Be alert for Kawasaki disease

Author(s): Beth McVey, DNP, APRN, ACNP, ENP

Early detection is key to avoiding heart disease.

Takeaways:

  • If Kawasaki disease (KD) is recognized and treated early, children can recover quickly with a decreased chance of developing long-term complications and heart disease.
  • Heart disease develops in 1 out of 4 children diagnosed with KD.
  • The diagnosis of complete KD is based on the presence of ≥ 5 days of fever and ≥ 4 principal clinical features (extremity changes, rash, conjunctivitis, oral changes, and cervical lymphadenopathy).
  • Nurses with strong assessment skills may be the key to early KD diagnosis of Kawasaki Disease.

Editor’s note: This is web exclusive is an early release article for an upcoming issue of the American Nurse Journal. 

Kawasaki disease (KD) is an acute, self-limited vasculitis that primarily affects infants and children. (See Facts about Kawasaki disease.) It’s not contagious. Recent reports of children experiencing a KD-like inflammatory syndrome that seems to be associated with COVID-19 mean nurses need to understand KD diagnosis and management. (See COVID-19-associated multisystem inflammatory syndrome.)

Facts about Kawasaki disease
  • In the United States, approximately 25 children (younger than 5 years old) per 100,000 are diagnosed with the disease. Over 6,000 children are diagnosed with KD in the United States per year.
  • It occurs in boys more often than girls.
  • It is reported globally, but the greatest incidence occurs among children of Japanese descent.
  • In North America, the disease occurs seasonally, most often in winter and early spring.

Source: Modesti, 2019.

The etiology of KD remains uncertain; however, it may have an autoimmune component resulting from a viral or bacterial infection in genetically predisposed children. According to Turnier and colleagues, over 40% of children diagnosed with KD have tested positive for viral respiratory pathogens. KD sometimes is called “mucocutaneous lymph node syndrome” because it also affects the skin glands, which swell during an infection, and the mucous membranes inside the mouth, nose, and throat. One of the main concerns is the possibility of cardiac damage.

KD and heart disease

KD can cause inflammation of the heart, which can lead to weakened vessels and aneurysm formation. Aneurysms can cause blood clots, potentially resulting in internal bleeding or myocardial infarction. Approximately one in four children diagnosed with KD experiences heart disease (including vasculitis, myocarditis, and heart valve problems), making it one of the leading causes of acquired heart disease in children. The severity of heart disease is greatest in infants younger than 1 year old.

Fortunately, with prompt diagnosis and treatment, only a small percentage of those with KD cardiac complications will have lasting damage.

COVID-19-associated multisystem inflammatory syndrome

Patients with multisystem inflammatory syndrome in children (MIS-C) associated with Coronavirus Disease 2019 (COVID-19) often have features similar to Kawasaki disease, but may have atypical features as well. According to a May 14, 2020, health advisory from the Centers for Disease Control and Prevention (CDC) released the health advisory, patients have had persistent fever and a constellation of symptoms including hypotension, multiorgan (for example, cardiac, gastrointestinal, renal, hematologic, dermatologic, and neurologic) involvement, and elevated inflammatory markers.

The advisory provides the following case definition:

Case definition for MIS-C

  • An individual aged <21 years presenting with feveri, laboratory evidence of inflammationii, and evidence of clinically severe illness requiring hospitalization, with multisystem (>2) organ involvement (cardiac, renal, respiratory, hematologic, gastrointestinal, dermatologic or neurological); AND
  • No alternative plausible diagnoses; AND
  • Positive for current or recent SARS-CoV-2 infection by RT-PCR, serology, or antigen test; or COVID-19 exposure within the 4 weeks prior to the onset of symptoms

iFever >38.0°C for ≥24 hours, or report of subjective fever lasting ≥24 hours
iiIncluding, but not limited to, one or more of the following: an elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), fibrinogen, procalcitonin, d-dimer, ferritin, lactic acid dehydrogenase (LDH), or interleukin 6 (IL-6), elevated neutrophils, reduced lymphocytes and low albumin

Additional comments

  • Some individuals may fulfill full or partial criteria for Kawasaki disease but should be reported if they meet the case definition for MIS-C
  • Consider MIS-C in any pediatric death with evidence of SARS-CoV-2 infection

Healthcare providers who have cared or are caring for patients younger than 21 years of age meeting MIS-C criteria should report suspected cases to their local, state, or territorial health department. Much is still unknown about MIS-C, and nurses are encouraged to seek out updates.

Source: CDC 2020

Assessment and diagnosis

A thorough history and physical examination are essential for diagnosis. Fever of at least 5 days’ duration is a key KD diagnostic finding, so if a caretaker reports absence of fever, ask about antipyretic use; lack of fever may be secondary to its use. Unfortunately, a delayed diagnosis is common in older children, putting them at higher risk for coronary artery abnormalities. These abnormalities are of particular concern because they can lead to complications such as thrombosis. In fact, the principal cause of death from KD is myocardial infarction.

Classic signs of KD include bilateral, bulbar conjunctivitis; red, swollen hands and feet; red, cracked lips; redness inside the mouth and pharynx; red tongue (“strawberry” tongue); high fever; flaky skin; cervical lymphadenopathy and diffuse rash that may or not be raised. (See KD: Criteria for diagnosis.) Clinicians unfamiliar with this rare disease may miss these signs. Your organization may find it helpful to post an image of them as a reminder; access an image here.

Other signs of KD include malaise, cold-like symptoms, vague abdominal pain, vomiting, diarrhea, jaundice, cough, and decreased breath sounds on lung auscultation. The child or infant also may have significant irritability.

KD: Criteria for diagnosis

Kawasaki disease (KD) diagnosis requires that a person has had a fever for at least 5 days, along with four out of five of these symptoms:

  • rash
  • cervical lymphadenopathy
  • conjunctival infection
  • oral mucosal changes
  • peripheral extremity changes.

The presentation of KD in adults may differ from that seen in children, as noted by Wolff and colleagues. Typical findings in both groups include fever, conjunctivitis, pharyngitis, and skin erythema progressing to a desquamating rash on the palms and soles. Adults more frequently present with cervical adenopathy, hepatitis, and arthralgia and are less likely to experience meningitis, thrombocytosis, and coronary artery aneurysms.

Possible findings from diagnostic tests that point to KD include the following:

  • Blood test. Elevated white blood cell (WBC) count with increased neutrophils, increased C-reactive protein, increased erythrocyte sedimentation rate, increased ferritin, decreased albumin, transaminitis, mild anemia, and increased platelets
  • Urine test. WBC with absence of leukocyte esterase, proteinuria, and sterile pyuria
  • ST or T wave abnormalities, Q waves
  • Chest X-ray. Cardiomegaly, pulmonary infiltrates or effusions
  • ECG. Pericardial effusion, coronary aneurysms, mitral insufficiency and/or aortic valvular insufficiency, and, rarely, peripheral aneurysms.

Differential diagnoses for KD include measles, adenovirus, enterovirus, scarlet fever, toxic shock syndrome, Stevens-Johnson syndrome, juvenile idiopathic arthritis, Rocky Mountain spotted fever, rickettsia, and leptospirosis.

Treatment

Because of the risks associated with KD, treatment is provided in the hospital setting. The primary treatment goals are to eliminate fever and decrease systemic inflammatory reaction.

I.V. immunoglobulin (IVIG) can lower the risk of coronary artery problems. According to an American Heart Association scientific statement, patients should be treated with IVIG 2 g/kg as a single infusion, usually given over 10 to 12 hours. Fever usually resolves within 36 hours after IVIG administration is completed. IVIG is most effective when given within the first 10 days of symptoms. Administering moderate (30-50 mg/kg/day) to high (80-100 mg/kg/day) doses of aspirin should be continued until the patient is afebrile; aspirin also will reduce inflammation and decrease pain.

After the fever has subsided, a child may be placed on a 6-week or longer low-dose aspirin regimen. (Note that this is an exception to the normal best practice of not using aspirin in children to avoid Reye’s syndrome.) Follow-up after discharge will be needed, including referral to a pediatric cardiologist to monitor heart health.

Nurses’ role in early diagnosis

KD may present as a typical childhood febrile illness, but it can have deadly consequences. Nurses in all settings—the community, medical office, and hospital—can play a leadership role in interdisciplinary collaboration, communicating all findings to healthcare team members. Subtle findings on your history or examination of a child can influence decision-making in practice. Your strong assessment skills may be the key to an early KD diagnosis. The more quickly patients receive a correct diagnosis and proper treatment, the sooner they’ll return to optimal quality of life.

Beth McVey is the advanced practice collaborative leader at Emergent Medical Associates in Temecula, California, and adjunct faculty for Simmons University in Boston.

References

Centers for Disease Control and Prevention. Kawasaki disease: About Kawasaki disease. October 24, 2018. cdc.gov/kawasaki/about.html

Centers for Disease Control and Prevention. Multisystem inflammatory syndrome in children (MIS-C) associated with Coronavirus Disease 2019 (COVID-19). Health Advisory. May 14, 2020. emergency.cdc.gov/han/2020/han00432.asp?deliveryName=USCDC_511-DM28431

Dietz SM, van Stijn D, Burgner D, et al. Dissecting Kawasaki disease: A state-of-the-art review. Eur J Pediatr. 2017;176(8):995-1009.

Greco A, De Virgilio A, Rizzo MI, et al. Kawasaki disease: An evolving paradigm. Autoimmun Rev. 2015;14(8):703-9.

Kawasaki Kids Foundation. Kawasaki disease symptoms. kawasakikidsfoundation.org/kawasaki-disease-symptoms

McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A scientific statement for health professionals from the American Heart Association. Circulation. 2017;135(17):e927-99.

Modesti AM, Plewa MC. Kawasaki disease. StatPearls. 2019. ncbi.nlm.nih.gov/books/NBK537163

Phend C. Kawasaki disease from COVID-19 in kids: How common? MedPageToday.com. May 8, 2020. medpagetoday.com/infectiousdisease/covid19/86393

Turnier JL, Anderson MS, Heizer HR, Jone PN, Glodé MP, Dominguez SR. Concurrent respiratory viruses and Kawasaki disease. Pediatrics. 2015;136(3):e609-14.

University of California San Diego School of Medicine. Kawasaki Disease Research Center. Resources for physicians. medschool.ucsd.edu/som/pediatrics/research/centers/kawasaki-disease/Physicians/Pages/default.aspx

Wilder MS, Palinka LA, Kao AS, et al. D Delayed diagnosis by physicians contributes to the development of coronary artery aneurysms in children with Kawasaki syndrome. Pediatr Infect Dis J. 2007;26(3):256-60.

Wolff AE, Hansen KE, Zakowski L. Acute Kawasaki disease: Not just for kids. J Gen Intern Med. 2007;22(5):681-84.

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