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Atypical Kawasaki disease: an often missed diagnosis
K Boven1, E R De Graeff-Meeder, W Spliet
1Department of Immunology and Rheumatology, University Hospital for Children and Youth, Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
Insights
Atypical Kawasaki disease (KD) can present with coronary vasculitis and few other symptoms, delaying diagnosis. Early recognition of subtle signs is crucial for timely treatment and improved outcomes in pediatric vasculitis.
Area of Science:
- Cardiology
- Pediatrics
- Rheumatology
Background:
- Coronary vasculitis is a hallmark of Kawasaki disease (KD).
- Diagnosis of KD typically relies on specific clinical criteria.
- Atypical presentations of KD can pose diagnostic challenges.
Observation:
- Four patients presented with vasculitis affecting coronary and medium-sized arteries.
- These patients exhibited few of the classic clinical signs associated with Kawasaki disease.
- One patient developed a ruptured iliac artery aneurysm, while three succumbed to cardiac failure.
Findings:
- The study highlights cases of "atypical" Kawasaki disease where coronary vasculitis was present without typical symptoms.
- Delayed diagnosis occurred due to the lack of fulfilling standard clinical criteria for KD.
- The findings underscore the variability in KD presentation.
Implications:
- Early recognition of subtle clinical features is vital for prompt diagnosis of atypical KD.
- Timely intervention in pediatric vasculitis can potentially prevent severe complications.
- This research emphasizes the need to consider KD even in non-classic cases presenting with vasculitis.
Abstract:
We describe four patients with vasculitis of the coronary and other medium sized arteries. Three of them died as a consequence of cardiac failure and the fourth had a ruptured aneurysm of the left common iliac artery. Coronary vasculitis is pathognomonic for Kawasaki disease (KD), but our patients had few other signs of this disorder, suggesting so called atypical KD. Because the described patients lacked most of the clinical criteria, the diagnosis was delayed. We focus on other clinical features in these patients and stress the importance of early recognition and treatment.