoms that may represent a psychiatric and/or neurological prodrome to SLE.”

The researchers were hoping to discover two things from the exercise: consistent prodromal features within each patient, and common prodromal features among patients.

There was too much variation among patients to satisfy the latter, and a “prospective quantitative study is therefore now required to test the theory of an NP prodrome in SLE and further explore NP symptoms in other [autoimmune diseases]”.

It’s fascinating, but still very messy, is the takeaway.

And just when you’re ready to throw it all in and go and grow potatoes, the authors remind you; “It is also important to consider the increasing evidence that an episode of severe psychiatric illness, an infection, or a stressful life event, may induce immune dysregulation and thus precipitate the onset/flare of an autoimmune disease.”

It’s enough to make any doctor cut up their lupus textbook to stash drugs in.

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