Sep 01, 2021 Issue
 
 

Kawasaki Disease and Multisystem Inflammatory Syndrome in Children: An Overview and Comparison

 

JOHN B. DARBY, MD, and JENNIFER M. JACKSON, MD, Wake Forest School of Medicine, Winston-Salem, North Carolina

Am Fam Physician. 2021 Sep ;104(2):244-252.

Published online August 12, 2021.

Author disclosure: No relevant financial affiliations.

Article Sections

Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) are inflammatory conditions that present diagnostic and therapeutic challenges to the physician. Although many of their features overlap, they are two distinct conditions. KD is a febrile illness most commonly affecting children younger than five years. It manifests with prolonged fever and at least four of the following features: bilateral bulbar conjunctivitis, mucositis, diffuse maculopapular rash, extremity changes, and cervical lymphadenopathy of 1.5 cm or more in diameter. Patients with MIS-C may have many of the same manifestations but tend to have higher rates of gastrointestinal and neurocognitive symptoms and signs of shock on presentation. Both conditions are associated with cardiac sequelae, including coronary artery aneurysms, although children with MIS-C are at high risk of developing ventricular dysfunction and depressed cardiac output. Lymphocytopenia, thrombocytopenia, elevated troponin, and elevated B-type natriuretic peptide are key laboratory findings of MIS-C that can help distinguish it from KD. The use of intravenous immune globulin is well established in KD and also appears to have a role in the treatment of MIS-C. Aspirin has been used in KD for an anti-inflammatory effect, and low-dose aspirin is recommended for MIS-C to reduce the risk of thrombosis. In addition to supportive care, patients with MIS-C may benefit from immunomodulatory medications, although data on this topic are evolving.

First described in Japan in 1967, Kawasaki disease (KD) is a vasculitis affecting small- and medium-sized vessels; it is predominantly seen in children younger than five years.1 This febrile illness is characterized by systemic inflammation and is the most common cause of acquired coronary artery disease in children.2 With the emergence of multisystem inflammatory syndrome in children (MIS-C), a new inflammatory syndrome associated with COVID-19, KD has received increased attention because features of the diseases overlap.

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SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComments

A diagnosis of classic KD is made in the presence of prolonged fever (five or more days) and four or more of the following principal features: oral mucosal inflammation; bilateral bulbar conjunctivitis; a diffuse maculopapular rash; extremity changes, including erythema and edema of the hands and feet; and cervical lymphadenopathy of 1.5 cm or more in diameter.2

C

Consensus opinion, society guidelines

For patients with a suspected diagnosis of KD or MIS-C, echocardiography should be performed promptly but should not delay treatment.2,7

C

Expert guidelines that included studies in limited populations and with few randomized controlled trials; benefits greatly outweigh risks

Physicians concerned about MIS-C should pursue a diagnostic workup that includes complete blood count, complete metabolic panel, erythrocyte sedimentation rate, C-reactive protein, and SARS-CoV-2 polymerase chain reaction and/or serologies; additional workup may be necessary, including B-type natriuretic peptide, troponin, ferritin, prothrombin time, partial thromboplastin time, d dimer, fibrinogen, lactate dehydrogenase, cytokine panel, electrocardiography, and chest radiography.19

C

Expert opinion, society guidelines

Patients with KD should receive IVIG (2 g per kg given as a single dose).2,20–24

C

Multiple randomized controlled trials and meta-analyses looking at coronary artery abnormalities

Suspected cases of MIS-C should be referred promptly to the hospital. Management of MIS-C requires a multidisciplinary team of specialists including, but not limited to, pediatric critical care physicians, pediatric cardiologists, pediatric hospitalists, pediatric rheumatologists, pediatric infectious disease specialists, and pediatric hematologists.19

C

Expert opinion

Patients with MIS-C should receive IVIG (2 g per kg given as a single dose), and concurrent corticosteroids should be considered.19

C

Expert opinion, observational studies

IVIG = intravenous immune globulin; KD = Kawasaki disease; MIS-C = multisystem inflammatory syndrome in children.

A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to https://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingComments

A diagnosis of classic KD is made in the presence of prolonged fever (five or more days) and four or more of the following principal features: oral mucosal inflammation; bilateral bulbar conjunctivitis; a diffuse maculopapular rash; extremity changes, including erythema and edema of the hands and feet; and cervical lymphadenopathy of 1.5 cm or more in

The Authors

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JOHN B. DARBY, MD, is an assistant professor in the Department of Pediatrics at Wake Forest School of Medicine, Winston-Salem, N.C....

JENNIFER M. JACKSON, MD, is the assistant dean for Curricular Innovation and an associate professor in the Department of Pediatrics at Wake Forest School of Medicine.

Address correspondence to Jennifer M. Jackson, MD, Wake Forest School of Medicine, Medical Center Blvd., Winston-Salem, NC 27157 (email: jstancil@wakehealth.edu). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

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