Complete Regression of Giant Aneurysms in an Infant with Delayed Diagnosis and Refractory Kawasaki Disease via Combination Anticytokine Therapy: Case Report and Review of Similar Cases

Abstract

Background. Kawasaki disease (KD) is an inflammatory vasculitis and is the most common cause of acquired childhood heart disease in developed countries. Current treatment with intravenous immunoglobulin (IVIG) is often ineffective in patients with delayed or refractory disease. We present a case of combination anticytokine therapy in an infant with delayed and refractory KD. Case Presentation. A 3-month-old infant presented with refractory KD with giant aneurysms after a delayed diagnosis of one month. Use of combination anticytokine therapy led to resolution of giant aneurysms over approximately 6 months. Conclusions. Our case is unique in effective use of anticytokine therapy in very delayed disease with giant aneurysms. Additionally, we review other cases for a broader perspective. Prospective study of anticytokine therapy for patients with giant aneurysms may be warranted.

COVID-19-associated vasculitis and vasculopathy

“…Kawasaki Disease is a systemic vasculitis that predominantly affects small and medium-sized arteries. It si associated with the development of coronary artery aneurysms in up to one-third of untreated patients, and both systemic and cerebral artery aneurysms in 1–2% of patients [ 74 ]. The cause of KD has not been determined, but a framework for making the diagnosis is as follows [ 75 ]: a genetic predisposition to KD, immunomodulation related to habitual exposures and environmental factors and contact with disease triggers.…”

Kawasaki-like diseases and thrombotic coagulopathy in COVID-19: delayed over-activation of the STING pathway?

“…Indeed: 1-even incomplete form of KD (KD-like disorders) can lead to severe, and sometimes lethal, delayed aneurysms; 2-the STING pathway is activated in aortic aneurysms, especially those induced by infections, and it contributes to their pathogenesis [33]. As giant coronary aneurysms in children with KD can regress following the use of anti-cytokines [21], drugs inhibiting the cytokine storm occurring in severe COVID-19 might be also useful to prevent the delayed onset of similar coronary or cerebral aneurysms, either in children with KD-like features and/or adults with COVID-19.…”

“…Kawasaki disease (KD) is a systemic vasculitis of unknown aetiology which predominantly affects medium and small-sized muscular arteries, mostly in children, and much less frequently in adults. KD is associated with the delayed occurrence of arterial aneurysms: coronary aneurysms (up to giant coronary aneurysms [ 21 ]) in roughly 30% of untreated patients, and systemic arterial aneurysms in 2%. Asymptomatic cerebral vasculitis is also more common in KD than previously believed, and cerebral aneurysms have been reported, although much less frequently than coronary aneurysms.…”

Multiresistant Kawasaki Disease Complicated With Facial Nerve Palsy, Bilateral Giant Coronary Artery Aneurysms, and Stenosis of the Right Coronary Artery in an Infant

“…A novel approach for treatment of refractory KD is the combination of IVIG, steroids, and 2 biologicals (infliximab or etanercept combined with anakinra). 9,10 In fact, successful treatment of refractory KD with interleukin 1-blocking agents such as anakinra has been reported, and this treatment may change the current treatment approach. 11 A review of patients requiring multiple treatments for KD is summarized in Table 1.…”

“…Gitiaux et al 8 report the case of a 4-year-old girl who presented with refractory KD complicated with central nervous system vasculitis and neuropathy and required multiple doses of IVIG, steroids, and 2 immunosuppressant drugs (azathioprine and cyclophosphamide). A novel approach for treatment of refractory KD is the combination of IVIG, steroids, and 2 biologicals (infliximab or etanercept combined with anakinra) 9,10 . In fact, successful treatment of refractory KD with interleukin 1–blocking agents such as anakinra has been reported, and this treatment may change the current treatment approach 11 .…”