Kawasaki's Disease
Click here to load reader
-
Upload
david-cheng -
Category
Documents
-
view
213 -
download
1
Transcript of Kawasaki's Disease
KAWASAKI DISEASE
DEFINITION Kawasaki disease, is an acute, febrile, self-
limiting systemic vasculitis of unkown origin that almost exclusively affects young children.
Medium sized vessel vasculitis
RISK FACTORS Asian ancestry Age 3 months to 4 years
o Peak age: < 5 years Male sex
PATHOPHYSIOLOGY Kawasaki disease is a systemic vasculitis Most serious complication is the
development of an acute coronary artery vasculitis with dilatation or aneurysm formation.
In the early phase of the disease, there is development of oedema and neutrophil infiltration in the coronary arterial wall, with a rapid transition to mononuclear cells.
This is followed by local production of matrix metalloproteinases that cause destruction of the interanal elastic lamina and media, with progress to fibrous connective tissue replacement of the intima and media, leading to aneurysm formation, scarring and stenosis
HISTORY AND EXAMINATION Characterised by fever (more than 5 days)
Plus 4 out of 5: Polymorphic rash
Bilateral (nonlimbal) scleral injectiono Perilimbal sparingo Non exudative (Dry conjunctivitis is
almost pathognomonic with KD in children
Mucosal erythema with strawberry tongue
Peripheral changes e.g. erythema of the palms or soles, oedema of the hands or feet and in convalescence desquamation
o In the acute phase, erythema of palms and soles and oedema of hands and feet
o In the subacute phase, desquamation of hands and feet
Unilateral cervical lymphadenopathyo Painfulo > 1.5 cm in diameter o non-purulent o usually single
The diagnostic features of Kawasaki disease can occur sequentially and may not all be present at the same time.
NOTE: these children are frequently inconsolably irritable.
DIAGNOSTIC CRITERIA: Warm CREAM fever > 5 d (warm) C – conjunctivitis (dry/non-purulent, bilateral)
(i.e. bilateral scleral injection – non-exudative; perilimbal sparing)
R – rash (polymorphic) E – erythema of palms/soles; edema of hands
& feet (= acute); subacute = desquamation of hands/ feet i.e. peripheral changes
A – adenopathy (unilateral, cervical, painful) M – mucosal involvement (red fissured lips,
strawberry tongue, diffuse redness or oral or pharyngeal mucosa)
Other symptoms or signs may include: Fever and extreme irritability Coronary artery aneurysms Arthritis Uveitis Diarrhoeal illness & vomiting Coryza and cough Hydropic gall bladder
PHASES1. acute phase (as long as fever persists, about
10d) most Dx criteria present irritability, aseptic meningitis, myocarditis,
pericarditis, CHF diarrhoea, gallbladder hydrops, pancreatitis,
urethritis, arthritis
2. subacute phase (resolution of fever, peeling of skin(desquamation), elevated ESR & platelets, usually day 11-21/2nd week of illness) arthritis beau’s lines seen on nail growth
3. convalescent phase (lasts until ESR and platelets normalise, >21d) coronary artery aneurysms aneurysm rupture MI, CHF
PREVALANCE Leading cause of acquired heart disease in
children under 5 years of age
INVESTIGATIONS All patients should have:
o Anti-Streptolysin O Titre (ASOT) and Anti DNaseB
Rule out Streptoccocal infection
o Echocardiography At least twice: at initial
presentation and if negative again at 6-8 weeks
Coronary artery aneursym Look for evidence of
myocarditis, valvulitis or pericardial effusion
o Platelet count Marked thrombocytosis
common in second week of illness
Other investigations that are not as useful but may be seen:
o Neutrophiliao Raised ESR and CRPo Mild normochromic, normocytic
anaemiao Hypoalbuminaemiao Elevated liver enzymes –
transaminases o CXR
If pericarditis or pneumonitis is suspected
o ECG Conduction abnormalities
o Urinalysiso LP
Exclude meningitis
MANAGEMENT Hospital admission 1st line: IV Immunoglobulin
o Preferbaly within 10d of onseto Most useful treatment in reducing
the development of coronary aneurysms (from around 30% to 3%)
o This may be repeated if the fever persists or recrs 36 hours or more after the completion of the initial immunoglobulin infusion
Methylprednisoloneo Used if fever persists after a second
dose of IVIg.
o Methylprednisolone may be repeated daily for up to 3 consecutive days.
1st line: High dose Aspirino 3-5mg/kg once a day for at least 6-
8wkso Used in the acute situation. o Continued until 48 hours after the
fever has resolvedo There is a greater risk of clotting so
aspirin is usedo Low-dose aspirin therapy is
continued during the subacute phase when there is marked thrombocytosis
o Aspirin therapy is continued if coronary artery changes are found
o In the absence of coronary artery changes it is normally continued for 3 months.
COMPLICATIONS Coronary aneurysms that develop in 20% to
25% of untreated patients Myocarditis Pericarditis with small pericardial effusions Gall bladder disease Pneumonitis Valvulitis Arthritis Hepatic dysfunction, rarely jaundice Aseptic meningitis Coronary thrombosis/MI
Bowel ischaemia and necrosis
PROGNOSIS Kawasaki disease, is an acute, self-limiting
illness In untreated patients it is associated with
significant morbidity and mortality Overall the mortality rate is less than 0.5%