Rapid Inflammation–Control and Regression of Giant Aneurysms in an Infant with Atypical Kawasaki's Disease Treated with TNF-Alpha Antagonist

Background: Kawasaki's disease is an acute vasculitis and a leading cause of acquired heart disease in children that causes inflammation in the walls of medium-sized arteries throughout the body. Its early symptoms include high fever, mucocutaneous inflammation, and cervical adenopathy. The development of giant aneurisms of the coronary arteries is a threatening complication which appears at a later stage of disease progression and may lead to development of coronary artery obstructive lesions, an important factor associated with increased morbidity and mortality.

Method: We report on a 4-month-old male patient with incomplete Kawasaki's disease who was initially treated with corticosteroid, intravenous immunoglobulin (IVIG), and aspirin. Under this therapeutic regimen, the fever has resolved. However, a few days later, the patient developed a new febrile peak. An echocardiogram demonstrated giant aneurisms at the following localizaitons: right coronary artery (RCA) max 7.5 mm (Z-score = +16), left coronary artery (LCA) maximum 8 mm (Z-score = +18). We performed a cardiac catheterization with angiography of the coronary arteries and intracoronary fibrinolytic therapy due to thrombus formation and reduced blood flow. We started a treatment with corticosteroid, aspirin, and low molecular heparin. Because of persistence of fever and elevated level of C-reactive protein, we additionally treated our patient with a TNF-α-antagonist. Upon reassessment after 24 hours, clinical signs of inflammation had subsided and 48 hours later the CRP-level was back to normal. A repeat echocardiography showed a slow regression of the coronary aneurisms, LCA = 6.5 mm (Z-score = +11); RCA = 6.5 mm (Z-score = +10). After 7 months, our patient is on therapy with aspirin and phenprocoumon (INR: 2–2.5), the echocardiography showed relevant regression of the coronary artery diameter: RCA = 4.5 mm (Z-score = +7.7), LCA = 4.5 mm (Z-score = +6.9). No clinical signs of Inflammation.

Conclusion: Due to the unknown etiology of Kawasaki's disease, the treatment goal is to reduce inflammation and prevent damage to coronary arteries. TNF-α-antagonist therapy is recognized as a rescue-therapy by IVIG resistance. Threating our patient with a TNF-α-antagonist, rapid inflammation control, regression of the aneurisms, improvement of clinic, laborytory values (CRP) and echocardiographic findings was achieved.

Publication History

Article published online:
12 February 2022