Kawasaki Disease Clinical Presentation
- Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Russell W Steele, MD more...
Most children with Kawasaki disease are brought to medical attention because of prolonged fever. Diagnosis of complete cases requires fever of at least 5 days’ duration (though many believe that the diagnosis can be made earlier in otherwise classic presentations). Affected children are usually more irritable than would be expected for the degree of fever. Antibiotic therapy may have been initiated for other diagnoses, but fever persists.
Parents may note that the fever began abruptly. Baker et al found that nonspecific symptoms commonly precede the onset of fever by several days. In order of decreasing frequency, these included the following :
Decreased oral intake
The clinical presentation of Kawasaki disease varies over time, with the clinical course conventionally divided into 3 stages: acute, subacute, and convalescent (see the image below). Some authors add a fourth, chronic, phase.
Stage 1: acutefebrilestage
The acute stage begins with an abrupt onset of fever and lasts approximately 7-14 days. The fever is typically high-spiking and remittent, with peak temperatures ranging from 102-104°F (39-40°C) or higher. This fever is not responsive to antibiotics or antipyretics and can persist for up to 3-4 weeks if untreated. With appropriate therapy, high-dose aspirin, and intravenous immunoglobulin (IVIG), the fever typically remits within 48 hours.
In addition to fever, signs and symptoms of this phase may include the following:
Nonexudative bilateral conjunctivitis (90%)
Anterior uveitis (70%)
Perianal erythema (70%)
Erythema and edema on the hands and feet; the latter impedes ambulation
Strawberry tongue and lip fissures
Hepatic, renal, and GI dysfunction
Myocarditis and pericarditis
Lymphadenopathy (75%), generally a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm
The mucocutaneous changes and lymphadenopathy are most evident during the acute phase. However, note that erythema and edema of the hands and feet may be the last finding to develop. The diagnosis should be made in this phase.
Stage 2: subacute stage
The subacute stage begins when the fevers have abated, and it continues until week 4-6. The hallmarks of this stage are desquamation of the digits, thrombocytosis (the platelet count may exceed 1 million/μL), and the development of coronary aneurysms. The risk for sudden death is highest at this stage.
Other characteristics of the subacute stage are by persistent irritability, anorexia, and conjunctival injection. Persistence of fever beyond 2-3 weeks may be an indication of recrudescent Kawasaki disease. If fever persists, the outcome is less favorable because of a greater risk of cardiac complications.
Stage 3: convalescent phase
The convalescent phase is marked by complete resolution of clinical signs of the illness, usually within 3 months of presentation. This stage begins with the return to baseline of the acute phase reactants (eg, erythrocyte sedimentation rate, C-reactive protein) and other laboratory abnormalities. During this stage, most of the clinical findings resolve; however, deep transverse grooves across the nails (Beau lines) may become apparent 1-2 months after the onset of fever.
During the convalescent stage, cardiac abnormalities may still be apparent. Smaller coronary artery aneurysms tend to resolve on their own (60% of cases), but larger aneurysms may expand, and myocardial infarction may occur. In patients whose echocardiograms were previously normal, however, detection of new aneurysms is unusual after week 8 of the illness.
This stage is of clinical importance only in patients who have developed cardiac complications. Its duration is of lifetime significance because an aneurysm formed in childhood may rupture in adulthood. In some cases of aneurysms rupturing in adult life, careful reviews of past medical histories have revealed febrile childhood illnesses of unknown etiology.
Because no specific test can be performed for Kawasaki disease and no clinical feature is pathognomonic, the diagnosis of Kawasaki disease is based on the presence of a constellation of clinical findings.[1, 2, 3, 4, 5] The diagnostic criteria established by the American Heart Association (AHA) include fever lasting longer than 5 days (fever is an absolute criterion) and 4 of the 5 main clinical features, after diseases with similar findings have been excluded. The 5 major clinical findings are as follows:
Changes in the peripheral extremities: Initial reddening or edema of the palms and soles, followed by membranous desquamation of the finger and toe tips or transverse grooves across the fingernails and toenails (Beau lines)
Polymorphous rash (not vesicular): Usually generalized but may be limited to the groin or lower extremities
Oropharyngeal changes: Erythema, fissuring, and crusting of the lips; strawberry tongue; diffuse mucosal injection of the oropharynx
Bilateral, nonexudative, painless bulbar conjunctival injection
Acute nonpurulent cervical lymphadenopathy with lymph node diameter greater than 1.5 cm, usually unilateral
The most recent version of the AHA guidelines suggest that if a patient presents with 4 or more of the principal criteria, Kawasaki disease can be diagnosed on day 4 of the fever. Experienced clinicians who have treated many Kawasaki disease patients may establish diagnosis before day 4. Patients who present with coronary artery disease can be diagnosed with Kawasaki disease if they have at least 3 of the 5 major diagnostic criteria.
Kawasaki disease is a diagnosis of exclusion, so other diseases with similar findings (see Differentials) must first be ruled out. Patients with fever who appear to have Kawasaki disease but do not meet diagnostic criteria and have no other identified cause of their illness are said to have atypical or incomplete Kawasaki disease. Laboratory testing supports the diagnosis in these cases (see Workup).
In published cases, 10-45% of patients have incomplete or atypical clinical presentations. The 2 findings that are most commonly absent are cervical lymphadenopathy and polymorphous rash. Cervical lymphadenopathy is the least common of all clinical features, occurring in approximately 40% of cases in the United States; it is more common in Asia. Least likely to be absent are mucous membrane changes, which occur in more than 90% of patients with either typical or atypical forms of the disease.
Japanese researchers have reported a severe form of Kawasaki disease presenting as only fever and cervical lymphadenopathy. These patients are at increased risk of nonresponse to IVIG therapy or development of a coronary artery abnormality.
The erythema, edema, and desquamation in the extremities may limit movement and cause the child to refuse to bear weight. Desquamation of the fingers and toes begins in the periungual region, may involve the palms and soles, and is usually observed 1-2 weeks after the onset of fever. Occurrence is approximately 75%. (See the image below of peeling and erythema.)
Cervical lymphadenopathy is usually greater than 1.5 cm and unilateral. Changes in the lips and oral cavity include pharyngeal erythema, dry/fissured or swollen lips, and strawberry tongue (occurrence is approximately 90%). The tongue is described as a strawberry tongue because of the diffuse erythema and prominent papillae. (See the images below of related oral manifestations.)
Central nervous system findings are reported. Nearly all children with Kawasaki disease are extremely irritable, 33% can present with lethargy, and the neck may be stiff enough to raise suspicion of meningitis. Aseptic meningitis (ie, with cerebrospinal fluid pleocytosis) may be present in half of all patients. Less frequently (and often a temporary finding), patients develop unilateral facial palsy or high-frequency sensorineural hearing loss. Facial palsy has been reported.
Gastrointestinal system findings include diarrhea, vomiting, and abdominal pain in 33% of patients. Rarely, hepatic enlargement and jaundice may occur.
Musculoskeletal system findings occur early in the illness, with patients experiencing diffuse arthralgia that involves multiple joints, both large and small. In the subacute phase, 20-40% of patients present with arthralgia or arthritis, both of which tend to favor the large weight-bearing joints.
Erythema and induration at the site of bacillus Calmette-Guérin (BCG) inoculation is commonly observed in Japan. This is rare in the United States.
In addition to the classic mucocutaneous signs, unusual dermatologic manifestations may include the following :
Erythema multiforme–like lesions
Scarlet fever–like rash
Pustulovesicular skin eruption
Other clinical features of the disease may include the following:
Dehydration from fever and anorexia
Meatitis, vulvitis, urethritis
Peripheral extremity gangrene (extremely rare)
A study that reported on the role of respiratory viruses in the pathogenesis of Kawasaki disease found that more than 40% of children with Kawasaki disease also tested positive for respiratory viruses when they were admitted to the hospital. As a result the authors note that a positive respiratory viral PCR or presence of respiratory symptoms at the time of presentation should not be used to exclude a diagnosis of Kawasaki disease.[69, 70]
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