Neuropsychiatric systemic lupus erythematosus

Neuropsychiatric systemic lupus erythematosus or NPSLE refers to the neurological and psychiatric manifestations of systemic lupus erythematosus. SLE is a disease in which the immune system attacks the body's own cells and tissues. It can affect various organs or systems of the body. It is estimated that over half of people with SLE have neuropsychiatric involvement.[1]

Neuropsychiatric systemic lupus erythematosus
Other namesCNS lupus, lupus cerebritis
SpecialtyRheumatology, Neurology

Classification

The American College of Rheumatology (ACR) has outlined 19 syndromes that are seen in NPSLE. These syndromes encompass disorders of the central and peripheral nervous systems:[2]

Each of the 19 syndromes are also stand-alone diagnoses, which can occur with or without lupus.

The majority of cases involve the central nervous system (CNS), which consists of the brain and spinal cord.[2] The most common CNS syndromes are headache and mood disorder.[1]

Though neuropsychiatric lupus is sometimes referred to as "CNS lupus", it can also affect the peripheral nervous system (PNS). Between 10-15% of people with NPSLE have PNS involvement.[3] Mononeuropathy and polyneuropathy are the most common PNS syndromes.[1]

Other syndromes

Some neurological syndromes outside of the ACR classification may also be considered NPSLE manifestations. These include neuromyelitis optica, posterior reversible encephalopathy syndrome, small fiber neuropathy,[4] and Lambert–Eaton myasthenic syndrome.[5]

Pathogenesis

There are several possible mechanisms that underlie the nervous system manifestations of lupus. Specific syndromes may be vasculopathic, autoantibody-mediated, or inflammatory in nature.

There is evidence that the blood–brain barrier, which protects the central nervous system, is compromised in patients with NPSLE. As a result of this, autoantibodies are able to infiltrate the CNS and cause damage.[6]

Diagnosis

For diagnosis of NPSLE, it must be determined whether neuropsychiatric symptoms are indeed caused by SLE, whether they constitute a separate comorbid condition, or whether they are an adverse effect of disease treatment. In addition, onset of neuropsychiatric symptoms may happen prior to the diagnosis of lupus.[7] Due to the lack of uniform diagnostic standards, statistics about NPSLE vary widely.[8]

Tests which aid in diagnosis include MRI, electrophysiological studies, psychiatric evaluation, and autoantibody tests.[9]

Treatment

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, and may include immunosuppressants, anticoagulants, and symptomatic therapy.[9]

See also

References
  1. Unterman, Avraham; Nolte, Johannes ES; Boaz, Mona; Abady, Maya; Shoenfeld, Yehuda; Zandman-Goddard, Gisele (August 2011). "Neuropsychiatric Syndromes in Systemic Lupus Erythematosus: A Meta-Analysis". Seminars in Arthritis and Rheumatism. 41 (1): 1–11. doi:10.1016/j.semarthrit.2010.08.001. ISSN 0049-0172. PMID 20965549.
  2. Liang, Matthew H; Corzillius, Michael; Bae, Sang Cheol; Lew, Robert A; Fortin, Paul R; et al. (April 1999). "The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes". Arthritis and Rheumatism. 42 (4): 599–608. doi:10.1002/1529-0131(199904)42:4<599::AID-ANR2>3.0.CO;2-F. ISSN 0004-3591. PMID 10211873.
  3. Kivity, Shaye; Agmon-Levin, Nancy; Zandman-Goddard, Gisele; Chapman, Joab; Shoenfeld, Yehuda (March 2015). "Neuropsychiatric lupus: a mosaic of clinical presentations". BMC Medicine. 13 (1): 43. doi:10.1186/s12916-015-0269-8. ISSN 1741-7015. PMC 4349748. PMID 25858312.
  4. Bortoluzzi, Alessandra; Scirè, Carlo Alberto; Govoni, Marcello (14 March 2018). "Attribution of Neuropsychiatric Manifestations to Systemic Lupus Erythematosus". Frontiers in Medicine. 5: 68. doi:10.3389/fmed.2018.00068. PMC 5861139. PMID 29594125.
  5. West, Sterling G; Hanly, John G (2019). "Lupus and the Nervous System: Clinical Aspects, Psychopathology, and Imaging". In Wallace, Daniel J; Hahn, Bevra Hannahs (eds.). Dubois' Lupus Erythematosus and Related Syndromes (Ninth ed.). Elsevier. pp. 434–456. doi:10.1016/B978-0-323-47927-1.00036-0. ISBN 978-0-323-47927-1. S2CID 80722393.
  6. Stock, Ariel D; Wen, Jing; Putterman, Chaim (December 2013). "Neuropsychiatric lupus, the blood brain barrier, and the TWEAK/Fn14 pathway". Frontiers in Immunology. 4: 484. doi:10.3389/fimmu.2013.00484. ISSN 1664-3224. PMC 3872310. PMID 24400009.
  7. Bertsias, GK; Ioannidis, JPA; Aringer, M; Bollen, E; Bombardieri, S; et al. (2010). "EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs". Annals of the Rheumatic Diseases. 69 (12): 2074–2082. doi:10.1136/ard.2010.130476. ISSN 0003-4967. PMID 20724309.
  8. Gulinello, Maria; Wen, Jing; Putterman, Chaim (September 2012). "Neuropsychiatric Symptoms in Lupus". Psychiatric Annals. 42 (9): 322–328. doi:10.3928/00485713-20120906-05. PMC 4302271. PMID 25620816.
  9. Magro-Checa, Cesar; Zirkzee, Elisabeth J; Huizinga, Tom W; Steup-Beekman, Gerda M (2016). "Management of Neuropsychiatric Systemic Lupus Erythematosus: Current Approaches and Future Perspectives". Drugs. 76 (4): 459–483. doi:10.1007/s40265-015-0534-3. ISSN 0012-6667. PMC 4791452. PMID 26809245.
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