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Kawa-shocky

Lindsay Miller

CASE

The patient is a 5 year-old female with a past medical history significant only for torticollis, who presented to the emergency department with fever, emesis, diarrhea, fatigue, and rash. The patient began having fevers as high as 102 Fahrenheit five days prior to arrival. The patient’s mother reported that four days ago, the patient began having diarrhea and non-bloody, non-bilious emesis. Two days ago, she began having decreased PO intake, continued fevers and emesis, anuria, and bilateral neck pain. The morning of arrival, the patient’s mother noted a rash on the patient’s back and bilateral conjunctival injection. She brought the patient to her pediatrician’s office, who sent her to the emergency department for further evaluation and management.

On arrival to the emergency department, review of systems was positive for activity change, appetite change, fatigue, fever, rhinorrhea, cough, diarrhea, nausea and vomiting, decreased urine volume, neck pain and neck stiffness, color change, pallor, rash, weakness, and adenopathy. Physical exam as below:

Vitals:

  • Temp 101.7F (38.7C), HR 148, BP 78/52, Resp 40, O2 sat 98% on RA

General:

  • She is ill-appearing but awake and alert

HEENT:

  • Nose: Congestion and rhinorrhea present

  • Mouth: Mucous membranes are dry

  • Pharynx: Posterior oropharynx erythematous

  • Eyes: Bilateral conjunctival erythema with no exudate

Neck:

  • Musculoskeletal: Neck rigidity and muscular tenderness present bilaterally

Cardiovascular:

  • Rate and Rhythm: Tachycardic, regular

Pulmonary:

  • Tachypneic.  Lungs clear bilaterally.

Lymph:

  • Cervical: Tender bilateral anterior cervical adenopathy present.

 Skin:

  • General: Skin is cool, dry and pale. Capillary refill takes more than 3 seconds.

  • Macular, blanching rash over entire back. 

Labs significant for Sodium 130, potassium 3.3, Bicarb 15, Anion Gap 19, BUN 49, Cr 0.74, LFTs WNL, CRP 215.93, ESR 125, WBC 22.4 (18.1 neutrophils), normal hgb w/ schistocytes on peripheral smear

Respiratory pathogen panel: positive for Coronavirus NL63 and Human Rhino/enterovirus

UA: Trace ketones, protein present

Chest X-ray: unremarkable

Neck X-ray: unremarkable

CT neck: significant for bilateral cervical lymphadenopathy

DIAGNOSIS

Kawasaki Disease Shock Syndrome

DISCUSSION

Kawasaki Disease is a systemic vasculitis that occurs in children. While the etiology of the disease is largely unknown, it is felt to be an autoimmune disorder that is often idiopathic, but may be triggered by a variety of viral illnesses.  The diagnosis is based on a constellation of clinical features.

Diagnostic criteria

FEVER of >= 5 days and at least 4 out of 5 of the principal features:

1.     Erythema and edema of hands and feet OR membranous desquamation of fingertips

2.     Rash

3.     Bilateral, painless bulbar conjunctival injection without exudate

4.     Oral mucosal changes which include: Erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosa

5.     Unilateral (usually) cervical lymphadenopathy

Atypical, or incomplete, Kawasaki Disease is more difficult to diagnose, with a diagnostic criteria of at least 5 days of fever, two or three other diagnostic criteria, and certain laboratory criteria, which normally include elevated CRP, ESR, WBC, platelets, ALT; anemia, albumin <3 or UA WBC >10 (with no organisms; aka sterile pyruia).  Other causes of these laboratory abnormalities must be ruled out. [1]

While not part of the diagnostic criteria, coronary artery abnormalities are the findings which convey the highest morbidity and mortality. Prevalence varies in different studies from 13% [2,3] to 19% [4] for “abnormalities” which range from mild dilatation to discrete aneurysms.

Kawasaki Disease Shock Syndrome is characterized by hypotension or clinical signs of hypoperfusion. It was named for its similarities to Toxic Shock Syndrome. It was first recognized as a distinct syndrome in 2009 and although it is not well-understood, it is known to be associated with some key features. These include: more likely to be present in older patients, male sex, non-Asian/non-white patients, and more likely to be associated with resistance to IVIG. Thus, despite lack of clear evidence, most experts recommend adding empiric high-dose steroid treatment to IVIG and high-dose aspirin therapy when Kawasaki Shock Syndrome is present. [5]

IVIG is one of the few treatments that is effective in reversing the cardiac abnormalities seen in Kawasaki. In patients who have not yet developed coronary artery abnormalities, IVIG is effective in preventing those sequelae. It is more effective than high-dose aspirin alone. This treatment has been the mainstay of therapy for Kawasaki disease for over 30 years to help reduce inflammation of the blood vessels. More recently, drug trials have attempted to identify patient’s at higher risk of failing IVIG treatment who might respond to newer drug treatments. [6]  Patients resistant to IVIG are more likely to require concurrent treatment with high-dose steroids.

Treatment

This patient was initially treated supportively in the ED for her shock syndrome while clinical and diagnostic evaluations took place.  She received oxygen, IV fluids and was started on an epinephrine drip for blood pressure support.  As is typical with Kawasaki Disease, treatment in the ED is mainly supportive until a definitive diagnosis has been confirmed and other causes of similar presentations have been ruled out.  A Chest X-ray, Neck X-ray, CT neck, and abdominal and pelvic (trans-abdominal) US were performed, which did not demonstrate an alternative cause of this patient’s presentation.

Disposition

Disposition of patients with Kawasaki Disease depends on their level of support. Mild cases, or cases where the diagnosis is suspected but not confirmed, may be able to go to the pediatric floor.  If a patient requires a higher level of support, or if there is significant concern for cardiac involvement, patients should go the Pediatric ICU. Due to this patient’s need for vasopressor support with epinephrine, the patient went to the Pediatric ICU. Cardiology was consulted early in the patient’s workup, even before a definitive diagnosis was made, as early identification of coronary artery abnormalities allows for early initiation of IVIG treatment.

CASE RESOLUTION

An echocardiogram by cardiology revealed dilatation of the coronary arteries without discrete aneurysm. Without an alternative cause of the symptoms, the patient met the diagnostic criteria for Kawasaki Disease. She was started on high dose aspirin, methylprednisolone and IVIG in the PICU. She continued to improve and was discharged on hospital day #6 on continued low-dose aspirin. At cardiology follow up, she had a prominent LCA without any dilatation.

TAKE-AWAYS

  • Kawasaki Disease is a systemic vasculitis that occurs in children

  • It should be considered in patients presenting with fever of >= 5 days and at least 4 out of 5 of the principal features:

    • Erythema and edema of hands and feet OR membranous desquamation of fingertips

    • Rash

    • Bilateral, painless bulbar conjunctival injection without exudate

    • Oral mucosal changes

    • Unilateral cervical lymphadenopathy

  • Kawasaki Disease Shock Syndrome is characterized by hypotension or clinical signs of hypoperfusion

  • IVIG is one of the few treatments that is effective in reversing the cardiac abnormalities seen in Kawasaki

Author: Lindsay Miller, MD is a third year emergency medicine resident at Brown University/Rhode Island Hospital.

Faculty Reviewer: Robyn Wing, MD is an emergency medicine attending at Hasbro Children’s Hospital.

REFERENCES

  1. Yu JJ. Diagnosis of incomplete Kawasaki disease. Korean J Pediatr. 2012 Mar; 55(3): 83-87

  2. Patel A, Holman RC, Callinan LS, Sreenivasan N, Schonberger LB, Fischer TK, Belay ED. Evaluation of clinical characteristics of Kawasaki syndrome and risk factors for coronary artery abnormalities among children in DenmarkExternal. Acta Paediatr. 2013 Apr;102(4):385-390. doi: 10.1111/apa.12142. Epub 2013 Jan 21.

  3. Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB. Kawaskaki syndrome and risk factors for coronary artery abnormalitiesExternal, United States, 1994-2003. Pediatr Infect Dis J. 2006;25(3):245-249.

  4. Callinan LS, Tabnak F, Holman RC, Maddox RA, Kim JJ, Schonberger LB, Vugia DJ, Belay ED. Kawasaki syndrome and factors associated with coronary artery abnormalities in CaliforniaExternal Pediatr Infect Dis J. 2012 Sep;31(9):894-898.

  5. Kanegaye JT, Wilder M, Molkara D, Frazer JR, Pancheri J, Tremoulet A, Watson VE, Best BM, Burns JC. Recognition of a Kawasaki disease shock syndrome. Pediatrics 2009;123(5):e783-9

  6. Hamada H et al. Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial. The Lancet. 2019;393(10176)P1128-1137

  7. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, Yanagawa H. A new infantile acute febrile mucocutaneous lymph node syndrome prevailing in Japan. Pediatrics. 1974;54:271-276

  8. Melish ME, Hicks RM, Larson EJ. Mucocutaneous lymph node syndrome in the United States.External Am J Dis Child. 1976;130:599-607.

  9. Newburger JW et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med. 1986 Aug7;315(6):341-7