Kawasaki disease (Penyakit Kawasaki) adalah suatu penyakit yang langka, dan ditemukan pada

anak-anak. Nama lainnya adalah Sindrom Kawasaki atau mucocutaneous lymph node syndrome.
Penyakit ini menyebabkan radang pada pembuluh darah disekujur tubuh penderita. Sekitar 80%
penderita Sindrom Kawasaki ini adalah anak-anak di bawah usia 5 tahun dan paling banyak terdapat
pada usia antar 9-12 bulan. Penyakit ini jarang menyerang anak-anak yang sudah berusia di atas 8
tahun. Dan anak-anak dari etnis Asia beresiko lebih tinggi terkena penyakit ini.

Penyakit ini dinamakan sesuai nama seorang pediatris Jepang bernama Tomisaku Kawasaki, yang
menemukannya pada tahun 1967. Sejak saat itu diketahui berbagai kasus serupa ternyata ditemukan
di berbagai negara lainnya.

Penderita ditandai dengan sebagian dari gejala-gejala berikut:

 Demam tinggi berhari-hari, hingga 39^ C, sedikitnya selama 5 hari berturut-turut dan diikuti
dengan gejala radang kemerahan pada mata (conjunctivitis),
 Telapak tangan dan kaki akan berwarna kemerahan atau bengkak,
 Diikuti kulit terkelupas setelah sekitar 2 minggu sejak penyakit ini terjadi,
 Ruam kemerahan pada tubuh,
 Kadang-kadang disertai pembengkakan kelenjar limpa (cervical lymphadenopathy),
 Radang pada bibir atau membran mucus di mulut,
 Bibir pecah-pecah, kadang terkelupas dan bisa berdarah,
 Radang pada tenggorokan,
 Atau warna merah seperti strawberry pada lidah (strawberry tangue),
 Sakit pada persendian.

Apa yang menyebabkan timbulnya sindrom Kawasaki ini pada anak-anak belum begitu jelas. Ada
yang berpendapat ini disebabkan oleh infeksi virus, teori lain mengatakan penyebabnya adalah
kelainan pada sistem kekebalan tubuh akibat reaksi oleh racun dari lingkungan sekitar. Serta berbagai
versi penyebab lainnya. Baru-baru ini penelitian menunjukkan bahwa sejenis virus yang disebut
coronavirus mungkin berperan atas timbulnya penyakit ini.

Heart Problem

Jika dibiarkan, sindrom Kawasaki dapat menyebabkan kerusakan jantung dan pembengkakkan pada
pembuluh darah menuju jantung (koroner) yang disebut aneurysm. Aneurysm pada arteri koroner
disebabkan oleh radang pada pembuluh arteri tersebut disebut vasculitis. Inilah yang membuat
sindrom Kawasaki menjadi penyebab utama timbulnya penyakit jantung bawaan (PJB) pada anak-
anak di negara berkembang. Sekitar 25% dari penderita akan memiliki masalah pada jantungnya dan
1% akan meninggal, jika tidak mendapat perawatan lebih lanjut.

Bila terjadi aneurysm, perkembangannya akan berlangsung dalam seminggu setelah gejala-gejala
demam dan yang lainnya bermula. Jika tindakan perawatan dilakukan kurang dari 10 hari, maka
komplikasi ke jantung akan dapat dicegah. Itu sebabnya diagnosa awal terhadap penyakit ini akan
sangat banyak membantu.

Berita baiknya adalah, bila mendapat perawatan sesegera mungkin, hanya sekitar 5-10% dari seluruh
kasus sindrom Kawasaki yang akan berkembang menjadi masalah jantung.

Komplikasi lain mungkin saja terjadi, karena vasculitis juga dapat terjadi ditempat lain selain dari
arteri. Berbagai jenis komplikasi lain juga mungkin terjadi akibat sindrom Kawasaki ini namun
kemungkinan kasus-kasus ini terjadi sangat langka.

Berbagai kemungkinan komplikasi dari penyakit kawakasi adalah sebagai berikut:

 Inflamasi (radang) pada pembuluh darah (valculitis).
 Inflamasi pada otot jantung (mycarditis).
 Inflamasi pada selaput pembungkus jantung (pericarditis).
 Inflamsi pada sendi (arthritis).
 Inflamsi pada membran otak dan spinal cord (meningitis).
 Aneurysm pada arteri yang memicu pembekuan darah atau menimbulkan penyakit jantung.
 Arrhythmia atau detak jantung tidak normal akibat adanya kelainan pada katup jantung.
Treatment

Pengobatan yang diberikan kepada penderita tujuan utamanya adalah untuk memperkecil ataupun
mencegah resiko komplikasi ke arah yang lebih serius, selain untuk menyembuhkan gangguan akibat
gejala-gejalanya juga tentunya. Umumnya pengobatan yang diberikan ada 2:

 Aspirin
Aspirin dapat membantu mengurangi radang yang timbul pada aneurysme pada arteri
koroner. Kegunaan lainnya juga untuk mengatasi demam, radang pada persendian, ruam
pada tubuh dan mencegah terjadinya pembekuan darah.

 Intravenous Gammaglobulin
Sejenis antibodi yang diberikan melalui aliran darah. Jika diberikan sesegera mungkin akan
mengurangi resiko kerusakan pada arteri koroner dan mencegah berlanjutnya penyakit ini ke
tingkat yang berbahaya.

Masih ada cara pengobatan lainnya seperti penggunaan cortisone (kortisol), yaitu sejenis hormon
yang dihasilkan oleh korteks adrenal pada ginjal, yang memiliki efek anti inflamasi dan anti alergi.
Bantuan dari obat-obatan lain sejenis ibuprofen dan naproxen juga terkadang digunakan untuk
mengatasi rasa nyeri persendian yang berkepanjangan.

Plasmapheresis atau pergantian plasma digunakan bila aspirin dan gammablogulin tidak berhasil
mengatasi penyakit ini. Ini sangat membantu, karena penyakit kawasaki yang tidak bereaksi terhadap
pengobatan aspirin dan gammablobulin biasanya cukup berbahaya.

Setelah diobati, pada umumnya akan terjadi pengelupasan kulit pada tangan atau kaki. Namun tidak
perlu khawatir, karena peristiwa ini sangat umum terjadi. Yang paling penting adalah memberikan
perhatian yang cukup bagi anak tersebut agar ia merasa nyaman.

Ada beberapa kejadian dimana penyakit ini bisa kambuh setelah beberapa minggu atau beberapa
bulan kemudian. Segeralah hubungi dokter bila ini terjadi.

kawasaki disease is an illness that involves the skin, mouth, and lymph nodes, and most often
affects kids under age 5. The cause is unknown, but if the symptoms are recognized early,
kids with Kawasaki disease can fully recover within a few days. Untreated, it can lead to
serious complications that can affect the heart.

Kawasaki disease occurs in 19 out of every 100,000 kids in the United States. It is most
common among children of Japanese and Korean descent, but can affect all ethnic groups.

Signs and Symptoms

Kawasaki disease can't be prevented, but usually has telltale symptoms and signs that appear
in phases.

The first phase, which can last for up to 2 weeks, usually involves a persistent fever higher
than 104° Fahrenheit (39° Celsius) and lasts for at least 5 days.

Other symptoms that typically develop include:

 severe redness in the eyes

 a rash on the stomach, chest, and genitals
 red, dry, cracked lips
 swollen tongue with a white coating and big red bumps
  sore, irritated throat
 swollen palms of the hands and soles of the feet with a purple-red color
 swollen lymph nodes

During the second phase, which usually begins within 2 weeks of when the fever started, the
skin on the hands and feet may begin to peel in large pieces. The child also may experience
joint pain, diarrhea, vomiting, or abdominal pain. If your child shows any of these symptoms,
call your doctor.

Complications

Doctors can manage the symptoms of Kawasaki disease if they catch it early. Symptoms often
disappear within just 2 days of the start of treatment. If Kawasaki disease is treated within 10
days of the onset of symptoms, heart problems usually do not develop.

Cases that go untreated can lead to more serious complications, such as vasculitis, an
inflammation of the blood vessels. This can be particularly dangerous because it can affect the
coronary arteries, which supply blood to the heart.

In addition to the coronary arteries, the heart muscle, lining, valves, and the outer membrane
that surrounds the heart can become inflamed. Arrhythmias (changes in the normal pattern of
the heartbeat) or abnormal functioning of some heart valves also can occur.

Diagnosis

No single test can detect Kawasaki disease, so doctors usually diagnose it by evaluating the
symptoms and ruling out other conditions.

Most kids diagnosed with Kawasaki disease will have a fever lasting 5 or more days and at
least four of these symptoms:

 redness in both eyes

 changes around the lips, tongue, or mouth
 changes in the fingers and toes, such as swelling, discoloration, or peeling
 a rash in the trunk or genital area
 a large swollen lymph node in the neck
 red, swollen palms of hands and soles of feet

If Kawasaki disease is suspected, the doctor may order tests to monitor heart function (such as
an echocardiogram) and might take blood and urine samples to rule out other conditions, such
as scarlet fever, measles, Rocky Mountain spotted fever, juvenile rheumatoid arthritis, or an
allergic drug reaction.

Treatment

Treatment should begin as soon as possible, ideally within 10 days of when the fever begins.
Usually, a child is treated with intravenous doses of gamma globulin (purified antibodies), an
ingredient of blood that helps the body fight infection. The child also might be given a high
dose of aspirin to reduce the risk of heart problems.
Reviewed by: Joel Klein, MD
Date reviewed: October 2008

Kawasaki's Disease History

Kawasaki syndrome (KS), also known as Kawasaki disease, is an acute febrile illness of
unknown etiology that primarily affects children younger than 5 years of age. KS was first
described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were
reported in Hawaii in 1976.

KS occurs worldwide, with the highest incidence in Japan, and it most often affects boys and
younger children. KS may have a winter-spring seasonality, and community-wide outbreaks
have been reported occasionally. In the continental United States, population-based and
hospitalization studies have estimated an incidence of KS ranging from 9 to 19 per 100,000
children younger than 5 years of age. Approximately 4248 hospitalizations with KS, of which
3277 (77%) were for children under 5 years of age, were estimated among children younger
than 18 years of age in the United States in the year 2000. In 2006, the number of
hospitalizations with KS was 5523 (standard error [SE] 289) and the percentage of children
under 5 years of age remained the same.

SOURCE: CDC

What is Kawasaki's disease?

Kawasaki's disease is an uncommon illness in children that is characterized by high fever of at

least five days' duration together with at least four of the following five findings:

1. Inflammation with reddening of the whites of the eyes (conjunctivitis) without pus

2. Redness or swelling of the hands or feet, or generalized skin peeling

3. Rash

4. Lymph node swelling in the neck

5. Cracking inflamed lips or throat, or red "strawberry" tongue

The above criteria are used to make a diagnosis of Kawasaki's disease. The terminology
"incomplete Kawasaki's disease" is sometimes used for patients with only some features of
classical Kawasaki's disease.

What is mucocutaneous lymph node syndrome?

Mucocutaneous lymph node syndrome is the original name for Kawasaki's disease. The
original name was quite descriptive because the disease is characterized by the typical
changes in the mucous membranes that line the lips and mouth and by the enlarged and tender
lymph nodes.
What are the usual symptoms and signs of Kawasaki's disease?

The usual symptoms and signs of Kawasaki's disease include

 fever,

 reddening of the eyes,

 cracked and inflamed lips and mucous membranes of the mouth with an inflamed
"strawberry" tongue,

 ulcerative gum disease (gingivitis),

 swollen lymph nodes in the neck (cervical lymphadenopathy),

 and a rash that is raised and bright red.

The rash appears in a glove-and-sock fashion over the skin of the hands and feet. The rash
becomes hard, swollen (edematous), and then peels off.

What are the less common findings?

Most of the common symptoms described above will resolve without complications, even if
untreated. Less common findings include inflammation of the lining of the heart (pericarditis),
joints (arthritis), or covering of the brain and spinal cord (meningitis), and the other heart
complications listed below in the section on serious complications. The outlook (prognosis)
for an individual affected is primarily determined by the seriousness of any heart
complications.

What is the difference between Kawasaki's disease and Kawasaki's syndrome?

They are the same. Kawasaki's disease is also referred to as Kawasaki's syndrome. It was first
described in the late 1960s in Japan by the pediatrician Tomisaku Kawasaki.

What causes Kawasaki's disease?

The cause is not known. Microorganisms and toxins have been suspected, but none has been
identified to date. Kawasaki's disease is not felt to be a contagious illness. Genetic factors and
the immune system seem to play roles in the disease, which is characterized by immune
activation.

Who develops Kawasaki's disease?

Kawasaki disease typically affects children under 5 years of age. Only rarely does it affect
children over 8 years of age, in which case it is sometimes referred to as atypical Kawasaki's
disease.
How can Kawasaki's disease cause serious complications?

Children with Kawasaki's disease can develop inflammation of the arteries of various parts of
the body. This inflammation of the arteries is called vasculitis. Arteries that can be affected
include the arteries that supply blood to the heart muscle (the coronary arteries). Vasculitis
can cause weakening of the blood vessels and lead to areas of vessel widening (aneurysms).
Coronary aneurysms have been reported in up to 25% of those with Kawasaki's disease.

Because of the potential for heart injury and coronary aneurysms, special tests are performed
to examine the heart. Children are typically evaluated with an electrocardiogram (EKG) and
an ultrasound test of the heart (echocardiogram). Other arteries that can become inflamed
include the arteries of the lungs, neck, and abdomen. These effects can lead to breathing
problems, headaches, and pain in the belly, respectively.

What is the treatment for Kawasaki's disease?

Kawasaki's disease is treated with high doses of aspirin (salicylic acid) to reduce
inflammation and to mildly thin the blood to prevent blood clot formation. Also used in
treatment is gamma globulin administered through the vein (intravenous immunoglobulin or
IVIG), together with fluids. This treatment has been shown to decrease the chance of
developing aneurysms in the coronary arteries. Sometimes cortisone medications are given.
Persisting joint pains are treated with anti-inflammatory drugs, such as ibuprofen (Advil) or
naproxen (Aleve).

Plasma exchange (plasmapheresis) has been reported as effective in patients who were not
responding to aspirin and gammaglobulin. Plasmapheresis is a procedure whereby the
patient's plasma is removed from the blood and replaced with protein-containing fluids. By
taking out portions of the patient's plasma, the procedure also removes antibodies and proteins
that are felt to be part of the immune reaction that is causing the inflammation of the disease.
Kawasaki's disease that is not responding to the traditional aspirin and gammaglobulin
treatments can be deadly. Medications that block the effects of TNF (tumor necrosis factor),
one of the messenger molecules in the inflammatory response, are being studied for use in
these situations. Examples of TNF-blocking drugs are infliximab (Remicade) and etanercept
(Enbrel). Further research is needed to design treatment programs for those who are failing
conventional treatments. Pentoxifylline (Trental) is also being studied as a possible treatment
for Kawasaki's disease.
Kawasaki disease
From Wikipedia, the free encyclopedia

Kawasaki disease

Classification and external resources

A child showing characteristic lip changes, and red eyes

seen in Kawasaki disease

ICD-10 M30.3

ICD-9 446.1

OMIM 611775

DiseasesDB 7121

MedlinePlus 000989

eMedicine ped/1236

MeSH D009080
Kawasaki disease (KD), also known as Kawasaki syndrome, lymph node syndrome and
mucocutaneous lymph node syndrome,[1] is an autoimmune disease that manifests as a
systemic necrotizing medium-sized vessel vasculitis and is largely seen in children under five
years of age. It affects many organ systems, mainly those including the blood vessels, skin,
mucous membranes and lymph nodes; however, its most serious effect is on the heart where it
can cause severe coronary artery aneurysms in untreated children. Without treatment,
mortality may approach 1%, usually within six weeks of onset. With treatment, the mortality
rate is less than 0.01% in the U.S.[2] There is often a pre-existing viral infection that may play
a role in its pathogenesis.[3] The conjunctivae and oral mucosa, along with the epidermis
(skin), become erythematous (red and inflamed). Edema is often seen in the hands and feet
and one or both of the cervical lymph nodes are often enlarged. Also, a remittant fever, often
40℃ (104°F) or higher, is characteristic of the acute phase of the disease.[4] In untreated
children, the febrile period lasts on average approximately 10 days, but may range from five
to 25 days.[4] The disorder was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.[5]

Contents
[hide]

 1 Classification
 2 Signs and symptoms
o 2.1 Complications
 3 Causes
 4 Diagnosis
o 4.1 Investigations
 5 Treatment
 6 Prognosis
 7 Epidemiology
 8 References
 9 External links

[edit] Classification

Systemic vasculitis is an inflammatory condition affecting both veins and arteries throughout
the body, and is usually caused by a proliferation of cells associated with an immune response
to a pathogen, or autoimmunity.[6] Systemic vasculitides may be classified according to the
type of cells involved in the proliferation, as well as the specific type of tissue damage
occurring within the vein or arterial walls.[6] Under this classification scheme for systemic
vasculitis, Kawasaki disease is considered to be a necrotizing vasculitis (also called
necrotizing angeititis), which may be identified histologically by the occurrence of necrosis
(tissue death), fibrosis, and proliferation of cells associated with inflammation in the inner
layer of the vascular wall.[6][7] Other diseases featuring necrotizing vasculitis include
Polyarteritis nodosa, Wegener's granulomatosis, Henoch-Schönlein purpura and Churg-
Strauss syndrome.[6] Kawasaki disease may be further classified as a medium-sized-vessel
vasculitis, affecting medium and small sized blood vessels,[8][9][10] such as the smaller
cutaneous vasculature (veins and arteries in the skin) that range from 50 to 100µm in
diameter.[11][12] KD is also considered to be a primary childhood vasculitis, a disorder
associated with vasculitis that mainly affects children under the age of 18.[13][14] A recent,
consensus-based evaluation of vasculitides occurring primarily in children resulted in a
classification scheme for these disorders, to both distinguish them and suggest a more
concrete set of diagnostic criteria for each.[14] Within this classification of childhood
vasculitides, Kawasaki disease is, again, a predominantly medium-sized vessel vasculitis.[14]

It is also an autoimmune form of vasculitis,[4] and is not associated with ANCA antibodies,
unlike other vasculitic disorders associated with them, such as wegener's granulomatosis,
microscopic polyangiitis, and Churg-Strauss syndrome.[6][15] This categorization is considered
essential for appropriate treatment.[16]

[edit] Signs and symptoms

(A) Bilateral, non-exudative conjunctivitis with perilimbal sparing - "conjunctival injection". (B)
Strawberry tongue and bright red, swollen lips with vertical cracking and bleeding. (C) Erythematous
rash involving perineum. (D) Erythema of the palms, which is often accompanied by painful, brawny
edema of the dorsa of the hands. (E) Erythema of the soles, and swelling dorsa of the feet. (F)
Desquamation of the fingers. (G) Erythema and induration at the site of a previous vaccination with
Bacillus Calmette-Guérin (BCG). (H) Perianal erythematous desquamation.[4]

Kawasaki disease often begins with a high and persistent fever that is not very responsive to
normal treatment with paracetamol (acetaminophen) or ibuprofen.[17][18] The fever may persist
steadily for up to two weeks and is normally accompanied by irritability.[17][18] Affected
children develop red eyes because of non-suppurative conjunctivitis, iritis[19] and bilateral
anterior uveitis.[20] Inflammation of the mucous membranes in the mouth,[4] along with
erythema (redness), edema (swelling) with fissures (cracks in the lip surface), desquamation
(peeling) and exsudation of the lips are also evident. The oropharynx mucosa has enanthema
and the tongue maintains an unusual red appearance termed "strawberry tongue" (marked
erythema with prominent gustative papillae).[12] Keratic precipitates (detectable by a slit lamp
but usually too small to be seen by the unaided eye), and swollen lymph nodes may also be
present and can be the first manifestation of the disease.[17][21] Rashes occur early in the
disease, and the cutaneous rash observed in patients with KD is non-specific, polymorphic,
non-itchy and normally observed up to the fifth day of fever. Cutaneous exanthema may
comprise macular-papular erythematous and fissure lesions, the most common type, in
addition to urticariform type rash, purpuric, multiform-like erythema.[22] and peeling of the
skin in the genital area, hands, and feet (especially around the nails and on the palms and
soles) may occur in later phases.[17][23] Some of these symptoms may come and go during the
course of the illness. It is a syndrome affecting multiple organ systems, and in the acute stage
of KD, systemic inflammatory changes are evident in many organs.[9] Myocarditis,[24]
pericarditis, valvulitis, aseptic meningitis, pneumonitis, lymphadenitis, and hepatitis may be
present and are manifested by the presence of inflammatory cells in the affected tissues.[9] If
left untreated, some symptoms will eventually relent, but coronary artery aneurysms will not
improve, resulting in a significant risk of death or disability due to myocardial infarction
(heart attack).[12] If treated in a timely fashion, this risk can be mostly avoided and the course
of illness cut short.[25]

Less common manifestations

System Manifestations

Diarrhea, abdominal pain,

vomiting, liver
dysfunction, pancreatitis,
Hydrops gallbladder,
GIT
cholangitis,
intussusception, intestinal
pseudo-obstruction,
ascites, splenic infarction.

Polyarthritis and
MSS
arthralgia.

Myocarditis, pericarditis,
CVS
valvular heart disease.

Urethritis, prostatitis,
cystitis, priapism,
GU Interstitial nephritis,
orchitis, nephrotic
syndrome.

Aseptic meningitis, and

CNS
sensorineural deafness.

Influenza-like illness,
RS plural effusion,
Atelectasis.

Erythema and induration

Skin at BCG vaccine site, Beau's
lines, and finger gangrene.

Source: review,[12] table.[26]

 High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F),[12] The
duration of fever is on average one to two weeks; in the absence of treatment, it may extend
 for three to four weeks.[12] However, when appropriate therapy is started the fever is gone
after two days.[17]
 Red eyes (conjunctivitis) bilateral without pus or drainage, also known as "conjunctival
injection".[19]
 Anterior uveitis.[19]
 Bright red, chapped, or cracked lips.[12]
 Red mucous membranes in the mouth.[12]
 Strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the
back of the tongue.[12]
 Red palms of the hands and the soles of the feet.[12]
 Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the
nails.[4][17]
 Rash which may take many forms, non-specific, polymorphic, non-itchy, but not vesicle-
bullous lesions, and appears on the trunk.[12]
 Swollen lymph nodes (frequently only one lymph node is swollen, and is usually on one side),
particularly in the neck area.[23]
 Joint pain (arthralgia) and swelling, frequently symmetrical, Also arthritis can occur.[12]
 Irritability.[12]
 Tachycardia (rapid heart beat).[12]
 Beau's lines (transverse grooves on nails).[12]
 May find breathing difficult.[12]

[edit] Complications

X-ray showing Aneurysmal enlargement of the coronary arteries, which is a complication in a

Kawasaki syndrome

The cardiac complications are the most important aspect of the disease. Kawasaki disease can
cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and
subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction
(heart attack) even in young children. Overall, about 10–18% of children with Kawasaki
disease develop coronary artery aneurysms with much higher prevalence among patients who
are not treated early in the course of illness. Kawasaki disease and rheumatic fever are the
most common causes of acquired heart disease among children in the United States.[27][28]
[edit] Causes

Like all autoimmune diseases, the cause of Kawasaki disease is presumably the interaction of
genetic and environmental factors, possibly including an infection. The specific cause is
unknown,[29][30][31] but current theories center primarily on immunological causes for the
disease. Evidence increasingly points to an infectious etiology,[32] but debate continues on
whether the cause is a conventional antigenic substance or a superantigen.[33] Children's
Hospital Boston reported that "[s]ome studies have found associations between the occurrence
of Kawasaki disease and recent exposure to carpet cleaning or residence near a body of
stagnant water; however, cause and effect have not been established."[28]

An association has been identified with a SNP in the ITPKC gene, which codes an enzyme
that negatively regulates T-cell activation.[34] An additional factor that suggests genetic
susceptibility is the fact that regardless of where they are living, Japanese children are more
likely than other children to contract the disease.[28] The HLA-B51 serotype has been found to
be associated with endemic instances of the disease.[35]

[edit] Diagnosis
Criteria for Diagnosis of Kawasaki
Disease

Fever of ≥5 days' duration

associated with at least 4† of the
following 5 changes

Bilateral nonsuppurative
conjunctivitis

One of more changes of the

mucous membranes of the upper
respiratory tract, including
pharyngeal injection, dry fissured
lips, injected lips, and
"strawberry" tongue

One or more changes of the

extremities, including peripheral
erythema, peripheral edema,
periungual desquamation, and
generalized desquamation

Polymorphous rash, primarily

truncal

Cervical lymphadenopathy
>1.5 cm in diameter
Disease cannot be explained by
some other known disease
process

†A diagnosis of Kawasaki disease

can be made if fever and only 3
changes are present in
conjunction with coronary artery
disease documented by two-
dimensional echocardiography or
coronary angiography.

Source: Nelson's essentials of

pediatrics,[36] Review[14]

Kawasaki disease can only be diagnosed clinically (i.e. by medical signs and symptoms).
There exists no specific laboratory test for this condition. It is difficult to establish the
diagnosis, especially early in the course of the illness, and frequently children are not
diagnosed until they have seen several health care providers. Many other serious illnesses can
cause similar symptoms, and must be considered in the differential diagnosis, including
scarlet fever, toxic shock syndrome, juvenile idiopathic arthritis, and childhood mercury
poisoning (infantile acrodynia).[citation needed]

Classically, five days of fever[37] plus four of five diagnostic criteria must be met in order to
establish the diagnosis. The criteria are: (1) erythema of the lips or oral cavity or cracking of
the lips; (2) rash on the trunk; (3) swelling or erythema of the hands or feet; (4) red eyes
(conjunctival injection) (5) swollen lymph node in the neck of at least 15 millimeters.

Many children, especially infants, eventually diagnosed with Kawasaki disease do not exhibit
all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease
even if only three days of fever have passed and at least three diagnostic criteria are present,
especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the
diagnosis can be made purely by the detection of coronary artery aneurysms in the proper
clinical setting.

[edit] Investigations

A physical examination will demonstrate many of the features listed above.

Blood tests

 Complete blood count (CBC) may reveal normocytic anemia and eventually thrombocytosis
 Erythrocyte sedimentation rate (ESR) will be elevated
 C-reactive protein (CRP) will be elevated
 Liver function tests may show evidence of hepatic inflammation and low serum albumin

Other optional tests

  Electrocardiogram may show evidence of ventricular dysfunction or, occasionally, arrhythmia
due to myocarditis
 Echocardiogram may show subtle coronary artery changes or, later, true aneurysms.
 Ultrasound or computerized tomography may show hydrops (enlargement) of the
gallbladder
 Urinalysis may show white blood cells and protein in the urine (pyuria and proteinuria)
without evidence of bacterial growth
 Lumbar puncture may show evidence of aseptic meningitis
 Angiography was historically used to detect coronary artery aneurysms and remains the gold
standard for their detection, but is rarely used today unless coronary artery aneurysms have
already been detected by echocardiography.

[edit] Treatment

Children with Kawasaki disease should be hospitalized and cared for by a physician who has
experience with this disease. When in an academic medical center, care is often shared
between pediatric cardiology and pediatric infectious disease specialists (although no specific
infectious agent has been identified as yet).[28] It is imperative that treatment be started as
soon as the diagnosis is made to prevent damage to the coronary arteries.

Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease[38] and is
administered in high doses with marked improvement usually noted within 24 hours. If the
fever does not respond, an additional dose may have to be considered. In rare cases, a third
dose may be given to the child. IVIG by itself is most useful within the first seven days of
onset of fever, in terms of preventing coronary artery aneurysm.

Salicylate therapy, particularly aspirin, remains an important part of the treatment (though
questioned by some)[39] but salicylates alone are not as effective as IVIG. Aspirin therapy is
started at high doses until the fever subsides, and then is continued at a low dose when the
patient returns home, usually for two months to prevent blood clots from forming. Except for
Kawasaki disease and a few other indications, aspirin is otherwise normally not recommended
for children due to its association with Reye's syndrome. Because children with Kawasaki
disease will be taking aspirin for up to several months, vaccination against varicella and
influenza is required, as these infections are most likely to cause Reye's syndrome.[40]

Corticosteroids have also been used,[41] especially when other treatments fail or symptoms
recur, but in a randomized controlled trial, the addition of corticosteroid to immune globulin
and aspirin did not improve outcome.[42] Additionally, corticosteroid use in the setting of
Kawasaki disease is associated with increased risk of coronary artery aneurysm, and so its use
is generally contraindicated in this setting. In cases of kawasaki disease refractory to IVIG,
cyclophosphamide and plasma exchange have been investigated as possible treatments, with
variable outcomes.

There are also treatments for iritis and other eye symptoms. Another treatment may include
the use of Infliximab (Remicade). Infliximab works by binding tumour necrosis factor
alpha.[43]
[edit] Prognosis

With early treatment, rapid recovery from the acute symptoms can be expected and the risk of
coronary artery aneurysms greatly reduced. Untreated, the acute symptoms of Kawasaki
disease are self-limited (i.e. the patient will recover eventually), but the risk of coronary artery
involvement is much greater. Overall, about 2% of patients die from complications of
coronary vasculitis. Patients who have had Kawasaki disease should have an echocardiogram
initially every few weeks, and then every one or two years to screen for progression of cardiac
involvement.

It is also not uncommon that a relapse of symptoms may occur soon after initial treatment
with IVIG. This usually requires re-hospitalization and re-treatment. Treatment with IVIG can
cause allergic and non-allergic acute reactions, aseptic meningitis, fluid overload and, rarely,
other serious reactions. Overall, life-threatening complications resulting from therapy for
Kawasaki disease are exceedingly rare, especially compared with the risk of non-treatment.
There is also evidence that Kawasaki disease produces altered lipid metabolism that persists
beyond clinical resolution of the disease.

[edit] Epidemiology

Kawasaki disease affects boys more than girls with people of Asian ethnicity, particularly
Japanese and Korean most susceptible as well as people of Afro-Caribbean ethnicity. The
disease was rare in Caucasians until the last few decades and incidence rate fluctuates from
country to country.

By far the highest incidence of Kawasaki disease occurs in Japan (175 per 100,000).

However its incidence in the United States is increasing. Kawasaki disease is predominantly a
disease of young children, with 80% of patients younger than five years of age.
Approximately 2,000-4,000 cases are identified in the United States each year.[27][28]

In the United Kingdom, estimates of incidence rate vary because of the rarity of Kawasaki
disease. However Kawasaki disease is believed to affect fewer than 1 in every 25,000
people.[44] Incidence of the disease doubled from 1991 to 2000 however, with 4 cases in per
100,000 children in 1991 compared with a rise of 8 cases per 100,000 in 2000.[45]