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

NCT07491926 | Not Yet Recruiting

• 1 other identifier: MASKd
• Study Type: observational
• Target: 150
• Locations: 1 country
• Sites: 1

Brief 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 (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.

Conditions

Kawasaki Disease; Macrophage Activation Syndrome (MAS)

Outcome Measures

Primary Outcomes (3)

• MAS-KD population

  Definition of the proportion of patients with KD who develop MAS in the study population.

  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

  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.

  From the study initiation date onward for 36 months

Secondary Outcomes (6)

• Heterogeneity of MAS KD population

  From the study initiation date onward for 36 months

• Applicability and diagnostic performance of currently available MAS classification criteria

  From the study initiation date onward for 36 months

• MAS diagnosis

  From the study initiation date onward for 36 months

• Treatment and clinical response of MAS KD patients

  From the study initiation date onward for 36 months

• Heterogeneity of MAS KD population

  From the study initiation date onward for 36 months

Study Arms (3)

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.

Eligibility Criteria

• Age: 4 Weeks - 17 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17)
• Sampling Method: Non-Probability Sample

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.

You may qualify if:

• 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.

You may not qualify if:

• 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

Sponsors & Collaborators

• Meyer Children's Hospital IRCCS: lead

Study Sites (1)

Azienda Ospedaliero-Universitaria IRCCS Meyer

Florence, Italy, 50139, Italy

Location

MeSH Terms

Conditions

Mucocutaneous Lymph Node Syndrome; Macrophage Activation Syndrome

Condition Hierarchy (Ancestors)

Vasculitis; Vascular Diseases; Cardiovascular Diseases; Lymphatic Diseases; Hemic and Lymphatic Diseases; Skin Diseases, Vascular; Skin Diseases; Skin and Connective Tissue Diseases; Lymphoproliferative Disorders; Immunoproliferative Disorders; Immune System Diseases

Central Study Contacts

Teresa Giani, MD

CONTACT

+390555662924; teresa.giani@meyer.it

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: OTHER
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: MD

Study Record Dates

• First Submitted: January 20, 2026
• First Posted: March 25, 2026
• Study Start: February 1, 2026
• Primary Completion (Estimated): November 1, 2027
• Study Completion (Estimated): February 1, 2029
• Last Updated: March 25, 2026

Record last verified: 2026-03

Data Sharing

• IPD Sharing: Will not share

Locations

IT (1)