Evidence map›Paper›PMID 26400031›Full record

ReviewIndian journal of pediatrics2016

Macrophage Activation Syndrome.

Ethan S Sen, Sarah L N Clarke, Athimalaipet V Ramanan

Open access · bronzeAbstract readReview
PubMed Publisher
In one paragraph

Review in Indian journal of pediatrics, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
37citing papers in PubMed, 1 pooled it
2.3field-weighted citation impact, top 11% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

37 citing papers in PubMed, 1 synthesis or guideline pooled it, 57 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Review
  4. Article
  5. Article
  6. Article
  7. Review
  8. Review
  9. Article
  10. Article
  11. Article
  12. Cytokine Storm Syndrome Associated with Hemorrhagic Fever and Other Viruses.Advances in experimental medicine and biology · 2024
    Review
  13. Article
  14. Review
  15. [Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2023
    Article
  16. Review
  17. Review
  18. Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment.International journal of molecular sciences · 2022
    Review
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

3 authors at 1 institution in 1 country.

Ethan S SenDepartment of Pediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK.
Sarah L N ClarkeDepartment of Pediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK.
Athimalaipet V RamananDepartment of Pediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK. avramanan@hotmail.com.
Bristol Royal Hospital for Children · GB

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases such as systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus. It is often considered a type of secondary hemophagocytic lymphohistiocytosis (HLH) and results from over-activation of T lymphocytes and macrophages leading to a "cytokine storm". Characteristic features are persistent fever, lymphadenopathy, hepatosplenomegaly, cytopenias (anemia, leucopenia, thrombocytopenia), raised C-reactive protein, falling erythrocyte sedimentation rate, hypofibrinogenemia, transaminitis, hypertriglyceridemia and extreme hyperferritinemia often associated with multi-organ impairment. Key to its management is early recognition of MAS which may be difficult due to similarity to systemic sepsis or flares of the underlying rheumatic disease. To aid with this process, criteria for the diagnosis of MAS in patients with sJIA derived by international consensus have been published. Although bone marrow biopsy showing hemophagocytosis is strongly supportive it is not essential for diagnosis. Together with appropriate supportive care, first-line treatment is high-dose intravenous corticosteroids with cyclosporin or intravenous immunoglobulin (IVIg) added if there is not initial response. Although etoposide is used by hematologists in treatment of HLH, there are concerns regarding organ toxicity and bone marrow suppression which weigh against its use in initial management of MAS. With increasing understanding of the pathogenesis of MAS, use of drugs targeting specific cytokines has been reported in case series. The relatively rapid effectiveness of anakinra, a recombinant IL-1 receptor antagonist, has been documented. Further studies of this and other biologic agents are required to identify the most effective and safest treatment option for refractory MAS.

Indexed as

Antibodies, MonoclonalCytokinesGlucocorticoidsHumansImmunoglobulins, IntravenousMacrophage Activation SyndromeAntibodies, MonoclonalCytokinesGlucocorticoidsImmunoglobulins, IntravenousHemophagocytic lymphohistiocytosisHyperferritinemiaInterleukin-1Macrophage activation syndromeSystemic juvenile idiopathic arthritis

Identifiers

PMID26400031
OpenAlexW2114259834

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.