Kawasaki Disease



Kawasaki Disease


Susa Benseler



Introduction



  • Kawasaki disease (KD) is an inflammatory syndrome that causes a systemic vasculitis with predilection of the coronary arteries


  • Etiology unclear; however, a wide variety of infections is associated with development of KD in children


  • Primarily affects young children, 80% < 5 years


  • Japan has highest incidence


  • Most common cause of acquired heart disease in Western countries


  • Kawasaki’s is a potential complication/common pathway of multiple triggers/infections; should be considered in all children with prolonged fever


Diagnostic Criteria

Clinical diagnosis based on presence of fever for at least 5 days plus 4/5 criteria

Fever ≥ 5 days plus:



  • Conjunctival injection/nonpurulent conjunctivitis (red eyes)


  • Oral changes: red, cracked lips/oral erythema, prominent follicles of lip (strawberry tongue), diffuse redness of oral cavity


  • Cervical lymphadenopathy (> 1.5 cm in diameter)


  • Polymorphous rash


  • Swelling/redness of hands and feet

Infants < 1 year and older children > 9 years often do not meet all criteria


Clinical Presentation

Frequency of clinical features:



  • Fever in all patients, mean duration 6.5 days



  • < 5 days of fever: 11.8%


  • Oral changes in 94%


  • Conjunctivitis in 92%


  • Rash in 90%


  • Extremity changes in 77%


  • Cervical lymphadenopathy in 64%








Table 40.1 Definitions















Typical KD

Fever for at least 5 days plus 4/5 criteria


Atypical KD

Not fully meeting KD criteria (i.e., fever + 3/5 KD criteria), but evidence of coronary artery lesions on echocardiogram


Incomplete KD

Does not fully meet KD criteria

No echocardiogram data are implicated in this term


May also present with:



  • Cardiac: hypotension, tachycardia, myocarditis, pericarditis, pericardial effusion, arrhythmia, valvulitis (< 1%), mitral regurgitation


  • CNS: headaches, aseptic meningitis, acute encephalitis


  • LN: lymph node abscess/severe adenitis, generalized lymphadenopathy


  • Lungs: pneumonitis, pleuritis


  • Abdominal symptoms: gallbladder hydrops, noninfectious hepatitis, mesenteric vasculitis, ischemic strictures, pseudo-obstruction


  • MSK: arthritis, myositis


  • Systemic vasculitis (1%)

Associated features may be presenting symptom: risk of missing diagnosis

Consider Kawasaki disease in every child with ≥ 5 days of fever


Tags:
Jun 22, 2016 | Posted by in EMERGENCY MEDICINE | Comments Off on Kawasaki Disease

Full access? Get Clinical Tree
Get Clinical Tree app for offline access