The management of neuropsychiatric lupus in the 21st century: still so many unmet needs?

Abstract

Neuropsychiatric (NP) events occur in the majority of patients with SLE and predominantly affect the CNS in addition to the peripheral and autonomic systems. Approximately 30% of all NP events are attributable to SLE (NPSLE) and present most frequently around the time of SLE onset. NPSLE is associated with increased morbidity and mortality and the proposed pathogenesis includes both ischaemic and neuroinflammatory mechanisms. Following diagnosis and causal attribution, the treatment of NPSLE is tailored to the type of NP event, the predominant putative pathogenic pathway and the activity and severity of the clinical event. There is a dearth of controlled clinical trials to guide management, but therapeutic options include symptomatic, antithrombotic and immunosuppressive agents that are supported by observational cohort studies. Our objective was to review what is currently known about NPSLE and to identify deficiencies in diagnostic biomarkers, novel therapies and clinical trials for this manifestation of SLE.

Neuropsychiatric Systemic Lupus Erythematosus: A 2021 Update on Diagnosis, Management, and Current Challenges

“…Other autoantibodies and their association with NPSLE are under analysis (Table 2 ) [ 26 - 29 ]. There is a dire need to develop and confirm an array of specific and sensitive laboratory serum or CSF biomarkers, radiological imaging changes for reliable apprehension of all different aspects of NPSLE heterogeneousness and further improve prevention, diagnosis, and management [ 30 ].…”

Coexistence of Systemic Lupus Erythematosus and Syringomyelia: A Case From a Rural Center in Nigeria

“…The most prevalent CNS manifestation is intractable headache, which accounts for more than half of all cases [ 7 ]. Others are cerebrovascular accidents, seizures, depression, and psychosis, aseptic meningitis, demyelinating syndromes including transverse myelitis, Guillain-Barre syndrome, and optic neuritis, myasthenia gravis, movement disorders, and neuropathies [ 8 ]. The exact pathogenic etiology of neuropsychiatric SLE is not clear but could be multifactorial involving genetic, immunological, and environmental factors [ 8 , 9 ].…”

“…Others are cerebrovascular accidents, seizures, depression, and psychosis, aseptic meningitis, demyelinating syndromes including transverse myelitis, Guillain-Barre syndrome, and optic neuritis, myasthenia gravis, movement disorders, and neuropathies [ 8 ]. The exact pathogenic etiology of neuropsychiatric SLE is not clear but could be multifactorial involving genetic, immunological, and environmental factors [ 8 , 9 ]. Several inflammatory cytokines and pathogenic autoantibodies have been implicated.…”

“…Several inflammatory cytokines and pathogenic autoantibodies have been implicated. Pathologic CNS findings include thrombotic multifocal infarcts of the small intracranial vessels, endothelial proliferation, and perivascular inflammation [ 8 , 9 ]. There have also been reports of SLE coexisting with multiple sclerosis, though rarely [ 10 ].…”

A case of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) with a high antinuclear antibody titer

“…White matter involvement, adult-onset age, and various progressive symptoms make it difficult to differentiate ALSP from NP-SLE when ANA is positive, and no other systems are involved. NP-SLE has been associated with a vast number of heterogeneous syndromes, and to date, there are no laboratory or neuroimaging biomarkers shown to be sufficiently accurate or reliable for use in the clinic [ 2 ].…”