COVID-19 Inflammatory Syndrome With Clinical Features Resembling Kawasaki Disease Robert Spencer, MD,a Ryan C. Closson, MD,a Mark Gorelik, MD,b Alexis D. Boneparth, MD,b Rebecca F. Hough, MD, PhD,c Karen P. Acker, MD,d Usha Krishnan, MDa We describe 2 patients with coronavirus disease who had multiple clinical abstract features suggestive of Kawasaki disease (KD). Both patients presented with fever lasting .5 days and were found to have rash, conjunctival injection, and swollen lips. One patient also had extremity swelling, whereas the other Divisions of aPediatric Cardiology, bAllergy, Immunology, and fi Rheumatology, and cPediatric Critical Care Medicine, developed desquamation of the ngers. In both cases, laboratory results were Department of Pediatrics, Columbia University Irving similar to those seen in KD. These patients had highly unusual but similar Medical Center and Morgan Stanley Children’s Hospital, New d features, and both appeared to respond favorably to treatment. It remains York, New York; and Division of Pediatric Infectious Diseases, Department of Pediatrics, Weill Cornell Medicine, unclear whether these patients had true KD or manifestations of coronavirus New York, New York disease that resembled KD. Dr Spencer was the consulting cardiology fellow who evaluated 1 of the 2 patients, and he was the primary author of this case report; Dr Closson was the consulting cardiology fellow who evaluated the As of May 18, 2020, nearly 1.5 million of his hands and feet developed. other patient, and he contributed references and cases of coronavirus disease (COVID- Two days later, he developed revisions to the manuscript; Dr Gorelik was the fi consulting rheumatology attending who helped 19) have been con rmed in the a polymorphous rash on his torso, manage one of these patients, and he contributed 1 United States. Although ∼2% of cases extremities, and face. The patient references and revisions to the manuscript; Dr are among individuals aged ,18 contacted his primary care physician, Boneparth was the consulting rheumatology years, and the majority of those who recommended supportive care. attending who helped manage these patients, and he patients experience mild symptoms, Three days later, symptoms increased contributed references and revisions to the manuscript; Dr Hough was the critical care our understanding of the wide in severity, and the patient began to attending who primarily managed these patients in spectrum of clinical patterns and have confusion, severe headache, and the ICU, and she contributed references and spectrum of disease in this population neck stiffness, prompting revisions to the manuscript; Dr Acker was the remains limited.2,3 We report 2 cases presentation to the emergency consulting infectious disease attending who helped of children who tested positive for department. manage these patients, and she contributed revisions to the manuscript; Dr Krishnan was the severe acute respiratory syndrome On admission, his temperature was precepting attending for Dr Spencer and senior coronavirus 2 (SARS-CoV-2) on 39.3°C, with a heart rate of 145 beats author, and she conceptualized and contributed reverse transcription polymerase revisions to the manuscript; and all authors per minute and blood pressure of chain reaction (RT-PCR) but approved the final manuscript as submitted and 78/42. He had a respiratory rate of presented with a multisystem agree to be accountable for all aspects of the work. 24 breaths per minute and oxygen inflammatory syndrome that also DOI: https://doi.org/10.1542/peds.2020-1845 saturation of 97% while he was may fulfill diagnostic criteria for Accepted for publication May 26, 2020 breathing ambient air. On examination, Kawasaki disease (KD). he appeared unwell and in moderate Address correspondence to Robert Spencer, MD, Division of Pediatric Cardiology, Morgan Stanley distress with grunting. He had nuchal Children’s Hospital of New York-Presbyterian, rigidity, bilateral bulbar conjunctival PATIENT I Columbia University Irving Medical Center, 3959 injection without exudate, and diffuse Broadway, New York, NY 10032. E-mail: ros9225@ An 11-year-old boy with a history of maculopapular rash (Fig 1). His lips nyp.org febrile seizures had been well until were initially noted to be swollen and 5 days before his presentation, when red, although this was not subsequently To cite: Spencer R, Closson RC, Gorelik M, et al. fever, sore throat, rhinorrhea, dry observed. He did not have cervical COVID-19 Inflammatory Syndrome With Clinical cough, emesis, diarrhea, myalgia, adenopathy, and extremity edema was Features Resembling Kawasaki Disease. Pediatrics. 2020;146(3):e20201845 bilateral conjunctivitis, and swelling not noted. Downloaded from www.aappublications.org/news by guest on September 29, 2021 PEDIATRICS Volume 146, number 3, September 2020:e20201845 CASE REPORT Pertinent laboratory results on with a 5-day course of intravenous presentation of the patient (patient 1) corticosteroids for cytokine storm to the emergency department are resulting from COVID-19. The summarized in Table 1. They patient’s fever resolved within included a normal white blood cell 24 hours of receiving IVIg, and his count with neutrophilic noncardiac symptoms have continued predominance and lymphopenia, to improve. A repeat echocardiogram hyponatremia, mild transaminitis did not reveal any further coronary and hypoalbuminemia, elevated abnormalities. inflammatory markers, and elevated N-terminal B-type natriuretic peptide PATENT II (NT-ProBNP). Urinalysis and cerebrospinal fluid (CSF) cell counts, The second patient was a previously which were obtained because of his healthy 7-year-old girl who presented nuchal rigidity and severe headache, with 5 days of fever, sore throat, were unremarkable. Urine, blood, and severe abdominal pain, emesis and CSF cultures were negative for diarrhea, and an erythema infection. COVID-19 was diagnosed multiforme-like rash on the face, on the basis of RT-PCR for SARS-CoV- FIGURE 1 trunk, and back. Two days earlier, her Rash on the arm of patient 1 (shared with parents took her to a local emergency 2. Electrocardiogram showed normal parental permission). sinus rhythm with nonspecific ST- department, where she tested and T-wave changes. Chest positive for SARS-CoV-2 on RT-PCR. radiography revealed patchy perihilar venous line and an arterial line were On examination, in addition to her opacities in both lungs. He was placed. Although preparations were rash (Fig 2), she was found to have started on antimicrobial therapy, made to intubate for impending conjunctival erythema, cracked lips, including coverage for herpes simplex respiratory failure in the setting of and strawberry tongue. Laboratory virus, toxic shock syndrome, and shock, he improved, and his results included leukopenia, rickettsial disease, with vancomycin, vasoactive infusions were weaned thrombocytopenia, hyponatremia, ceftriaxone, acyclovir, clindamycin, within hours. Transthoracic mild transaminitis and and doxycycline. He was fluid echocardiography revealed low- hypoalbuminemia, elevated troponin, resuscitated, and a dopamine infusion normal systolic function of the left and elevated inflammatory markers. was added for hypotension. ventricle and a shortening fraction of Because of severe abdominal pain Supplemental oxygen was provided 29% (normal range, 25–45) on concerning for acute appendicitis, through a nasal cannula at a rate of vasoactive support, with moderate abdominal ultrasound and MRI were 2 L per minute. mitral regurgitation and a small both obtained and demonstrated pericardial effusion. The right acute, aggressive colitis, and ileitis. The patient continued to have fever coronary artery was noted to be Shortly after admission, the patient and developed desquamation of his prominent with lack of tapering but developed agitation and confusion fingertips. He soon developed had a z score of 1.4 (normal value, concerning for encephalopathy. On leukocytosis (white blood cell count, ,2.0), whereas the proximal left telemetry, she was noted to have 19 440 per cubic millimeter) and coronary artery appeared normal; no frequent premature ventricular continued to have thrombocytopenia. aneurysms were evident. contractions. Transthoracic On hospital day 2, his high-sensitivity echocardiography revealed low- troponin T increased to 136 ng/L Given the patient’s persistent fevers normal systolic function of the left (normal value, ,22), whereas NT- and mucocutaneous findings, his ventricle and a shortening fraction of ProBNP rose to 35 137 000 pg/L supportive laboratory data 27%, mild mitral regurgitation, and (normal range, 10 000–242 000). He (leukocytosis with neutrophilic normal coronary arteries. In the continued to have worsening predominance, hyponatremia, context of the previous patient with hypotension and was transitioned hypoalbuminemia, elevated hyperinflammatory syndrome and from dopamine to high-dose inflammatory markers) and his evidence of significant multisystem epinephrine, norepinephrine, and echocardiographic findings (mitral involvement in the setting of COVID- vasopressin infusions. Cefepime was regurgitation and pericardial 19, treatment with intravenous added for increased Gram-negative effusion), there was concern for corticosteroids was initiated, with coverage, and he was given stress- possible KD. He was treated with prompt fever defervescence and dose hydrocortisone. A central intravenous immunoglobulin (IVIg) improvement in her clinical Downloaded from www.aappublications.org/news by guest on September