Cardiovascular

Refractory Kawasaki Disease in Infants Complicated with Giant Coronary Artery Aneurysms and Takayasu Arteritis: A Rare Case Report and Literature Review.

TL;DR

A 45-day-old male infant developed refractory Kawasaki disease complicated by Kawasaki disease shock syndrome, macrophage activation syndrome, giant coronary artery aneurysms, and subsequent Takayasu arteritis, representing an extremely rare co-occurrence of these conditions.

Key Findings

A 45-day-old male infant was diagnosed with refractory Kawasaki disease complicated by Kawasaki disease shock syndrome (KDSS) and macrophage activation syndrome (MAS).

  • The patient was a 45-day-old male infant, representing an extremely young age of presentation for Kawasaki disease.
  • The case involved simultaneous occurrence of refractory KD, KDSS, and MAS, which are each individually serious complications.
  • 10% to 20% of children with KD present with refractory disease, and only a few critically ill children develop KDSS or MAS.
  • The complete clinical diagnosis and treatment process was elaborated in detail in the case report.

After conventional treatment, the infant's condition relapsed and progressed to giant coronary artery aneurysms (GCAA).

  • Coronary artery aneurysm (CAA) is described as the most serious complication of KD.
  • Giant coronary artery aneurysm (GCAA) has an extremely poor prognosis.
  • The progression to GCAA occurred despite conventional treatment, consistent with the refractory nature of the disease.
  • The relapse and progression to GCAA followed initial conventional treatment failure.

The infant subsequently developed Takayasu arteritis (TAK) following the Kawasaki disease course, representing an extremely rare co-occurrence in an infant.

  • TAK is a chronic granulomatous vasculitis mainly involving the aorta and its branches.
  • TAK is described as extremely rare in infants, with no specific early symptoms and prone to delayed diagnosis.
  • The combination of infant KD and TAK is described as extremely rare, as there is overlap in vascular involvement, making it difficult to differentiate clinically.
  • The sequential occurrence of KD followed by TAK in a 45-day-old infant has not been well characterized in the literature.

The overlapping vascular involvement of Kawasaki disease and Takayasu arteritis creates significant clinical diagnostic challenges, particularly in infants.

  • Both KD and TAK involve vascular inflammation but differ in their typical age of onset, chronicity, and vessel distribution.
  • TAK has no specific early symptoms, making it prone to delayed diagnosis even without concurrent KD.
  • The overlap in vascular involvement between KD and TAK makes clinical differentiation difficult.
  • The authors conducted a literature review to analyze diagnosis and treatment to guide clinical practice.

Refractory Kawasaki disease affects 10% to 20% of children with KD, with a subset developing severe complications including KDSS and MAS.

  • 10% to 20% of children present with refractory KD, defined as failure to respond to conventional treatment.
  • A few critically ill children may develop Kawasaki disease shock syndrome (KDSS) or macrophage activation syndrome (MAS).
  • KD is described as a common acute systemic vasculitis in children.
  • The co-occurrence of refractory KD with both KDSS and MAS in a 45-day-old infant represents a particularly severe clinical presentation.

What This Means

This research describes the case of a 45-day-old baby boy who developed an unusually severe and complex set of inflammatory blood vessel diseases. He was diagnosed with Kawasaki disease (KD), a condition that causes widespread inflammation of blood vessels and is one of the more common serious inflammatory illnesses in young children. His case was particularly severe because he also developed two dangerous complications at the same time: Kawasaki disease shock syndrome (where the body goes into a state of cardiovascular collapse) and macrophage activation syndrome (where the immune system becomes dangerously overactive). When standard treatments failed to control the disease, it progressed to form giant aneurysms — dangerous balloon-like bulges — in the arteries of his heart, which carry a very poor outlook. He then went on to develop a second distinct vascular disease called Takayasu arteritis, a chronic inflammatory condition affecting the body's major arteries that is itself extremely rare in infants. This research matters because the combination of Kawasaki disease and Takayasu arteritis occurring together in an infant is extraordinarily rare and presents major diagnostic challenges for doctors. Both diseases inflame blood vessels, and their symptoms can overlap significantly, making it very hard to tell them apart or recognize when both are present at the same time. Takayasu arteritis in particular often has no clear early warning signs in infants, leading to delayed diagnosis. By documenting this case in detail and reviewing related medical literature, the authors aim to help clinicians better recognize and manage this rare combination of conditions. This research suggests that in infants with Kawasaki disease that does not respond to standard treatment and continues to worsen, doctors should be alert to the possibility of additional overlapping vascular diseases such as Takayasu arteritis. Early recognition of these rare but serious combinations may allow for more timely and appropriate treatment, potentially improving outcomes for critically ill infants with complex inflammatory vascular disease.

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Citation

Zhu Y, Niu M, Chen Y, Li F, Li D, Dong S, et al.. (2026). Refractory Kawasaki Disease in Infants Complicated with Giant Coronary Artery Aneurysms and Takayasu Arteritis: A Rare Case Report and Literature Review.. Vascular health and risk management. https://doi.org/10.2147/VHRM.S624409