CCC Diagnosing Kawasaki Disease Strawberries and Symptoms Discussion

Peer response to Adam

• A 10-year-old Asian patient presents with an erythematous maculopapular rash, conjunctivitis, a mild fever of 102.1 and a strawberry tongue. The rash started 4 days ago. How are you going to evaluate this patient? What is the differential diagnosis for this patient? Describe your treatment plan based on current guidelines.

The “strawberry tongue” noted in the prompt immediately draws one’s mind to Kawasaki disease (KD). This thought is then supported by the other information given. Diagnostic criteria for KD include fever for at least 5 days along with 4 out of 5 other criteria to include: 1) conjunctival injection without exudate, 2) oral or pharyngeal erythema or strawberry tongue, 3) erythema or edema in the feet and hands or periungual desquamation, 4) maculopapular rash, and 5) cervical lymphadenopathy (Gorelik et al., 2022). The patient’s Asian ancestry also makes KD more likely (Owens & Plewa, 2023). The patient has fever and 3 other diagnostic criteria, although we do not know how long the fever has been present. If cervical lymphadenopathy or feet and hand erythema and edema are present, complete criteria for KD would be present (Gorelik et al., 2022).

Evaluation for suspected KD should include a CBC, CMP, ESR, CRP, and UA. Expected findings in KD would be low hemoglobin, elevated serum WBC, elevated platelet count, low serum albumin, elevated liver enzymes, elevated inflammatory markers, and sterile pyuria (Owens & Plewa, 2023). Other findings that may be present on labs are elevated bilirubin, elevated Pro-BNP, and a positive finding on a viral respiratory panel (Owens & Plewa, 2023). Current or recent viral respiratory infection is a common finding in patients diagnosed with KD and siblings of patients that have had KD are 10-20 times more likely to experience the illness (Owens & Plewa, 2023). These facts make a thorough history very important. If KD is diagnosed, an echocardiogram is the only necessary test and it will rule out coronary artery aneurysm (Owens & Plewa, 2023).

There are many differentials based on clinical findings of KD. Some of these include peritonsillar abscess, retropharyngeal abscess, group A strep pharyngitis, measles, scarlet fever, rheumatic fever, rocky mountain spotted fever, adenovirus, enterovirus, meningitis, and mononucleosis, among others (Owens & Plewa, 2023). The large number of possible differentials makes the history and thorough physical examination even more important.

If a diagnosis of KD is made, the most current clinical practice guidelines were released in 2021 by the American College of Rheumatology and the Vascular Foundation. Treatment is somewhat based on echocardiogram findings, so at diagnosis, an echo should be done (Gorelik et al., 2022).  Intravenous immunoglobulin (IVIG) is the standard treatment for KD (Gorelik et al., 2022). 2 gm/kg over 10-12 hours has been suggested (Owens & Plewa, 2023). If the patient is less than 6 months old or has a Z-score greater than or equal to 2.5 in the left descending or right coronary artery following echocardiogram, either glucocorticoids or an immunomodulatory agent should be added to the IVIG treatment (Gorelik et al., 2022). With persistent fever or a return of fever within 36 hours of this treatment, it should be repeated (Gorelik et al., 2022). A third treatment of only glucocorticoid or immunomodulatory agent may be considered if the fever again returns or persists within 36-48 hours after the second treatment (Gorelik et al., 2022). Use of high dose aspirin is also strongly recommended in acute KD while fever persists (Gorelik et al., 2022). Doses of aspirin range from 80-100 mg/kg/day divided every 6 hours (Owens & Plewa, 2023).

References

Gorelik, M., Chung, S. A., Ardalan, K., Binstadt, B. A., Friedman, K., Hayward, K., Imundo, L. F., Lapidus, S. K., Kim, S., Son, M. B., Sule, S., Tremoulet, A. H., Van Mater, H., Yildirim?Toruner, C., Langford, C. A., Maz, M., Abril, A., Guyatt, G., Archer, A. M., … Mustafa, R. A. (2022). 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of Kawasaki Disease. Arthritis & Rheumatology, 74(4), 586–596. https://doi.org/10.1002/art.42041

Owens, A. M., & Plewa, M. C. (2023). Kawasaki Disease. In: StatPearls [Internet]. StatPearls Publishing. https://www.ncbi.nlm.nih.gov/books/NBK537163/

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