Kawasaki Disease - Signs and Symptoms

Signs and Symptoms

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal treatment with paracetamol (acetaminophen) or ibuprofen. It is the most prominent symptom in Kawasaki disease, is a characteristic sign of the acute phase of the disease, is normally high (above 39-40°C), remittent and followed by extreme irritability. Recent reports says it is even present in patients with atypical or incomplete KD, nevertheless recent reports says it is not present on 100% of cases. The first day of fever is considered the first day of illness, and the duration of fever is on average one to two weeks; in the absence of treatment, it may extend for three to four weeks, Prolonged fever is associated with higher incidence of cardiac involvement. It responds partially to antipyretic drugs and does not cease with the introduction of antibiotics. However, when appropriate therapy is started – intravenous immunoglobulin (IVIG) and aspirin – the fever is gone after two days.

Bilateral conjunctival injection was reported by many publications to be the most common symptom after fever. It typically involves the bulbar conjunctivae, is not accompanied by suppuration, and is not painful. It usually begins shortly after the onset of fever during the acute stage of the disease. Anterior uveitis may be present on slit-lamp examination. iritis can occur too. Keratic precipitates is another eye manifestation (detectable by a slit lamp but usually too small to be seen by the unaided eye.

Kawasaki disease presents with set of oral manifestations, the most characteristic changes are the bright red (erythema), swollen lips (edema) with vertical cracking (fissures) and bleeding. The mucosa of the oropharynx may be bright red, and the tongue may have a typical "strawberry tongue" appearance (marked erythema with prominent gustative papillae). These oral manifestations are caused by the typical necrotizing microvasculitis with fibrinoid necrosis.

Cervical lymphadenopathy is seen in approximately 50 to 75% of patients, whereas the other features are estimated to occur in 90% of patients. But sometimes it can be the dominant presenting symptom. According to the definition of the diagnostic criteria, there should be more than one impaired lymph node and > 1.5 cm in diameter. Affected lymph nodes are not painful or little painful, not-fluctuating and non-suppurative; erythema of the neighboring skin may occur. We should be attentive to those children with fever and neck adenitis who do not respond to antibiotics, because Kawasaki disease should be part of the differential diagnoses.

In the acute phase of the disease changes in the peripheral extremities can include erythema of the palms and soles, which is often striking with sharp demarcation, and often accompanied by painful, brawny edema of the dorsa of the hands or feet in the acute phase of the disease, and it is why affected children frequently refuse to hold objects in their hands or to bear weight on their feet. Later during the convalescent or the subacute phase desquamation of the fingers and toes usually begins in the periungual region within two to three weeks after the onset of fever and may extend to include the palms and soles, and around 11% of children affected by the disease may continue skin peeling for many years. 1 to 2 months after the onset of fever deep transverse grooves across the nails may develop (Beau’s lines), and occasionally nail are shed.

The most common cutaneous manifestation is a diffuse macular-papular erythematous rash, which is quite nonspecific. The rash varies over time and is characteristically located on the trunk and may further spread to involve the face, extremities, and perineum. Many other forms of cutaneous lesions have been reported also; they may include Scarlatiniform, popular, urticariform, multiform-like erythema, and purpuric lesions, even micropustules were reported. It can be polymorphic, non-itchy and normally observed up to the fifth day of fever. however it is never bullous or vesicular

The syndrome affecting multiple organ systems, and in the acute stage of KD, systemic inflammatory changes are evident in many organs. Joint pain (arthralgia) and swelling, frequently symmetrical, Also arthritis can occur. Myocarditis, diarrhea, pericarditis, valvulitis, aseptic meningitis, pneumonitis, lymphadenitis, and hepatitis may be present and are manifested by the presence of inflammatory cells in the affected tissues. If left untreated, some symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short.

Other reported non-specific symptoms include cough, rhinorrhea, sputum, vomiting, headache, and seizure.

The course of the disease can be divided into three clinical phases. The acute febrile phase, which usually lasts for 1 to 2 weeks, is characterized by fever, conjunctival injection, erythema of the oral mucosa, erythema and swelling of the hands and feet, rash, cervical adenopathy, aseptic meningitis, diarrhea, and hepatic dysfunction. Myocarditis is common during this time, and a pericardial effusion may be present. Coronary arteritis may be present, but aneurysms are generally not yet visible by echocardiography. The subacute phase begins when fever, rash, and lymphadenopathy resolve at about 1 to 2 weeks after the onset of fever, but irritability, anorexia, and conjunctival injection persist. Desquamation of the fingers and toes and thrombocytosis are seen during this stage, which generally lasts until about 4 weeks after the onset of fever. Coronary artery aneurysms usually develop during this time, and the risk for sudden death is highest during this stage. The convalescent stage begins when all clinical signs of illness have disappeared and continues until the sedimentation rate returns to normal, usually at 6 to 8 weeks after the onset of illness.

There is differences in clinical presentation between adults and children, as adults have more affection of neck lymph nodes (93% of adults versus 15% of children); hepatitis (65% versus 10%), and arthralgia (61% versus 24-38%). Some patients have atypical presentations and may not have the classical symptoms and this occurs in particular in young infants, and those patients are especially at higher risk for cardiac artery aneurysms.