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NCT Number: NCT07491926

MASKd: a Study on Kawasaki Disease (KD) Complicated by Macrophage Activation Syndrome (MAS)

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.

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Key information

Conditions

Kawasaki Disease Cardiovascular Diseases Hemic and Lymphatic Diseases Immune System Diseases Immunoproliferative Disorders Lymphatic Diseases Lymphoproliferative Disorders Macrophage Activation Syndrome Macrophage Activation Syndrome (MAS) Mucocutaneous Lymph Node Syndrome Skin Diseases Skin Diseases, Vascular Skin and Connective Tissue Diseases Vascular Diseases Vasculitis

Age range

4 week–17 year

Sex eligibility

All sexes

Study type

Observational

Primary location

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • 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.

Exclusion criteria

  • 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

Treatment and study plan

Primary outcomes

  1. 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.

  2. 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

  3. 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.

Secondary outcomes

  1. Heterogeneity of MAS KD population

    Time frame: From the study initiation date onward for 36 months

    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.

  2. 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.

  3. MAS diagnosis

    Time frame: From the study initiation date onward for 36 months

    Time from KD onset to MAS diagnosis and its correlation with clinical outcomes;

  4. Treatment and clinical response of MAS KD patients

    Time frame: From the study initiation date onward for 36 months

    Description of treatments used for MAS (e.g., corticosteroids, IVIG, biologics) and the corresponding clinical response.

  5. Heterogeneity of MAS KD population

    Time frame: From the study initiation date onward for 36 months

    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.

  6. Heterogeneity of MAS KD population

    Time frame: From the study initiation date onward for 36 months

    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.

Study contacts

Contact information is provided by the study sponsor or research team.

Teresa Giani, MD

CONTACT

[email protected]

+390555662924

Sponsors and collaborators

Lead sponsor

Meyer Children's Hospital IRCCS

Other

Registry information

Official study title

MACROPHAGE ACTIVATION SYNDROME IN KAWASAKI DISEASE: Features, Treatment, Outcome, Predictive and Diagnostic Factors (The MASKd Study)

Important dates

Study start
2026
Primary completion
2027
Study completion
2029
First posted
Mar 25, 2026
Registry last updated
Mar 25, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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