Abstract

Background:Distinguishing primary NPSLE (neuropsychiatric systemic lupus erythematosus) from secondary causes remains challenging (1). Attribution models were developed in order to aim clinicians in correct classification of NPSLE cases (2).Objectives:To investigate the prevalence of primary NPSLE manifestations assigned with Italian model of attribution (2).Methods:We retrospectively assessed clinical details of 164 patients with SLE classified with 2012 SLICC (Systemic Lupus International Collaborating Clinics) classification criteria, 21 were excluded due to incomplete information. Data was gathered with a questionnaire comprising demographics, medical history, laboratory results (concentrations of antibodies against double stranded DNA – anti-dsDNA, complement components C3 and C4), disease activity measured with Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and Physician Global Assessment (PGA) and damage determined with SLICC/ACR (American College of Rheumatology) Damage Index (SDI). Neuropsychiatric manifestations were categorized in accordance with 1999 ACR glossary and attribution of manifestations was performed with the use of Italian model with the score ≥7 out of 10 points enabling assignment to primary NPSLE group (2). Statistical analysis was conducted with Statistica v.13.3 using Mann-Whitney U, chi-square and Fisher exact test.Results:We encountered 155 NP manifestations in our cohort and 52 (34%) were attributed to SLE. Characteristics of the study groups are presented in Table 1. Exact manifestations and their attribution rates are presented on Graph 1. Patients with attributable NPSLE were younger, had earlier disease onset, presented higher disease activity, lower damage accrual without taking NP damage into account and more often had increased anti-dsDNA serum concentration.Table 1.Demographic and laboratory characteristics with disease activity and damage of the study groups, N(%) or mean(±SD).CharacteristicPatients with attributed NPSLE manifestationsPatients without attributed NPSLE manifestationsPatients34 (23.8%)109 (76.2%)Sex, female30 (88.2%)102 (93.6%)Age (years)37.6 (±11.7)44.3 (±13.9)*Age of disease onset (years)32.5 (±11.4)37.6 (±12.6)*Disease duration (years)5.1 (±4.1)6.8 (±5.6)SLEDAI-2K29.2 (±10.7)12.2 (±8.1)*patients with clinically active disease (defined as SLEDAI-2K≥6 in clinical manifestations)34 (100%)93 (85.3%)*SLEDAI-2K without NP manifestations14.8 (±8.4)11.0 (±6.7)*PGA2.1 (±1.0)1.2 (±1.0)*SDI0.5 (±0.8)0.7 (±1.1)SDI without NP damage0.3 (±0.6)0.7 (±1.1)*low C3/C4 complement component concentration in serum21 (61.8%)55 (50.4%)elevated anti-dsDNA autoantibody concentration in serum27 (79.4%)55 (50.4%)*NPSLE – neuropsychiatric systemic lupus erythematosus, SLEDAI-2K – Systemic Lupus Erythematosus Disease Activity Index version 2000, PGA – physician global assessment, SDI – SLICC/ACR (Systemic Lupus International Collaborating Clinics/American College of Rheumatology) Damage Index*p<0,05, Mann-Whitney U, χ2or Fisher’s exact test, as appropriateConclusion:Primary NP manifestations in patients with SLE occur mainly in young patients with high disease activity. Cerebrovascular disease, seizures, psychosis and cranial neuropathy are most frequent primary NPSLE manifestations.

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