Macrophage Activation SyndromeAMETHYSTNCT06405152
Assessment of Macrophage Activation syndromE in STill's Disease
An observational study of Macrophage Activation Syndrome and Still's Disease, Adult-Onset, run by Swedish Orphan Biovitrum at 11 sites in 7 countries.
Trial summary
Assessment of Macrophage activation syndrome in STill's disease: retrospective chart analysis of patient History, Symptom resolution and Treatment characteristics Read more about this study
- Who is it for?
- Ages 6 months to 80 years
- Conditions the study is for
- Macrophage Activation Syndrome, Still's Disease, Adult-Onset, Stills Disease, Juvenile-Onset
- What is being tested?
- Chart review
- How many people
- 75 participants
- Where
- 11 sitesin 7 countries
- Study dates
- 27 Sep 2023to 26 Sep 2024
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 months to 80 years
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Study population: The study population will include patients with Still's disease who were diagnosed with MAS that was refractory to GC treatment.
Who may be able to join
- Age >6 months and ≤80 years at the beginning of the index MAS episode.
- Diagnosis of Still's disease (sJIA (systemic Juvenile Idiopathic Arthritis) or AOSD (Adult Onset Stills Disease) diagnosis).
- Diagnosis of MAS according to treating physician in the medical record.
- Patients who have received at least 3 consecutive days of GC after diagnosis of MAS and/or are judged by the Investigator to be refractory to GC due to clinical worsening of patient's condition .
- The onset of the index MAS episode occurred between 01 January 2012 and 30 September 2022.
Who cannot join
- A diagnosis of primary Hemophagocytic Lymphohistiocytosis (HLH) prior to the beginning of the index MAS episode.
- Confirmed malignancy prior to the beginning of the index MAS episode.
- Patient treated with any investigational product as a part of clinical trial during the index MAS episode.
Trial locations and contacts
11 sites in 7 countries.
11 sites in 7 countries
United States4 sites
Swedish Orphan Biovitrum Research Site
Atlanta, Georgia, 30329
Swedish Orphan Biovitrum Research Site
Cincinnati, Ohio, 45229
Swedish Orphan Biovitrum Research Site
Los Angeles, California, 90095
Swedish Orphan Biovitrum Research Site
Philadelphia, Pennsylvania, 19104
Italy2 sites
Swedish Orphan Biovitrum Research Site
Milan
Swedish Orphan Biovitrum Research Site
Rome
About this study
Official title: Assessment of Macrophage Activation syndromE in STill's Disease: Retrospective Chart Analysis of Patient HistorY, Symptom Resolution and Treatment Characteristics
Assessment of Macrophage activation syndrome in STill's disease: retrospective chart analysis of patient History, Symptom resolution and Treatment characteristics
Read the detailed description
This is an observational, retrospective cohort study on the treatment utilization and outcomes of Macrophage Activation Syndrome (MAS) refractory to glucocorticoids (GC) in patients with Still's disease. The study will be conducted entirely through medical chart abstraction; all data will be taken from the patient's medical record, with no additional assessments.
- Conditions
- Macrophage Activation Syndrome, Still's Disease, Adult-Onset, Stills Disease, Juvenile-Onset
- Being tested
- Chart review
- Medical subject headings
- Macrophage Activation Syndrome, Still's Disease, Adult-Onset, Arthritis, Juvenile
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Chart reviewOther
Retrospective Chart Review
What is being measured
The primary outcome is the main question the study is designed to answer.
Overall survival
The proportion of patients alive after 1-year of follow-up
Measured: 1 year
Time to laboratory value normalization
Time to normalization for the key laboratory values
Measured: From Index date up to 26 weeks
Time to Macrophage Activation Syndrome (MAS) laboratory remission
Time to normalization for all key laboratory values
Measured: From Index date up to 26 weeks
Time to partial MAS laboratory remission
Time to normalization for at least 3 key laboratory values
Measured: From Index date up to 26 weeks
Time to tapering of Glucocorticoids (GCs)
From index date to the last of 7 consecutive days receiving ≤1 mg/kg/day of prednisone (PDN).
Measured: from index date to the last of 7 consecutive days
Number of recurrent MAS episodes
Occurring any time after the end of the data collection period for the index MAS episode
Measured: through study completion
Administration of organ support care
Proportion of patients receiving hemodialysis, assisted ventilation, cardiac support, or inotropic drugs
Measured: from the index date until either the end of hospitalization or 26 weeks, whichever occurs later
Characteristics of MAS treatment
description of treatment patterns of MAS-related treatments for both index and recurrent MAS episodes.
Measured: for MAS episodes occurring during the data collection period
Clinical signs
No of MAS symptoms (Fever, Skin rash, Hemorrhagic manifestations, Evidence of CNS involvement) present
Measured: At index date and 8 weeks after index date or at the earliest time this data becomes available between 8 and 12 weeks
Secondary outcomes (3)
Time to hospital discharge
Defined as time from the index date to discharge from the hospital
Measured: Up to 26 weeks
Time to intensive care unit (ICU) discharge
For patients who are admitted to the ICU during the index MAS episode, this is defined as time from index date or ICU admission (whichever comes later) to ICU discharge
Measured: Up to 26 weeks
Duration of Clinical Response
defined as the time (in days) from the date of first documented MAS laboratory remission until the date of first documented occurrence of a new MAS episode as per investigator's assessment.
Measured: Until week 26
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted8 May 2024
- Study start27 Sep 2023
- Primary completion26 Sep 2024
- Study completion26 Sep 2024
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Macrophage Activation SyndromeStudies of the same condition, open and under way.
- More trials run by Swedish Orphan BiovitrumThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Other
- Time perspective
- Retrospective
- Enrolment
- 75 participants (actual)
Sponsor and investigators
- Lead sponsor
- Swedish Orphan Biovitrum
- Sponsor type
- Industry (pharma, biotech, devices)
- Responsible party
- Sponsor
- Investigators
- Uwe Ullman, MD, Study director, Swedish Orphan Biovitrum AG Riehenring 182, 4058 Basel, Switzerland
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06405152
- Sponsor's protocol ID
- Sobi.Emapalumab-105
- Acronym
- AMETHYST
- Status last verified
- Oct 2024
- First submitted
- 9 Apr 2024
- Last updated
- 5 Oct 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 5 Oct 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Assessment of Macrophage Activation syndromE in STill's Disease: Retrospective Chart Analysis of Patient HistorY, Symptom Resolution and Treatment Characteristics
- ClinicalTrials.gov ID
- NCT06405152
- Acronym / sponsor ID
- AMETHYST / Sobi.Emapalumab-105
- Conditions
- Macrophage Activation Syndrome, Still's Disease, Adult-Onset, Stills Disease, Juvenile-Onset
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 75 participants (actual)
- Lead sponsor
- Swedish Orphan BiovitrumIndustry (pharma, biotech, devices)
- Study start
- 27 Sep 2023 (actual)
- Primary completion
- 26 Sep 2024 (actual)
- Study completion
- 26 Sep 2024 (actual)
- First posted
- 8 May 2024 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Assessment of Macrophage activation syndrome in STill's disease: retrospective chart analysis of patient History, Symptom resolution and Treatment characteristics
Detailed description
This is an observational, retrospective cohort study on the treatment utilization and outcomes of Macrophage Activation Syndrome (MAS) refractory to glucocorticoids (GC) in patients with Still's disease. The study will be conducted entirely through medical chart abstraction; all data will be taken from the patient's medical record, with no additional assessments.
Study design
- Observational model
- Other
- Time perspective
- Retrospective
Who may qualify
- Ages
- 6 months to 80 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
- Study population
- The study population will include patients with Still's disease who were diagnosed with MAS that was refractory to GC treatment.
Key inclusion criteria (5 listed)
- Age >6 months and ≤80 years at the beginning of the index MAS episode.
- Diagnosis of Still's disease (sJIA (systemic Juvenile Idiopathic Arthritis) or AOSD (Adult Onset Stills Disease) diagnosis).
- Diagnosis of MAS according to treating physician in the medical record.
- Patients who have received at least 3 consecutive days of GC after diagnosis of MAS and/or are judged by the Investigator to be refractory to GC due to clinical worsening of patient's condition .
- The onset of the index MAS episode occurred between 01 January 2012 and 30 September 2022.
Key exclusion criteria (3 listed)
- A diagnosis of primary Hemophagocytic Lymphohistiocytosis (HLH) prior to the beginning of the index MAS episode.
- Confirmed malignancy prior to the beginning of the index MAS episode.
- Patient treated with any investigational product as a part of clinical trial during the index MAS episode.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Chart reviewRetrospective Chart Review | Other | – |
Primary outcome
Overall survival
Time frame: 1 year
The proportion of patients alive after 1-year of follow-up
Time to laboratory value normalization
Time frame: From Index date up to 26 weeks
Time to normalization for the key laboratory values
Time to Macrophage Activation Syndrome (MAS) laboratory remission
Time frame: From Index date up to 26 weeks
Time to normalization for all key laboratory values
Time to partial MAS laboratory remission
Time frame: From Index date up to 26 weeks
Time to normalization for at least 3 key laboratory values
Time to tapering of Glucocorticoids (GCs)
Time frame: from index date to the last of 7 consecutive days
From index date to the last of 7 consecutive days receiving ≤1 mg/kg/day of prednisone (PDN).
Number of recurrent MAS episodes
Time frame: through study completion
Occurring any time after the end of the data collection period for the index MAS episode
Administration of organ support care
Time frame: from the index date until either the end of hospitalization or 26 weeks, whichever occurs later
Proportion of patients receiving hemodialysis, assisted ventilation, cardiac support, or inotropic drugs
Characteristics of MAS treatment
Time frame: for MAS episodes occurring during the data collection period
description of treatment patterns of MAS-related treatments for both index and recurrent MAS episodes.
Clinical signs
Time frame: At index date and 8 weeks after index date or at the earliest time this data becomes available between 8 and 12 weeks
No of MAS symptoms (Fever, Skin rash, Hemorrhagic manifestations, Evidence of CNS involvement) present
3 secondary outcomes
Time to hospital discharge
Time frame: Up to 26 weeks
Time to intensive care unit (ICU) discharge
Time frame: Up to 26 weeks
Duration of Clinical Response
Time frame: Until week 26
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 5 Oct 2026.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Uwe Ullman, MD study director
Swedish Orphan Biovitrum AG Riehenring 182, 4058 Basel, Switzerland
11 sites in 7 countries: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Macrophage Activation Syndrome and Still's Disease, Adult-Onset, run by Swedish Orphan Biovitrum at 11 sites in 7 countries.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 5 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 5 Oct 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 11 sites in United States, Italy, Canada, France, Germany and 2 more countries. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Overall survival (time frame: 1 year). The study lists 9 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 5 Oct 2026.