Pericarditis: Cause Symptoms Diagnosis Treatment Complication

  Kawasaki disease 

Kawasaki disease (KD) is an acute self-limiting vasculitis, of the small- and medium-sized arteries. The diagnosis is clinical and should be considered in the differential diagnosis of a childhood rash because of the potentially serious complications. Coronary complications are reduced by the use of intravenous immunoglobulin plus aspirin.

Epidemiology of Kawasaki disease

Kawasaki disease is one of the commonest childhood vasculitides. The typical age at presentation is 6 months to 5 years of age. It is rare in adolescents, adults, and children younger than 6 months.

Aetiology of Kawasaki disease

The aetiology is unknown. However, there are two current hypotheses. The first is that KD is caused by an infectious agent, such as Propionibacterium acnes, Rickettsiae, Epstein–Barr virus, or retrovirus. The second is that the patient is genetically or immunologically susceptible to generating an immune-mediated response to a superantigen of an infectious agent.

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Clinical features of Kawasaki disease

Diagnosis is made using the Centers for Disease Control and Prevention guidelines and must include, as the presenting symptom, a fever of 5 days or more without other explanation (which occurs in at least 95% of patients) plus at least four of five of the following:

•  A widespread rash that may present with a variety of morphologies (polymorphic exanthem).

•  Changes of peripheral extremities:

•  Acute phase: erythema and/or indurative oedema of the palms and soles.

•  Convalescent phase: desquamation from finger tips.

•  Bilateral non-exudative conjunctival injection.

•  Changes in the oropharynx, such as injected or fissured lips, a ‘strawberry tongue’, and an injected pharynx.

•  Acute non-suppurative cervical lymphadenopathy (>1.5 cm in diameter).

The fever is of sudden onset and may spike several times a day. Usually, the polymorphous widespread rash is present at onset and occurs predominantly on the trunk and extremities. The rash may be scarlatiniform (i.e. innumerable small red papules that are widely and diffusely distributed), generalized erythema, a papular rash, or erythema multiforme like. There may be fine pustules on the soles of the feet, with associated erythema and oedema. Fusiform swelling of the digits may be present.

Pericarditis: Cause Symptoms Diagnosis Treatment Complication

Other clinical features include:

•  General:

•  Swollen hands and feet, crusted lips.

•  Cardiovascular:

•  Cardiac murmurs, gallop rhythm, cardiomegaly, ECG changes, pericardial effusion, coronary and peripheral artery aneurysms, angina, and myocardial infarction.

•  Central nervous system:

•  Irritability, lethargy, meningism, and cranial nerve palsies.

•  Gastrointestinal:

•  Vomiting, diarrhoea, abdominal pain, and paralytic ileus.

•  Respiratory:

•  Cough, rhinorrhoea.

•  Musculoskeletal:

•  Arthralgia and arthritis, usually large joints.

The differential diagnosis includes:

•  Measles—Koplik’s spots (white spots on buccal mucosa), exudative conjunctivitis, and cough. The rash starts behind the ears.

•  Scarlet fever—streptococcal pharyngitis and rapid response to penicillin.

•  Toxic shock syndrome—hypotension and renal impairment.

•  Staphylococcal scalded skin syndrome—hypotension and renal impairment, responds to flucloxacillin.

•  Erythema multiforme.

•  Stevens-Johnson syndrome—mucosal involvement.

•  Drug reactions.

•  Juvenile rheumatoid arthritis—evanescent salmon-pink rash.

•  Rocky Mountain spotted fever.

•  Leptospirosis.

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Investigations of Kawasaki disease

Laboratory findings are non-specific. During the first week, the following may be present:

•  Neutrophil leucocytosis.

•  Mild normochromic normocytic anaemia.

•  High ESR.

•  Raised C reactive protein.

•  Raised alpha-1-antitrypsin.

•  Sterile pyuria.

A high platelet count develops in the second week and coincides with the highest risk of coronary artery thrombosis.

Patients with ECG abnormalities (PR-QT prolongation, abnormal Q, low voltage, ST-T changes, and dysrhythmias) require echocardiography to assess ventricular function and to detect any pericardial effusion.

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Treatment of Kawasaki disease

Intravenous gammaglobulin decreases the incidence and severity of coronary artery lesions. It is given as a single infusion of 2 g/kg body weight. Aspirin at a dose of 80–100 mg/kg per day during the acute febrile phase, can be reduced to 3–5 mg/kg per day when the fever subsides. Antibiotics and corticosteroids, as a monotherapy are contraindicated, as they are associated with a worse cardiac outcome. Corticosteroids may be beneficial as an adjunctive treatment.

Prognosis

Most cases, if untreated, will resolve without sequelae. However, 25% develop coronary artery abnormalities, with a 1–2% mortality rate in the acute phase.