SCIENTIFIC
A Case of Refractory Kawasaki Disease in a Patient with Confounding Laboratory Studies
AUTHORS:
ABSTRACT
Tatiana Henriksson, MD 1 Catherine Brown, MD FAAP 2
Pediatric Hospitalist and Assistant Professor of Pediatrics
AFFILIATIONS:
West Virginia University School of Medicine
West Virginia University Department of Pediatrics
CORRESPONDING AUTHOR:
Catherine Brown ckelly13 @ hsc. wvu. edu
DISCLOSURES: None
ETHICS STATEMENT:
Written informed consent for publication has been obtained from the patient’ s parents.
FUNDING: None
CASE PRESENTATION
A previously healthy 2-year-old male was admitted for four days of fever, ranging from 38-40 ° C, and viral prodrome. The patient was ill-appearing without distress with normal vital signs, coarse breath sounds bilaterally, and a small oral mucosal ulceration. Initial work-up revealed elevated white blood cell count( WBC) at 21.0 x 10 ³/µ L, liver enzyme levels with aspartate aminotransferase( AST) at 359 U / L and alanine aminotransferase at 257 U / L, creatinine at 0.59 mg / dL, C-reactive protein( CRP) at 50.8 mg / L, and procalcitonin at 13.81 ng / mL. Rhinovirus and coronavirus were detected on a viral diagnostic polymerase chain reaction( PCR). There was no sterile pyuria, anemia, or thrombocytosis( platelet count was 246 × 10 ³/µ L). Herpes simplex virus PCR from his oral lesion was negative. Empiric cef-
Kawasaki disease( KD) is an acute vasculitis predominantly affecting children, characterized by prolonged fever and mucocutaneous inflammation. We present a case of a previously healthy 2-year-old male who initially presented with fever and viral symptoms along with rhinovirus and coronavirus detected on a respiratory panel, complicating the diagnosis. Despite supportive care for a presumed viral illness, the patient developed classic KD features and was treated with intravenous immunoglobulin( IVIG) and high-dose aspirin. Persistent fever met criteria for IVIG-resistant KD, prompting
triaxone was started for suspected bacterial pneumonia in polyviral illness. Blood and urine cultures yielded no growth and the patient continued to experience temperatures up to 40 ° C despite antimicrobial treatment.
On day five of illness, the patient developed irritability, bilateral non-purulent conjunctivitis( Figure 1), morbilliform rash on his abdomen and groin, fissured lips( Figure 2), extremity swelling, and cervical lymphadenopathy. The patient was started on intravenous immunoglobulin( IVIG) infusion of 2 g / kg and high dose aspirin( 30 mg / kg / day) for presumed Kawasaki disease( KD). Same-day echocardiogram was negative for coronary dilation or aneurysm. Following infusion, his oral intake, rash, and chapped lips improved, but daily fevers continued up to 39 ° C in the evenings over the next two days. Repeat laboratory studies demonstrated improving trends in WBC at 8.3 × 10 ³/µ L, AST at 43 U / L, ALT at 74 U / L, creatinine at 0.44 mg / dL, and procalcitonin at 7.59 ng / mL. CRP remained elevated at 105.5 mg / L and follow up platelet count was stable at 241 × 10 ³/µ L. The patient received a second round of IVIG on day seven of illness due to persistent fevers and was transferred from an outside facility to our academic center for higher level of care. Due to our patient’ s persistent symptomology despite
further evaluation that revealed gallbladder hydrops, reinforcing the diagnosis. Therapy was escalated with corticosteroids and infliximab, resulting in rapid clinical improvement and normalization of inflammatory markers. Echocardiograms remained normal throughout the patient’ s course. This case highlights the importance of clinicians’ early recognition of IVIG resistance, particularly in patients with confounding laboratory findings, such as a positive viral panel, to guide timely escalation of therapy and prevent coronary complications.
treatment with two courses of IVIG in the context of a positive viral panel, pediatric infectious disease( ID) and rheumatology specialists were consulted.
The pediatric ID specialist recommended the following studies: serologies for other potential bacterial and viral serologies, an abdominal ultrasound, and a repeat echocardiogram. Epstein-Barr virus, cytomegalovirus, adenovirus, and enterovirus serologies were negative. Repeat echocardiogram on day eight was normal. Coronary arteries were within normal limits( left main coronary artery z-score of 0.24, left main and left anterior descending coronary artery z-score of 0.97). Abdominal ultrasound revealed gallbladder hydrops. On day nine of illness, pediatric rheumatology recommended methylprednisolone 1 mg / kg IV for 5 days, along with a single dose of infliximab 7 mg / kg IV. The patient demonstrated rapid clinical improvement with fever cessation to temperatures < 37 ° C and significant inflammatory marker improvement over the next 48 hours. CRP was now at 82.7 mg / L, AST at 36 U / L, and ALT at 35 U / L. The patient was discharged with a four-week prednisolone taper and continued low-dose aspirin( 60.75 mg / day) for 6-8 weeks. Repeat echocardiogram 1 week later revealed normal cardiac anatomy without formation of coronary artery aneurysm( CAA).
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