Kawasaki Disease Shock Syndrome

Kawasaki Disease Shock SyndromeEvaluating and managing the child in SHOCK is certainly challenging. Identifying it (ex, Cap Refill, Shock Index, Lactate level) is challenging. Treating it is also challenging (see, Epi over Dopa). The challenge of determining the cause, though, should not be underestimated. While Sepsis, Trauma (Abdominal, Chest, Aortic), PE, Tamponade, Myocarditis, Heart Failure, Intussusception, Adrenal Crisis all deserve a place on the list of differential diagnoses, there is another condition to keep in mind- Kawasaki Disease Shock Syndrome:

 

Kawasaki Disease: Quick Review

  • Kawasaki Disease is also called mucocutaneous lymph node syndrome.
  • It is is a SYSTEMIC vascular inflammatory disease.
  • It is characterized by:
    • Unexplained Fever lasting > 5 days with:
      • Bilateral, non-exudative conjunctivitis
      • Oral mucosa membrane changes (ex, strawberry tongue, red lips)
      • Cervical lymphadenopathy 
      • Skin Rash
      • Swelling of the hands and feet 
    • Desquamation can be noted as well.
    • Often clinical findings do not occur contemporaneously.
    • Can also present in an Incomplete manner.
  • Can develop significant cardiovascular complications.
    • Coronary artery aneurysms (CAA) may develop in 20-25% of untreated patients.
      • Can lead to occlusion of vessel and cardiac ischemia.
      • Can also lead to coronary artery rupture.
    • Appropriate treatment with IVIG and ASA can reduce incidence of CAA to 3-5%.

 

Kawasaki Disease Shock Syndrome

  • In 2009, a shock syndrome was attributed to Kawasaki Disease during its acute phase. [Kanegaye, 2009].
  • Hypotension and Shock defined by either:
  • The diagnosis of Kawasaki Disease is made using the typical clinical features, but recognition of it in a patient in shock can be difficult. [Ma, 2017]
    • Missed diagnosis and delayed diagnosis can occur. [Ma, 2017]
    • Can look similar to Toxic Shock Syndrome. [Lin, 2015; Yim, 2010]
    • Can also have incomplete presentation (just to complicate your day further) [Taddio, 2017]
      • Look for sterile pyuria, gallbladder hydrous, uveitis
      • May also be seen in kids < 6 months of age.
  • The cause is yet unclear, but may be multifactorial. [Ma, 2017]
    • Systemic inflammatory vasculitis leads to:
      • Increased vascular permeability
      • Hypoproteinemia (albumin leaked from capillary beds)
    • Myocardial damage, mitral regurgitation, and cardiac systolic dysfunction [Kanegaye, 2009]
    • Acute myocarditis can also occur.
  • The immune inflammatory response has been found to be more intense in patients with Kawasaki Disease Shock Syndrome compared to KD without shock. [Ma, 2017; Chen, 2013; Kanegaye, 2009]
    • Higher inflammatory markers.
    • Patients found to be more resistant to initial treatment of IVIG.
      • May require additional doses of IVIG
      • May require adjunctive therapy as well (ex, steroids).
      • While initial response is poor, therapy can resolve cardiovascular abnormalities. [Taddio, 2016]

 

Red Flags for Kawasaki Disease Shock Syndrome

  • History and Physical Exam:
    • Kawasaki Disease occurring in older children
    • Prolonged fever duration (when compared to KD without shock)
  • Laboratory Studies
    • Higher levels of inflammatory markers (ex, CRP, PCT) – 3 fold higher!
    • Lower albumin levels
    • Anemia
    • Consumptive coagulopathy
    • Bandemia
    • Hyponatremia [Schuster, 2017]
  • Clinical Findings
    • More severe skin rash
    • Myocardial dysfunction (ex, higher BNP and Troponin I levels, arrhythmias)
    • More severe coronary artery involvement (Echo will be helpful!)
    • Poor response to IVIG (more than 50% do not respond initially)

 

Moral of the Morsel

  • Remain vigilant! Not all hypotension is from bacterial infections.
  • Look at the entire picture. Put the pieces of the puzzle together. Does it add up to sepsis… or something else?
  • Kawasaki Disease just augmented its game! Put Kawasaki Disease on your list of items that lead to a child being critically ill and hypotensive!
  • Take a look! Echocardiogram may help distinguish Kawasaki Disease Shock Syndrome from Toxic Shock Syndrome.

 

References

Taddio A1,2, Rossi ED3, Monasta L4, Pastore S4, Tommasini A4, Lepore L3, Bronzetti G5, Marrani E6, Mottolese BD4, Simonini G6, Cimaz R6, Ventura A3,4. Describing Kawasaki shock syndrome: results from a retrospective study and literature review. Clin Rheumatol. 2017 Jan;36(1):223-228. PMID: 27230223. [PubMed] [Read by QxMD]

Schuster JE1, Palac HL2, Innocentini N3, Rowley AH3, Young LT3, Shulman ST3. Hyponatremia Is a Feature of Kawasaki Disease Shock Syndrome: A Case-Control Study. J Pediatric Infect Dis Soc. 2017 Feb 8. PMID: 28177492. [PubMed] [Read by QxMD]

Ma L1, Zhang YY1, Yu HG1. Clinical Manifestations of Kawasaki Disease Shock Syndrome. Clin Pediatr (Phila). 2017 Sep 1:9922817729483. PMID: 28905639. [PubMed] [Read by QxMD]

Yang HF1, Chen WL, Chang CN, Chen SJ, Fan HC. Kawasaki disease shock syndrome: case report. Paediatr Int Child Health. 2016 Feb;36(1):76-8. PMID: 25695906. [PubMed] [Read by QxMD]

Chen PS1, Chi H2, Huang FY1, Peng CC3, Chen MR3, Chiu NC4. Clinical manifestations of Kawasaki disease shock syndrome: a case-control study. J Microbiol Immunol Infect. 2015 Feb;48(1):43-50. PMID: 23927822. [PubMed] [Read by QxMD]

Lin YJ1, Cheng MC, Lo MH, Chien SJ. Early Differentiation of Kawasaki Disease Shock Syndrome and Toxic Shock Syndrome in a Pediatric Intensive Care Unit. Pediatr Infect Dis J. 2015 Nov;34(11):1163-7. PMID: 26222065. [PubMed] [Read by QxMD]

Gatterre P1, Oualha M, Dupic L, Iserin F, Bodemer C, Lesage F, Hubert P. Kawasaki disease: an unexpected etiology of shock and multiple organ dysfunction syndrome. Intensive Care Med. 2012 May;38(5):872-8. PMID: 22273753. [PubMed] [Read by QxMD]

Yim D1, Ramsay J, Kothari D, Burgner D. Coronary artery dilatation in toxic shock-like syndrome: the Kawasaki disease shock syndrome. Pediatr Cardiol. 2010 Nov;31(8):1232-5. PMID: 20706709. [PubMed] [Read by QxMD]

Kanegaye JT1, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, Watson VE, Best BM, Burns JC. Recognition of a Kawasaki disease shock syndrome. Pediatrics. 2009 May;123(5):e783-9. PMID: 19403470. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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3 Responses

  1. Cindy says:

    thank you, so impressed!

  2. Matthew says:

    Dr Fox,

    What are your thoughts on IVCS as a first line medication in the setting of suspected kawasaki shock? A meta-analysis performed in 2012 demonstrated improvement in outcomes clinical course without alteration in the incidence of coronary artery aneurysms. The analysis also included studies where CS were used in lieu of IVIG and no inferiority was demonstrated (though that may just be a lack of power or a limitation in study design). We have recently begun using more IVCS as adjunctive treatment inpatient and, in fact, have debated whether this should be a matter of general practice (which some may find interesting as, in the past, it was believe that CS might be deleterious in this disease process). Given the CV stability benefits offered by corticosteroids in the setting of shock do you think that it is within reason to make this one of the first supportive treatment administered to these patients? (additionally there is anecdotal evidence that patients presenting with shock syndrome are, in general, more likely to be recalcitrant to IVIG only treatment)

    https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3284666/

    • Sean Fox says:

      Dr. Coghill,
      Thank you for your response and insight. Yes, as you report, patients with KD Shock Syndrome are more likely to be recalcitrant to first line therapies and, thus, require adjunct therapies. Whether I would list corticosteroids as “first line” is another question, as there is currently not enough data to definitively advocate for that status… BUT… that being said, I don’t think that it is unreasonable. In the end, these kids can be very sick and are often misdiagnosed and have delayed therapies delivered, so the mere consideration of KD Shock Syndrome on a Ddx I believe is an advancement!

      Thank you,
      sean

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