How Is Neuropsychiatric Lupus (NPSLE) Diagnosed?

Medically Reviewed on 6/7/2022
neuropsychiatric lupus
Most people have a normal lifespan and an excellent quality of life if NPSLE is treated early.

The most basic approaches to detect nervous system abnormalities in systemic lupus erythematosus (SLE) are cognitive tests and a magnetic resonance imaging evaluation. However, a diagnosis of neuropsychiatric lupus (NPSLE) might be difficult to establish.

The doctor may order tests to rule out an infection or other health issues.

8 tests for proper diagnosis of NPSLE

  1. Blood tests
  2. Imaging tests, such as computed tomography scan or magnetic resonance imaging
  3. Electroencephalogram
  4. Nerve conduction tests
  5. Spinal tap
  6. Electrophysiological studies
  7. Psychiatric evaluation
  8. Autoantibody tests

To diagnose NPSLE, it must be identified if neuropsychiatric symptoms are caused by SLE, a distinct comorbid disorder, or an unfavorable outcome of disease therapy. Furthermore, the beginning of neuropsychiatric symptoms may occur before the diagnosis of lupus.

Statistics on NPSLE vary greatly due to a lack of common diagnostic criteria.

What is neuropsychiatric lupus (NPSLE)?

Lupus that affects the brain, spinal cord, or nerves is referred to as neuropsychiatric lupus (NPSLE). Systemic lupus erythematosus (SLE) is a chronic autoimmune condition with an unpredictable clinical course that includes periods of remission and intense flares. Lupus may impact each organ system in the body, but one of the most prevalent forms of the illness is neuropsychiatric symptoms that are still poorly understood.

  • NPSLE is a kind of lupus that has neuropsychiatric symptoms that are believed to be caused by this systemic autoimmune illness.
  • NPSLE symptoms might last for a long time or come and go. They might range in severity from minor to severe.

13 possible signs and symptoms of NPSLE

  1. Headaches not improved by over-the-counter medication
  2. Anxiety
  3. Depression
  4. Confusion
  5. Attention problems
  6. Seizures
  7. Stroke
  8. Abnormal gait (walking)
  9. Tremors
  10. Psychosis
  11. Vision problems
  12. Lupus fog is a combination of memory loss, fatigue, confusion, and trouble expressing thoughts
  13. Blood vessel inflammation (called vasculitis) is a rare form of NPSLE and can cause high fevers, seizures, and strokes, as well as problems with thinking, attention, and memory


Systemic Lupus Erythematosus Browse our medical image collection to see of photos of autoimmune, vascular, and other systemic conditions See Images

19 NPSLE syndromes

Following a diagnosis of systemic lupus erythematosus (SLE), many neurological and psychiatric symptoms could be discovered. The American College of Rheumatology (ACR) developed a set of classifications for 19 NPSLE syndromes, which were then classified as either central nervous system (CNS) or peripheral nervous system (PNS) presentations of this illness.

12 CNS syndromes observed in NPSLE

  1. Aseptic meningitis
  2. Cerebrovascular disease
  3. Demyelinating syndrome
  4. Headaches, migraines, and intracranial hypertension
  5. Movement disorders
  6. Myelopathy
  7. Seizures
  8. Acute confusional state
  9. Anxiety disorders
  10. Cognitive dysfunction
  11. Mood disorders
  12. Psychosis

7 syndromes that affect the PNS

  1. Guilliain-Barré syndrome
  2. Autonomic disorder
  3. Mononeuritis
  4. Myasthenia gravis
  5. Cranial neuropathy
  6. Plexopathy
  7. Polyneuropathy

These issues typically develop during lupus flares in other bodily organs as well (such as arthritis, skin rash, or kidney disease). To be sure, physicians rule out other possibilities, such as atherosclerosis, stroke, or tumor.

What causes neuropsychiatric lupus (NPSLE)?

It is unclear how lupus produces neuropsychiatric issues. Autoantibodies could be the cause in many situations (antibodies that are against the body). In some situations, these antibodies might reach the brain and trigger inflammation.

In other circumstances, the difficulties are caused by the release of proteins that promote inflammation within the brain. In general, these issues arise when the lupus is particularly active (flaring).

9 risk factors of NPSLE

  1. Genes
  2. Hormonal imbalance
  3. Age 15 to 44 years
  4. Women are more prone to NPSLE
  5. Some prescription drugs
  6. Exposure to sunlight
  7. Stress
  8. Infections
  9. Cigarette smoke and secondhand smoke

Although the underlying processes are still being studied, various pathogenic pathways have been found that may cause NPSLE.

3 common pathways that may lead to NPSLE

  1. Antibody-mediated neurotoxicity
  2. Vasculopathy is caused by anti-phospholipid antibodies
  3. Cytokine-induced neurotoxicity

What are the treatment options for neuropsychiatric lupus (NPSLE)?

Management of neuropsychiatric lupus is similar to the management of neuropsychiatric disease in patients without lupus. Treatment depends on the underlying causes of a patient’s disease.

13 treatment options to manage NPSLE

  1. Nonsteroidal anti-inflammatory drugs
  2. Corticosteroids
  3. Anticoagulants
  4. Blood thinners
  5. Symptomatic therapy
  6. Intravenous immunoglobulin
  7. Plasmapheresis
  8. Conventional and biologic immunosuppressive medications
  9. Antimalarial-like drugs
  10. Antiseizure medications
  11. Antidepressants
  12. Lifestyle changes and diets as prescribed by doctors and therapists
  13. Associating with support groups

Novel therapies, including small-molecule inhibitors and biologic agents that target inflammatory pathways, are currently being explored to target NPSLE specifically.

Prognosis of NPSLE

  • Most people have a normal lifespan and an excellent quality of life if NPSLE is treated early. When one therapy does not work, physicians will try another.
  • Neuropsychiatric SLE is a significant lupus condition that requires immediate attention and treatment. Good communication between the patient and the doctor is critical for early diagnosis and appropriate treatment.
  • For the optimal management of these patients, the rheumatologist frequently collaborates with a neurologist. When patients are treated in this manner, the outcomes are frequently quite positive and pleasant for both patients and clinicians.
  • Neuropsychiatric symptoms afflict roughly half of patients with systemic lupus erythematosus. However, the impact on disease severity, quality of life, and prognosis are enormous.
  • Neuropsychiatric lupus symptoms can range from moderate to severe and life-threatening.

Some lupus therapies may raise the chance of getting potentially deadly infections. However, the vast majority of people with lupus may expect to have a normal or near-normal life.

Medically Reviewed on 6/7/2022
Image Source: iStock image

Kivity S, Agmon-Levin N, Zandman-Goddard G, Chapman J, Shoenfeld Y. Neuropsychiatric lupus: a mosaic of clinical presentations. BMC Med. 2015;13:43.

Yoon S, Kang DH, Choi TY. Psychiatric Symptoms in Systemic Lupus Erythematosus: Diagnosis and Treatment. J Rheum Dis. 2019; 26(2): 93-103.

Kirou KA. Neuropsychiatric SLE: Lupus and the Brain. Hospital for Special Surgery.