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Kawasaki Disease

Updated : August 24, 2023





Background

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a rare but serious condition that primarily affects children under the age of five. It is characterized by inflammation of blood vessels throughout the body, particularly in the coronary arteries that supply blood to the heart.

The exact cause of Kawasaki disease is unknown, but it is thought to be related to an abnormal immune response to an infection or other trigger. If left untreated, Kawasaki disease can lead to serious complications, including heart damage and aneurysms, which can be life-threatening. Early diagnosis and treatment are crucial to improve outcomes and prevent complications.

Epidemiology

Kawasaki disease (KD) is a rare condition, but it is one of the leading causes of acquired heart disease in children. It occurs worldwide, with the highest incidence rates reported in Japan, Korea, and other countries in East Asia. The incidence of KD varies by geographic region, with rates ranging from 4.5 to 25 cases per 100,000 children under the age of five. In the United States, the incidence of KD is estimated to be around 19 cases per 100,000 children under the age of five.

The condition is more common in boys than in girls, and it typically affects children under the age of five, with the highest incidence rates observed in children between six months and two years of age. Although the exact cause of KD is unknown, it is thought to be related to an abnormal immune response to an infection or other trigger, and certain genetic factors may also play a role in its development.

Anatomy

Pathophysiology

The exact cause of Kawasaki disease (KD) is unknown, but it is thought to be related to an abnormal immune response to an infection or other trigger. The disease is characterized by inflammation of blood vessels throughout the body, particularly in the coronary arteries that supply blood to the heart.

The pathophysiology of KD involves three distinct phases:

  • Acute phase: The acute phase of KD typically lasts for one to two weeks and is characterized by fever, rash, red eyes, swollen lymph nodes, and redness and swelling of the hands and feet. During this phase, there is widespread inflammation of the blood vessels throughout the body, including the coronary arteries, which can lead to the formation of aneurysms.
  • Subacute phase: The subacute phase of KD typically lasts from two to four weeks after the onset of symptoms. During this phase, the fever subsides, but other symptoms may persist. The coronary arteries may continue to be inflamed, which can lead to the development of aneurysms or other complications.
  • Convalescent phase: The convalescent phase of KD begins when the symptoms have resolved, and it can last for several weeks or months. During this phase, the inflammation of the blood vessels subsides, and the body begins to heal.

The inflammation of the blood vessels in KD can lead to the formation of aneurysms in the coronary arteries, which can result in long-term heart damage or even death. The exact mechanisms that lead to the development of aneurysms are not well understood but may involve a combination of inflammation, immune dysfunction, and genetic factors. Prompt diagnosis and treatment are essential to prevent long-term complications and improve outcomes for children with KD.

Etiology

The exact cause of Kawasaki disease (KD) is unknown, but it is thought to be related to an abnormal immune response to an infection or other trigger. Some researchers believe that KD may be caused by a viral or bacterial infection, although no specific infectious agent has been definitively identified as the cause of the disease.

There is also evidence to suggest that genetic factors may play a role in the development of KD. Certain genes have been identified that are associated with an increased risk of developing the disease. Additionally, KD tends to occur more frequently in people of Asian descent, which suggests that there may be a genetic predisposition to the condition.

Other possible factors that have been implicated in the development of KD include environmental triggers, such as exposure to certain toxins or pollutants, as well as other medical conditions or treatments. However, more research is needed to fully understand the etiology of KD.

Genetics

Prognostic Factors

Several factors can influence the prognosis of Kawasaki disease (KD), including the child’s age, the duration of the illness before treatment, and the presence of certain laboratory and clinical findings. Some of the key prognostic factors for KD include:

  • Age: Infants younger than 6 months and adolescents older than 12 years are at higher risk for complications and a more severe course of the disease.
  • Duration of illness before treatment: Children who are not treated promptly with IVIG and aspirin within 10 days of symptom onset are at higher risk for developing coronary artery aneurysms and other complications.
  • Laboratory findings: Children with elevated levels of certain inflammatory markers, such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white blood cell count, are at higher risk for complications. Children with low levels of albumin, an important blood protein, may also be at higher risk for more severe disease.
  • Clinical findings: Children with more severe symptoms, such as persistent fever, respiratory distress, and gastrointestinal symptoms, may be at higher risk for complications. Children with certain physical exam findings, such as coronary artery abnormalities, may also be at higher risk for long-term complications.

Other factors that may influence the prognosis of KD include the child’s response to treatment, the presence of other medical conditions, and the presence of any complications. With prompt and appropriate treatment, the majority of children with KD recover fully without long-term complications. However, a small percentage of children may develop coronary artery aneurysms or other complications, which can have significant long-term consequences.

Clinical History

Clinical history

The clinical history of Kawasaki disease (KD) typically begins with the onset of a fever that lasts for at least five days and is accompanied by other symptoms. Some of the key features of the clinical history of KD include:

  • Fever: The fever associated with KD typically lasts for at least five days and may be high, reaching 39-40°C (102-104°F).
  • Rash: A rash is one of the most common symptoms of KD, and it typically appears during the acute phase of the illness. The rash is often described as a diffuse, maculopapular rash that may be more prominent in the groin area.
  • Conjunctivitis: Inflammation of the conjunctiva, the thin membrane that lines the inside of the eyelids and covers the white part of the eye, is common in KD and typically appears early in the course of the illness.
  • Redness and swelling of the hands and feet: This is a characteristic feature of KD and is often referred to as “strawberry tongue” or “glove-and-sock” syndrome.
  • Swollen lymph nodes: Enlarged lymph nodes are common in KD and are often found in the neck.
  • Irritability: Children with KD may be irritable and may not want to eat or drink.
  • Abdominal pain: Abdominal pain is common in KD and may be severe enough to be mistaken for appendicitis or another acute abdominal condition.
  • Joint pain: Joint pain and swelling may be present in some cases of KD.

It is important to note that not all children with KD will have all of these symptoms, and some may have additional symptoms that are not listed here. Additionally, the clinical history of KD may vary depending on the age of the child and the severity of the illness.

Physical Examination

Physical examination

The physical examination of a child with Kawasaki disease (KD) typically includes a thorough assessment of the skin, eyes, mouth, hands, feet, heart, and other organs. Some of the key findings on physical examination that may suggest the diagnosis of KD include:

  • Skin rash: A diffuse, maculopapular rash is one of the most common findings on physical examination in KD. The rash may be more prominent in the groin area and may be accompanied by peeling of the skin on the hands and feet.
  • Conjunctivitis: Inflammation of the conjunctiva, the thin membrane that lines the inside of the eyelids and covers the white part of the eye, is common in KD and is typically bilateral and non-purulent.
  • Redness and swelling of the hands and feet: This is a characteristic feature of KD and is often referred to as “strawberry tongue” or “glove-and-sock” syndrome.
  • Oral cavity: Children with KD may have redness and swelling of the lips, tongue, and mucous membranes of the mouth.
  • Cervical lymphadenopathy: Enlarged lymph nodes in the neck are common in KD and are often tender.
  • Cardiac examination: Children with KD may have a rapid or irregular heartbeat, murmurs, or other signs of cardiac involvement.
  • Abdominal examination: Abdominal pain and tenderness are common in KD and may be accompanied by hepatosplenomegaly or other signs of gastrointestinal involvement.

It is important to note that not all children with KD will have all of these findings on physical examination, and some may have additional findings that are not listed here. Additionally, the physical examination of a child with suspected KD should be performed by a healthcare provider with experience in the diagnosis and management of the condition.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential diagnosis

Kawasaki disease (KD) can be difficult to diagnose because its symptoms can resemble those of other childhood illnesses. Some of the conditions that may be considered in the differential diagnosis of KD include:

  • Scarlet fever: This is a bacterial infection that can cause a fever, rash, and other symptoms similar to those of KD.
  • Toxic shock syndrome: This is a rare but potentially life-threatening condition that can cause fever, rash, and other symptoms that are similar to those of KD.
  • Measles: This is a viral infection that can cause fever, rash, and other symptoms that can be mistaken for those of KD.
  • Stevens-Johnson syndrome: This is a rare but serious skin condition that can cause a fever, rash, and other symptoms similar to those of KD.
  • Adenovirus infection: This is a viral infection that can cause fever, rash, and other symptoms that may be similar to those of KD.
  • Juvenile idiopathic arthritis: This is a chronic inflammatory condition that can cause fever, rash, and other symptoms that may resemble those of KD.
  • Rocky Mountain spotted fever: This is a bacterial infection that can cause fever, rash, and other symptoms that can be mistaken for those of KD.
  • Systemic lupus erythematosus: This is an autoimmune disorder that can cause fever, rash, and other symptoms that may be similar to those of KD.

It is important to consider KD in the differential diagnosis of any child with fever and unexplained rash, particularly if there are other clinical features that suggest the possibility of KD, such as redness and swelling of the hands and feet, conjunctivitis, and swollen lymph nodes. Prompt diagnosis and treatment are crucial to prevent complications and improve outcomes for children with KD.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 
 

aspirin

Initial: 80-100 mg/kg orally in divided doses 14 days
Maintenance: 3-6 mg/kg orally as a single dose daily



 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK537163/

Kawasaki Disease

Updated : August 24, 2023




Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a rare but serious condition that primarily affects children under the age of five. It is characterized by inflammation of blood vessels throughout the body, particularly in the coronary arteries that supply blood to the heart.

The exact cause of Kawasaki disease is unknown, but it is thought to be related to an abnormal immune response to an infection or other trigger. If left untreated, Kawasaki disease can lead to serious complications, including heart damage and aneurysms, which can be life-threatening. Early diagnosis and treatment are crucial to improve outcomes and prevent complications.

Kawasaki disease (KD) is a rare condition, but it is one of the leading causes of acquired heart disease in children. It occurs worldwide, with the highest incidence rates reported in Japan, Korea, and other countries in East Asia. The incidence of KD varies by geographic region, with rates ranging from 4.5 to 25 cases per 100,000 children under the age of five. In the United States, the incidence of KD is estimated to be around 19 cases per 100,000 children under the age of five.

The condition is more common in boys than in girls, and it typically affects children under the age of five, with the highest incidence rates observed in children between six months and two years of age. Although the exact cause of KD is unknown, it is thought to be related to an abnormal immune response to an infection or other trigger, and certain genetic factors may also play a role in its development.

The exact cause of Kawasaki disease (KD) is unknown, but it is thought to be related to an abnormal immune response to an infection or other trigger. The disease is characterized by inflammation of blood vessels throughout the body, particularly in the coronary arteries that supply blood to the heart.

The pathophysiology of KD involves three distinct phases:

  • Acute phase: The acute phase of KD typically lasts for one to two weeks and is characterized by fever, rash, red eyes, swollen lymph nodes, and redness and swelling of the hands and feet. During this phase, there is widespread inflammation of the blood vessels throughout the body, including the coronary arteries, which can lead to the formation of aneurysms.
  • Subacute phase: The subacute phase of KD typically lasts from two to four weeks after the onset of symptoms. During this phase, the fever subsides, but other symptoms may persist. The coronary arteries may continue to be inflamed, which can lead to the development of aneurysms or other complications.
  • Convalescent phase: The convalescent phase of KD begins when the symptoms have resolved, and it can last for several weeks or months. During this phase, the inflammation of the blood vessels subsides, and the body begins to heal.

The inflammation of the blood vessels in KD can lead to the formation of aneurysms in the coronary arteries, which can result in long-term heart damage or even death. The exact mechanisms that lead to the development of aneurysms are not well understood but may involve a combination of inflammation, immune dysfunction, and genetic factors. Prompt diagnosis and treatment are essential to prevent long-term complications and improve outcomes for children with KD.

The exact cause of Kawasaki disease (KD) is unknown, but it is thought to be related to an abnormal immune response to an infection or other trigger. Some researchers believe that KD may be caused by a viral or bacterial infection, although no specific infectious agent has been definitively identified as the cause of the disease.

There is also evidence to suggest that genetic factors may play a role in the development of KD. Certain genes have been identified that are associated with an increased risk of developing the disease. Additionally, KD tends to occur more frequently in people of Asian descent, which suggests that there may be a genetic predisposition to the condition.

Other possible factors that have been implicated in the development of KD include environmental triggers, such as exposure to certain toxins or pollutants, as well as other medical conditions or treatments. However, more research is needed to fully understand the etiology of KD.

Several factors can influence the prognosis of Kawasaki disease (KD), including the child’s age, the duration of the illness before treatment, and the presence of certain laboratory and clinical findings. Some of the key prognostic factors for KD include:

  • Age: Infants younger than 6 months and adolescents older than 12 years are at higher risk for complications and a more severe course of the disease.
  • Duration of illness before treatment: Children who are not treated promptly with IVIG and aspirin within 10 days of symptom onset are at higher risk for developing coronary artery aneurysms and other complications.
  • Laboratory findings: Children with elevated levels of certain inflammatory markers, such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white blood cell count, are at higher risk for complications. Children with low levels of albumin, an important blood protein, may also be at higher risk for more severe disease.
  • Clinical findings: Children with more severe symptoms, such as persistent fever, respiratory distress, and gastrointestinal symptoms, may be at higher risk for complications. Children with certain physical exam findings, such as coronary artery abnormalities, may also be at higher risk for long-term complications.

Other factors that may influence the prognosis of KD include the child’s response to treatment, the presence of other medical conditions, and the presence of any complications. With prompt and appropriate treatment, the majority of children with KD recover fully without long-term complications. However, a small percentage of children may develop coronary artery aneurysms or other complications, which can have significant long-term consequences.

Clinical history

The clinical history of Kawasaki disease (KD) typically begins with the onset of a fever that lasts for at least five days and is accompanied by other symptoms. Some of the key features of the clinical history of KD include:

  • Fever: The fever associated with KD typically lasts for at least five days and may be high, reaching 39-40°C (102-104°F).
  • Rash: A rash is one of the most common symptoms of KD, and it typically appears during the acute phase of the illness. The rash is often described as a diffuse, maculopapular rash that may be more prominent in the groin area.
  • Conjunctivitis: Inflammation of the conjunctiva, the thin membrane that lines the inside of the eyelids and covers the white part of the eye, is common in KD and typically appears early in the course of the illness.
  • Redness and swelling of the hands and feet: This is a characteristic feature of KD and is often referred to as “strawberry tongue” or “glove-and-sock” syndrome.
  • Swollen lymph nodes: Enlarged lymph nodes are common in KD and are often found in the neck.
  • Irritability: Children with KD may be irritable and may not want to eat or drink.
  • Abdominal pain: Abdominal pain is common in KD and may be severe enough to be mistaken for appendicitis or another acute abdominal condition.
  • Joint pain: Joint pain and swelling may be present in some cases of KD.

It is important to note that not all children with KD will have all of these symptoms, and some may have additional symptoms that are not listed here. Additionally, the clinical history of KD may vary depending on the age of the child and the severity of the illness.

Physical examination

The physical examination of a child with Kawasaki disease (KD) typically includes a thorough assessment of the skin, eyes, mouth, hands, feet, heart, and other organs. Some of the key findings on physical examination that may suggest the diagnosis of KD include:

  • Skin rash: A diffuse, maculopapular rash is one of the most common findings on physical examination in KD. The rash may be more prominent in the groin area and may be accompanied by peeling of the skin on the hands and feet.
  • Conjunctivitis: Inflammation of the conjunctiva, the thin membrane that lines the inside of the eyelids and covers the white part of the eye, is common in KD and is typically bilateral and non-purulent.
  • Redness and swelling of the hands and feet: This is a characteristic feature of KD and is often referred to as “strawberry tongue” or “glove-and-sock” syndrome.
  • Oral cavity: Children with KD may have redness and swelling of the lips, tongue, and mucous membranes of the mouth.
  • Cervical lymphadenopathy: Enlarged lymph nodes in the neck are common in KD and are often tender.
  • Cardiac examination: Children with KD may have a rapid or irregular heartbeat, murmurs, or other signs of cardiac involvement.
  • Abdominal examination: Abdominal pain and tenderness are common in KD and may be accompanied by hepatosplenomegaly or other signs of gastrointestinal involvement.

It is important to note that not all children with KD will have all of these findings on physical examination, and some may have additional findings that are not listed here. Additionally, the physical examination of a child with suspected KD should be performed by a healthcare provider with experience in the diagnosis and management of the condition.

Differential diagnosis

Kawasaki disease (KD) can be difficult to diagnose because its symptoms can resemble those of other childhood illnesses. Some of the conditions that may be considered in the differential diagnosis of KD include:

  • Scarlet fever: This is a bacterial infection that can cause a fever, rash, and other symptoms similar to those of KD.
  • Toxic shock syndrome: This is a rare but potentially life-threatening condition that can cause fever, rash, and other symptoms that are similar to those of KD.
  • Measles: This is a viral infection that can cause fever, rash, and other symptoms that can be mistaken for those of KD.
  • Stevens-Johnson syndrome: This is a rare but serious skin condition that can cause a fever, rash, and other symptoms similar to those of KD.
  • Adenovirus infection: This is a viral infection that can cause fever, rash, and other symptoms that may be similar to those of KD.
  • Juvenile idiopathic arthritis: This is a chronic inflammatory condition that can cause fever, rash, and other symptoms that may resemble those of KD.
  • Rocky Mountain spotted fever: This is a bacterial infection that can cause fever, rash, and other symptoms that can be mistaken for those of KD.
  • Systemic lupus erythematosus: This is an autoimmune disorder that can cause fever, rash, and other symptoms that may be similar to those of KD.

It is important to consider KD in the differential diagnosis of any child with fever and unexplained rash, particularly if there are other clinical features that suggest the possibility of KD, such as redness and swelling of the hands and feet, conjunctivitis, and swollen lymph nodes. Prompt diagnosis and treatment are crucial to prevent complications and improve outcomes for children with KD.

aspirin

Initial: 80-100 mg/kg orally in divided doses 14 days
Maintenance: 3-6 mg/kg orally as a single dose daily



https://www.ncbi.nlm.nih.gov/books/NBK537163/