George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69–86.

Article  PubMed  Google Scholar 

Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med. 2012;63:233–46.

Article  CAS  PubMed  Google Scholar 

Faitelson Y, Grunebaum E. Hemophagocytic lymphohistiocytosis and primary immune deficiency disorders. Clin Immunol. 2014;155(1):118–25.

Article  CAS  PubMed  Google Scholar 

Lin CI, Yu HH, Lee JH, Wang LC, Lin YT, Yang YH, et al. Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. Clin Rheumatol. 2012;31(8):1223–30.

Article  PubMed  Google Scholar 

Minoia F, Davi S, Horne A, Demirkaya E, Bovis F, Li C, et al. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014;66(11):3160–9.

Article  PubMed  Google Scholar 

Aytac S, Batu ED, Unal S, Bilginer Y, Cetin M, Tuncer M, et al. Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis and systemic lupus erythematosus. Rheumatol Int. 2016;36(10):1421–9.

Article  CAS  PubMed  Google Scholar 

Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood. 2020;135(16):1332–43.

Article  PubMed  Google Scholar 

Xu XJ, Wang HS, Ju XL, Xiao PF, Xiao Y, Xue HM, Shi HY, Gao YJ, Jia GC, Li XR, Zhao WH, Wang NL, Tang YM; Histiocytosis Study Group of the Chinese Pediatric Society. Clinical presentation and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in China: A retrospective multicenter study. Pediatr Blood Cancer. 2017;64(4). https://doi.org/10.1002/pbc.26264.

Harnchoowong S, Soponkanaporn S, Vilaiyuk S, Lerkvaleekul B, Pakakasama S. Central nervous system involvement and thrombocytopenia as predictors of mortality in children with hemophagocytic lymphohistiocytosis. Front Pediatr. 2022;10: 941318.

Article  PubMed  Google Scholar 

Cheng H, Chen D, Peng X, Wu P, Jiang L, Hu Y. Clinical characteristics of Epstein-Barr virus infection in the pediatric nervous system. BMC Infect Dis. 2020;20(1):886.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Martelius T, Lappalainen M, Palomaki M, Anttila VJ. Clinical characteristics of patients with Epstein Barr virus in cerebrospinal fluid. BMC Infect Dis. 2011;11: 281.

Article  PubMed  PubMed Central  Google Scholar 

Oguz MM, Sahin G, Altinel Acoglu E, Polat E, Yucel H, Oztek Celebi FZ, et al. Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single center experience and factors that influenced patient prognosis. Pediatr Hematol Oncol. 2019;36(1):1–16.

Article  PubMed  Google Scholar 

Volpi A. Epstein-Barr virus and human herpesvirus type 8 infections of the central nervous system. Herpes. 2004;11(Suppl 2):120A-A127.

PubMed  Google Scholar 

Weinberg A, Li S, Palmer M, Tyler KL. Quantitative CSF PCR in Epstein-Barr virus infections of the central nervous system. Ann Neurol. 2002;52(5):543–8.

Article  CAS  PubMed  Google Scholar 

Hongbo C, Hongzhen M, Lingzhi H, Maosheng X, Mei C. Secondary neuropsychiatric manifestations caused by Epstein-Barr virus encephalitis in a new onset systemic lupus erythematosus patient. Rheumatol Int. 2012;32(8):2321–3.

Article  PubMed  Google Scholar 

Gladman DD, Ibanez D, Urowitz MB. Systemic lupus erythematosus disease activity index 2000. J Rheumatol. 2002;29(2):288–91.

PubMed  Google Scholar 

Uribe AG, Vila LM, McGwin G Jr, Sanchez ML, Reveille JD, Alarcon GS. The systemic lupus activity measure-revised, the Mexican Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and a modified SLEDAI-2K are adequate instruments to measure disease activity in systemic lupus erythematosus. J Rheumatol. 2004;31(10):1934–40.

PubMed  Google Scholar 

Nikolopoulos D, Kitsos D, Papathanasiou M, Kapsala N, Garantziotis P, Pieta A, et al. Demyelinating syndromes in systemic lupus erythematosus: data from the “Attikon” Lupus Cohort. Front Neurol. 2022;13: 889613.

Article  PubMed  PubMed Central  Google Scholar 

Pastula DM, Burish M, Reis GF, Bollen A, Cha S, Ralph J, et al. Adult-onset central nervous system hemophagocytic lymphohistiocytosis: a case report. BMC Neurol. 2015;15:203.

Article  PubMed  PubMed Central  Google Scholar 

Benson LA, Li H, Henderson LA, Solomon IH, Soldatos A, Murphy J, et al. Pediatric CNS-isolated hemophagocytic lymphohistiocytosis. Neurol Neuroimmunol Neuroinflamm. 2019;6(3): e560.

Article  PubMed  PubMed Central  Google Scholar 

Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124–31.

Article  PubMed  Google Scholar 

Parodi A, Davi S, Pringe AB, Pistorio A, Ruperto N, Magni-Manzoni S, et al. Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients. Arthritis Rheum. 2009;60(11):3388–99.

Article  CAS  PubMed  Google Scholar 

Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613–20.

Article  PubMed  Google Scholar 

Imashuku S, Morimoto A, Ishii E. Virus-triggered secondary hemophagocytic lymphohistiocytosis. Acta Paediatr. 2021;110(10):2729–36.

Article  CAS  PubMed  Google Scholar 

El-Mallawany NK, Curry CV, Allen CE. Haemophagocytic lymphohistiocytosis and Epstein-Barr virus: a complex relationship with diverse origins, expression and outcomes. Br J Haematol. 2022;196(1):31–44.

Article  PubMed  Google Scholar 

Yu HH, Lee JH, Wang LC, Yang YH, Chiang BL. Neuropsychiatric manifestations in pediatric systemic lupus erythematosus: a 20-year study. Lupus. 2006;15(10):651–7.

Article  CAS  PubMed  Google Scholar 

Schwartz N, Stock AD, Putterman C. Neuropsychiatric lupus: new mechanistic insights and future treatment directions. Nat Rev Rheumatol. 2019;15(3):137–52.

Article  PubMed  PubMed Central  Google Scholar 

Kisaarslan AP, Cicek SO, Batu ED, Sahin S, Gurgoze MK, Cetinkaya SB, et al. Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: a multicenter study. Joint Bone Spine. 2023;90(4): 105559.

Article  PubMed  Google Scholar 

Jiang M, Shi X, Gao X, Niu J, Hu X, Zhao L, et al. Clinical features of central nervous system infections and experience in differential diagnosis from neuropsychiatric lupus erythematosus in a cohort of 8491 patients with systemic lupus erythematosus. Arthritis Res Ther. 2019;21(1):189.

Article  PubMed  PubMed Central  Google Scholar 

Fang H, Lan L, Qu Y, Zhang Q, Lv J. Differences between central nervous system infection and neuropsychiatric systemic lupus erythematosus in patients with systemic lupus erythematosus. J Int Med Res. 2018;46(1):485–91.

Article  PubMed  Google Scholar 

Borgia RE, Gerstein M, Levy DM, Silverman ED, Hiraki LT. Features, treatment, and outcomes of macrophage activation syndrome in childhood-onset systemic lupus erythematosus. Arthritis Rheumatol. 2018;70(4):616–24.

Article  CAS  PubMed  Google Scholar 

Giani T, Smith EM, Al-Abadi E, Armon K, Bailey K, Ciurtin C, et al. Neuropsychiatric involvement in juvenile-onset systemic lupus erythematosus: Data from the UK Juvenile-onset systemic lupus erythematosus cohort study. Lupus. 2021;30(12):1955–65.

Article  CAS  PubMed  Google Scholar 

Doja A, Bitnun A, Ford Jones EL, Richardson S, Tellier R, Petric M, et al. Pediatric Epstein-Barr virus-associated encephalitis: 10-year review. J Child Neurol. 2006;21(5):384–91.

Article  PubMed  Google Scholar 

Labouret M, Costi S, Bondet V, Trebossen V, Le Roux E, Ntorkou A, et al. Juvenile Neuropsychiatric Systemic Lupus Erythematosus: Identification of Novel Central Neuroinflammation Biomarkers. J Clin Immunol. 2023;43(3):615–24.

Article