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Kawasaki Disease and Coronary Complications
Research Guide
What is Kawasaki Disease and Coronary Complications?
Kawasaki disease and coronary complications refer to an acute vasculitis of childhood that leads to coronary artery aneurysms in approximately 25% of untreated cases and is the leading cause of acquired heart disease in children in developed countries.
Kawasaki disease presents with fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy, with coronary artery aneurysms or ectasia developing in about 25% of untreated patients. The field encompasses 28,372 papers focused on diagnosis, treatment, long-term management, epidemiological features, immunological aspects, and associations with multisystem inflammatory syndrome and SARS-CoV-2-related pediatric inflammatory syndrome. Treatment guidelines emphasize intravenous immunoglobulin to reduce coronary complications.
Topic Hierarchy
Research Sub-Topics
Intravenous Immunoglobulin Resistance in Kawasaki Disease
This sub-topic analyzes predictors, genetic factors, and alternative therapies for patients unresponsive to initial IVIG treatment. Researchers conduct cohort studies on adjunct corticosteroids and novel biologics like infliximab.
Coronary Artery Lesions Long-term Management
Focuses on imaging surveillance, endothelial function assessment, and risk stratification for aneurysms post-Kawasaki disease. Longitudinal studies track cardiovascular events into adulthood using echocardiography and MRI.
Immunological Pathogenesis of Kawasaki Disease
Investigates cytokine profiles, T-cell activation, and genetic polymorphisms driving vascular inflammation. Research integrates omics data to identify biomarkers and therapeutic targets.
Multisystem Inflammatory Syndrome in Children (MIS-C)
This area studies clinical phenotypes, SARS-CoV-2 antibody correlations, and outcomes of MIS-C resembling Kawasaki disease. Cohort analyses compare treatment responses and cardiac sequelae.
Epidemiological Trends in Kawasaki Disease Incidence
Examines seasonal patterns, geographic variations, and temporal changes in incidence pre- and post-COVID-19. Studies use registries to assess environmental and infectious triggers.
Why It Matters
Kawasaki disease causes coronary artery abnormalities, making it the leading cause of acquired heart disease in children in developed countries, with aneurysms occurring in ≈25% of untreated cases. McCrindle et al. (2017) in "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association" provide revised guidelines for diagnosis and intravenous immunoglobulin treatment within 10 days of fever onset, reducing aneurysm risk to 5%. Newburger et al. (2004) in "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease" established early protocols that lowered coronary ectasia rates from 25% to under 5% with prompt therapy. Recent papers link SARS-CoV-2 to Kawasaki-like multisystem inflammatory syndrome, as in Verdoni et al. (2020) reporting severe cases at the Italian epicenter with 30 children showing cardiac involvement, highlighting needs for updated pediatric protocols during pandemics.
Reading Guide
Where to Start
"Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association" by McCrindle et al. (2017) provides the foundational AHA guidelines on diagnosis, treatment, and management, including coronary risk stratification, making it the ideal starting point for understanding clinical practice.
Key Papers Explained
Newburger et al. (2004) in "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease" established core diagnostic criteria and IVIG protocols reducing aneurysms to under 5%. McCrindle et al. (2017) in "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association" revises and expands these with updated risk scores and long-term surveillance. Verdoni et al. (2020) and Whittaker et al. (2020) connect classic Kawasaki disease to SARS-CoV-2-associated syndromes, showing overlapping coronary aneurysm presentations in pandemic contexts.
Paper Timeline
Most-cited paper highlighted in red. Papers ordered chronologically.
Advanced Directions
Recent papers emphasize SARS-CoV-2 links to Kawasaki-like disease with cardiac shock, as in Verdoni et al. (2020) and Feldstein et al. (2020), but no preprints from the last 6 months are available. Frontiers involve inflammasome roles in vasculitis from Brož and Dixit (2016) and Lamkanfi and Dixit (2014), informing potential immunomodulatory therapies.
Papers at a Glance
| # | Paper | Year | Venue | Citations | Open Access |
|---|---|---|---|---|---|
| 1 | COVID-19: consider cytokine storm syndromes and immunosuppression | 2020 | The Lancet | 10.0K | ✓ |
| 2 | Diagnosis, Treatment, and Long-Term Management of Kawasaki Dis... | 2017 | Circulation | 3.5K | ✕ |
| 3 | Inflammasomes: mechanism of assembly, regulation and signalling | 2016 | Nature reviews. Immuno... | 3.3K | ✕ |
| 4 | Multisystem Inflammatory Syndrome in U.S. Children and Adolesc... | 2020 | New England Journal of... | 2.6K | ✓ |
| 5 | Hyperinflammatory shock in children during COVID-19 pandemic | 2020 | The Lancet | 2.5K | ✓ |
| 6 | An outbreak of severe Kawasaki-like disease at the Italian epi... | 2020 | The Lancet | 2.5K | ✓ |
| 7 | Mechanisms and Functions of Inflammasomes | 2014 | Cell | 2.4K | ✓ |
| 8 | Clinical Characteristics of 58 Children With a Pediatric Infla... | 2020 | JAMA | 2.0K | ✓ |
| 9 | Juvenile idiopathic arthritis | 2007 | The Lancet | 2.0K | ✕ |
| 10 | Diagnosis, Treatment, and Long-Term Management of Kawasaki Dis... | 2004 | Circulation | 1.9K | ✓ |
Frequently Asked Questions
What are the diagnostic criteria for Kawasaki disease?
Kawasaki disease diagnosis requires fever lasting at least 5 days plus four of five principal features: bilateral nonexudative conjunctivitis, oral mucosal changes, peripheral extremity changes, polymorphous rash, and cervical lymphadenopathy. Newburger et al. (2004) in "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease" note that incomplete cases with fewer features require supplementary lab and echocardiographic findings. Coronary artery involvement guides urgent evaluation.
How does intravenous immunoglobulin treat Kawasaki disease?
Intravenous immunoglobulin reduces coronary artery aneurysm formation from ≈25% in untreated cases to 5% when given within 10 days of fever onset. McCrindle et al. (2017) in "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association" recommend a single 2 g/kg dose plus aspirin. This therapy targets vasculitis and inflammation effectively.
What coronary complications arise in Kawasaki disease?
Coronary artery aneurysms or ectasia develop in ∼25% of untreated Kawasaki disease patients, leading to acquired heart disease. Whittaker et al. (2020) in "Clinical Characteristics of 58 Children With a Pediatric Inflammatory Multisystem Syndrome Temporally Associated With SARS-CoV-2" observed coronary artery aneurysms in cases resembling Kawasaki disease. Long-term management involves echocardiographic surveillance per AHA guidelines.
How is Kawasaki disease linked to SARS-CoV-2?
SARS-CoV-2 infection associates with multisystem inflammatory syndrome in children featuring Kawasaki-like symptoms including coronary abnormalities. Verdoni et al. (2020) in "An outbreak of severe Kawasaki-like disease at the Italian epicentre of the SARS-CoV-2 epidemic: an observational cohort study" described 30 cases with shock and cardiac involvement post-SARS-CoV-2. Feldstein et al. (2020) in "Multisystem Inflammatory Syndrome in U.S. Children and Adolescents" reported serious illness in previously healthy children.
What is the current state of Kawasaki disease treatment guidelines?
AHA scientific statements outline diagnosis, acute treatment with IVIG and aspirin, and long-term coronary monitoring based on risk levels. McCrindle et al. (2017) updated prior guidelines from Newburger et al. (2004), emphasizing early intervention to prevent aneurysms. Risk scores predict IVIG resistance for intensified therapy.
Open Research Questions
- ? What immunological mechanisms drive coronary artery aneurysm formation in Kawasaki disease?
- ? How does SARS-CoV-2 trigger Kawasaki-like multisystem inflammatory syndrome, and what differentiates it from classic Kawasaki disease?
- ? Which patients with Kawasaki disease develop IVIG resistance, and what alternative therapies prevent coronary complications?
- ? What long-term cardiovascular risks persist in adults after childhood Kawasaki disease with aneurysms?
- ? How do genetic factors influence susceptibility to coronary abnormalities in Kawasaki disease?
Recent Trends
The field has 28,372 papers with no reported 5-year growth rate.
High-citation works from 2020, such as Verdoni et al. with 2454 citations on SARS-CoV-2-linked Kawasaki-like outbreaks and Feldstein et al. (2020) with 2625 citations on U.S. multisystem inflammatory syndrome, highlight pandemic-era shifts toward viral triggers of coronary complications.
2020No recent preprints or news from the last 12 months available.
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