Kawasaki disease: symptoms, causes, treatments

The Kawasaki disease is vasculitis multisystem character (Delgado Rubio, 2016), to a specific level, is a pediatric disease that causes inflammation of the arterial walls of the body (Mayo Clinic, 2014).

Clinically, this disease can affect arteries, lymph nodes, mucous membranes and even the nervous system (Mayo Clinic, 2014). Thus,  some of the most common signs and symptoms usually include fever, conjunctival injection, oral abnormalities, skin pathologies, cervical lymphadenopathy,  among others (García Rodríguez et al., 2016).

Kawasaki syndrome

The origin of Kawasaki disease is not yet known with accuracy, however, several ongoing investigations relate its etiological cause with immunological and infectious factors (Arias Cabello, Fernández Álvarez and Ordaz Favila, 2016).

On the other hand, although early identification is complex, confirmation of this disorder is usually made based on clinical criteria related to the clinical and temporal course. Furthermore, additional tests are often performed as blood analysis, the study of cerebrospinal fluid or the echocardiogram (Bou, 2014).

In terms of treatment, therapeutic approaches are aimed at both the treatment of ongoing pathologies and the control and minimization of medical sequelae. Thus, some of the methods used include the administration of gamma globulin, acetylsalicylic acid, corticoid or monoclonal antibodies (Bou, 2014).

Characteristics of Kawasaki disease

Kawasaki disease is a type of vasculitis, it is also a rare or infrequent pathology that affects mainly the pediatric population and,  especially, children under the age of 5 (The Royal Childre’s Hospital Melbourne, 2016).

Vasculitis is a type of disorder that involves a pathological inflammation of the body’s blood vessels, altering the efficient functioning of capillaries, veins or arteries (National Institutes of Health, 2016).

The different structures of our circulatory system are responsible for transporting and delivering blood and, therefore, nutrients and oxygen to the entire body, essential for survival.

Thus, when a factor related to infectious processes, immune system abnormalities or other medical conditions, results in the  development of an inflammatory process in blood vessels, these can be altered in different ways (National Institutes of Health, 2016):

  • Partial or complete closure of the blood channel that hinders the passage of blood to one or several body regions.
  • Distension or weakening of the walls of the blood conduit, facilitating the development of aneurysms or malformations.

In this way, when blood flow fails to reach all organs normally, a wide variety of medical pathologies will occur, which will vary depending on the affected areas.

Some of the medical complications can include cardiac alterations, fever, blood draws due to aneurysms, stroke, among others.

Specifically, the first time that Kawasaki disease was described clinically was in 1961. Dr. Tomisaku Kawasaki referred to the case of a child who was less than 5 years of age and presented asymptomatic group composed of pathological increase in temperature body,  cervical adenopathy, skin rashes, oral abnormalities, anemia, etc., all of which are compatible with a vasculitis (Prego Petit, 2003).

However, this pathology was not defined explicitly until 1967. Kawasaki, in his clinical report, described 50 cases of children with a  clinical course characterized by febrile episodes, adenopathies, mucocutaneous alteration and desquamation of the fingers (Bou, 2014 ).

Although there have been numerous cases in the medical literature, it has not yet been possible to establish the etiological causes that give rise to this pathology. However, its course and clinical criteria have been specified by a multitude of authors and medical institutions.

Statistics

Kawasaki disease is considered one of the most frequent types of vasculitis in the pediatric age (García Rodríguez et al., 2016). Thus, in more than  85% of cases, those affected are less than 5 years of age (Bou, 2014).

However, it is a pathology that can occur at any age, although it is rare during the neonatal phase (before three months) or in the adolescent stage (Prego Petit, 2003).

In addition, a clear pattern of geographic distribution can be observed in Kawasaki disease since it is a pathology that primarily affects the  Asian population (Bou, 2014).

In a recent national survey in the Japanese region, an incidence of approximately 26.9 cases per 100,000 inhabitants was observed in children, especially between 0 and 4 years of age (Arias Cabello, Fernández Álvarez, Ordaz Favila, 2016).

Apart from this, the most recent epidemiological investigations show that, at present, Kawasaki disease presents a more universal distribution, affected to any ethnic or racial group (Bou, 2014).

In addition, from the first descriptions of this pathology until 2015, more than 900 clinical reports have been published related to cases diagnosed with Kawasaki disease (Sotelo-Cruz, 2016).

On the other hand, in terms of gender distribution, it has been observed that it is a predominant pathology in males, with a ratio of 1.5 / 2.1: 1,  compared to females (Bou, 2014).

In the United States, Kawasaki disease is considered the most frequent type of acquired heart disease, specifically, its incidence reaches an approximate figure of 9 to 19 cases per 100,000 children aged less than 5 years (Centers for Disease Control and Prevention, 2016).

Specifically, in the year 2000, there were about 4248 hospitalizations associated with Kawasaki disease, in North American medical centers  (Centers for Disease Control and Prevention, 2016).

Have you read Optic Neuritis: Symptoms, Causes, and Treatments (optic neuritis treatment)?

Characteristic signs and symptoms

Kawasaki disease is considered a multisystemic pathology, mainly due to the variability of areas and bodily organs that can be affected by the inflammation of the circulatory system and consequently, by the reduction of efficient blood flow. However, some of the  most common tend to include (Bou, 2014, Delgado Rubio, 2016, Prego Petit, 2003):

a) Cardiac Manifestations

As we have indicated previously, Kawasaki disease is a type of vasculitis, so the cardinal medical complication of this disorder is inflammation of the walls of the blood vessels.

Specifically, this pathology affects primarily the median arteries, although it is also possible to observe a significant alteration of small and medium-sized blood vessels, including arteries, capillaries, and veins. In addition, one of the most affected arteries is the coronary one, reason why the probability of development of myocardial infarction is high.

In addition, within cardiac manifestations some of the most frequent are:

  • Myocarditis: in this case, the term myocarditis is used to refer to the presence of significant inflammation of the myocardium or cardiac muscle area, as a consequence, various manifestations may appear such as arrhythmia or heart failure.
  • Pericarditis: in this case, the term pericarditis is used to refer to the presence of significant inflammation of the pericardium or tissue that covers the cardiac structures, as a result, there may be various secondary manifestations such as myocardial infarction or myocarditis.
  • Valvulitis: in this case, the term valvulitis is used to refer to the presence of significant inflammation of the heart valves (mitral, aortic, pulmonary and tricuspid), responsible for controlling the direction of blood flow. As a consequence, various pathologies can appear such as valvular heart disease, valvular stenosis, valvular insufficiency or endocarditis.
  • Aneurysms: in this case, the term aneurysm is used to refer to the development of a weak or thinner area in the walls of blood vessels. With the blood flow, the weakened area can acquire a sack shape, protruding and, therefore, with a high probability of rupture and bleeding in any area of ​​the organism. Specifically, one of the most serious types is those that appear in the cerebral circulatory system,  due to the possibility of hemorrhage and mechanical pressure in nervous areas.

b) Eye involvement

Ophthalmological manifestations are one of the most common medical findings in Kawasaki disease, some of them are characterized  by the development of:

  • Conjunctival injection and hyperthermia: the presence of a reddening of the ocular areas, simulating the presence of lines or points of blood material is a common finding in Kawasaki disease. In addition, this type of alterations can produce episodes of eye pain or loss of visual capacity.
  • Conjunctivitis: in this case, the term conjunctivitis is used to refer to the presence of significant inflammation of the transparent tissue that covers both the eyeball and the inside of the eyelid. As a result, various pathologies related to ocular redness, inflammation or episodes of pain can appear.

c) Fever

The pathological increase in body temperature is one of the earliest medical symptoms of Kawasaki disease.

At a clinical level, the body temperature is extremely high, in some cases, they can reach 40 ᵒC or even higher temperatures.

Although it occurs in recurrent episodes and responds to drug treatment, body temperature tends to remain around 38  degrees.

Specifically, the duration of episodes of fever if no type of therapeutic approach is used is usually about two weeks, however, it can reach a longer duration, around 4 weeks.

d) Skin manifestations

The anomaly in the blood circulation can also affect cutaneous and musculoskeletal integrity. Some of the most common pathological findings in Kawasaki disease include:

  • Cutaneous erythema: in this case, the term erythema is used to refer to the presence of focal cutaneous inflammation and reddening of some cutaneous areas. It usually affects the extremities, especially the hands and feet.
  • Edema in the extremities: this pathology is a type of skin disorder that manifests as inflammation and redness that is usually accompanied by the development of bumps, pustules, and fever.
  • Skin flaking: in this case, this cutaneous pathology is characterized by the loss or detachment of the outermost cutaneous layers. It is usually superficial and spontaneous and affects mainly the fingers.

e) Oral manifestations

Buccal involvement is one of the most characteristic findings of Kawasaki disease, some pathologies include:

  • Fissured lips: it is common to observe dryness and significant bleeding of the oral structures, especially accentuated by physical contact.
  • Aframbuesa tongue: the tongue usually has an inflamed appearance, taking a darker color than usual, with an increased size of the taste buds.

f) Lymphatic manifestations

The lymphatic system is primarily responsible for transporting the lymph from the circulatory system to the different body organs.

The lymph is an aqueous substance that comes from cardiac material and whose main function is to act as an intermediary in the exchanges of nutrients.

Thus, in Kawasaki disease, the presence of abnormalities in this system, has as a consequence the development of some pathologies, such as cervical adenoma.

Specifically, in Kawasaki disease it is common to detect the development of cervical adenopathy, that is, an abnormal inflammation of the lymph nodes found at the cervical level.

g) Neurological manifestations

Although the involvement of the nervous system is less common than the previous ones, in many cases various alterations and neurological pathologies can be detected, including meningitis or cerebral aneurysms.

Clinical course

In the typical clinical evolution of Kawasaki disease, three fundamental phases can be distinguished (Delgado Rubio, 2016):

  • Acute or febrile phase: the first phase of the disease usually lasts approximately one to two weeks. It is usually characterized by the presence of febrile episodes, adenopathy, conjunctive injection, erythema of the hands and feet, oral lesions, meningitis, and the development of other alterations related to irritability and anorexia.
  • Subacute phase: after overcoming the first two weeks, some of the above symptoms may persist, especially ocular involvement or adenopathy and, in addition, new ones such as desquamation develop. On the other hand, some secondary medical complications can be observed, including myocardial infarctions or aneurysms.
  • Recovery or convalescence phase: in the final phase of Kawasaki disease the clinical manifestations tend to remit. However, some complications may appear recurrently or require a longer time to resolve, as is the case of aneurysms.

Causes

At present, the specific etiologic cause of Kawasaki disease has not yet been identified (American Heart Association, 2015).

No genetic and hereditary patterns have been observed, however, its clinical course seems to suggest the presence of infectious or immunological etiological factors  (American Heart Association, 2015).

Some of the current hypotheses propose that the presence of an infectious episode, probably respiratory by a viral agent,  results in the development of an inflammatory vascular process in genetically predisposed persons (Boraveli and Chiaverini, 2014).

Diagnosis

The diagnosis of Kawasaki disease is fundamentally clinical, and is based on the following criteria (Delgado Rubio, 2016):

1. Presence of fever for 5 days or more.

2. Presence of conjunctival injection

3. Presence of cervical adenopathy.

4. Presence of cutaneous examine.

5. Presence of labial and buccal anomalies (dry lips, reddened, fissured, pharyngeal erythema, arabinose tongue)

Furthermore, to confirm the diagnosis and rule out other types of medical pathologies, laboratory tests are usually used: echocardiogram, blood analysis, magnetic resonance imaging, urinalysis, cerebrospinal fluid extraction, etc. (Bou, 2014).

Treatment

Kawasaki disease usually responds favorably to treatments prescribed by health professionals (Boraveli and Chiaverini, 2014).

Normally the initial treatment usually lasts about 5 days, however, it can be prolonged if the medical complication persists. This usually uses the intravenous administration of immunoglobulin and acetylsalicylic acid (aspirin) (Boraveli and Chiaverini, 2014).

If the symptoms persist, we start with the parallel administration of corticosteroids, calcineurin inhibitors or plasma exchanges (Boraveli and Chiaverini, 2014).

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