Kawasaki disease

kawasaki ds

1. Dr.MANOJ PRABHAKAR Resident , Dept. of Paediatrics

2. CASE A 2-year-old boy is brought into the emergency room with a complaint of fever for 6 days and development of a limp. On examination, he is found to have an erythematous macular exanthem over his body, ocular conjunctivitis, dry and cracked lips, a red throat, and cervical lymphadenopathy . There is a grade II/VI vibratory systolic ejection murmur at the lower left sternal border. A white blood cell count and differential show predominant neutrophils with increased platelets on smear.

3. Tomisaku Kawasaki

4. Kawasaki disease (KD), formerly known as Mucocutaneous lymph node syndrome and Infantile polyarteritis nodosa , is an acute febrile illness characterized by inflammation of blood vessels throughout the body that primarily affects young children and infants . It is now the most common cause of acquired heart disease in children.

5. Epidemiology • 80% of cases in children < 4 yrs • Males:females = 2:1 Highest incidence occurring in Asian children. • Positive family history in 1% but 13% risk of occurrence in twins . Infants <6 mo and children >5 yr were at the highest risk for CAA • Seasonal variation – More cases in winter and spring but occurs throughout the year

6. Signs & Symptoms • Respiratory – Rhinorrhea , cough, pulmonary infiltrate • GI – Diarrhea, vomiting, abdominal pain , jaundice etc • Neurologic – Irritability, aseptic meningitis, facial palsy, hearing loss • Musculoskeletal – Myositis , arthralgia , arthritis

7. Phases of Disease A) Acute Febrile Phase (1-2 weeks from onset) – characterized by fever and the other acute signs of illness.

8. B) Subacute Phase (lasts about 3 wk ) – associated with desquamation,thrombocytosis , the development of CAA, the highest risk of sudden death in patients in whom aneurysms have developed . C) Convalescent (6-8 wk after the onset of illness.)

9. Desquamation of the fingers Indurative edema of the hands

10. Description of the Rash Appears within the first five days of illness and is truncal , often with accentuation in the groin region  . Most commonly, the rash is erythematous and maculopapular .

11. Diagnostic C riteria Fever of ≥ 5 days duration + four of five criteria 1 . Oropharyngeal changes 2.Changes in peripheral extremities 3.B/L non-purulent conjunctival injection 4.Polymorphous rash 5 . Cervical lymphadenopathy (~75% of cases)

12. Differential Diagnosis VIRAL INFECTIONS • Adenovirus • Enterovirus • Measles • Epstein-Barr virus • Cytomegalovirus BACTERIAL INFECTIONS • Scarlet fever • Rocky Mountain spotted fever • Leptospirosis OTHER • Toxic shock syndromes • Staphylococcal scalded skin syndrome • Drug hypersensitivity reactions • Stevens-Johnson syndrome Behçet disease

13. Cardiovascular Manifestations of Acute Kawasaki Disease • EKG changes – Arrhythmias – Prolonged PR and/or QT intervals – Low voltage – ST-T–wave changes . • CXR– cardiomegaly

14. Coronary Arterial Changes • Vary in severity from echogenicity due to thickening and edema or asymptomatic coronary artery ectasia to giant aneurysms. • May lead to myocardial infarction, sudden death, or ischemic heart disease.

15. Coronary Aneurysms • Patients most likely to develop aneurysms – Younger than 6 months, older than 5 years – Males – Fevers persist for greater than 14 days – Persistently elevated ESR – Thrombocytosis – Pts who manifest s/s of cardiac involvement

16. Coronary angiogram demonstrating giant aneurysm of the left anterior descending coronary artery (LAD) with obstruction and giant aneurysm of the right coronary artery (RCA) with an area of severe narrowing in 6 yr old boy

17. Lab Picture Leukocytosis with neutrophilia Elevated ESR Elevated CRP Anemia Abnormal plasma lipids Hypoalbuminemia ; Hyponatremia Thrombocytosis after week 1 Sterile pyuria Elevated serum transaminases Elevated serum gamma glutamyl transpeptidase

18. Atypical or Incomplete Kawasaki Disease • Present with < 4 of 5 diagnostic criteria • Compatible laboratory findings • Still develop coronary artery aneurysms • No other explanation for the illness • More common in children < 1 year of age

19. Management ACUTE STAGE • IVIG : (2 gm/kg single dose) within 10 days of onset of symptoms. Aspirin 80-100 mg/kg/day divided every 6 hr orally until patient is afebrile for at least 48 hr. CONVALESCENT STAGE • Aspirin 3-5 mg/kg once daily orally until 6-8 wk after illness onset if normal coronary findings throughout course

20. IMPORTANCE : Effective in treating, and preventing cardiac consequences. Significant decrease in new coronary artery abnormalities. Significant reduction in duration of fever. 

21. Biomarkers for Diagnosis of Kawasaki Disease Although the disease is known for a lot of years the diagnosis remains challenging. There are no markers that are specific for Kawasaki disease. Recent studies say that Elevated levels of NT-pro-BNP and IL-17 may serve as a useful adjunct for differentiation of Kawasaki disease, especially in its incomplete form, from other confounding infectious diseases of children.

22. Kawasaki Disease in India Prospectively collated data from several Asian countries have suggested that incidence of KD has continued to increase over the last 20 years. Should be considered proactively in all children (especially infants) with fever beyond 5 days. High time to realize that a large component of this cardiac burden is preventable by early diagnosis and treatment .

23. Information and education campaigns to increase awareness . All children with a history of KD should be counseled regarding a heart-healthy diet, adequate amounts of exercise, tobacco avoidance, and intermittent lipid monitoring.