From the Guidelines
The etiology of Kawasaki disease remains unknown, but current evidence suggests it is likely the result of an abnormal immune response to an infectious trigger in genetically susceptible individuals. The disease is characterized by a self-limited, generally nonrecurring illness that manifests itself by fever, rash, enanthem, conjunctival injection, and cervical adenopathy, which fits well with an infectious etiology or trigger 1. Several infectious agents have been proposed as potential triggers, including viruses and bacteria, but no single pathogen has been definitively identified.
Key Factors in Etiology
- Genetic factors likely play a role in susceptibility, as evidenced by higher rates among Asian populations, particularly Japanese children, and familial clustering in some cases 1.
- Environmental factors may also contribute, as suggested by seasonal patterns of disease occurrence with peaks in winter and spring in many regions 1.
- The immune-mediated vasculitis characteristic of Kawasaki disease leads to the classic clinical manifestations including fever, rash, lymphadenopathy, and cardiac complications, particularly coronary artery aneurysms 1.
Evidence Summary
The American Heart Association guidelines suggest that Kawasaki disease is an acute vasculitis of childhood that predominantly affects the coronary arteries, with an unknown etiology but a suspected infectious agent and genetic predisposition 1. The guidelines also highlight the importance of clinical criteria and supporting ancillary studies in diagnosis, as well as the potential for incomplete or atypical presentations of the disease 1.
Clinical Implications
Understanding the etiology of Kawasaki disease is crucial for developing effective diagnostic and treatment strategies, particularly given the potential for cardiac complications and the fact that it is the leading cause of acquired heart disease among children in developed countries 1. Further research is needed to elucidate the exact mechanisms underlying the disease and to identify potential infectious triggers and genetic susceptibility factors.
From the Research
Etiology of Kawasaki Disease
The etiology of Kawasaki disease is still largely unknown, but various studies suggest that it may be triggered by an infectious agent 2, 3.
- The exact cause of the disease remains unidentified, despite extensive research and the use of increasingly sensitive molecular methods for identifying microbial nucleic acids and proteins in tissue samples 2.
- Epidemiologic and clinical observations suggest that one or more infectious agents may trigger the disease, but no links between any individual agents and the disease have been established irrefutably 4, 3.
- The disease is characterized as an acute febrile illness and systemic vasculitis of unknown aetiology, predominantly affecting young children and causing coronary artery aneurysms 5.
- Various infectious agents, including bacterial, viral, and Rickettsial organisms, have been implicated as potential causes, but the evidence is not conclusive 3.
- The development of Kawasaki disease is likely to be multifactorial in nature, requiring certain genetic and immunological factors, and possibly a vector 3.
Research Goals and Challenges
- Identifying the etiology of Kawasaki disease remains the single most important research goal in the field, as it would improve diagnosis, treatment, and prevention of the disease 2.
- The lack of specific diagnostic tests and biomarkers for Kawasaki disease makes early diagnosis and treatment challenging 5.
- Experimental mouse models of Kawasaki disease vasculitis have improved our understanding of the pathology of the disease and helped characterize the cellular and molecular immune mechanisms contributing to cardiovascular complications 5.