The DOI matches its Crossref record; 81 of the 81 references it cites were found in a registry or an index.
Macrophage activation syndrome in the era of biologic therapy
Open accessAbstract
Macrophage activation syndrome (MAS) refers to acute overwhelming inflammation caused by a 'cytokine storm'. Although increasingly recognized as a life-threatening complication of various rheumatic diseases, clinically, MAS is strikingly similar to primary and secondary forms of haemophagocytic lymphohistiocytosis (HLH). Not surprisingly, many rheumatologists prefer the term secondary HLH rather than MAS to describe this condition, and efforts to change the nomenclature are in progress. The pathophysiology of MAS remains elusive, but observations in animal models, as well as data on the effects of new anticytokine therapies on rates and clinical presentations of MAS in patients with systemic juvenile idiopathic arthritis (sJIA), provide clues to the understanding of this perplexing clinical phenomenon. In this Review, we explore the latest available evidence and discuss potential diagnostic challenges in the era of increasing use of biologic therapies.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis
Henter, J., Horne, A., Aricó, M., Egeler, R. M., Filipovich, A. H., Imashuku, S., Ladisch, S., McClain, K., Webb, D., Winiarski, J., et al. (2007) · Pediatric Blood & Cancer · doi:10.1002/pbc.21039 · Cited by 5,386
Self-citationHematological Problems in Pediatric Intensive Care
Revel-Vilk, S., Cox, P., Robitaille, N., & Blanchette, V. (2014) · Pediatric and Adolescent Medicine · doi:10.1159/000350346 · Cited by 3,508
Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis
Stepp, S. E., Dufourcq-Lagelouse, R., Deist, F. L., Bhawan, S., Certain, S., Mathew, P. A., Henter, J.-I., Bennett, M., Fischer, A., Basile, G. D. S., et al. (1999) · Science · doi:10.1126/science.286.5446.1957 · Cited by 1,241
How I treat hemophagocytic lymphohistiocytosis
Jordan, M. B., Allen, C. E., Weitzman, S., Filipovich, A. H., & McClain, K. L. (2011) · Blood · doi:10.1182/blood-2011-03-278127 · Cited by 1,148
Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome
Ménasché, G., Pastural, E., Feldmann, J., Certain, S., Ersoy, F., Dupuis, S., Wulffraat, N., Bianchi, D., Fischer, A., Le Deist, F., et al. (2000) · Nature Genetics · doi:10.1038/76024 · Cited by 962
Feldmann, J., Callebaut, I., Raposo, G., Certain, S., Bacq, D., Dumont, C., Lambert, N., Ouachée-Chardin, M., Chedeville, G., Tamary, H., et al. (2003) · Cell · doi:10.1016/s0092-8674(03)00855-9 · Cited by 943
Pascual, V., Allantaz, F., Arce, E., Punaro, M., & Banchereau, J. (2005) · The Journal of Experimental Medicine · doi:10.1084/jem.20050473 · Cited by 922
Contemporary classification of histiocytic disorders
Favara, B. E., Feller, A. C., Pauli, M., Jaffe, E. S., Weiss, L. M., Arico, M., Bucsky, P., Egeler, R. M., Elinder, G., Gadner, H., et al. (1997) · Medical and Pediatric Oncology · doi:10.1002/(sici)1096-911x(199709)29:3<157::aid-mpo1>3.0.co;2-c · Cited by 837
Yokota, S., Imagawa, T., Mori, M., Miyamae, T., Aihara, Y., Takei, S., Iwata, N., Umebayashi, H., Murata, T., Miyoshi, M., et al. (2008) · The Lancet · doi:10.1016/s0140-6736(08)60454-7 · Cited by 789
Ravelli, A., Minoia, F., Davì, S., Horne, A., Bovis, F., Pistorio, A., Aricò, M., Avcin, T., Behrens, E. M., De Benedetti, F., et al. (2016) · Arthritis & Rheumatology · doi:10.1002/art.39332 · Cited by 773
Self-citation
Cited by
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
Nakra, N., Blumberg, D., Herrera-Guerra, A., & Lakshminrusimha, S. (2020) · Children · doi:10.3390/children7070069 · Cited by 512
Macrophage Activation-Like Syndrome: A Distinct Entity Leading to Early Death in Sepsis
Karakike, E., & Giamarellos-Bourboulis, E. J. (2019) · Frontiers in Immunology · doi:10.3389/fimmu.2019.00055 · Cited by 304
Interleukin-1β is a potential therapeutic target for periodontitis: a narrative review
Cheng, R., Wu, Z., Li, M., Shao, M., & Hu, T. (2020) · International Journal of Oral Science · doi:10.1038/s41368-019-0068-8 · Cited by 298
COVID-19 gone bad: A new character in the spectrum of the hyperferritinemic syndrome?
Colafrancesco, S., Alessandri, C., Conti, F., & Priori, R. (2020) · Autoimmunity Reviews · doi:10.1016/j.autrev.2020.102573 · Cited by 268
Metadata
- Authors
- Grom, A. A., Horne, A., & De Benedetti, F.
- Venue
- Nature Reviews Rheumatology
- Published
- 2016-03-24
- Volume
- 12
- Issue
- 5
- Pages
- 259-268
- ISSN
- 1759-4790 (ISSN-L)
- 1759-4804
- OpenAlex
- W2304277276
- Cited by
- 448
- References
- 81
- FWCI
- 5.68
- Type
- journal article
- Language
- en
- Open access
- green
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
How to cite
Cite CoChat for the checks on this page, and the paper for its own findings.
- This page
- CoChat. (2026). Citation check: Macrophage activation syndrome in the era of biologic therapy. https://app.cochat.ai/papers/10.1038/nrrheum.2015.179
- The paper
- Grom, A. A., Horne, A., & De Benedetti, F. (2016). Macrophage activation syndrome in the era of biologic therapy. Nature Reviews Rheumatology. https://doi.org/10.1038/nrrheum.2015.179
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
