Kawasaki disease is a small-medium vessel vasculitis that commonly affects children. It is a clinical diagnosis.
Stages of Kawasaki disease
| Stage | Description |
|---|---|
| Acute febrile stage | Fever abates. There is also desquamation of the digits, thrombocytosis, and development of coronary artery aneurysms |
| Subacute stage | Involves monitoring and follow-up, particularly in patients who develop cardiac complications |
| Convalescent phase | Clinical manifestatin resolve at around 3 months. Beau lines become more apparent, and small coronary artery aneurysms may resolve |
| Chronic stage | Involves monitoring and follow-up; particularly in patients who develop cardiac complications |
Diagnostic criteria of Kawasaki disease (AHA)
- Changes in peripheral extremities: Initial erythema of palms and soles → desquamation and Beau lines
- Polymorphic rash: non-vesicular and generalized (may be limited to groin and lower limbs)
- Oropharyngeal changes: erythemal; fissuring and crusting of the lips; strawberry tongue; diffuse mucosal injection of the oropharynx
- Bilatera, non-exudative, painless bulbar conjunctival injection
- Acute purulent cervical lymphadenopathy: unilateral with a diameter of > 15 mm
- Risk factors
- Genetic predisposition
- Viral infection causes Kawasaki disease in genetically susceptible individuals
- Signs and symptoms
- High-grade fever lasting more than 5 days – it is characteristically resistant to antipyretics
- Conjunctival injection
- Bright red, cracked lips
- Strawberry tongue
- Cervical lymphadenopathy
- Red palms and soles, which desquamate
- Investigations
- Complete blood count
- Neutrophilia
- Normocytic anaemia
- Thombocytosis
- CRP an ESR
- Markedly raised
- Liver function tests
- Elevated transaminases
- Urinalysis
- Sterile pyria
- Blood culture to rule out sepsis or other infectious causes
- Echocardiography to detect coronary artery aneurysm – this is repeated at intervals
- Chest X-ray if there are respiratory symptoms or cardiac dysfunction
- Complete blood count
- Treatment
- High-dose aspirin
- Intravenous immunoglobulin (IVIG)
- Echocardiogram to screen for a coronary artery aneurysm
- Complications
- Cardiovascular
- Coronary artery aneurysm
- Coronary artery stenosis
- Coronary artery thrombosis
- Myocardial infarction
- Myocarditis
- Pericarditis and pericardial effusion
- Valvular (mitral) regurgitation
- Peripheral artery aneurysm
- Musculoskeletal
- Gastrointestinal
- Acute gallbladder hydrops
- Intestinal pseudo-obstruction
- Neurological
- Aseptic meningitis
- Facial nerve palsy
- Seizures
- Cerebral infarction
- Cardiovascular
