The diagnostic significance of soluble CD163 and soluble interleukin‐2 receptor α‐chain in macrophage activation syndrome and untreated new‐onset systemic juvenile idiopathic arthritis

Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis

Stepp, S. E., Dufourcq-Lagelouse, R., Deist, F. L., Bhawan, S., Dogniaux, S., Mathew, P. A., Henter, J., Bennett, M., Fischer, A., Basile, G. D. S., et al. (1999) · Science · doi:10.1126/science.286.5446.1957 · Cited by 1,236 · see citing works

Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban 1997

Petty, R. E., Southwood, T. R. E., Baum, J., Bhettay, E., Glass, D. N., Manners, P. J., Maldonado-Cocco, J. A., Suarez-Almazor, M. E., Orozco-Alcalá, J., & Prieur, A. (2001) · CRC Press eBooks · doi:10.3109/9780203214237-62 · Cited by 1,175 · see citing works

An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder

Jordan, M. B., Hildeman, D., Kappler, J., & Marrack, P. (2004) · Blood · doi:10.1182/blood-2003-10-3413 · Cited by 712 · see citing works

Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Ravelli, A., Magni‐Manzoni, S., Pistorio, A., Besana, C., Foti, T., Ruperto, N., Viola, S., & Martini, A. (2005) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2004.12.016 · Cited by 409 · see citing works

Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-γ-producing lymphocytes and IL-6- and TNF-α-producing macrophages

Billiau, A., Roskams, T., Damme‐Lombaerts, R. V., Matthys, P., & Wouters, C. H. (2004) · Blood · doi:10.1182/blood-2004-08-2997 · Cited by 306 · see citing works

Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: Possible relationship to drugs or infection

Hadchouel, M., Prieur, A., & Griscelli, C. (1985) · The Journal of Pediatrics · doi:10.1016/s0022-3476(85)80072-x · Cited by 251 · see citing works

Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome

Villanueva, J. M., Lee, S., Giannini, E. H., Graham, T. B., Passo, M. H., Filipovich, A. H., & Grom, A. A. (2004) · Arthritis Research & Therapy · doi:10.1186/ar1453 · Cited by 240 · see citing works

Elevated soluble interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes

Komp, D., McNamara, J., & Buckley, P. J. (1989) · Blood · doi:10.1182/blood.v73.8.2128.bloodjournal7382128 · Cited by 227 · see citing works

Natural killer cell dysfunction: A common pathway in systemic‐onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis?

Grom, A. A. (2004) · Arthritis & Rheumatism · doi:10.1002/art.20198 · Cited by 209 · see citing works

Familial and Acquired Hemophagocytic Lymphohistiocytosis

Janka, G., & Stadt, U. Z. (2005) · Hematology · doi:10.1182/asheducation-2005.1.82 · Cited by 203 · see citing works

Soluble hemoglobin–haptoglobin scavenger receptor CD163 as a lineage‐specific marker in the reactive hemophagocytic syndrome

Schaer, D. J., Schleiffenbaum, B. E., Kurrer, M. O., Imhof, A., Bächli, E., Fehr, J., Møller, H. J., Moestrup, S. K., & Schaffner, A. (2004) · European Journal Of Haematology · doi:10.1111/j.1600-0609.2004.00318.x · Cited by 203 · see citing works

Soluble CD163: a marker molecule for monocyte/macrophage activity in disease

Møller, H. J., Aerts, J. M. F. G., Grøbæk, H., Peterslund, N. A., Petersen, P. H., Hornung, N., Renjmark, L., Jabbarpour, E., & Moestrup, S. K. (2002) · Scandinavian Journal of Clinical and Laboratory Investigation · doi:10.1080/003655102762377466 · Cited by 162 · see citing works

Defective Natural Killer Cell Function in Patients with Hemophagocytic Lymphohistiocytosis and in First Degree Relatives

Sullivan, K. E., DeLaat, C., Douglas, S. D., & Filipovich, A. H. (1998) · Pediatric Research · doi:10.1203/00006450-199810000-00001 · Cited by 138 · see citing works

Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood

Avčin, T., Tse, S. M. L., Schneider, R., Ngan, B., & Silverman, E. D. (2006) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2005.12.070 · Cited by 125 · see citing works

Is macrophage activation syndrome a new entity?

Athreya, B. H. (2003) · PubMed · Cited by 80 · see citing works

Hematological Problems in Pediatric Intensive Care

Revel‐Vilk, S., Cox, P. N., Robitaille, N., & Blanchette, V. S. (2013) · Pediatric and adolescent medicine · doi:10.1159/000350346 · Cited by 3,526 · see citing works

How I treat hemophagocytic lymphohistiocytosis

Jordan, M. B., Allen, C. E., Weitzman, S. S., Filipovich, A. H., & McClain, K. L. (2011) · Blood · doi:10.1182/blood-2011-03-278127 · Cited by 1,145 · see citing works

Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome: Reanalysis of a Prior Phase III Trial*

Shakoory, B., Carcillo, J. A., Chatham, W. W., Amdur, R. L., Zhao, H., Dinarello, C. A., Cron, R. Q., & Opal, S. M. (2016) · Critical Care Medicine · doi:10.1097/ccm.0000000000001402 · Cited by 867 · see citing works

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., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avčin, T., Behrens, E. M., Benedetti, F. D., et al. (2015) · Arthritis & Rheumatology · doi:10.1002/art.39332 · Cited by 770 · see citing works

The Immunology of Macrophage Activation Syndrome

Crayne, C. B., Albeituni, S., Nichols, K. E., & Cron, R. Q. (2019) · Frontiers in Immunology · doi:10.3389/fimmu.2019.00119 · Cited by 633 · see citing works

CD163 and Inflammation: Biological, Diagnostic, and Therapeutic Aspects

Etzerodt, A., & Moestrup, S. K. (2012) · Antioxidants and Redox Signaling · doi:10.1089/ars.2012.4834 · Cited by 609 · see citing works

Macrophage activation syndrome in the era of biologic therapy

Grom, A. A., Horne, A., & Benedetti, F. D. (2016) · Nature Reviews Rheumatology · doi:10.1038/nrrheum.2015.179 · Cited by 450 · see citing works

Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

Minoia, F., Davì, S., Horne, A., Demirkaya, E., Bovis, F., Li, C., Lehmberg, K., Weitzman, S. S., Insalaco, A., Wouters, C. H., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38802 · Cited by 430 · see citing works

Juvenile Idiopathic Arthritis

Benedetti, F. D., & Ravelli, A. (2000) · BioDrugs · doi:10.2165/00063030-200014020-00003 · Cited by 430 · see citing works

Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)

Jordan, M. B., Allen, C. E., Greenberg, J., Henry, M., Hermiston, M. L., Kumar, A. R., Hines, M., Eckstein, O. S., Ladisch, S., Nichols, K. E., et al. (2019) · Pediatric Blood & Cancer · doi:10.1002/pbc.27929 · Cited by 428 · see citing works

Large-scale genetic fine mapping and genotype-phenotype associations implicate polymorphism in the IL2RA region in type 1 diabetes

Lowe, C. E., Cooper, J. D., Brusko, T. M., Walker, N. M., Smyth, D. J., Bailey, R., Bourget, K., Plagnol, V., Field, S., Atkinson, M. A., et al. (2007) · Nature Genetics · doi:10.1038/ng2102 · Cited by 427 · see citing works

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

Ravelli, A., Minoia, F., Davì, S., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avčin, T., Behrens, E. M., Benedetti, F. D., et al. (2016) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2015-208982 · Cited by 409 · see citing works

Pathogenesis of Macrophage Activation Syndrome and Potential for Cytokine- Directed Therapies

Schulert, G. S., & Grom, A. A. (2014) · Annual Review of Medicine · doi:10.1146/annurev-med-061813-012806 · Cited by 401 · see citing works

Mechanisms, biomarkers and targets for adult-onset Still’s disease

Feist, E., Mitrovic, S., & Fautrel, B. (2018) · Nature Reviews Rheumatology · doi:10.1038/s41584-018-0081-x · Cited by 397 · see citing works

Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice

Behrens, E. M., Canna, S. W., Slade, K., Rao, S., Kreiger, P. A., Paessler, M., Kambayashi, T., & Koretzky, G. A. (2011) · Journal of Clinical Investigation · doi:10.1172/jci43157 · Cited by 392 · see citing works

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

Ravelli, A., Grom, A. A., Behrens, E. M., & Cron, R. Q. (2012) · Genes and Immunity · doi:10.1038/gene.2012.3 · Cited by 381 · see citing works

Scavenger receptor CD163, a Jack-of-all-trades and potential target for cell-directed therapy

Gorp, H. V., Delputte, P., & Nauwynck, H. (2010) · Molecular Immunology · doi:10.1016/j.molimm.2010.02.008 · Cited by 358 · see citing works

Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy

Griffin, G., Shenoi, S., & Hughes, G. C. (2020) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2020.101515 · Cited by 352 · see citing works

Soluble CD163

Møller, H. J. (2011) · Scandinavian Journal of Clinical and Laboratory Investigation · doi:10.3109/00365513.2011.626868 · Cited by 343 · see citing works

Hemophagocytic Lymphohistiocytosis

Rosado, F., & Kim, A. S. (2013) · American Journal of Clinical Pathology · doi:10.1309/ajcp4zdkj4icouat · Cited by 338 · see citing works

Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions

Mellins, E., Macaubas, C., & Grom, A. A. (2011) · Nature Reviews Rheumatology · doi:10.1038/nrrheum.2011.68 · Cited by 330 · see citing works

Review: Cytokine Storm Syndrome: Looking Toward the Precision Medicine Era

Behrens, E. M., & Koretzky, G. A. (2017) · Arthritis & Rheumatology · doi:10.1002/art.40071 · Cited by 304 · see citing works

Histiocytic Disorders: Recent Insights into Pathophysiology and Practical Guidelines

Filipovich, A. H., McClain, K. L., & Grom, A. A. (2009) · Transplantation and Cellular Therapy · doi:10.1016/j.bbmt.2009.11.014 · Cited by 294 · see citing works

Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty‐eight patients

Parodi, A., Davì, S., Pringe, A. B., Pistorio, A., Ruperto, N., Magni‐Manzoni, S., Miettunen, P., Bader‐Meunier, B., Espada, G., Sterba, G., et al. (2009) · Arthritis & Rheumatism · doi:10.1002/art.24883 · Cited by 291 · see citing works

Molecular Mechanisms in Genetically Defined Autoinflammatory Diseases: Disorders of Amplified Danger Signaling

de Jesus, A. A., Canna, S., Liu, Y., & Goldbach‐Mansky, R. T. (2015) · Annual Review of Immunology · doi:10.1146/annurev-immunol-032414-112227 · Cited by 277 · see citing works

Secondary hemophagocytic lymphohistiocytosis and severe sepsis/systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation

Castillo, L., & Carcillo, J. A. (2009) · Pediatric Critical Care Medicine · doi:10.1097/pcc.0b013e3181a1ae08 · Cited by 272 · see citing works

Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment

Carter, S. J., Tattersall, R. S., & Ramanan, A. V. (2018) · Lara D. Veeken · doi:10.1093/rheumatology/key006 · Cited by 263 · see citing works

Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis

Shimizu, M., Yokoyama, T., Yamada, K., Kaneda, H., Wada, H., Wada, T., Toma, T., Ohta, K., Kasahara, Y., & Yachie, A. (2010) · Rheumatology · doi:10.1093/rheumatology/keq133 · Cited by 255 · see citing works

Juvenile Idiopathic Arthritis: Diagnosis and Treatment

Giancane, G., Consolaro, A., Lanni, S., Davì, S., Schiappapietra, B., & Ravelli, A. (2016) · Rheumatology and Therapy · doi:10.1007/s40744-016-0040-4 · Cited by 247 · see citing works

Gene expression profiling of peripheral blood from patients with untreated new‐onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome

Fall, N., Barnes, M., Thornton, S., Luyrink, L., Olson, J., Ilowite, N. T., Gottlieb, B. S., Griffin, T., Sherry, D. D., Thompson, S., et al. (2007) · Arthritis & Rheumatism · doi:10.1002/art.22981 · Cited by 245 · see citing works

Hemophagocytic Lymphohistiocytosis: Advances in Pathophysiology, Diagnosis, and Treatment

Chandrakasan, S., & Filipovich, A. H. (2013) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2013.06.053 · Cited by 235 · see citing works

Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management

Morimoto, A., Nakazawa, Y., & Ishii, E. (2016) · Pediatrics International · doi:10.1111/ped.13064 · Cited by 230 · see citing works

Macrophage Activation Syndrome

Ravelli, A., Davì, S., Minoia, F., Martini, A., & Cron, R. Q. (2015) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2015.06.010 · Cited by 211 · see citing works

M1 and M2 Monocytes in Rheumatoid Arthritis: A Contribution of Imbalance of M1/M2 Monocytes to Osteoclastogenesis

Fukui, S., Iwamoto, N., Takatani, A., Igawa, T., Shimizu, T., Umeda, M., Nishino, A., Horai, Y., Hirai, Y., Koga, T., et al. (2018) · Frontiers in Immunology · doi:10.3389/fimmu.2017.01958 · Cited by 209 · see citing works

Macrophage activation syndrome: advances towards understanding pathogenesis

Grom, A. A., & Mellins, E. (2010) · Current Opinion in Rheumatology · doi:10.1097/01.bor.0000381996.69261.71 · Cited by 209 · see citing works

Whole‐Exome Sequencing Reveals Overlap Between Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis and Familial Hemophagocytic Lymphohistiocytosis

Kaufman, K. M., Linghu, B., Szustakowski, J. D., Husami, A., Yang, F., Zhang, K., Filipovich, A. H., Fall, N., Harley, J. B., Nirmala, N. R., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38793 · Cited by 207 · see citing works

Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH

Hayden, A. R., Lin, M., Park, S., Pudek, M. R., Schneider, M., Jordan, M. B., Mattman, A., & Chen, L. Y. C. (2017) · Blood Advances · doi:10.1182/bloodadvances.2017012310 · Cited by 203 · see citing works

Advances in understanding the pathogenesis of HLH

Usmani, G. N., Woda, B. A., & Newburger, P. E. (2013) · British Journal of Haematology · doi:10.1111/bjh.12293 · Cited by 202 · see citing works

Making Sense of the Cytokine Storm: A Conceptual Framework for Understanding, Diagnosing, and Treating Hemophagocytic Syndromes

Canna, S., & Behrens, E. M. (2012) · Pediatric Clinics of North America · doi:10.1016/j.pcl.2012.03.002 · Cited by 197 · see citing works

Approach to Hemophagocytic Syndromes

Weitzman, S. S. (2011) · Hematology · doi:10.1182/asheducation-2011.1.178 · Cited by 197 · see citing works

The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis

Lin, Y., Wang, C.-T., Gershwin, M. E., & Chiang, B. (2011) · Autoimmunity Reviews · doi:10.1016/j.autrev.2011.02.001 · Cited by 195 · see citing works

Hemoglobin and Heme Scavenger Receptors

Nielsen, M. J., Møller, H. J., & Moestrup, S. K. (2009) · Antioxidants and Redox Signaling · doi:10.1089/ars.2009.2792 · Cited by 191 · see citing works

The Conserved Scavenger Receptor Cysteine-Rich Superfamily in Therapy and Diagnosis

Martínez, V. G., Moestrup, S. K., Holmskov, U., Mollenhauer, J., & Lozano, F. (2011) · Pharmacological Reviews · doi:10.1124/pr.111.004523 · Cited by 187 · see citing works

Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy

Gupta, S., & Weitzman, S. S. (2009) · Expert Review of Clinical Immunology · doi:10.1586/eci.09.58 · Cited by 183 · see citing works

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13‐4 polymorphisms

Zhang, K., Biroschak, J., Glass, D. N., Thompson, S. D., Finkel, T. H., Passo, M. H., Binstadt, B. A., Filipovich, A. H., & Grom, A. A. (2008) · Arthritis & Rheumatism · doi:10.1002/art.23734 · Cited by 182 · see citing works

An open label trial of anakinra to prevent respiratory failure in COVID-19

Kyriazopoulou, E., Panagopoulos, P., Metallidis, S., Dalekos, G. Ν., Poulakou, G., Gatselis, N., Karakike, E., Saridaki, M., Loli, G., Stefos, A., et al. (2021) · eLife · doi:10.7554/elife.66125 · Cited by 182 · see citing works

Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Shimizu, M., Nakagishi, Y., Inoue, N., Mizuta, M., Ko, G., Saikawa, Y., Kubota, T., Yamasaki, Y., Takei, S., & Yachie, A. (2015) · Clinical Immunology · doi:10.1016/j.clim.2015.06.005 · Cited by 179 · see citing works

Systemic Juvenile Idiopathic Arthritis

Lee, J. J. Y., & Schneider, R. (2018) · Pediatric Clinics of North America · doi:10.1016/j.pcl.2018.04.005 · Cited by 168 · see citing works

Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management

Henderson, L. A., & Cron, R. Q. (2019) · Pediatric Drugs · doi:10.1007/s40272-019-00367-1 · Cited by 168 · see citing works

Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis

Allen, C. E., & McClain, K. L. (2015) · Hematology · doi:10.1182/asheducation-2015.1.177 · Cited by 164 · see citing works

Macrophage activation syndrome and cytokine-directed therapies

Schulert, G. S., & Grom, A. A. (2014) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2014.03.002 · Cited by 163 · see citing works

Macrophage activation syndrome: early diagnosis is key

Lerkvaleekul, B., & Vilaiyuk, S. (2018) · Open Access Rheumatology Research and Reviews · doi:10.2147/oarrr.s151013 · Cited by 151 · see citing works

An International Consensus Survey of Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

Davì, S., Consolaro, A., Guseinova, D., Pistorio, A., Ruperto, N., Martini, A., Cron, R. Q., & Ravelli, A. (2011) · The Journal of Rheumatology · doi:10.3899/jrheum.100996 · Cited by 149 · see citing works

Life-threatening complications of adult-onset Still’s disease

Efthimiou, P., Kadavath, S. M., & Mehta, B. Y. (2014) · Clinical Rheumatology · doi:10.1007/s10067-014-2487-4 · Cited by 148 · see citing works

Managing Macrophages in Rheumatoid Arthritis by Reform or Removal

Li­, ­., Hsu, H., & Mountz, J. D. (2012) · Current Rheumatology Reports · doi:10.1007/s11926-012-0272-4 · Cited by 149 · see citing works

Genetic Defects in Cytolysis in Macrophage Activation Syndrome

Zhang, M., Behrens, E. M., Atkinson, T. P., Shakoory, B., Grom, A. A., & Cron, R. Q. (2014) · Current Rheumatology Reports · doi:10.1007/s11926-014-0439-2 · Cited by 148 · see citing works

Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review

Lin, M., Park, S., Hayden, A. R., Giustini, D., Trinkaus, M. A., Pudek, M. R., Mattman, A., Schneider, M., & Chen, L. Y. C. (2017) · Annals of Hematology · doi:10.1007/s00277-017-2993-y · Cited by 150 · see citing works

A case of macrophage activation syndrome successfully treated with anakinra

Kelly, A., & Ramanan, A. V. (2008) · Nature Clinical Practice Rheumatology · doi:10.1038/ncprheum0919 · Cited by 147 · see citing works

Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults

Hines, M., Greenwood, T. V. B., Beutel, G., Beutel, K., Hays, J. A., Horne, A., Janka, G., Jordan, M. B., van Laar, J. A. M., Lachmann, G., et al. (2021) · Critical Care Medicine · doi:10.1097/ccm.0000000000005361 · Cited by 144 · see citing works

Association of a Mutation in LACC1 With a Monogenic Form of Systemic Juvenile Idiopathic Arthritis

Wakil, S. M., Monies, D., Abouelhoda, M. I., Tassan, N. A. A., Al‐Dusery, H., Naim, E. A., Al‐Younes, B., Shinwari, J. M., Al-Mohanna, F. A., Meyer, B. F., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38877 · Cited by 142 · see citing works

Cytokine profile in adult-onset Still's disease: Comparison with systemic juvenile idiopathic arthritis

Inoue, N., Shimizu, M., Tsunoda, S., Kawano, M., Matsumura, M., & Yachie, A. (2016) · Clinical Immunology · doi:10.1016/j.clim.2016.05.010 · Cited by 140 · see citing works

Systemic JIA: new developments in the understanding of the pathophysiology and therapy

Vastert, S. J., Kuis, W., & Grom, A. A. (2009) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2009.08.003 · Cited by 138 · see citing works

Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis

Lehmberg, K., & Ehl, S. R. (2012) · British Journal of Haematology · doi:10.1111/bjh.12138 · Cited by 137 · see citing works

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

Schulert, G. S., Minoia, F., Bohnsack, J. F., Cron, R. Q., Hashad, S., Koné‐Paut, I., Kostik, M. M., Lovell, D. J., Maritsi, D., Nigrovic, P. A., et al. (2017) · Arthritis Care & Research · doi:10.1002/acr.23277 · Cited by 137 · see citing works

The macrophages in rheumatic diseases

Lurati, A. M., Laria, A., Marrazza, M., Re, K. A., Mazzocchi, D., & Scarpellini, M. (2016) · Journal of Inflammation Research · doi:10.2147/jir.s82320 · Cited by 136 · see citing works

Tolerance and efficacy of off-label anti-interleukin-1 treatments in France: a nationwide survey

Rossi‐Semerano, L., Fautrel, B., Wendling, D., HACHULLA, E., Galeotti, C., Semerano, L., Touitou, I., Koné‐Paut, I., & the MAIL1 (Maladies Auto-inflammatoires et Anti-IL-1) study Group on the behalf of CRI (Club Rhumatisme et Inflammation) (2015) · Orphanet Journal of Rare Diseases · doi:10.1186/s13023-015-0228-7 · Cited by 136 · see citing works

Review of haemophagocytic lymphohistiocytosis

Freeman, H. R., & Ramanan, A. V. (2010) · Archives of Disease in Childhood · doi:10.1136/adc.2009.176610 · Cited by 131 · see citing works

Cytokine Release Syndrome in COVID-19 Patients, A New Scenario for an Old Concern: The Fragile Balance between Infections and Autoimmunity

Diamanti, A. P., Rosado, M. M., Pioli, C., Sesti, G., & Laganà, B. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21093330 · Cited by 129 · see citing works

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

Grom, A. A., Ilowite, N. T., Pascual, V., Brunner, H. I., Martini, A., Lovell, D. J., Ruperto, N., Leon, K., Lheritier, K., & Abrams, K. B. (2015) · Arthritis & Rheumatology · doi:10.1002/art.39407 · Cited by 129 · see citing works

Differential Expression of CD163 on Monocyte Subsets in Healthy and HIV-1 Infected Individuals

Tippett, E., Cheng, W., Westhorpe, C. L. V., Cameron, P. U., Brew, B. J., Lewin, S. R., Jaworowski, A., & Crowe, S. M. (2011) · PLoS ONE · doi:10.1371/journal.pone.0019968 · Cited by 126 · see citing works

Performance of Current Guidelines for Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

Davì, S., Minoia, F., Pistorio, A., Horne, A., Consolaro, A., Rosina, S., Bovis, F., Cimaz, R., Gámir, M. L. G., Ilowite, N. T., et al. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38769 · Cited by 116 · see citing works

Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Minoia, F., Bovis, F., Davì, S., Horne, A., Fischbach, M., Frosch, M., Huber, A. M., Jelušić, M., Sawhney, S., McCurdy, D., et al. (2019) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2019-215211 · Cited by 113 · see citing works

Synovial cellular and molecular markers in rheumatoid arthritis

Amin, M. A., Fox, D. A., & Ruth, J. H. (2017) · Seminars in Immunopathology · doi:10.1007/s00281-017-0631-3 · Cited by 109 · see citing works

Inflammatory biomarkers in COVID-19-associated multisystem inflammatory syndrome in children, Kawasaki disease, and macrophage activation syndrome: a cohort study

Rodriguez‐Smith, J., Verweyen, E. L., Clay, G. M., Esteban, Y., de Loizaga, S. R., Baker, E. J., Do, T., Dhakal, S., Lang, S. M., Grom, A. A., et al. (2021) · The Lancet Rheumatology · doi:10.1016/s2665-9913(21)00139-9 · Cited by 109 · see citing works

Recognition and management of macrophage activation syndrome in juvenile arthritis

Kelly, A., & Ramanan, A. V. (2007) · Current Opinion in Rheumatology · doi:10.1097/bor.0b013e32825a6a79 · Cited by 107 · see citing works

Hemophagocytic Lymphohistiocytosis: Clinical Presentations and Diagnosis

Risma, K., & Marsh, R. A. (2018) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2018.11.050 · Cited by 106 · see citing works

New Markers for Adult-Onset Still's Disease

Mitrovic, S., & Fautrel, B. (2017) · Joint Bone Spine · doi:10.1016/j.jbspin.2017.05.011 · Cited by 100 · see citing works

Approaching hemophagocytic lymphohistiocytosis

Chinnici, A., Beneforti, L., Pegoraro, F., Trambusti, I., Tondo, A., Favre, C., Coniglio, M. L., & Sieni, E. (2023) · Frontiers in Immunology · doi:10.3389/fimmu.2023.1210041 · Cited by 99 · see citing works

Clinical features and correct diagnosis of macrophage activation syndrome

Cron, R. Q., Davì, S., Minoia, F., & Ravelli, A. (2015) · Expert Review of Clinical Immunology · doi:10.1586/1744666x.2015.1058159 · Cited by 97 · see citing works

Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Ravelli, A., Minoia, F., Davì, S., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avčin, T., Behrens, E. M., Benedetti, F. D., et al. (2016) · RMD Open · doi:10.1136/rmdopen-2015-000161 · Cited by 96 · see citing works

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

Boom, V., Antón, J., Lahdenne, P., Quartier, P., Ravelli, A., Wulffraat, N. M., & Vastert, S. J. (2015) · Pediatric Rheumatology · doi:10.1186/s12969-015-0055-3 · Cited by 95 · see citing works

Hemophagocytic Syndrome and Critical Illness

Tóthová, Z., & Berliner, N. (2014) · Journal of Intensive Care Medicine · doi:10.1177/0885066613517076 · Cited by 93 · see citing works

The genetics of macrophage activation syndrome

Schulert, G. S., & Cron, R. Q. (2020) · Genes & Immunity · doi:10.1038/s41435-020-0098-4 · Cited by 91 · see citing works

Monocyte MicroRNA Expression in Active Systemic Juvenile Idiopathic Arthritis Implicates MicroRNA‐125a‐5p in Polarized Monocyte Phenotypes

Schulert, G. S., Fall, N., Harley, J. B., Shen, N., Lovell, D. J., Thornton, S., & Grom, A. A. (2016) · Arthritis & Rheumatology · doi:10.1002/art.39694 · Cited by 88 · see citing works

Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases

Lin, C., Yu, H., Lee, J., Wang, L., Lin, Y., Yang, Y., & Chiang, B. (2012) · Clinical Rheumatology · doi:10.1007/s10067-012-1998-0 · Cited by 87 · see citing works

Differentiating Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis from Other Forms of Hemophagocytic Lymphohistiocytosis

Lehmberg, K., Pink, I., zu Eulenburg, C., Beutel, K., Maul‐Pavicic, A., & Janka, G. (2013) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2012.11.081 · Cited by 85 · see citing works

sCD163 in AOSD: a biomarker for macrophage activation related to hyperferritinemia

Colafrancesco, S., Priori, R., Alessandri, C., Astorri, E., Perricone, C., Blank, M., Agmon‐Levin, N., Shoenfeld, Y., & Valesini, G. (2014) · Immunologic Research · doi:10.1007/s12026-014-8563-7 · Cited by 83 · see citing works

Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis

Bennett, T. D., Fluchel, M. N., Hersh, A. O., Hayward, K. N., Hersh, A. L., Brogan, T. V., Srivastava, R., Stone, B. L., Korgenski, E. K., Mundorff, M. B., et al. (2012) · Arthritis & Rheumatism · doi:10.1002/art.34661 · Cited by 80 · see citing works

Proteomic profiling of MIS-C patients indicates heterogeneity relating to interferon gamma dysregulation and vascular endothelial dysfunction

Diorio, C. J., Shraim, R., Vella, L. A., Giles, J. R., Baxter, A. E., Oldridge, D. A., Canna, S., Henrickson, S. E., McNerney, K. O., Balamuth, F., et al. (2021) · Nature Communications · doi:10.1038/s41467-021-27544-6 · Cited by 77 · see citing works

Convergent pathways of the hyperferritinemic syndromes

Schulert, G. S., & Canna, S. (2018) · International Immunology · doi:10.1093/intimm/dxy012 · Cited by 77 · see citing works

Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

Minoia, F., Davì, S., Horne, A., Bovis, F., Demirkaya, E., Akikusa, J. D., Ayaz, N. A., Al-Mayouf, S. M., Barone, P., Bica, B., et al. (2015) · The Journal of Rheumatology · doi:10.3899/jrheum.141261 · Cited by 76 · see citing works

Complications of adult-onset Still’s disease and their management

Mitrovic, S., & Fautrel, B. (2018) · Expert Review of Clinical Immunology · doi:10.1080/1744666x.2018.1465821 · Cited by 72 · see citing works

Review of biomarkers in systemic juvenile idiopathic arthritis: helpful tools or just playing tricks?

Gohar, F., Kessel, C., Lavrič, M., Holzinger, D., & Foell, D. (2016) · Arthritis Research & Therapy · doi:10.1186/s13075-016-1069-z · Cited by 71 · see citing works

Macrophage activation syndrome: Serological markers and treatment with anti-thymocyte globulin

Coca, A., Bundy, K. W., Marston, B. A., Huggins, J., & Looney, R. J. (2009) · Clinical Immunology · doi:10.1016/j.clim.2009.02.005 · Cited by 69 · see citing works

Macrophage activation syndrome in rheumatic disease: What is the role of the antigen presenting cell?

Behrens, E. M. (2008) · Autoimmunity Reviews · doi:10.1016/j.autrev.2007.11.025 · Cited by 69 · see citing works

Pediatric macrophage activation syndrome, recognizing the tip of the Iceberg

Crayne, C. B., & Cron, R. Q. (2019) · European Journal of Rheumatology · doi:10.5152/eurjrheum.2019.19150 · Cited by 67 · see citing works

Proteolytic shedding of the macrophage scavenger receptor CD163 in multiple sclerosis

Fabriek, B. O., Møller, H. J., Vloet, R. P. M., van Winsen, L. M. L., Hanemaaijer, R., Teunissen, C. E., Uitdehaag, B. M. J., van den Berg, T. K., & Dijkstra, C. D. (2007) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2007.04.016 · Cited by 65 · see citing works

Elevated Granzyme B in Cytotoxic Lymphocytes is a Signature of Immune Activation in Hemophagocytic Lymphohistiocytosis

Mellor-Heineke, S., Villanueva, J. M., Jordan, M. B., Marsh, R. A., Zhang, K., Bleesing, J., Filipovich, A. H., & Risma, K. (2013) · Frontiers in Immunology · doi:10.3389/fimmu.2013.00072 · Cited by 64 · see citing works

Application of the 2016 EULAR/ACR/PRINTO Classification Criteria for Macrophage Activation Syndrome in Patients with Adult-onset Still Disease

Ahn, S. S., Yoo, B.-W., Jung, S. M., Lee, S., Park, Y., & Song, J. J. (2017) · The Journal of Rheumatology · doi:10.3899/jrheum.161286 · Cited by 64 · see citing works

Advances in the pathogenesis and treatment of systemic juvenile idiopathic arthritis

Correll, C. K., & Binstadt, B. A. (2013) · Pediatric Research · doi:10.1038/pr.2013.187 · Cited by 63 · see citing works

Potential markers for macrophage activation syndrome in systemic JIA

(2007) · Nature Clinical Practice Rheumatology · doi:10.1038/ncprheum0489

Which Children with JIA Are At Risk for a Potentially Fatal Complication?

(2007) · AAP Grand Rounds · doi:10.1542/gr.18-3-29-a

Soluble CD 25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis

Reddy, V. V., Myles, A., Cheekatla, S. S., Singh, S., & Aggarwal, A. (2014) · International Journal of Rheumatic Diseases · doi:10.1111/1756-185x.12196 · Cited by 27 · see citing works

Soluble CD163, a unique biomarker to evaluate the disease activity, exhibits macrophage activation in systemic juvenile idiopathic arthritis

Sakumura, N., Shimizu, M., Mizuta, M., Inoue, N., Nakagishi, Y., & Yachie, A. (2018) · Cytokine · doi:10.1016/j.cyto.2018.05.017 · Cited by 44 · see citing works

Characteristic elevation of soluble TNF receptor II : I ratio in macrophage activation syndrome with systemic juvenile idiopathic arthritis

Shimizu, M., Inoue, N., Mizuta, M., Nakagishi, Y., & Yachie, A. (2017) · Clinical & Experimental Immunology · doi:10.1111/cei.13026 · Cited by 47 · see citing works