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Kawasaki disease
- 1. Kawasaki Disease: Dr.B.BALAGOBI
- 2. KAWASAKI DISEASE • Vasculitis of unknown etiology • Multisystem involvement and inflammation of small and medium sized arteries with aneurysm formation • More common among children of Asian decent • Usually children <5 years; peak 2-3 years
- 3. • KAWASAKI DISEASE In children < 3 months of age – Usually see atypical course leading to rapid and severe coronary artery damage (CAD) – ECHO mandatory if considered in this age group; diagnosis very difficult • Age is independent risk factor for CAD • CAD develops in 5% of timely treated patients • Prolonged fever is hallmark of the disease • Lymphadenopathy is least common finding (seen in 75% of cases compared with 90% for other signs) • Coronary lesions are usually not present until 10 days; therefore decision to treat made prior to knowledge of cardiac outcome • Other useful signs – Extreme irritability – Inflammation of BCG scar
- 4. Etiology • Infectious agent most likely – Age-restricted susceptible population – Seasonal variation – Well-defined epidemics – Acute self-limited illness similar to known infections • No causative agent identified – Bacterial, retroviral, superantigenic bacterial toxin – Immunologic response triggered by one of several microbial agents
- 5. KAWASAKI DISEASE – CLINICAL PRESENTATION • Acute phase (1-2 weeks) – Sudden onset of high fever followed by conjunctival erythema, mucosal changes, cervical adenopathy, swelling of hands and feet – Irritability – Abdominal pain, hydrops of gall bladder – Arthritis – Carditis – tachycardia, CHF, giant coronary artery aneurysms
- 6. KAWASAKI DISEASE – CLINICAL PRESENTATION • Subacute phase – Lasts up to 4th week – Resolution of fever and other symptoms – Desquamation of fingers and toes – Elevation of platelet count – Coronary artery aneurysms • Convalescent phase – Disappearance of clinical symptoms – 6-8 weeks after initial symptoms
- 7. Diagnostic Criteria • Fever for at least 5 days • At least 4 of the following 5 features: 1. Changes in the extremities Edema, erythema, desquamation 2. Polymorphous exanthem, usually truncal 3. Conjunctival injection 4. Erythema&/or fissuring of lips and oral cavity 5. Cervical lymphadenopathy • Illness not explained by other known disease process
- 8. Trager, J. D. N Engl J Med 333(21): 1391. 1995.
- 10. Trager, J. D. N Engl J Med 333(21): 1391. 1995.
- 11. ANGULAR CHELITIS
- 14. DESQUAMATION OF THE SKIN
- 17. • Infectious Differential Diagnosis – Measles & Group A beta-hemolytic strep can closely resemble KD – Bacterial: severe staph infections w/toxin release – Viral: adenovirus, enterovirus, EBV, roseola – Spirocheteal: Lyme disease, Leptospirosis – Parasitic: Toxoplasmosis – Rickettsial: Rocky Mountain spotted fever, Typhus • Immunological/Allergic – JRA (systemic onset) – Atypical ARF – Hypersensitivity reactions – Stevens-Johnson syndrome
- 18. KAWASAKI DISEASE - COMPLICATIONS • Coronary artery thrombosis and coronary and peripheral artery Coronary artery thrombosis and coronary and peripheral artery aneurysm • Myocardial infarction • Myopericarditis • Congestive heart failure • Hydrops of gall bladder • Aseptic meningitis • Arthritis • Sterile pyuria (urethritis) • Thrombocytosis • Diarrhea • Pancreatitis • Peripheral gangrene
- 21. Kawasaki Disease: Labs • Late • Early – Thrombocytosis – Leukocytosis – Elevated CRP – Left shift – Mild anemia • EKG changes – Thrombocytosis – Arrhythmias – Elevated ESR – Abnormal Q waves – Elevated CRP – Prolonged PR and/or QT – Hypoalbuminemia intervals – Elevated transaminases – Low voltage – Sterile pyuria – ST-T–wave changes. • CXR–cardiomegaly
- 22. KAWASAKI DISEASE - TREATMENT • IV Immunoglobulin (mechanism unknown) – Single dose of 2 g/kg over 12 hours • Rapid defervescence and symptom resolution • Reduces incidence of coronary artery aneurysm • Aspirin 80-100 mg/kg/day divided q 6 hours for 48 hours then reduce by ½ – Continue for 6-8 weeks until cardiac ECHO shows no evidence of cardiac pathology
- 23. T/F features of kawasaki disease? A. Coronary artery aneurysm commonly occurs during acute phase. B. High dose Aspirin is used during acute phase. C. Cause polycythaemia D. Desquamation of the skin is a feature. E. Common after 10 years of age
- 24. T/F regarding Kawasaki disease – Thrombocytosis is seen within 7days of the illness – Erythema and induration at the BCG immunization site may be seen – Early treatment with aspirin intravenous globulins will reduce the development of coronary arterial aneurysms – Desquamation may involve palms and soles – Pericarditis and pericardial effusion is a recognized complication
Notas del editor
- A number of each of these has been implicated in individual cases or clinical series but typically data are conflicting. A variant strain of Propionibacterium acnes with dust mites playing a role as vectors has been proposed as the causative agent by several investigators but no causative link has been established despite multiple investigations.
- The diagnosis of Kawasaki Disease requires fever persisting at least 5 days (although US and Japanese experts now agree that only 4 days of treatment are necessary before initiating treatment) and the presence of at least 4 of the following 5 principal features: changes in extremities, a polymorphous rash, conjunctival injection, changes in lips and oral cavity, and cervical lymphadenopathy. In addition, other diagnoses must also be ruled out. For example scarlet fever, toxic shock syndrome, and adenoviral infections have similar presentation. The fever is usually high, greater than 102°F, does not respond well to antipyretics, and can last 1-2 weeks. The important thing to remember though is that Kawasaki disease is a diagnosis of exclusion without confirmatory tests s and based on a constellation of signs and symptoms and/or consistent ultrasound findings
- Acute changes seen in the hands and feet are usually seen several days into the course. There is erythema and edema of hands and feet, usually on the dorsal surfaces. Children may also refuse to walk or have trouble putting on their shoes. Erythema of palms & soles &/or painful edema of hands/feet Usually start 3-5 days after onset of fevers Subungual desquamation in subacute phase Beau lines may be seen 2-3 months after onset
- In the first week, patients can develop bilateral, painless bulbar conjunctival injection without exudate. There tends to be no redness adjacent to the pupil and these patients may also have anterior uveitis which is shown on acute phase slit lamp exam in 80% In the first week, they may also have erythema and cracking of lips, a strawberry tongue, and/or diffuse injection of oral and pharyngeal mucosae.
- An abdominal ultrasound was obtained which revealed an extremely distended gallbladder (11 cm) without wall thickening, and no visible stone nor enlargement of the common bile duct or biliary tree. This picture suggested gallbladder hydrops.
- Angiography is the gold standard and can define proximal and distal coronary anatomy better than echocardiography. However, performing angiography on all patients is unreasonable. In addition, angiography may miss abnormalities in the arterial wall, including thromboses or dysfunction.
- In acute phase, the most common blood laboratory findings are: a leukocytosis with left shift, mild anemia, an increased erythrocyte sedimentation rate or C-reactive protein, hypoalbuminemia, elevation of liver transaminases Later in the illness, the platelet count tends to rise during the second week and can remain elevated for longer than 6-8 weeks. Finally, the urine can show sterile pyuria of urethral origin and occasional proteinuria.