Background/Purpose: Systemic lupus erythematosus (SLE) patients may experience a wide variety of neurological and psychiatric manifestations (neuropsychiatric [NP] SLE [NPSLE]), which pose diagnostic and therapeutic challenges to clinicians leading to significant heterogeneity in their management. We have recently published evidence and expert based recommendations on NPSLE. We sought to measure them against usual care by auditing the management in our centers prior to issuing these recommendations.

Methods: NPSLE events were reviewed in two lupus centers in Iraklio, Greece and Cluj, Romania. Non-SLE-related events were excluded. We compared the diagnostic and treatment decisions in our cohort with the recommendations issued by the European League Against Rheumatism (EULAR) for specific NPSLE manifestations.

Results:

A total of 105 NP events attributed to SLE were recorded in 89 patients (89% female, mean age 41.1 years, mean time from SLE onset to NPSLE 5.2 years) by cxahrt review over the last decade (2001-11). Most common events included cerebrovascular disease (n=19, 18%), cognitive dysfunction (n=17, 16%), intractable headache (n=10, 9.5%), psychosis (n=10, 9.5%), and transverse myelitis (n=10, 9.5%). Overall, the concordance between clinical decisions and the recommendations was 74% for diagnostic work-up and 67% for treatment of NPSLE (Table 1). Regarding diagnosis, lower concordance rates were noted in cases of cognitive dysfunction with only 5/17 (29%) SLE patients undergoing the recommended neuropsychological testing, and also in mood disorder where 4/7 (57%) patients had brain neuroimaging despite limited evidence for its usefulness. In contrast, the diagnostic work-up of major NPSLE such as seizure disorders, cranial neuropathy, and peripheral neuropathy, was generally in accordance with the recommendations. In terms of treatment, 40% of patients with seizure disorder and 40% of patients with cerebrovascular disease underwent intensification of immunosuppressive therapy without clear evidence of generalized SLE activity, as the recommendations suggest.  Antiplatelet or anticoagulant treatment was initiated in 34/39 (87%) of patients with NPSLE and positive antiphospholipid antibodies in accordance with EULAR recommendations. In contrast, the management of cognitive dysfunction did not include the recommended psycho-educational support in the majority of cases.

Conclusion:

The diagnostic and therapeutic decisions in NPSLE patients managed in two European centers were often not in concordance with the existing EULAR recommendations. Applications of these recommendations may decrease unnecessary testing and curve intensifications of immunosuppressive therapy in cases no clear benefit has been documented. Longitudinal studies to further validate  the impact of these recommendations in improving outcomes are needed.

Table 1   Concordance of clinical practice with recommendations   N Diagnostic work-up Treatment Cardiovascular disease 19 (18%) 14/19 (74%)a 9/19 (47.3%)b, 6/11 (55.5%)d Cognitive disorder 17 (16%) 5/17 (29.4%)f 17/17 (100%)g, 1/17 (6%)h Psychosis 10 (9.5%) 7/10 (70%)a 8/8 (100%)b Seizure disorder 9 (8.5%) 8/9 (88.9%)c 3/5 (60%)b, 0/2 (0%)d Mood disorder 7 (6.6%) 4/7 (57%)a                      5/7 (71%)b     Transverse myelitis 10 (9.5%) 7/10 (70%), 8/10 (80%)e 6/10 (70%), 7/10 (80%)b Cranial neuropathy 8 (7.5%) 2/2 (100%)i 2/2 (100%)b Peripheral neuropathy 6 (5.7%) 6/6 (100%)j 5/6 (83%)b Abbreviations: a, brain imaging; b,  glucocorticoids and immunosuppressants; c, MRI and EEG; d, antiplatelet/anticoagulation; e, MRI and CSF; f, neuropsychological tests; g, disease and risk management; h, psycho-educational support. i:ophtalmological evaluation, j: electromyography and nerve conduction studies

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/application-of-european-league-against-rheumatism-recommendations-for-the-management-of-systemic-lupus-erythematosus-patients-with-neuropsychiatric-involvement-may-limit-unnecessary-diagnostic-testing/