{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T13:13:30Z","timestamp":1770815610376,"version":"3.50.1"},"reference-count":77,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T00:00:00Z","timestamp":1770768000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background: Childhood-onset systemic lupus erythematosus (cSLE) is a rare, serious autoimmune disease characterized by multiorgan involvement and long-term morbidity. Although several studies have examined this condition in Japan, a comprehensive summary of recent findings remains lacking. Methods: PubMed was searched for Japanese publications on cSLE published between 2015 and 2025, including clinical studies, case reports, translational research, basic science studies, systematic reviews, clinical practice guidance, and transition care guidance. Results: Sixty publications met the inclusion criteria: 20 clinical studies, 30 case reports, 6 translational studies, 1 basic science study, 1 systematic review, 1 clinical practice guidance, and 1 transition care guidance. Most clinical studies were retrospective, although multicenter and registry-based designs have increased in recent years. Lupus nephritis remained the primary research focus, with accumulating evidence supporting mycophenolate mofetil, tacrolimus, and early belimumab as glucocorticoid (GC)-sparing approaches. Case reports illustrated the broad clinical spectrum of cSLE, with hematological and vascular complications being the most frequently reported. Translational studies highlighted the pathogenic role of type I interferon signaling and cytokine dysregulation, particularly in macrophage activation syndrome. Despite these advances, prospective studies and standardized assessment methods for pediatric-specific practice remain limited. Conclusions: Over the past decade, cSLE research in Japan has contributed to a deeper understanding of its clinical and immunological characteristics. However, treatment-related complications and long-term organ damage remain important challenges. Continued multicenter collaboration and domestic data accumulation may strengthen the evidence base, facilitate optimization of GC-sparing approaches, improve clinical management, and provide background information to support future discussions on clinical practice guidelines.<\/jats:p>","DOI":"10.3390\/children13020250","type":"journal-article","created":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T09:15:12Z","timestamp":1770801312000},"page":"250","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Childhood-Onset Systemic Lupus Erythematosus Research over the Past Decade in Japan"],"prefix":"10.3390","volume":"13","author":[{"given":"Tasuku","family":"Tamai","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Oita University Faculty of Medicine, 1-1 Idaigaoka Hasama-machi, Yufu 879-5593, Oita, Japan"},{"name":"Division of General Pediatrics and Emergency Medicine, Department of Pediatrics, Oita University Faculty of Medicine, 1-1 Idaigaoka Hasama-machi, Yufu 879-5593, Oita, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1534-2583","authenticated-orcid":false,"given":"Hiroyuki","family":"Wakiguchi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Oita University Faculty of Medicine, 1-1 Idaigaoka Hasama-machi, Yufu 879-5593, Oita, Japan"},{"name":"Division of General Pediatrics and Emergency Medicine, Department of Pediatrics, Oita University Faculty of Medicine, 1-1 Idaigaoka Hasama-machi, Yufu 879-5593, Oita, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8831-1991","authenticated-orcid":false,"given":"Kenji","family":"Ihara","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Oita University Faculty of Medicine, 1-1 Idaigaoka Hasama-machi, Yufu 879-5593, Oita, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,2,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2326","DOI":"10.1016\/S0140-6736(24)00398-2","article-title":"Systemic lupus erythematosus","volume":"403","author":"Hoi","year":"2024","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1093\/mr\/roab002","article-title":"Clinical practice guidance for childhood-onset systemic lupus erythematosus-secondary publication","volume":"32","author":"Takei","year":"2022","journal-title":"Mod. Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1038\/s41584-021-00668-1","article-title":"Global epidemiology of systemic lupus erythematosus","volume":"17","author":"Barber","year":"2021","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1007\/s11882-024-01169-3","article-title":"Childhood-onset systemic lupus erythematosus (cSLE): An international perspective","volume":"24","author":"Aggarwal","year":"2024","journal-title":"Curr. Allergy Asthma Rep."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1093\/mr\/roaf020","article-title":"Prevalence of systemic lupus erythematosus and age-stratified treatment trends in the Japanese population: A cross-sectional study based on the National Database of Health Insurance Claims","volume":"35","author":"Matsushita","year":"2025","journal-title":"Mod. Rheumatol."},{"key":"ref_6","unstructured":"Study Group for Childhood-Onset Systemic Lupus Erythematosus in the Japanese Ministry of Health (2018). Clinical Practice Guidance for Childhood-Onset Systemic Lupus Erythematosus 2018, Yodosha. (In Japanese)."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1725","DOI":"10.1002\/art.1780400928","article-title":"Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus","volume":"40","author":"Hochberg","year":"1997","journal-title":"Arthritis Rheum."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1097\/01.ASN.0000108969.21691.5D","article-title":"The classification of glomerulonephritis in systemic lupus erythematosus revisited","volume":"15","author":"Weening","year":"2004","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1016\/j.kint.2017.11.023","article-title":"Revision of the International Society of Nephrology\/Renal Pathology Society classification for lupus nephritis: Clarification of definitions, and modified National Institutes of Health activity and chronicity indices","volume":"93","author":"Bajema","year":"2018","journal-title":"Kidney Int."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1093\/mr\/roaf075","article-title":"Telemedicine for paediatric rheumatic diseases in Japan: A national survey of physicians\u2019 perspectives","volume":"36","author":"Inoue","year":"2025","journal-title":"Mod. Rheumatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"106970","DOI":"10.1016\/j.ejps.2024.106970","article-title":"Population pharmacokinetic analysis in children with different diseases treated with mycophenolate mofetil-Integrated analysis of clinical trials and real-world clinical data","volume":"204","author":"Saito","year":"2025","journal-title":"Eur. J. Pharm. Sci."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1111\/nep.14406","article-title":"Long-term outcome of tacrolimus-based immunosuppressive treatment for patients with paediatric-onset lupus nephritis","volume":"29","author":"Tanaka","year":"2024","journal-title":"Nephrology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1177\/09612033241238867","article-title":"Early add-on use of Belimumab as induction therapy for patients with childhood-onset systemic lupus erythematosus","volume":"33","author":"Hashimoto","year":"2024","journal-title":"Lupus"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2257","DOI":"10.1007\/s40620-023-01687-9","article-title":"Renal pathology in adult and paediatric population of Japan: Review of the Japan renal biopsy registry database from 2007 to 2017","volume":"36","author":"Goto","year":"2023","journal-title":"J. Nephrol."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Wakiguchi, H., Kaneko, U., Sato, S., Imagawa, T., Narazaki, H., and Miyamae, T. (2023). Clinical features of COVID-19 in pediatric rheumatic diseases: 2020\u20132022 survey of the pediatric rheumatology association of Japan. Viruses, 15.","DOI":"10.3390\/v15051205"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1007\/s10157-023-02349-7","article-title":"Analysis of the ratio of urinary beta-2-microglobulin to total protein concentration in children with isolated tubulointerstitial disease","volume":"27","author":"Nishimura","year":"2023","journal-title":"Clin. Exp. Nephrol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1093\/mr\/roac112","article-title":"Epidemiology conduction of paediatric rheumatic diseases based on the registry database of the Pediatric Rheumatology Association of Japan","volume":"33","author":"Narazaki","year":"2023","journal-title":"Mod. Rheumatol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2483","DOI":"10.1007\/s10067-022-06138-7","article-title":"Evaluation of the European League Against Rheumatism\/American College of Rheumatology-2019 classification criteria in patients with childhood-onset systemic lupus erythematosus: A single-center retrospective study","volume":"41","author":"Ohara","year":"2022","journal-title":"Clin. Rheumatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2131","DOI":"10.1007\/s00467-021-05350-y","article-title":"Glucocorticoid discontinuation in pediatric-onset systemic lupus erythematosus: A single-center experience","volume":"37","author":"Nishi","year":"2022","journal-title":"Pediatr. Nephrol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1093\/mr\/roab038","article-title":"Trends in actual medication use for child-onset systemic lupus erythematosus using the Japanese health insurance database 2009\u201318","volume":"32","author":"Kishi","year":"2022","journal-title":"Mod. Rheumatol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"rkz013","DOI":"10.1093\/rap\/rkz013","article-title":"Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus","volume":"3","author":"Sato","year":"2019","journal-title":"Rheumatol. Adv. Pract."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1080\/14397595.2018.1532861","article-title":"Clinicopathological characteristics and renal outcomes of childhood-onset lupus nephritis with acute kidney injury: A multicenter study","volume":"29","author":"Ishimori","year":"2019","journal-title":"Mod. Rheumatol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2333794X17736700","DOI":"10.1177\/2333794X17736700","article-title":"Risk factors for thyroid cancer in systemic lupus erythematosus","volume":"4","author":"Kawano","year":"2017","journal-title":"Glob. Pediatr. Health"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1007\/s10067-016-3491-7","article-title":"Treatable renal disease in children with silent lupus nephritis detected by baseline biopsy: Association with serum C3 levels","volume":"36","author":"Wakiguchi","year":"2017","journal-title":"Clin. Rheumatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1007\/s00467-016-3507-1","article-title":"Ten-year treatment with mycophenolate mofetil using therapeutic drug monitoring for childhood-onset lupus nephritis in Japan","volume":"32","author":"Fujinaga","year":"2017","journal-title":"Pediatr. Nephrol."},{"key":"ref_26","first-page":"858","article-title":"A national survey on current use of mycophenolate mofetil for childhood-onset systemic lupus erythematosus in Japan","volume":"25","author":"Hara","year":"2015","journal-title":"Mod. Rheumatol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1111\/ped.12588","article-title":"Incidence and prognosis of systemic lupus erythematosus in a 35 year period in Fukushima, Japan","volume":"57","author":"Kawasaki","year":"2015","journal-title":"Pediatr. Int."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"210","DOI":"10.3109\/14397595.2014.950810","article-title":"Mycophenolate mofetil as maintenance therapy for childhood-onset systemic lupus erythematosus patients with severe lupus nephritis","volume":"25","author":"Kizawa","year":"2015","journal-title":"Mod. Rheumatol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"rxaf006","DOI":"10.1093\/mrcr\/rxaf006","article-title":"12-year-old girl with systemic lupus erythematosus complicated by gangrene and intermittent claudication","volume":"9","author":"Uechi","year":"2025","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1177\/09612033251330094","article-title":"Anifrolumab for refractory cutaneous lupus lesions in pediatric systemic lupus erythematosus","volume":"34","author":"Shmizu","year":"2025","journal-title":"Lupus"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"85","DOI":"10.25259\/IJN_444_2024","article-title":"Effectiveness of Belimumab for glucocorticoid discontinuation in juvenile-onset lupus nephritis","volume":"35","author":"Kise","year":"2025","journal-title":"Indian J. Nephrol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1093\/mrcr\/rxac057","article-title":"Atrophic autoimmune thyroiditis complicated with systemic lupus erythematosus","volume":"7","author":"Wakatsuki","year":"2023","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1093\/mrcr\/rxac055","article-title":"Inappropriate secretion of fibroblast growth factor 23 despite hypophosphataemia with changes in bone turnover markers in a girl with systemic lupus erythematosus: Case report and review of the literature","volume":"7","author":"Kawaguchi","year":"2023","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"e4","DOI":"10.1053\/j.gastro.2022.08.022","article-title":"Neck pain with abdominal distention in a patient with systemic lupus erythematosus","volume":"164","author":"Uda","year":"2023","journal-title":"Gastroenterology"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"3059","DOI":"10.1007\/s00467-022-05555-9","article-title":"A girl with hearing loss, dizziness, hypertension, and pyelonephritis with ureteral edema: Answers","volume":"37","author":"Kaneko","year":"2022","journal-title":"Pediatr. Nephrol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"3057","DOI":"10.1007\/s00467-022-05545-x","article-title":"A girl with hearing loss, dizziness, hypertension, and pyelonephritis with ureteral edema: Questions","volume":"37","author":"Kaneko","year":"2022","journal-title":"Pediatr. Nephrol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1007\/s13730-021-00625-7","article-title":"Elevation of serum fibroblast growth factor 23 level in a pediatric patient with lupus nephritis","volume":"11","author":"Shimazaki","year":"2022","journal-title":"CEN Case Rep."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"e15338","DOI":"10.1111\/ped.15338","article-title":"Overt myositis in a girl with systemic lupus erythematosus","volume":"64","author":"Hoshi","year":"2022","journal-title":"Pediatr. Int."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"e15297","DOI":"10.1111\/ped.15297","article-title":"Thrombotic thrombocytopenic purpura as initial presentation of systemic lupus erythematosus","volume":"64","author":"Endo","year":"2022","journal-title":"Pediatr. Int."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"e14875","DOI":"10.1111\/ped.14875","article-title":"Septic arthritis of the pubic symphysis in a patient with SLE","volume":"64","author":"Shimizu","year":"2022","journal-title":"Pediatr. Int."},{"key":"ref_41","first-page":"36","article-title":"Anti-cyclic citrullinated peptide antibody-positive girl with chronic polyarthritis later diagnosed with juvenile systemic lupus erythematosus: A case report","volume":"47","author":"Kama","year":"2022","journal-title":"Tokai J. Exp. Clin. Med."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"S625","DOI":"10.1097\/RHU.0000000000001698","article-title":"Juvenile neuropsychiatric systemic lupus erythematosus with magnetic resonance imaging findings similar to acute necrotizing encephalopathy","volume":"27","author":"Korenaga","year":"2021","journal-title":"J. Clin. Rheumatol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.1111\/ped.14642","article-title":"Concurrent lupus enteritis and cystitis","volume":"63","author":"Shimizu","year":"2021","journal-title":"Pediatr. Int."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/s12959-021-00271-0","article-title":"Lupus anticoagulant hypoprothrombinemia syndrome associated with bilateral adrenal haemorrhage in a child: Early diagnosis and intervention","volume":"19","author":"Sakamoto","year":"2021","journal-title":"Thromb. J."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1007\/s12185-020-03004-7","article-title":"Bone marrow transplantation from a human leukocyte antigen-mismatched unrelated donor in a case with C1q deficiency associated with refractory systemic lupus erythematosus","volume":"113","author":"Matsumura","year":"2021","journal-title":"Int. J. Hematol."},{"key":"ref_46","first-page":"27","article-title":"Anti-MDA5 antibody-positive juvenile dermatomyositis overlap with neuropsychiatric systemic lupus erythematosus: A case report","volume":"39","author":"Uejima","year":"2021","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1007\/s13730-020-00475-9","article-title":"Refractory secondary thrombotic microangiopathy with kidney injury associated with systemic lupus erythematosus in a pediatric patient","volume":"9","author":"Kaneda","year":"2020","journal-title":"CEN Case Rep."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"e57","DOI":"10.1093\/rheumatology\/keaa051","article-title":"Induction therapy with rituximab for lupus nephritis due to prolidase deficiency","volume":"59","author":"Sato","year":"2020","journal-title":"Rheumatology"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1159\/000500516","article-title":"Severe complications from an unexpectedly high serum mycophenolic acid concentration in a patient with renal failure secondary to lupus nephritis: A case report","volume":"9","author":"Doi","year":"2019","journal-title":"Case Rep. Nephrol. Dial."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1111\/ped.13736","article-title":"Juvenile-onset systemic lupus erythematosus with severe hypertriglyceridemia induced by anti-apolipoprotein C-II antibody","volume":"61","author":"Inoue","year":"2019","journal-title":"Pediatr. Int."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1111\/nep.13175","article-title":"Coagulopathy as a complication of kidney biopsies in paediatric systemic lupus erythematosus patients with antiphospholipid syndrome","volume":"23","author":"Nagata","year":"2018","journal-title":"Nephrology"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1097\/MPH.0000000000000853","article-title":"Necrotizing ulcer after BCG vaccination in a girl with leukocyte-adhesion deficiency Type 1","volume":"40","author":"Kurosawa","year":"2018","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1111\/ped.13327","article-title":"Development of systemic lupus erythematosus 5 years after onset of thrombotic thrombocytopenic purpura","volume":"59","author":"Shingu","year":"2017","journal-title":"Pediatr. Int."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"e13","DOI":"10.1016\/j.semarthrit.2016.05.003","article-title":"A case of walled-off necrosis with systemic lupus erythematosus: Successful treatment with endoscopic necrosectomy","volume":"46","author":"Sato","year":"2016","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1111\/ped.12849","article-title":"Systemic lupus erythematosus presenting with mixed-type fulminant autoimmune hemolytic anemia","volume":"58","author":"Hirano","year":"2016","journal-title":"Pediatr. Int."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"617","DOI":"10.3109\/14397595.2014.906053","article-title":"Successful therapy of macrophage activation syndrome with dexamethasone palmitate","volume":"26","author":"Nakagishi","year":"2016","journal-title":"Mod. Rheumatol."},{"key":"ref_57","doi-asserted-by":"crossref","unstructured":"Yamazaki, S., Endo, A., Iso, T., Abe, S., Aoyagi, Y., Suzuki, M., Fujii, T., Haruna, H., Ohtsuka, Y., and Shimizu, T. (2015). Cytomegalovirus as a potential trigger for systemic lupus erythematosus: A case report. BMC Res. Notes, 8.","DOI":"10.1186\/s13104-015-1520-2"},{"key":"ref_58","first-page":"651803","article-title":"Successful treatment with mycophenolate mofetil and tacrolimus in juvenile severe lupus nephritis","volume":"2015","author":"Kise","year":"2015","journal-title":"Case Rep. Pediatr."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1002\/art.40930","article-title":"2019 European League Against Rheumatism\/American College of Rheumatology classification criteria for systemic lupus erythematosus","volume":"71","author":"Aringer","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"2677","DOI":"10.1002\/art.34473","article-title":"Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus","volume":"64","author":"Petri","year":"2012","journal-title":"Arthritis Rheum."},{"key":"ref_61","doi-asserted-by":"crossref","unstructured":"Kaneko, S., Hatano, M., Shimbo, A., Miyaoka, F., Irabu, H., Akutsu, Y., Hayashi, Y., Mizuta, M., Nakagishi, Y., and Akamine, K. (Arthritis Rheumatol., 2025). Serum cytokine profiling differentiates underlying diseases in cytokine storm syndrome, Arthritis Rheumatol., in press.","DOI":"10.1002\/art.43349"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1093\/rheumatology\/keaa299","article-title":"Comparison of serum cytokine profiles in macrophage activation syndrome complicating different background rheumatic diseases in children","volume":"60","author":"Mizuta","year":"2021","journal-title":"Rheumatology"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"108255","DOI":"10.1016\/j.clim.2019.108255","article-title":"Extensive serum biomarker analysis in patients with macrophage activation syndrome associated with systemic lupus erythematosus","volume":"208","author":"Usami","year":"2019","journal-title":"Clin. Immunol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"109329","DOI":"10.1016\/j.clim.2023.109329","article-title":"CD169 expression on monocytes as a marker for assessing type I interferon status in pediatric inflammatory diseases","volume":"250","author":"Sakumura","year":"2023","journal-title":"Clin. Immunol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s00467-020-04734-w","article-title":"Steroid treatment promotes an M2 anti-inflammatory macrophage phenotype in childhood lupus nephritis","volume":"36","author":"Ikezumi","year":"2021","journal-title":"Pediatr. Nephrol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/j.jaci.2016.09.029","article-title":"Abnormal hematopoiesis and autoimmunity in human subjects with germline IKZF1 mutations","volume":"140","author":"Hoshino","year":"2017","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"102794","DOI":"10.1016\/j.jaut.2022.102794","article-title":"Intracellular virus sensor MDA5 mutation develops autoimmune myocarditis and nephritis","volume":"127","author":"Ohto","year":"2022","journal-title":"J. Autoimmun."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"2021","DOI":"10.1007\/s00296-023-05425-z","article-title":"Promoting awareness of terminology related to unmet medical needs in context of rheumatic diseases in Japan: A systematic review for evaluating unmet medical needs","volume":"43","author":"Yamazaki","year":"2023","journal-title":"Rheumatol. Int."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1093\/mr\/roab071","article-title":"Transitioning from paediatric to adult rheumatological healthcare: English summary of the Japanese Transition Support Guide","volume":"32","author":"Mori","year":"2022","journal-title":"Mod. Rheumatol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"2959","DOI":"10.1007\/s00467-024-06405-6","article-title":"Development of clinical and laboratory biomarkers in an international cohort of 428 children with lupus nephritis","volume":"39","author":"Wenderfer","year":"2024","journal-title":"Pediatr. Nephrol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"3699","DOI":"10.1007\/s00467-023-06018-5","article-title":"International cohort of 382 children with lupus nephritis\u2014Presentation, treatment and outcome at 24 months","volume":"38","author":"Wenderfer","year":"2023","journal-title":"Pediatr. Nephrol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"2563","DOI":"10.1093\/rheumatology\/keab746","article-title":"Defining renal remission in an international cohort of 248 children and adolescents with lupus nephritis","volume":"61","author":"Wenderfer","year":"2022","journal-title":"Rheumatology"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1007\/s40272-021-00457-z","article-title":"An update on the management of childhood-onset systemic lupus erythematosus","volume":"23","author":"Trindade","year":"2021","journal-title":"Paediatr. Drugs"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1136\/ard-2023-224762","article-title":"EULAR recommendations for the management of systemic lupus erythematosus: 2023 update","volume":"83","author":"Fanouriakis","year":"2024","journal-title":"Ann. Rheum. Dis."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"e000817","DOI":"10.1136\/lupus-2022-000817","article-title":"Treatment patterns in paediatric and adult patients with SLE: A retrospective claims database study in the USA","volume":"10","author":"Brunner","year":"2023","journal-title":"Lupus Sci. Med."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1136\/ard-2022-223328","article-title":"Towards development of treat to target (T2T) in childhood-onset systemic lupus erythematosus: PReS-endorsed overarching principles and points-to-consider from an international task force","volume":"82","author":"Smith","year":"2023","journal-title":"Ann. Rheum. Dis."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1038\/s41584-024-01152-2","article-title":"Systemic lupus erythematosus genetics: Insights into pathogenesis and implications for therapy","volume":"20","author":"Olferiev","year":"2024","journal-title":"Nat. Rev. Rheumatol."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/13\/2\/250\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T10:05:13Z","timestamp":1770804313000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/13\/2\/250"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,11]]},"references-count":77,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,2]]}},"alternative-id":["children13020250"],"URL":"https:\/\/doi.org\/10.3390\/children13020250","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,11]]}}}