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Disease · MONDO_0015545

macrophage activation syndrome

6 sourcesdiseases-2026-09-18-0.1.0

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ConductScience Library. macrophage activation syndrome. Diseases, record MONDO_0015545. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0015545

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name
macrophage activation syndrome
Open Targets
Open Targets ID
MONDO_0015545
Open Targets
Other names
MAS, reactive hemophagocytic lymphohistiocytosis
Open Targets
MeSH
D055501
MeSH
NCI Thesaurus
C114471
NCI Thesaurus
Orphanet
ORPHA:158061
Orphanet
Highest stage of its drugs
Phase 3, for 3 of its 7 drugs, from ClinicalTrials.gov
Open Targets

Drugs7

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

DrugChEMBL IDTypeOpen Targets' stageStage reported byReportsOf them trialsTrials shown hereSource
EMAPALUMABCHEMBL3989977AntibodyPhase 3ClinicalTrials.gov333Drugs and targets
METHYLPREDNISOLONECHEMBL650Small moleculePhase 3ClinicalTrials.gov111Drugs and targets
RUXOLITINIBCHEMBL1789941Small moleculePhase 3ClinicalTrials.gov111Drugs and targets
ANAKINRACHEMBL1201570ProteinPhase 2ClinicalTrials.gov333Drugs and targets
INTERFERON GAMMA-1BCHEMBL1201564ProteinPhase 2ClinicalTrials.gov111Drugs and targets
TOCILIZUMABCHEMBL1237022AntibodyPhase 2ClinicalTrials.gov111Drugs and targets
ALDESLEUKINCHEMBL1201438ProteinPhase 1/2ClinicalTrials.gov111Drugs and targets

Targets13

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

TargetProteinIts drugs that act on itMechanismsHighest stage of those drugsGene in GenesSource
IFNGInterferon gamma1Interferon gamma inhibitorPhase 3https://conductscience.org/library/genes/3458Drugs and targets
IFNGR1Interferon gamma receptor1Interferon gamma receptor agonistPhase 2https://conductscience.org/library/genes/3459Drugs and targets
IFNGR2Interferon gamma receptor1Interferon gamma receptor agonistPhase 2https://conductscience.org/library/genes/3460Drugs and targets
IL1R1Interleukin-1 receptor type 11Interleukin-1 receptor antagonistPhase 2https://conductscience.org/library/genes/3554Drugs and targets
IL2RAInterleukin-2 receptor1Interleukin-2 receptor agonistPhase 1/2https://conductscience.org/library/genes/3559Drugs and targets
IL2RBInterleukin-2 receptor1Interleukin-2 receptor agonistPhase 1/2https://conductscience.org/library/genes/3560Drugs and targets
IL2RGInterleukin-2 receptor1Interleukin-2 receptor agonistPhase 1/2https://conductscience.org/library/genes/3561Drugs and targets
IL6RInterleukin-6 receptor subunit alpha1Interleukin-6 receptor alpha subunit inhibitorPhase 2https://conductscience.org/library/genes/3570Drugs and targets
JAK1Tyrosine-protein kinase JAK11Tyrosine-protein kinase JAK1 inhibitorPhase 3https://conductscience.org/library/genes/3716Drugs and targets
JAK2Tyrosine-protein kinase JAK21Tyrosine-protein kinase JAK2 inhibitorPhase 3https://conductscience.org/library/genes/3717Drugs and targets

1 to 10 of 13

See all 13 targets

Trials8

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

TrialTitlePhaseParticipantsResults first postedConditionsDrugsLinked bySource
NCT02069899A Study for Long-term Follow-up of Hemophagocytic Lymphohistiocytosis (HLH) Participants Who Received Treatment With Emapalumab (NI-0501), an Anti-interferon Gamma Monoclonal AntibodyPhase 2/3582022-06-28Hemophagocytic LymphohistiocytosisEMAPALUMABIts record names the diseaseClinicalTrials.gov
NCT05001737Evaluate Efficacy, Safety and Tolerability, PK and PD of Emapalumab in Children and Adults With MAS in Still's or SLEPhase 3332026-02-19Macrophage Activation Syndrome, Secondary Hemophagocytic Lymphohistiocytosis, Still Disease, Systemic Lupus Erythematosus, SJIA, AOSD, MASEMAPALUMABIts record names the diseaseClinicalTrials.gov
NCT03311854
A Study to Investigate the Safety and Efficacy of Emapalumab, an Anti-IFN-gamma mAb in Patients With Systemic Juvenile Idiopathic Arthritis (sJIA) or Adult-onset Still's Disease (AOSD) Developing Macrophage Activation Syndrome/Secondary HLH (MAS/sHLH)
Phase 2142022-01-26Macrophage Activation Syndrome, Lymphohistiocytosis, Hemophagocytic, Arthritis, Juvenile, Adult Onset Still DiseaseEMAPALUMABIts record names the diseaseClinicalTrials.gov
NCT04339712Personalised Immunotherapy for SARS-CoV-2 (COVID-19) Associated With Organ DysfunctionPhase 2102COVID-19, Virus Diseases, Macrophage Activation Syndrome, Corona Virus InfectionANAKINRA, TOCILIZUMABIts record names the diseaseClinicalTrials.gov
NCT05137496Ruxolitinib and Methylprednisolone as a First-line Treatment for Macrophage Activation SyndromePhase 340Macrophage Activation SyndromeMETHYLPREDNISOLONE, RUXOLITINIBIts record names the diseaseClinicalTrials.gov
NCT03332225A Trial of Validation and Restoration of Immune Dysfunction in Severe Infections and SepsisPhase 236Sepsis, Macrophage Activation SyndromeANAKINRA, INTERFERON GAMMA-1BIts record names the diseaseClinicalTrials.gov
NCT02780583Treatment of Macrophage Activation Syndrome (MAS) With AnakinraPhase 19Macrophage Activation SyndromeANAKINRAIts record names the diseaseClinicalTrials.gov
NCT02569463Low-dose IL-2 ( Interleukin-2) Treatment in Macrophage Activation Syndrome(MAS)Phase 1/26Macrophage Activation SyndromeALDESLEUKINIts record names the diseaseClinicalTrials.gov

Comparisons in its trials12

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

ComparisonGroup 1, with the outcomeGroup 2, with the outcomeParticipantsTrialSource
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Laboratory Parameters0 of 250 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Study Interruption Due to Safety Reasons1 of 251 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Withdrawal From the Study Due to Lack of Response0 of 250 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Adverse Events (AEs) (Serious and Non-serious).24 of 258 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Survival23 of 258 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Proportion of Subjects With Overall Response as Defined by CR or Partial Response (PR)16 of 256 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Proportion of Subjects With Complete Response (CR) at Week 8 After First Administration of Emapalumab11 of 254 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): deaths from any cause2 of 250 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): Number of Patients With Glucocorticoids (GC)s Tapering to a Dose Below 50% of Prednisolone (PDN) Equivalent at the Time of Emapalumab Start or to the Same (or Lower) Dose Being Administered Before the Occurrence of MAS Whichever Occurs First
15 of 257 of 833NCT05001737Trial comparisons
Cohort 1 (sJIA and AOSD) vs Cohort 2 (SLE): serious adverse events18 of 254 of 833NCT05001737Trial comparisons

Papers from its trials20

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMIDListed asTrials listing itSource
32374962RESULT1PubMed
28918754RESULT1PubMed
27856268RESULT1PubMed
27418577RESULT1PubMed
26903338RESULT1PubMed
26584195RESULT1PubMed
24183799RESULT1PubMed
22822024RESULT1PubMed
20597663RESULT1PubMed
16003060RESULT1PubMed

Data dates

The date of each source this release read.

Open Targets
2026-09-18
Open Targets
Drug links and their checks, from Drugs and targets
2026-09-18
Drugs and targets
ClinicalTrials.gov, from Trials
2026-11-12
Trials
Comparisons, from Trial comparisons
2026-03-23
Trial comparisons
PubMed, for the checks
2026-11-06
PubMed
MeSH, for the checks
2026-11-10
MeSH

Sources 6

  • ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
  • ChEMBL, through Open TargetsCC BY-SA 3.0
  • ClinicalTrials.govNo license stated
  • NLM's MeSHNLM's terms
  • Open TargetsCC0 1.0
  • PubMedNLM's terms
Each source's terms
  • ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
  • ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
  • ClinicalTrials.govnone stated
  • NLM's MeSHNLM's
  • Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
  • PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0015545

GET https://conductscience.org/api/diseases/v1/MONDO_0015545/drugs