Relevant domains and outcome measurement instruments in neuropsychiatric systemic lupus erythematosus: a systematic literature review

Abstract

Objectives Although neuropsychiatric involvement in Systemic Lupus Erythematosus (NPSLE) is one of the most complex and troubling manifestations of the disease, validated outcome instruments to be used as sensitive endpoints in controlled clinical trials are lacking. We set out a systematic literature review (SLR) to identify outcome measurement instruments and domains used to assess NPSLE. Methods The Preferred Reporting Items for systematic reviews and Meta-analysis (PRISMA) guidelines were used. Articles available in English (1967-2020), listed in PubMed, EMBASE, PsycINFO, Cochrane Library and EULAR outcome measures library were screened. All domains and outcome measurement instruments were characterized according to the OMERACT Filter 2.1, considering core areas (manifestations/abnormalities, life impact, death/lifespan, societal/resource use) and contextual factors. Results Of 3,392 abstracts evaluated, 83 studies were included in the SLR (15,974 patients, females 89.9%). Eligible studies included domains and instruments pertinent to all core areas defined by OMERACT, except for “societal/resource use”. The most common core areas were “manifestations/abnormalities”, covering 10 domains pertinent to laboratory and instrumental markers, indexes and neuropsychiatric dimension (cognitive, neurologic and psychiatric field), and “life impact”, covering 7 domains related to physical function (from both the perspective of the patient and the physician), pain and quality of life. Conclusion Our study revealed great heterogeneity in the instruments derived from populations with NPSLE and none of these had high-quality evidence. This supports the need to develop and further validate a core domain set and outcome measurement instruments to promote clinical research in this field, enhancing comparability across studies.

Prevalence and identification of neuropsychiatric symptoms in systemic autoimmune rheumatic diseases: an international mixed methods study

“…We suggest that there has been a self-perpetuating cycle, in which NP symptoms are under-elicited in clinic, under-identified in research and excluded from trial outcomes [ 33 ], and have limited [ 14 , 34 ] or non-inclusion in SARD criteria/guidelines [ 35 ]. Our data and clinician participants strongly endorse the calls to update the ACR NPSLE criteria [ 36 , 37 ], which remains widely used despite being reported as having low reliability and ‘quite unsatisfactory’ performance in clinical practice [ 6 ].…”

Cerebrospinal Fluid Analysis in Rheumatological Diseases with Neuropsychiatric Complications and Manifestations: A Narrative Review

“…The positive effect of hydroxychloroquine has been demonstrated in several studies, while specific symptomatic therapy is indicated according to the type of manifestation (e.g., antipsychotics, anti-depressants, anti-convulsants) [22]. The lack of properly validated outcome measures to be assessed in RCTs remains one of the main limitations to testing newer drugs in this challenging disease [37].…”

Neuropsychiatric Systemic Lupus Erythematosus: A Systematic Review

“…It remains difficult to discern whether some of these patients' neuropsychiatric manifestations such as headache, anxiety, and depression are a consequence of their SLE or are independent, and how this differentiation affects management and outcomes [21]. Even in patients whose symptoms have been formally attributed to SLE, many treatments for these conditions are still trial and error at this stage, and further investigations and large scale clinical trials are necessary to optimize outcomes for these patients [22]. This review highlights the diverse clinical presentation and severity of symptoms in patients with NPSLE and calls for more research on the topic to optimize patient diagnostics and outcomes.…”