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

Trial statusNot yet recruiting
Trial phaseNot listed
Trial typeObservational
Biological sexAll
Age4-17
SponsorMeyer Children's Hospital IRCCS

About this trial

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.

Eligibility criteria

Qualifiers

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.

Disqualifiers

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 design

Treatments tested in this trial

  • Not listed

Trial groups

150 Participants
are grouped into 3 trial groups