Complete & Incomplete Kawasaki Disease: Two sides of the same coin Journal Club Presentation Mekhala Ayya ST3 Paediatrics

Reasons for choosing this topic?? Multiple recent cases Having a Rheumatologist as an Educational Supervisor Parents asking me instead of the Rheumatology team

Kawasaki Disease ( Complete) Diagnosis based on fulfilment of set criteria At least 4 out of 5 clinical signs: (a)Bilateral conjunctival injection (b)Cervical Lymphadenopathy (c) Polymorphous rash (d) Changes to oral mucosa or lips (e) Changes in distal extremities

Kawasaki Disease (Complete) Associated Laboratory features: CRP ↑ ESR↑ Platelets ↑ WCC ↑ Albumin↓

Kawasaki Disease ( Incomplete) Interchangeable term with Atypical Variable definitions Mainly describes children who have complications such as coronary aneurysms in the absence of “ full clinical spectrum” May have lab/ image findings without clinical signs

Kawasaki Disease ( Incomplete) Occurs at the extremes of age < 1year of age >5- 9 years of age Laboratory findings: Albumin ↓ Anaemia ALT ↑ WCC ↑ Platelets ↑ Urine WBC ↑

Primary Outcomes Comparison of clinical findings Treatment response Increased risk of cardiac complications for those with incomplete KD

Methods Conducted between January 1990 – April 2007, Toronto, Canada All patients with diagnosed Kawasaki assessed Retrospective Didn’t need ethical approval as retrospective study Excluded patients diagnosed with KD pre-transfer

Methods- Classification Complete KD: 4 clinical signs Fever for at least 5 days Incomplete KD: 2 or 3 clinical signs Fever All patients received standardised treatment and follow up

Methods- Data Collected Clinical features Additional clinical findings Patient demographics Laboratory results Z-scores for RCA/ LCA/ LAD Aneurysms

Results Total 1374 KD cases 98 excluded due to insufficient clinical details 321 excluded as they were diagnosed pre-transfer 955 KD cases included Complete KD – 738 Incomplete KD- 217 ( 53 with 2 symptoms & 164 with 3 symptoms)

Table 1 from Study

Table 2& 3

Table 4

Limitations of study Conducted over a prolonged period Changes in the diagnostic criteria/ clinical features – incomplete KD mean that some patients may have been missed Not including those diagnosed pre-transfer (? More Incomplete KD)

Conclusions - Incomplete Cardiac outcomes are similar Patients at extremes of age Delay to diagnose – 1 day Less likely to be treated < 10 days after onset of illness Less likely to get IVIG but similar number of patients received Aspirin Similar length of hospital stays +ve correlation between Age ( < 1 yr) and maximum coronary z-score Do we need a more clear diagnostic criteria for Incomplete KD ?

CASP Tool Did the study address a clearly focused issue? YES Was the cohort recruited in an acceptable way? YES Was the exposure accurately measured to minimise bias? YES Was the outcome accurately measured to minimise bias? YES Have the authors identified all important confounding factors? NO ( Not all KD patients in that period were included in the study ie. The transfer patients) Have they taken into account confounding factors in design? YES Was the follow up of subjects complete enough? YES Was the follow up of subjects long enough? CAN’T TELL Do I believe the results? YES Can the results be applied locally? YES Do the study results fit with other available evidence? YES

Thank you