Kawasaki DiseaseNCT07491926
MASKd: a Study on Kawasaki Disease (KD) Complicated by Macrophage Activation Syndrome (MAS)
An observational study of Kawasaki Disease and Macrophage Activation Syndrome (MAS), run by Meyer Children's Hospital IRCCS at 1 site in Italy.
Trial summary
Kawasaki Disease (KD) is one of the most common vasculitides in childhood and represents a leading cause of acquired heart disease in developed countries. Macrophage Activation Syndrome (MAS) is a potentially life threatening hyperinflammatory condition belonging to the spectrum of hemophagocytic lymphohistiocytosis… Read more about this study
- Who is it for?
- Ages 4 weeks to 17 years
- Conditions the study is for
- Kawasaki Disease, Macrophage Activation Syndrome (MAS)
- How many people
- 150 participants
- Where
- 1 siteItaly
- Study dates
- Feb 2026to Feb 2029
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
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 4 weeks to 17 years
- All sexes
- No healthy volunteers
- Child
Study population: Since no validated or universally accepted diagnostic criteria currently exist for MAS occurring in the context of KD, the diagnosis of MAS will be based on the clinical judgment of the treating physician. To minimize the risk of misdiagnosis, all cases will be independently and critically reviewed by three experienced pediatric rheumatologists. Control groups will consist of age- and sex-matched patients, divided as follows: * Patients with KD resistant to first-line therapy * Patients with KD responsive to first-line therapy For each patient with KD complicated by MAS included in the study, 2 KD-responsive and 2 KD-resistant patients will be enrolled. The goal is to include at least 30 MAS-KD patients, along with 60 KD-resistant controls and 60 KD-responsive controls.
Who may be able to join
- Age between 4 weeks and under 18 years at the time of KD diagnosis
- Diagnosis of KD made according to the 2024 AHA guidelines
- Diagnosis of MAS made by the attending physician within 30 days from the onset of KD.
Who cannot join
- Unconfirmed diagnosis of KD (e.g., mimicking conditions)
- Primary (genetic) HLH
- Lack of informed consent
- MAS diagnosed more than 30 days after or more than 15 days before the onset of KD
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
Italy1 site
Azienda Ospedaliero-Universitaria IRCCS Meyer
Florence, Italy, 50139
Teresa Giani, MD, +390555662924, teresa.giani@meyer.it
About this study
Official title: MACROPHAGE ACTIVATION SYNDROME IN KAWASAKI DISEASE: Features, Treatment, Outcome, Predictive and Diagnostic Factors (The MASKd Study)
Kawasaki Disease (KD) is one of the most common vasculitides in childhood and represents a leading cause of acquired heart disease in developed countries. Macrophage Activation Syndrome (MAS) is a potentially life threatening hyperinflammatory condition belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), and it can complicate various rheumatologic diseases. Awareness of MAS in the context of KD has recently increased, supporting the hypothesis that it is an underdiagnosed complication. The study aims to define the epidemiology, clinical characteristics, management, and therapeutic strategies of MAS in patients with KD, through a multicenter data collection in Europe.
Read the detailed description
KD most frequently affects young children under the age of 5. Its epidemiology varies by geographical location and season. The course of KD can be complicated by the development of MAS.
Clinical similarities between KD-especially refractory KD-and MAS, combined with the lack of specific diagnostic criteria, may hinder accurate and timely identification of MAS in KD, complicating treatment decisions and worsening clinical outcomes. Given that MAS is associated with a significant risk of multi-organ failure (MOF), patient prognosis may be severely compromised, with increased morbidity and mortality. Therefore, early recognition of MAS is crucial in order to implement targeted therapeutic strategies as promptly as possible.
In this retrospective-prospective, observational, descriptive, international multicenter study, we aim to:
- Analyze the clinical features, management, and outcomes of patients with KD complicated by MAS to describe this complication and identify potential risk factors for MAS development;
- Evaluate the performance of currently available MAS diagnostic criteria in KD patients and identify specific diagnostic criteria for this condition.
The study will include international pediatric rheumatology centers affiliated with the PReS network.
- Medical subject headings
- Mucocutaneous Lymph Node Syndrome, Macrophage Activation Syndrome
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| MAS-KD patients. | Patients with diagnosis of KD made according to the 2024 AHA guidelines. | |
| KD-resistant controls | Patients with KD resistant to first-line therapy | |
| KD-responsive controls | Patients with KD responsive to first-line therapy. |
What is being measured
The primary outcome is the main question the study is designed to answer.
MAS-KD population
Definition of the proportion of patients with KD who develop MAS in the study population.
Measured: From the study initiation date onward for 36 months
Clinical and laboratory features of MAS KD patients
Adjusted Odds ratios of clinical and laboratory risk factors
Measured: From the study initiation date onward for 36 months
Applicability and diagnostic performance of currently available MAS classification criteria
Evaluation of diagnostic performance metrics (sensitivity, specificity, positive predictive value, negative predictive value) of existing MAS criteria when applied to KD patients.
Measured: From the study initiation date onward for 36 months
Secondary outcomes (6)
Heterogeneity of MAS KD population
Differences in clinical course based on e.g., intensive care unit admission (present/absent, days), duration of fever (days), coronary involvement (present/absent,) among three groups of Patients: patients with KD complicated by MAS; patients with KD resistant to first-line therapy; patients with KD responsive to first-line therapy.
Measured: From the study initiation date onward for 36 months
Applicability and diagnostic performance of currently available MAS classification criteria
Evaluation of diagnostic performance metrics (sensitivity, specificity, positive predictive value, negative predictive value) of existing MAS criteria when applied to KD patients.
Measured: From the study initiation date onward for 36 months
MAS diagnosis
Time from KD onset to MAS diagnosis and its correlation with clinical outcomes;
Measured: From the study initiation date onward for 36 months
Treatment and clinical response of MAS KD patients
Description of treatments used for MAS (e.g., corticosteroids, IVIG, biologics) and the corresponding clinical response.
Measured: From the study initiation date onward for 36 months
Heterogeneity of MAS KD population
Differences in treatment strategies based on e.g., intensive care unit admission (present/absent, days), duration of fever (days), coronary involvement (present/absent,) among three groups of Patients: patients with KD complicated by MAS; patients with KD resistant to first-line therapy; patients with KD responsive to first-line therapy.
Measured: From the study initiation date onward for 36 months
Heterogeneity of MAS KD population
Differences in outcomes based on e.g., intensive care unit admission (present/absent, days), duration of fever (days), coronary involvement (present/absent,) among three groups of Patients: patients with KD complicated by MAS; patients with KD resistant to first-line therapy; patients with KD responsive to first-line therapy.
Measured: From the study initiation date onward for 36 months
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted25 Mar 2026
- Study startFeb 2026 (estimated)
- Primary completionNov 2027 (estimated)
- Study completionFeb 2029 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Kawasaki DiseaseStudies of the same condition, open and under way.
- 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
- Case-control
- Time perspective
- Other
- Enrolment
- 150 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Meyer Children's Hospital IRCCS
- Sponsor type
- University, hospital or other
- Responsible party
- Principal investigator, Teresa Giani, Meyer Children's Hospital IRCCS
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07491926
- Sponsor's protocol ID
- MASKd
- Status last verified
- Mar 2026
- First submitted
- 20 Jan 2026
- Last updated
- 25 Mar 2026
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Study at a glance
From the registry record, last updated by the sponsor on 25 Mar 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- MACROPHAGE ACTIVATION SYNDROME IN KAWASAKI DISEASE: Features, Treatment, Outcome, Predictive and Diagnostic Factors (The MASKd Study)
- ClinicalTrials.gov ID
- NCT07491926
- Acronym / sponsor ID
- MASKd
- Study type
- Observational
- Recruitment status
- Not yet recruiting
- Enrolment
- 150 participants (estimated)
- Lead sponsor
- Meyer Children's Hospital IRCCSUniversity, hospital or other
- Study start
- Feb 2026 (estimated)
- Primary completion
- Nov 2027 (estimated)
- Study completion
- Feb 2029 (estimated)
- First posted
- 25 Mar 2026 (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.
Kawasaki Disease (KD) is one of the most common vasculitides in childhood and represents a leading cause of acquired heart disease in developed countries. Macrophage Activation Syndrome (MAS) is a potentially life threatening hyperinflammatory condition belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), and it can complicate various rheumatologic diseases. Awareness of MAS in the context of KD has recently increased, supporting the hypothesis that it is an underdiagnosed complication. The study aims to define the epidemiology, clinical characteristics, management, and therapeutic strategies of MAS in patients with KD, through a multicenter data collection in Europe.
Detailed description
KD most frequently affects young children under the age of 5. Its epidemiology varies by geographical location and season. The course of KD can be complicated by the development of MAS.
Clinical similarities between KD-especially refractory KD-and MAS, combined with the lack of specific diagnostic criteria, may hinder accurate and timely identification of MAS in KD, complicating treatment decisions and worsening clinical outcomes. Given that MAS is associated with a significant risk of multi-organ failure (MOF), patient prognosis may be severely compromised, with increased morbidity and mortality. Therefore, early recognition of MAS is crucial in order to implement targeted therapeutic strategies as promptly as possible.
In this retrospective-prospective, observational, descriptive, international multicenter study, we aim to:
- Analyze the clinical features, management, and outcomes of patients with KD complicated by MAS to describe this complication and identify potential risk factors for MAS development;
- Evaluate the performance of currently available MAS diagnostic criteria in KD patients and identify specific diagnostic criteria for this condition.
The study will include international pediatric rheumatology centers affiliated with the PReS network.
Study design
- Observational model
- Case-control
- Time perspective
- Other
- Number of arms
- 3
| Arm | Type | Receives |
|---|---|---|
| MAS-KD patients. | – | –Patients with diagnosis of KD made according to the 2024 AHA guidelines. |
| KD-resistant controls | – | –Patients with KD resistant to first-line therapy |
| KD-responsive controls | – | –Patients with KD responsive to first-line therapy. |
Who may qualify
- Ages
- 4 weeks to 17 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
- Study population
- Since no validated or universally accepted diagnostic criteria currently exist for MAS occurring in the context of KD, the diagnosis of MAS will be based on the clinical judgment of the treating physician. To minimize the risk of misdiagnosis, all cases will be independently and critically reviewed by three experienced pediatric rheumatologists. Control groups will consist of age- and sex-matched patients, divided as follows: * Patients with KD resistant to first-line therapy * Patients with KD responsive to first-line therapy For each patient with KD complicated by MAS included in the study, 2 KD-responsive and 2 KD-resistant patients will be enrolled. The goal is to include at least 30 MAS-KD patients, along with 60 KD-resistant controls and 60 KD-responsive controls.
Key inclusion criteria (3 listed)
- Age between 4 weeks and under 18 years at the time of KD diagnosis
- Diagnosis of KD made according to the 2024 AHA guidelines
- Diagnosis of MAS made by the attending physician within 30 days from the onset of KD.
Key exclusion criteria (4 listed)
- Unconfirmed diagnosis of KD (e.g., mimicking conditions)
- Primary (genetic) HLH
- Lack of informed consent
- MAS diagnosed more than 30 days after or more than 15 days before the onset of KD
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
MAS-KD population
Time frame: From the study initiation date onward for 36 months
Definition of the proportion of patients with KD who develop MAS in the study population.
Clinical and laboratory features of MAS KD patients
Time frame: From the study initiation date onward for 36 months
Adjusted Odds ratios of clinical and laboratory risk factors
Applicability and diagnostic performance of currently available MAS classification criteria
Time frame: From the study initiation date onward for 36 months
Evaluation of diagnostic performance metrics (sensitivity, specificity, positive predictive value, negative predictive value) of existing MAS criteria when applied to KD patients.
6 secondary outcomes
Heterogeneity of MAS KD population
Time frame: From the study initiation date onward for 36 months
Applicability and diagnostic performance of currently available MAS classification criteria
Time frame: From the study initiation date onward for 36 months
MAS diagnosis
Time frame: From the study initiation date onward for 36 months
Treatment and clinical response of MAS KD patients
Time frame: From the study initiation date onward for 36 months
Heterogeneity of MAS KD population
Time frame: From the study initiation date onward for 36 months
Heterogeneity of MAS KD population
Time frame: From the study initiation date onward for 36 months
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 25 Mar 2026.
- Individual participant dataSharing planned: No
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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.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Kawasaki Disease and Macrophage Activation Syndrome (MAS), run by Meyer Children's Hospital IRCCS at 1 site in Italy.
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 3 inclusion and 4 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: opening soon, as of the sponsor's last update on 25 Mar 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Italy. 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?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Teresa Giani, MD (+390555662924, teresa.giani@meyer.it), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
MAS-KD population (time frame: From the study initiation date onward for 36 months). The study lists 3 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 25 Mar 2026.