Systemic lupus erythematosus (also known as lupus or SLE) is an autoimmune disorder that can cause chronic disease in different parts of the body. While the exact mechanisms for lupus are unknown, the condition ultimately represents an immune system gone awry, attacking normal cells it mistakenly sees as dangerous.

The central nervous system is just one of the targets of this autoimmune response. When it happens, it can manifest with psychiatric symptoms that are strikingly similar to bipolar disorder. While the symptoms of the two disorders overlap (as do the drugs used to treat them), SLE and bipolar are in no way related.

Despite popular belief, SLE does not cause bipolar disorder. On the other hand, SLE is sometimes misdiagnosed as bipolar disorder. When this happens, a person may be exposed to unnecessary and inappropriate treatment.

Neuropsychiatric Symptoms of Lupus

When lupus affects the central nervous system, it can cause a variety of symptoms, both neurological and psychiatric. We refer to this condition as neuropsychiatric systemic lupus erythematosus (NPSLE). Symptoms can range from mild to severe and include:

  • Blurred visionClumsiness or unsteady gaitConfusion and deliriumHeadachesHearing and speech problemsLoss of cognitive functionMemory lossMood disorders, including depression and bipolar-type symptomsSeizuresStrokeTingling, numbness, burning, and painful nerve sensationsTremors, tics, and involuntary movement

NPSLE is considered a serious complication that leads to a reduced quality of life and an increased illness. Current research suggests NPSLE is associated with a nearly ten-fold increase in mortality compared to people in the general population.

NPSLE affects 80–90% of people with lupus, most frequently manifesting as headaches, depression, and general cognitive decline.

Causes

Rather than having one specific cause, NPLSE is due to a combination of factors including immune dysfunction, hormonal irregularities, vascular inflammation, and direct damage to nervous tissue. Even drug side effects may contribute to the symptoms.

Some of the symptoms of NPLSE may also be related to a condition called demyelinating syndrome in which the autoimmune response gradually strips away the myelin sheath (think of it as the insulating cover) of a nerve. Depending on where this occurs, it can trigger a variety of sensory, cognitive, and visual problems.

Moreover, the protective layer which surrounds the brain, called the blood-brain barrier, can be disrupted by lupus, allowing toxins to penetrate and damage neural tissue.

Diagnosis

Because it is difficult to distinguish between the various causes of NPSLE (including independent psychiatric disorders), there is no gold standard for diagnosis. As such, diagnosis is typically made by exclusion, exploring all other possible causes including infection, coincidental disease, and even drug side effects.

This is made on a case-by-case basis under the direction of a specialist experienced in NPSLE. If demyelination syndrome is suspected, tests may be performed to confirm the presence of autoimmune antibodies (autoantibodies) associated with myelin damage.

Treatment

Typically speaking, the medications used to treat psychiatric and mood disorders may also be used to treat the psychiatric symptoms of lupus. In the event of severe NPSLE, treatment will be focused on the use of medications that suppress and moderate the autoimmune response.

Options include high-dose corticosteroids (such as prednisone or dexamethasone with intravenous cyclophosphamide). Other standard treatments include rituximab, intravenous immunoglobulin (antibody) therapy, or plasmapheresis (plasma dialysis).

Mild to moderate symptoms may be treated with oral azathioprine or mycophenolate. It’s important to note, however, that high doses of corticosteroids may exacerbate mood disorders and, in rare cases, lead to psychosis.