febrile

febrile

IDDI Locked Key
Subscribe

Febrile #96 – Riddle Me This

96 Cover Art OPT

Summary

Drs. Jack Flores and Madan Kumar chat about the nuances of Kawasaki disease

Table of Contents

Credits

Host(s): Jack Flores, Sara Dong

Guests: Madan Kumar

Writing: Jack Flores

Content Editing, Cover Art: Sara Dong

Audio Editing: Bentley Brown

Produced by Sara Dong with support from the Infectious Diseases Society of America (IDSA)

Our Guests

John “Jack” Flores, MD

Dr. John “Jack” Flores is a third year Med-Peds ID fellow at the University of Chicago who is passionate about all infectious diseases, but sees himself as an academic MedPeds ID provider focusing on adolescent/young adult and maternal-fetal infections along with HIV syndemic care and travel medicine.

Twitter

Madan Kumar, DO

Dr. Madan Kumar is a pediatric infectious diseases specialist and Assistant Professor in Pediatrics at the University of Chicago’s Comer Children’s Hospital. His various scholarly focuses are with immunocompromised hosts, the intersection of infectious diseases and immune dysregulation, and the study of the microbiome.

Culture

Madan is an amateur woodworker and has recently made some furniture and basic wooden toys

 

Jack shared his dabbling in riddle-making!  Check it out below:

I can be a tree, a bridge, a lily pond, a battle, or a shelf,
Pieces of fruit, a chair, a woman, or even God himself,
These are just a few of the things that often make me sublime,
I’m simply a snapshot of someone’s emotions and perceptions of their world at that time,
Most of the time I’m free, but occasionally I’ll be a pretty price to pay
You can find me almost anywhere, from Dublin to New York, Paris to Mumbai

Solution: A painting!

Consult Notes

Case Summary

2 year old boy who presents with fever and was diagnosed with Kawasaki disease

Key Points

Resources related to KD:

This episode focused on Kawasaki disease (KD)! Let’s start with the definition and clinical manifestations

  • The diagnosis of KD is made with the following definition:  Fever for ≥5 days in addition to the presence of 4 of the following 5 clinical criteria:
      • Bilateral injection of bulbar conjunctivae with limbic sparing and without exudate
      • Erythematous mouth and pharynx, strawberry tongue, and red, cracked lips
      • A polymorphous, generalized, erythematous rash, often with accentuation in the groin (which can be morbilliform, maculopapular, scarlatiniform, or erythema multiforme-like)
      • Changes in the peripheral extremities consisting of erythema of the palms and soles and firm, sometimes painful, induration of the hands and feet, often with periungual desquamation usually beginning 10-14d after fever onset
      • Acute nonsuppurative (usually unilateral) anterior cervical lymphadenopathy with at least 1 node ≥1.5 cm in diameter
  • Madan discussed how remembering that this is a vasculitis can assist you in understanding what physical changes might manifest (rather than just memorizing the clinical criteria)!  The clinical features reflect inflammation of medium-sized muscular arteries
  • KD should be considered even before that 5th day of fever if several of the primary clinical criteria are present without an alternative explanation
      • The clinical manifestations may appear and self-resolve at different times (rather than all being present simultaneously) → make sure to ask about prior presence of relevant manifestations when you see a child for persistent fever
      • KD diagnosis is sometimes delayed, especially when fever and unilateral neck swelling is thought to be bacterial lymphadenitis or para- or retropharyngeal infection (a useful distinguishing feature would be that suppuration generally is NOT observed in KD)
      • Another common consideration in the post-COVID era is the possibility of MIS-C vs KD (remember that children with MIS-C often have a wider spectrum of symptoms, are typically older, and show greater elevation of inflammatory markers)
  • Coronary artery abnormalities are serious sequelae of Kawasaki disease, occurring in 20% to 25% of untreated children.  If coronary artery aneurysm or ectasia is evident (z score ≥2.5) in any patient evaluated for fever, a presumptive diagnosis of KD should be made
      • A normal early echo is typical and does not exclude the diagnosis but may be useful in evaluation of patients with suspected incomplete Kawasaki disease
      • In one study, 80% of patients with Kawasaki disease who ultimately developed coronary artery disease had abnormalities (z score ≥2.5) on an echocardiogram obtained during the first 10 days of illness. 
      • Increased risk of developing coronary artery abnormalities is associated with male sex; age <12 months or >8 years; fever for more than 10 days; white blood cell count >15 000/mm3; high relative neutrophil (>80%) and band count; low hemoglobin concentration (<10 g/dL); hypoalbuminemia, hyponatremia, or thrombocytopenia; and fever persisting or recurring >36 hours after completion of Immune Globulin Intravenous (IGIV) administration. 
      • Aneurysms of the coronary arteries most typically occur between 1 and 4 weeks after onset of illness; onset later than 6 weeks is extremely uncommon 
      • In children with only mild coronary artery dilation, coronary artery dimensions often return to baseline within 6-8 wks after onset of disease
  • Other notes on clinical features and diagnosis:
      • Madan and Jack discussed concurrent viral upper respiratory infections, which are sometimes present in patients with KD, should not delay diagnosis and treatment of KD
          • One possible exception is that in a patient with fever, exudative conjunctivitis, and exudative pharyngitis with +Adenovirus, it’s like that the syndrome represents Adenovirus (and that KD is unlikely)
      • There is no specific diagnostic test for KD.  Madan also discussed how you need to consider the clinical criteria in context of your patient → sometimes this might mean not following strict criteria and considering the diagnosis still in children that might look pathophysiologically like they have KD (that may benefit from treatment).  See more on incomplete KD below too

What about incomplete Kawasaki disease? Sometimes previously called “atypical” KD

Considering epidemiology of Kawasaki disease

What causes Kawasaki disease?

Management of Kawasaki disease

  • The goal of treatment during the acute phase is to decrease inflammation of the myocardium and coronary artery wall and provide supportive care → after the acute phase, therapy is directed at preventing coronary artery thrombosis
  • Primary treatment includes a single dose of IVIG 2g/kg, administered over 10-12 hrs, and aspirin
      • This typically results in rapid resolution of fever and lab changes in ~85% of patients
      • IVIG given within 10d of fever onset reduces the risk of coronary artery aneurysms from 17-25% down to <5%
      • IVIG is most effective if given within 7-10d of illness but still given after 10d in patients with persistent fever, ongoing signs of systemic inflammation, and/or coronary artery aneurysms
      • The mechanism is not totally clear
  • Aspirin is used for its anti-inflammatory and antithrombotic activity, although aspirin alone does not decrease the risk of coronary artery abnormalities.  There is variability in dosing of aspirin, as discussed on the show:
      • Some use 30-50 mg/kg/day (in 4 divided doses) while others use 80-100 mg/kg/day (in 4 divided doses) → There is little data to suggest which aspirin dose is superior
      • The lower dose of 30-50 mg/kg/d is recommended by AHA and AAP given higher doses have increased potential for adverse effects without confirmed benefits
      • Aspirin is held if there is a contraindication including infection with varicella or influenza

Platt B, Belarski E, Manaloor J, et al. Comparison of Risk of Recrudescent Fever in Children With Kawasaki Disease Treated With Intravenous Immunoglobulin and Low-Dose vs High-Dose Aspirin. JAMA Netw Open. 2020;3(1):e1918565. Published 2020 Jan 3. doi:10.1001/jamanetworkopen.2019.18565

A few additional follow-up considerations

  • Echocardiography should be performed at the time of suspected diagnosis and repeated at 2 weeks and 6 to 8 weeks after diagnosis in children with normal coronary arteries on initial evaluation. 
  • Long-term management and cardiac care of a child with KD will be based on extent of coronary artery involvement (which will be coordinated with your friendly neighborhood pediatric cardiologist!)
    • Development of giant coronary artery aneurysms (luminal diameter ≥8 mm or larger in a child, but smaller diameter in an infant based on relative body surface area, z score ≥10) usually requires addition of anticoagulant therapy, such as warfarin or low-molecular weight heparin, to prevent thrombosis. 
  • The average duration of fever in untreated Kawasaki disease is 10 days; however, fever can last 2 weeks or longer. 
    • After fever resolves, patients can remain anorexic and/or irritable with decreased energy for 2 to 3 weeks. 
    • During this phase, desquamation of fingers, toes, hands, and feet may occur. Transverse lines across the nails (Beau’s lines) sometimes are noted month(s) later. 
  • Recurrent disease develops in approximately 1-2% of children in the US at a median of 1.5 years after  index episode
  • Reminder/Pearl: Measles- and varicella-containing vaccines (MMRV) should be deferred until 11 months after receipt of IGIV, 2 g/kg, for treatment of Kawasaki disease because of possible interference with development of an adequate immune response

A few other references

Episode Art & Infographics

Infographics

Find all the Febrile infographics here!

Goal

Listeners will be able to explain the clinical presentation, diagnosis, and management of Kawasaki disease

Learning Objectives

After listening to this episode, listeners will be able to:

  • Recognize the clinical presentation of KD along with other diseases that may be on the differential
  • Discuss the use of diagnostic clinical criteria for KD and incomplete KD
  • Identify the importance of prompt IVIG administration in KD, which can prevent coronary aneurysms

Disclosures

Our guests as well as Febrile podcast and hosts report no relevant financial disclosures

Citation

Flores, J., Kumar, M., Dong, S. “#96: Riddle Me This”. Febrile: A Cultured Podcast. https://player.captivate.fm/episode/5058f3c5-8b3b-4757-8413-b0798cde32b8

Transcript

Scroll to Top