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Abstract Number: 1436

Application of European League Against Rheumatism Recommendations for the Management of Systemic Lupus Erythematosus Patients with Neuropsychiatric Involvement May Limit Unnecessary Diagnostic Testing and Curve Intensification of Immunosuppressive Therapy of Unclear Benefit

Cristina Pamfil1, Antonios Fanouriakis2, Argyro Repa3, Maria Melissourgaki2, Prodromos Sidiropoulos3, Ileana Filipescu1, Mirela Rinzis4, Simona Rednic4, George Bertsias3 and Dimitrios Boumpas5, 1Rheumatology, University of Medicine and Pharmacy, Cluj-Napoca, Romania, 2Rheumatology, Clinical Immunology, and Allergy, University of Crete, Iraklion, Greece, 3Rheumatology, Clinical Immunology, and Allergy, University of Crete, Heraklion, Greece, 4Rheumatology, Emergency County Clinical Hospital Cluj Napoca, Cluj-Napoca, Romania, 5Panepistimio Kritis, Rethymnon, Greece

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Neurologic involvement, neuropsychiatric disorders and systemic lupus erythematosus (SLE)

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Session Information

Title: Systemic Lupus Erythematosus: Clinical Aspects

Session Type: Abstract Submissions (ACR)

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


Disclosure:

C. Pamfil,
None;

A. Fanouriakis,
None;

A. Repa,
None;

M. Melissourgaki,
None;

P. Sidiropoulos,
None;

I. Filipescu,
None;

M. Rinzis,
None;

S. Rednic,
None;

G. Bertsias,
None;

D. Boumpas,
None.

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