Research & Literature

Rate and Clinical Presentation of Macrophage Activation Syndrome in Patients With Systemic Juvenile Idiopathic Arthritis Treated With Canakinumab.

Grom AA, Ilowite NT, Pascual V, et al.

Arthritis & rheumatology (Hoboken, N.J.) 2016; (68(1)):218-28 doi:10.1002/art.39407.

Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis.

Boom V, Anton J, Lahdenne P, et al.

Pediatric rheumatology online journal 2015; (13()):55 doi:10.1186/s12969-015-0055-3.

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

Ravelli A, Minoia F, Davì S, et al.

Annals of the rheumatic diseases 2016; (75(3)):481-9 doi:10.1136/annrheumdis-2015-208982.

Systemic Juvenile Idiopathic Arthritis: Diagnosis and Management.

Kumar S

Indian journal of pediatrics 2016; (83(4)):322-7 doi:10.1007/s12098-016-2060-z.

Outcome and Trends in Treatment of Systemic Juvenile Idiopathic Arthritis in the German National Pediatric Rheumatologic Database, 2000-2013.

Klotsche J, Raab A, Niewerth M, et al.

Arthritis & rheumatology (Hoboken, N.J.) 2016; (68(12)):3023-3034 doi:10.1002/art.39796.

Macrophage activation syndrome in a patient with systemic onset of the juvenile idiopathic arthritis.

Jain D, Aggarwal HK, Rao A, et al.

Reumatologia 2016; (54(1)):42-7 doi:10.5114/reum.2016.58763.

Serum ferritin levels as a useful diagnostic marker for the distinction of systemic juvenile idiopathic arthritis and Kawasaki disease.

Mizuta M, Shimizu M, Inoue N, et al.

Modern rheumatology 2016; (26(6)):929-932 doi:10.3109/14397595.2016.1159120.

Primary varicella infection in children with systemic juvenile idiopathic arthritis under tocilizumab therapy.

Nozawa T, Nishimura K, Ohara A, et al.

Modern rheumatology 2019; (29(3)):558-562 doi:10.1080/14397595.2016.1254314.

Early changes in gene expression and inflammatory proteins in systemic juvenile idiopathic arthritis patients on canakinumab therapy.

Brachat AH, Grom AA, Wulffraat N, et al.

Arthritis research & therapy 2017; (19(1)):13 doi:10.1186/s13075-016-1212-x.

Safety of tocilizumab in the treatment of juvenile idiopathic arthritis.

Machado SH, Xavier RM

Expert opinion on drug safety 2017; (16(4)):493-500 doi:10.1080/14740338.2017.1303479.

Protecting Bone Health in Pediatric Rheumatic Diseases: Pharmacological Considerations.

Zhang Y, Milojevic D

Paediatric drugs 2017; (19(3)):193-211 doi:10.1007/s40272-017-0219-3.

Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis.

Schulert GS, Minoia F, Bohnsack J, et al.

Arthritis care & research 2018; (70(3)):409-419 doi:10.1002/acr.23277.

Experience with etanercept, tocilizumab and interleukin-1 inhibitors in systemic onset juvenile idiopathic arthritis patients from the BIKER registry.

Horneff G, Schulz AC, Klotsche J, et al.

Arthritis research & therapy 2017; (19(1)):256 doi:10.1186/s13075-017-1462-2.

Validation of Classification Criteria of Macrophage Activation Syndrome in Japanese Patients With Systemic Juvenile Idiopathic Arthritis.

Shimizu M, Mizuta M, Yasumi T, et al.

Arthritis care & research 2018; (70(9)):1412-1415 doi:10.1002/acr.23482.

Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany.

Hinze CH, Holzinger D, Lainka E, et al.

Pediatric rheumatology online journal 2018; (16(1)):7 doi:10.1186/s12969-018-0224-2.

Reversal of Sepsis-Like Features of Neutrophils by Interleukin-1 Blockade in Patients With Systemic-Onset Juvenile Idiopathic Arthritis.

Ter Haar NM, Tak T, Mokry M, et al.

Arthritis & rheumatology (Hoboken, N.J.) 2018; (70(6)):943-956 doi:10.1002/art.40442.

The safety of live-attenuated vaccines in patients using IL-1 or IL-6 blockade: an international survey.

Jeyaratnam J, Ter Haar NM, Lachmann HJ, et al.

Pediatric rheumatology online journal 2018; (16(1)):19 doi:10.1186/s12969-018-0235-z.

Systemic juvenile idiopathic arthritis: New insights into pathogenesis and cytokine directed therapies.

Pardeo M, Bracaglia C, De Benedetti F

Best practice & research. Clinical rheumatology 2017; (31(4)):505-516 doi:10.1016/j.berh.2018.02.002.

Impact of systemic juvenile idiopathic arthritis/Still's disease on adolescents as evidenced through social media posts.

Modica RF, Lomax KG, Batzel P, Cassanas A

Open access rheumatology : research and reviews 2018; (10()):73-81 doi:10.2147/OARRR.S165010.

Systemic Juvenile Idiopathic Arthritis.

Lee JJY, Schneider R

Pediatric clinics of North America 2018; (65(4)):691-709 doi:10.1016/j.pcl.2018.04.005.

Macrophage activation syndrome: early diagnosis is key.

Lerkvaleekul B, Vilaiyuk S

Open access rheumatology : research and reviews 2018; (10()):117-128 doi:10.2147/OARRR.S151013.

Impact of biologics on disease course in systemic onset juvenile idiopathic arthritis.

Baris HE, Anderson E, Sozeri B, Dedeoglu F

Clinical rheumatology 2018; (37(12)):3263-3273 doi:10.1007/s10067-018-4297-6.

Glucocorticoid treatment in juvenile idiopathic arthritis.

Batu ED

Rheumatology international 2019; (39(1)):13-27 doi:10.1007/s00296-018-4168-0.

Tocilizumab in the treatment of systemic-onset juvenile idiopathic arthritis - single-centre experience.

Roszkiewicz J, Orczyk K, Smolewska E

Reumatologia 2018; (56(5)):279-284 doi:10.5114/reum.2018.79497.

PRECORDIAL PAIN, LEUKOCYTOSIS AND BICYTOPENIA IN A TEENAGER WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS UNDER IMMUNOSUPPRESSIVE THERAPY.

Vieira MS, Andrade FR, Brandão LMKR, et al.

Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo 2019; (37(2)):252-256 doi:10.1590/1984-0462/;2019;37;2;00004.

Treatment to Target Using Recombinant Interleukin-1 Receptor Antagonist as First-Line Monotherapy in New-Onset Systemic Juvenile Idiopathic Arthritis: Results From a Five-Year Follow-Up Study.

Ter Haar NM, van Dijkhuizen EHP, Swart JF, et al.

Arthritis & rheumatology (Hoboken, N.J.) 2019; (71(7)):1163-1173 doi:10.1002/art.40865.

Tofacitinib Treatment of Refractory Systemic Juvenile Idiopathic Arthritis.

Huang Z, Lee PY, Yao X, et al.

Pediatrics 2019; (143(5)) doi:10.1542/peds.2018-2845.

Molecular signature characterisation of different inflammatory phenotypes of systemic juvenile idiopathic arthritis.

Gohar F, McArdle A, Jones M, et al.

Annals of the rheumatic diseases 2019; (78(8)):1107-1113 doi:10.1136/annrheumdis-2019-215051.

Prognosis, complications and treatment response in systemic juvenile idiopathic arthritis patients: A single-center experience.

Barut K, Adrovic A, Sahin S, et al.

International journal of rheumatic diseases 2019; (22(9)):1661-1669 doi:10.1111/1756-185X.13649.

The frequency of macrophage activation syndrome and disease course in systemic juvenile idiopathic arthritis.

Çakan M, Karadağ ŞG, Tanatar A, Ayaz NA

Modern rheumatology 2020; (30(5)):900-904 doi:10.1080/14397595.2019.1660026.

Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.

Shimizu M, Mizuta M, Okamoto N, et al.

Pediatric rheumatology online journal 2020; (18(1)):2 doi:10.1186/s12969-020-0399-1.

Systemic onset juvenile idiopathic arthritis: a single center experience.

Sağ E, Uzunoğlu B, Bal F, et al.

The Turkish journal of pediatrics 2019; (61(6)):852-858.

Severe sepsis caused by serious gastrointestinal infection in sJIA patients treated with IL-6 receptor antagonist: a case report.

Xu M, Liu C, Guo L, Yang S

BMC pediatrics 2020; (20(1)):126 doi:10.1186/s12887-020-02032-w.

Th17 reprogramming of T cells in systemic juvenile idiopathic arthritis.

Henderson LA, Hoyt KJ, Lee PY, et al.

JCI insight 2020; (5(6)).

Experience with Cidofovir as an adjunctive therapy in a patient of adenovirus-induced macrophage activation syndrome in systemic arthritis.

Pal P, Bose N, Poddar A, et al.

Clinical rheumatology 2020; (39(8)):2449-2452 doi:10.1007/s10067-020-05133-0.

Efficacy and safety of canakinumab in systemic juvenile idiopathic arthritis: 48-week results from an open-label phase III study in Japanese patients.

Nishimura K, Hara R, Umebayashi H, et al.

Modern rheumatology 2021; (31(1)):226-234 doi:10.1080/14397595.2020.1783163.

Comparison of baseline laboratory findings of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis and multisystem inflammatory syndrome in children.

Aydın F, Çelikel E, Ekici Tekin Z, et al.

International journal of rheumatic diseases 2021; (24(4)):542-547 doi:10.1111/1756-185X.14078.

Macrophage activation syndrome in systemic juvenile idiopathic arthritis.

Shimizu M

Immunological medicine 2021; (44(4)):237-245 doi:10.1080/25785826.2021.1912893.

Short-Term Outcomes and Predictors of Effectiveness of Tocilizumab in Systemic Juvenile Idiopathic Arthritis: A Prospective Cohort Study.

Nada DW, Moghazy A, Allam AE, et al.

Frontiers in medicine 2021; (8()):665028 doi:10.3389/fmed.2021.665028.

Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study.

Otar Yener G, Paç Kısaarslan A, Ulu K, et al.

Rheumatology international 2022; (42(5)):879-889 doi:10.1007/s00296-021-04980-7.

MRP8/14 serum levels as diagnostic markers for systemic juvenile idiopathic arthritis in children with prolonged fever.

Park C, Miranda-Garcia M, Berendes R, et al.

Rheumatology (Oxford, England) 2022; (61(7)):3082-3092 doi:10.1093/rheumatology/keab729.

Bone Health in Children with Rheumatic Disorders: Focus on Molecular Mechanisms, Diagnosis, and Management.

Di Marcello F, Di Donato G, d'Angelo DM, et al.

International journal of molecular sciences 2022; (23(10)) doi:10.3390/ijms23105725.

Therapeutic plasma exchange in refractory macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a case-based review.

Liquidano-Perez E, Maza-Ramos G, Salazar-Bailón JL, et al.

Rheumatology international 2023; (43(1)):183-189 doi:10.1007/s00296-022-05231-z.

Case report: Emapalumab for active disease control prior to hematopoietic stem cell transplantation in refractory systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome.

Chellapandian D, Milojevic D

Frontiers in pediatrics 2023; (11()):1123104 doi:10.3389/fped.2023.1123104.

Overlapping clinical features of systemic juvenile idiopathic arthritis and SARS-CoV-2-related multisystem inflammatory syndrome in children.

Boyarchuk O, Kovalchuk T

Reumatologia 2023; (61(1)):63-70 doi:10.5114/reum/161185.

Systemic juvenile idiopathic arthritis: The Great Ormond Street Hospital experience (2005-2021).

Foley CM, McKenna D, Gallagher K, et al.

Frontiers in pediatrics 2023; (11()):1218312 doi:10.3389/fped.2023.1218312.

Anakinra and hepatotoxicity in pediatric rheumatology: a case series.

Martins FR, Azevedo AC, Ganhão S, et al.

Pediatric rheumatology online journal 2023; (21(1)):112 doi:10.1186/s12969-023-00891-y.

A novel serum calprotectin (MRP8/14) particle-enhanced immuno-turbidimetric assay (sCAL turbo) helps to differentiate systemic juvenile idiopathic arthritis from other diseases in routine clinical laboratory settings.

Foell D, Saers M, Park C, et al.

Molecular and cellular pediatrics 2023; (10(1)):14 doi:10.1186/s40348-023-00168-0.

Juvenile Idiopathic Arthritis.

Sudhakar M, Kumar S

Indian journal of pediatrics 2024; (91(9)):949-958 doi:10.1007/s12098-023-04939-5.

Case Report: Refractory macrophage activation syndrome requiring high-dose anakinra, emapalumab, and etoposide therapy in early-onset systemic juvenile idiopathic arthritis associated with adenoviremia.

Slaney ED, Modica R, Woolnough L, et al.

Frontiers in pediatrics 2023; (11()):1336554 doi:10.3389/fped.2023.1336554.

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy.

Ulu K, Aliyev E, Kılıç Könte E, et al.

Rheumatology (Oxford, England) 2024; (63(SI2)):SI167-SI172 doi:10.1093/rheumatology/keae124.

Single center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases.

Huang S, Liu Y, Yan W, et al.

Pediatric rheumatology online journal 2024; (22(1)):58 doi:10.1186/s12969-024-00991-3.

Extraarticular manifestations of juvenile idiopathic arthritis and their impact on health-related quality of life.

Tharwat S, Nassar MK, Salem KM, Nassar MK

Clinical rheumatology 2024; (43(7)):2295-2305 doi:10.1007/s10067-024-07008-0.

Genetics of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis.

Grom AA

Advances in experimental medicine and biology 2024; (1448()):121-126 doi:10.1007/978-3-031-59815-9_9.

Kawasaki Disease-Associated Cytokine Storm Syndrome.

Tsoukas P, Yeung RSM

Advances in experimental medicine and biology 2024; (1448()):365-383 doi:10.1007/978-3-031-59815-9_25.

EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.

Fautrel B, Mitrovic S, De Matteis A, et al.

Annals of the rheumatic diseases 2024; (83(12)):1614-1627 doi:10.1136/ard-2024-225851.

Utility of patient-reported outcomes for pulmonary symptoms and sleep disturbance and impairment in children with systemic juvenile idiopathic arthritis.

Nguyen K, Yasin S, Fall N, et al.

Pediatric rheumatology online journal 2025; (23(1)):74 doi:10.1186/s12969-025-01126-y.

Tofacitinib as an adjuvant treatment for pediatric Still's disease.

Hou L, Zhou P, Zhao C, et al.

Frontiers in pediatrics 2025; (13()):1650675 doi:10.3389/fped.2025.1650675.

Pan American League of Associations for Rheumatology treatment recommendations for systemic juvenile idiopathic arthritis.

Franco L, Rodrigues Fonseca A, Barzola ML, et al.

Rheumatology advances in practice 2025; (9(4)):rkaf087 doi:10.1093/rap/rkaf087.

Drug-induced liver injury secondary to anakinra in a patient with systemic juvenile idiopathic arthritis-Case report and literature overview.

Mannala S, Kehar M, Samson L, et al.

Canadian liver journal 2025; (8(4)):591-596 doi:10.3138/canlivj-2025-0039.

Effectiveness of Canakinumab for First-Line Steroid-Free Treatment in Systemic-Onset Juvenile Idiopathic Arthritis and Juvenile Still Disease.

Horneff G, Kallinich T, Hufnagel M, et al.

ACR open rheumatology 2026; (8(4)):e90028 doi:10.1002/acr2.90028.

Early intensification versus step-up biologic strategies in systemic juvenile idiopathic arthritis: a Bayesian network meta-analysis of remission and safety outcomes.

Liu X, Yang L, Huang H

Pediatric rheumatology online journal 2026; (24(1)).

Diagnostic value of basic and extended procedures in pediatric fever of unknown origin - results of a nation-wide surveillance study.

Bienioschek MK, Lejeune A, Ngoumou G, et al.

Molecular and cellular pediatrics 2026; (13(1)).

The Asia-Pacific League of Associations for Rheumatology Consensus Recommendations on the Management of Systemic Juvenile Idiopathic Arthritis (Juvenile Still's Disease).

Arkachaisri T, Teh KL, Vilaiyuk S, et al.

International journal of rheumatic diseases 2026; (29(8)):e70810 doi:10.1111/1756-185x.70810.

Diagnostic pitfalls in systemic juvenile idiopathic arthritis: insights from 6 misdiagnosed cases.

Zhu X, Guo Y, Guo J, et al.

Frontiers in pediatrics 2026; (14()):1885428 doi:10.3389/fped.2026.1885428.