Infliximab as an alternative therapy for refractory adult onset Kawasaki disease

Abstract

Rationale:Kawasaki disease (KD) is an acute febrile illness predominantly affecting children less than 5 years of age and characterized by systemic inflammation in all medium-sized arteries. Adult-onset KD (AKD) is rare with only 105 case reports published. Recently, the efficacy of infliximab (IFX) for patients with refractory KD has been demonstrated.Patient concerns:A previously healthy 24-year-old man was admitted because of a persistent fever, and elevated serum level of AST, ALT, LDH, and CRP.Diagnosis:The patients met the diagnostic criteria for KD based on the findings of persistent fever, polymorphous exanthema, unilateral cervical lymphadenopathy, non-purulent palpebral conjunctivitis and membranous desquamation. Echocardiogram revealed the dilatation at the proximal sites of the right coronary artery (7.9 mm) and left anterior descending artery (5 mm). The patient was treated with high-dose intravenous immunoglobulin (1 g/kg/day for 2 days) and ASA (100 mg daily). However, his fever and arthralgia persisted.Interventions:He was administered single 5 mg/kg doses of IFX.Outcomes:He became afebrile the next day and his arthralgia improved.Lessons:We report the first case of administration of IFX in a patient with AKD refractory to intravenous immunoglobulin (IVIG), and successful reduction of systemic inflammation. However, the effectiveness of IFX in the regression of coronary artery aneurysm remains to be determined.

Two Cases of Adult-onset Kawasaki Disease with a Difficult Diagnosis

“…AKD is said to present with specific symptoms that differ from those of infants. Table compares the results of the 26th National Kawasaki Disease Surveillance in 2021 with those of the AKD cases reported in Japan for which major symptoms have been described (2,(4)(5)(6)(7)(8)(9).…”

Two Distinct Cases of Adult-onset Kawasaki Disease

“…Therefore, Case 1 was treated with a low to intermediate dose of ASA, while Case 2 received an intermediate dose in response to the severity. IVIG-refractory AKD-myocarditis with an improved cardiac function is treated with infliximab ( 9 ). However, our Case 2 still showed progressive heart failure due to AKD-myocarditis.…”