Kawasaki Disease

Kawasaki disease is a systemic vascular disorder that causes widespread inflammation in small and medium-sized blood vessels. It primarily affects the coronary arteries and mainly occurs in children between one and five years old, although it can also affect adolescents and adults. It is the leading cause of acquired heart disease in children and has a particularly high incidence in individuals of Japanese descent.

It is sometimes referred to as mucocutaneous lymph node syndrome because the inflammation also affects the lymph nodes and the mucous membranes of the mouth, eyes, nose, and throat.

Symptoms

Kawasaki disease and its symptoms usually progress through three phases:

Acute phase: Lasts about ten days.

• Fever above 39°C (102.2°F) lasting at least five days and not subsiding without medication.
• Eye redness within one or two days.
• Rash within five days: red spots typically appearing on the torso, around the diaper area, and on mucous membranes.
• Throat redness.
• Red, dry, and cracked lips.
• Swollen papillae on the tongue.
• Red or purplish coloration of the palms of the hands and soles of the feet.
• Swelling of the hands and feet.

Subacute phase: Lasts between two and four weeks.

• Fever disappears.
• Skin peeling on the fingers after about ten days.
• Swollen lymph nodes.
• Joint inflammation and pain may occur.
• Other clinical manifestations include diarrhea, vomiting, meningitis, and inflammation of the ears, eyes, urethra, liver, and gallbladder.

Convalescent phase: Clinical signs disappear, and the phase lasts until six to eight weeks from the onset of the acute phase.

Causes

The cause of Kawasaki disease remains unknown. Studies suggest an infectious trigger, but no single causative pathogen has been identified. One of the most accepted theories proposes that the disease may be caused by an environmentally triggered infection, leading to an abnormal immune response in genetically predisposed individuals.

Risk Factors

Based on analyzed cases, the risk factors for Kawasaki disease include:

• Age: Children under five years old are more likely to develop it.
• Sex: Slightly more common in males.
• Ethnic background: Much more frequent in individuals of Asian descent.
• Climate: Often occurs in winter and early spring.

Complications

Kawasaki disease complications stem from heart damage when treatment is not administered:

• Coronary artery aneurysm: Bulging of the coronary arteries. These aneurysms can rupture or cause a thrombus, leading to a myocardial infarction and, in rare cases, death. Even if the aneurysms resolve, there is an increased risk of heart problems in adulthood.
• Mitral valve regurgitation: The valve does not close completely, causing some blood to flow backward instead of moving forward. This forces the heart to pump harder to compensate for the leakage, which can lead to heart failure.
• Pericardial effusion: Accumulation of fluid around the heart.

Prevention

Since the exact cause is unknown, there are no known ways to prevent this condition.

What Doctor Treats Kawasaki Disease?

Kawasaki disease is diagnosed and treated by specialists in pediatric rheumatology and pediatric cardiology.