Kawasaki is far more commonly associated with motorbikes than fever. So it’s not surprising that one is met with incredulous looks when one tells parents that their child (often infant) has Kawasaki Disease (Kawasaki Pani in Malayalam). This disease is fairly young in its recognition, though it must have been present earlier too.

A Japanese Paediatrician called Tomisaki Kawasaki wrote up his first collection of cases in 1967 and he had actually seen his first case in 1961! In parallel the evolution of Kawasaki motorcycles is between 1962-1967!!!

What Is Kawasaki Disease or KD?

It’s a systemic vasculitis notable by high grade persistent fever for several days despite antibiotics/ antipyretics. As the day’s pass, a series of other interesting changes happen- a dry, red eye, red tongue, red lips, a painful neck lymph node swelling, swelling of hands and feet and painful arthritis, rashes and other features too.

The vast majority of KD occurs in childhood and in younger children, though it can present at any age. Adult cases form less than 5%, so this is largely a paediatric disease.

The individual features can have similarities to other childhood infections but the pattern of occurrence is the key. These children look sick, irritable, and have very poor intake.

Diagnosis of Kawasaki Disease

It’s a clinical diagnosis, taking a detailed history in a child with continuous fever lasting several days. The clinical features can occur in series and the red eye might be gone when the lips and tongue (strawberry tongue) turn red. Along with this, baseline blood tests will establish high blood counts and very raised markers of inflammation.

As the disease goes on (sometimes in the recovery phase) a fairly characteristic kind of peeling is established starting on the sides of the fingernails. There are specific diagnostic criteria in KD to aid physicians to come to a diagnosis.

Why Is It So Important to Spot KD?

Left undiagnosed and untreated, about a third of these children are prone to developing swelling of the blood vessels to the heart. This can be a very serious life threatening problem both in the short term as well as the long term. Many of us Paediatricians who trained in the 90’s will possibly recall young infants with sudden cardiac death and it’s then that the realisation strikes- was this KD?

In many ways, I will call KD a partial success story. For such a young disease, it is possibly the best recognised paediatric rheumatic disease by Doctors. Not just because the features can be striking, but also because once Tomaisaki Kawasaki’s work began to be known and other nations had started recognising the problem, there was a fantastic awareness set up to get paediatricians to think KD in a febrile child. And thus it’s probably unlikely that there are many paediatricians who haven’t heard of KD.

These children have to be hospitalised, identified early. The younger the child, the less clear it might be- Incomplete and Atypical forms are frequently encountered in young infants, especially. We also know that these are the children who have higher risk for complications in the coronary arteries.

When inflammation is high, some children present in a state of macrophage activation or in a shock state called Kawasaki shock syndrome. The management of these has to be more aggressive.

Treatment for Kawasaki Disease

Along with traditionally used Aspirin (which in high doses is probably looking more decorative these days), IV immunoglobulin infusion has to be given. A continuous infusion of specified dosage. Giving IV IG early on in the disease process (ideally before coronary involvement) hugely reduces the inflammation and controls the vasculitis process and thus, the coronary aneurysm incidence.

As we learn more about KD today, we realise that children who are at higher risk for complications are children who have much higher inflammatory load- young infants, males, atypical and incomplete presentations, etc. It has therefore become equally important to identify the high risk nature of disease in addition to diagnosing KD. There are disease severity scores, mainly from Japanese teams to aid this.

About 15-20% of these children will not respond to the primary treatment above. A good number of these resistant children can actually be predicted by the scores I’ve mentioned. The trend now is to offer upfront combination therapy (ivIG+ Infliximab or ivIG + steroids) to reduce subsequent complications.

Does Every Child Need an Echo for the Diagnosis?

No. But in unclear cases, ECHO by experts can add value to the diagnosis.

When the diagnosis is clear, treatment must not wait till ECHO is done. But referral (to units where Paediatric Cardiologists are present is recommended) early for ECHO and followup ECHO as recommended is necessary.

The principle of primary treatment is to bring down the inflammation and control the vasculitic process and thus reduce the complications. The earlier the diagnosis and understanding of risk and commencement of therapy, the better the mitigation of complications.

Once coronary dilatation has already occurred, the child will require protective medications to reduce complications due to the same.

Children with significant dilatation will need to be followed up by the paediatric cardiologist long term.

What Are the Challenges in Managing Kawasaki Disease?

Making the diagnosis: As parents keep switching doctors because the child's fever isn’t getting better, clinical findings are missed. Parents think Kawasaki Disease is a prolonged fever!

Risk stratification at diagnosis: I would recommend that higher risk for IVIG resistance children are referred earlier or discussed at the outset with a paediatric rheumatologist regarding the treatment protocol.
 

Expense of treatment: IvIG (especially the marked up MRP) is very expensive. And if additional doses are required or Infliximab is required, it can get even more so. Most government medical colleges are usually empowered to provide free of cost to poor patients and public private partnerships could significantly improve the situation.

Keeping the pulse on the problem: Just giving the medicine isn’t everything. Understanding how to recognise improvement or the lack of it and discussing such issues with a paediatric rheumatologist is important. The paediatric rheumatologist will help to control the vasculitis, the paediatric cardiologist will help to alert and manage secondary complications. Working closely with them is important in the higher risk children.

Access to specialists can be difficult but establishing liaisons for when necessary is important. Pick up that phone and call.

The mainstay of KD management involves high-dose aspirin and intravenous immunoglobulin (IVIG), with emerging therapies for treatment-resistant cases. Early treatment, ideally before coronary involvement, can greatly reduce inflammation and subsequent complications. This makes timely diagnosis critical.

Amrita Hospital, Kochi provides exceptional services in managing Kawasaki Disease, offering advanced diagnostic and therapeutic options. Our multidisciplinary team, including paediatric rheumatologists and cardiologists, ensures comprehensive care for children with KD. The hospital is equipped to handle complex cases and offers support for families navigating the challenges of this disease. Increased awareness, prompt diagnosis, risk stratification, and access to specialist care are key to improving outcomes for children with KD.