Kawasaki disease (KD)

Kawasaki disease (KD):maple_leaf:

:sparkles: mucocutaneous lymph node syndrome and infantile polyarteritis nodosa, is an acute febrile illness of childhood .

:sparkles:KD is a vasculitis with a predilection for the
coronary arteries.

:cyclone:Approximately 20-25% of untreated children develop coronary artery abnormalities (CAA) including aneurysms.

:cyclone:<5% of children treated with intravenous gammaglobulin (IVIG) develop CAA.

:cyclone:KD is the leading cause of acquired heart
disease in children in most developed countries.


The cause of KD remains ((unknown))
:point_right:but certain epidemiologic and clinical features support an infectious origin.

:point_right:A genetic role in the pathogenesis of KD seems likely, as evidenced
by the higher risk of KD in Asian children.

:point_right:linkage studies and genome-wide association studies have identified significant associations between polymorphisms in the ITPKC gene, a T-cell regulator, with increased susceptibility to KD and to more-severe disease.

:bell:immunoglobulin G (FCGR2A) have also been identified as significant variants in KD patients.


KD is a vasculitis affects medium-size arteries.
The coronary arteries are the most commonly involved,also popliteal and brachial arteries.

((A 3-phase process ))to the arteriopathy of KD.

:lollipop:The 1st phase is in the :arrow_right:1st 2 wk of illness begins in the endothelium and moves
through the coronary wall.

:fire: Saccular aneurysms may form from this arteritis.

:lollipop:The 2nd phase is a subacute/chronic vasculitis may last weeks to years .

:fire:results in fusiform aneurysms.

:lollipop:The vessels affected by the subacute/chronic vasculitis cause progressive stenosis.
Thrombi may form in the lumen and obstruct blood flow.


:hole:Fever is characteristically :point_down:
high (≥38.3°C [101°F]),
unresponsive to antibiotics.
The duration of fever without treatment is generally 1-2 wk, but may persist for 3-4 wk.
:hole: the 5 principal clinical criteria of KD are:
:lollipop:bilateral nonexudative conjunctival injection with limbal sparing

:lollipop:erythema of the oral and pharyngeal mucosa with strawberry tongue and red, cracked lips;

:lollipop:edema and erythema of the hands and feet; :lollipop:rash of various forms (maculo-papular, erythema multiforme, or scarlatiniform

:lollipop:nonsuppurative cervical lymphadenopathy, usually unilateral, with node size >1.5 cm
(Table 166-1; Figs. 166-1 to 166-4).

:hole:Perineal desquamation is common in the acute phase.

:bell:Periungual desquamation of the fingers and toes begins(( 2-3 wk ))after the onset of illness .

:hole:Gastrointestinal symptoms (vomiting, diarrhea, or abdominal pain) occur in more than 60% of patients, and at least 1 respiratory symptom (rhinorrhea or cough) occurs in 35%.

Other findings
:hole:* significant irritability
:hole: * urethritis and meatitis with sterile pyuria.
:hole:* arthritis.
Small or large joints may be affected, and the arthralgias

KD can be divided into 3 clinical phases:
:maple_leaf:The acute febrile phase is
:v:fever + acute signs of illness and usually
lasts 1-2 wk.

:maple_leaf:The subacute phase 🤷
the development of CAA, the highest risk of sudden death from aneurysms
lasts about 3 wk.

:maple_leaf:The convalescent phase
begins when all clinical signs have disappeared and continues until (ESR) returns to normal.
typically about ((6-8 )) Wk after the onset of illness.