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Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: A multicenter study - 21/06/23

Doi : 10.1016/j.jbspin.2023.105559 
Ayşenur Paç Kısaarslan a, , Sümeyra Özdemir Çiçek b, Ezgi D. Batu c, Sezgin Şahin d, Metin K. Gürgöze e, Sibel Balcı Çetinkaya b, Miray Kışla Ekinci f, Bahriye Atmış g, Kenan Barut d, Amra Adrovic d, Buket Esen Ağar e, Nihal Şahin h, Ferhat Demir i, Esra Bağlan j, Mehtap Akbalık Kara k, Şenay Zırhlı Selçuk l, Semanur Özdel j, Elif Çomak m, Betül Akkoyunlu n, Gülçin Otar Yener o, Deniz Gezgin Yıldırım p, Kübra Öztürk q, Mehmet Yıldız d, Fatih Haşlak d, Seher Şener c, Hakan Kısaoğlu r, Özge Baba r, Zehra Kızıldağ s, Rana İşgüder s, Şengül Çağlayan t, Raziye B. Güven Bilgin u, Gülçin Aytaç u, Burcu Bozkaya Yücel v, Ayşe Tanatar w, Hafize E. Sönmez x, Mustafa Çakan y, Aslıhan Kara e, Ahmet T. Elmas l, Beltinge Demircioğlu Kılıç k, Nuray Aktay Ayaz w, Belde Kasap z, Banu Çelikel Acar aa, Ozan Ozkaya ab, Selçuk Yüksel ac, Sevcan Bakkaloğlu p, Özlem Aydoğ v, Güzide Aksu u, Sema Akman m, Osman Dönmez n, Mehmet Bülbül j, Mithat Büyükçelik k, Yılmaz Tabel l, Betül Sözeri t, Mukaddes Kalyoncu r, Yelda Bilginer c, Muammer H. Poyrazoğlu a, Erbil Ünsal s, Özgür Kasapçopur d, Seza Özen c, Ruhan Düşünsel ad
a Pediatric Rheumatology, Faculty of Medicine, Erciyes University, Kayseri, Turkey 
b Pediatric Rheumatology, Kayseri City Hospital, Kayseri, Turkey 
c Pediatric Rheumatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey 
d Pediatric Rheumatology, Cerrahpasa Faculty of Medicine, İstanbul University, Istanbul, Turkey 
e Pediatric Nephrology and Rheumatology, Faculty of Medicine, Fırat University, Elazıg, Turkey 
f Pediatric Rheumatology, Adana City Hospital, Adana, Turkey 
g Pediatric Nephrology, Faculty of Medicine, Çukurova University, Adana, Turkey 
h Pediatric Rheumatology, Derince Educational and Research Hospital, Kocaeli, Turkey 
i Pediatric Rheumatology, Acıbadem Hospital, Istanbul, Turkey 
j Pediatric Rheumatology, Doctor Sami-Ulus Maternity and Child Health and Diseases Research and Training Hospital, Ankara, Turkey 
k Pediatric Nephrology, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey 
l Pediatric Nephrology, Faculty of Medicine, İnönü University, Malatya, Turkey 
m Pediatric Nephrology and Rheumatology, Faculty of Medicine, Akdeniz University, Antalya, Turkey 
n Pediatric Nephrology and Rheumatology, Faculty of Medicine, Uludag University, Bursa, Turkey 
o Pediatric Rheumatology, Şanlıurfa Research and Training Hospital, Şanlıurfa, Turkey 
p Pediatric Rheumatology, Faculty of Medicine, Gazi University, Ankara, Turkey 
q Pediatric Rheumatology, Istanbul Medeniyet University, Göztepe Professor Doctor Süleyman-Yalçın City Hospital, İstanbul, Turkey 
r Pediatric Rheumatology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey 
s Pediatric Rheumatology, Faculty of Medicine, Dokuz Eylül University, Izmir, Turkey 
t Pediatric Rheumatology, Ümraniye Research and Training Hospital, İstanbul, Turkey 
u Pediatric Rheumatology, Faculty of Medicine, Ege University, İzmir, Turkey 
v Pediatric Rheumatology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey 
w Pediatric Rheumatology, Faculty of Medicine, Istanbul University, İstanbul, Turkey 
x Pediatric Rheumatology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey 
y Pediatric Rheumatology, University of Health Sciences, Zeynep Kamil Women and Children's Diseases Training and Research Hospital, İstanbul, Turkey 
z Pediatric Nephrology, Katip-Çelebi University, İzmir, Turkey 
aa Pediatric Nephrology and Rheumatology, Ankara City Hospital, Ankara, Turkey 
ab Pediatric Nephrology and Rheumatology, Faculty of Medicine, İstinye University, İstanbul, Turkey 
ac Pediatric Nephrology and Rheumatology, Faculty of Medicine, Pamukkale University, Denizli, Turkey 
ad Pediatric Nephrology and Rheumatology, Faculty of Medicine, Yeditepe University, Istanbul, Turkey 

Corresponding author.

Highlights

The prevalence of NPSLE is 13.5% in pediatric SLE patients in Turkey.
Clinical and laboratory features of SLE and NP involvement are grouped in five clusters.
APA positivity, high SLEDAI scores and plasmapheresis are risk factors for neurological sequelae.

Il testo completo di questo articolo è disponibile in PDF.

Abstract

Introduction

Neuropsychiatric (NP) involvement is a restricted area in juvenile-onset systemic lupus erythematosus (jSLE).

Aim

To investigate the prevalence, demographic and clinical features, and outcomes of the neurological involvement in the Turkish jSLE population.

Methods

This study was based upon 24 referral centers’ SLE cohorts, multicenter and multidisciplinary network in Turkey. Patient data were collected by a case report form which was standardized for NP definitions according to American Collage of Rheumatology (ACR). Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) neuropsychiatric part was used to determine NP damage. Variables were evaluated Ward's hierarchical clustering analyses, univariate, and multivariate logistic regression analyses.

Results

A hundred forty-nine of 1107 jSLE patients had NP involvement (13.5%). The most common NPSLE findings were headache (50.3%), seizure (38.3%), and acute confusional state (33.6%). Five clusters were identified with all clinical and laboratory findings. The first two clusters involved neuropathies, demyelinating diseases, aseptic meningitis, and movement disorder. Cluster 3 involved headache, activity markers and other SLE involvements. Idiopathic intracranial hypertension, cerebrovascular disease, cognitive dysfunction, psychiatric disorders and SLE antibodies were in the fourth, and acute confusional state was in the fifth cluster. In multivariate analysis, APA positivity; OR: 2.820, (%95CI: 1.002–7.939), P: 0,050, plasmapheresis; OR: 13.804 (%95CI: 2.785–68.432), P: 0,001, SLEDAI scores; OR: 1.115 (%95CI: (1.049–1.186), P: 0,001 were associated with increased risk for neurologic sequelae.

Conclusion

We detected the prevalence of juvenile NPSLE manifestations in Turkey. We have identified five clusters that may shed light pathogenesis, treatment and prognosis of NP involvements. We also determined risk factors of neurological sequelae. Our study showed that new definitions NP involvements and sequelae for childhood period are needed.

Il testo completo di questo articolo è disponibile in PDF.

Keywords : Cluster, Juvenile, Systemic lupus erythematosus, Neurologic manifestations


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