Disease of Kawasaki

Description:

Disease of Kawasaki - the system arteritis affecting large, average and small arteries, which is combined with a skin and mucous lymphatic syndrome.
For the first time the disease is described by Kawasaki in 1967 in Japan. Meets mainly at early children's age, it is preferential among representatives of yellow race.

Symptoms of the Disease of Kawasaki:

Distinguish three periods of a disease: acute - 1-1.5 weeks; subacute - 2-4 weeks; the recovery period - from several months to several years.

Main displays of a disease following.

    * Fever - without treatment proceeds about 2 weeks. Its longer existence is considered an adverse predictive factor.
    * Damage of skin and mucous membranes:
          - in the first 5 weeks from the beginning of fever polymorphic rash in the form of urtikarny eczema, makulopapulezny elements with localization preferential on a trunk, proximal departments of extremities, in a crotch develops; further there are an erythema, consolidation of skin in the field of soles and palms (very painful), limiting mobility of fingers; in 2-3 weeks in the field of erythematic sites the plentiful peeling begins;
          - within a week from an onset of the illness bilateral conjunctivitis develops, at some patients the combination to a front uveitis is possible;
          - hyperemia and dryness of a mucous membrane of an oral cavity, crack and bleeding of lips, reddening and increase in almonds, language brightly red (crimson).
    * Damage of lymph nodes - is most often observed one - or bilateral increase in cervical lymph nodes.
    * Defeat of cardiovascular system:
          - myocarditis (tachycardia, a cantering rhythm, heart arrhythmias, a cardiomegaly) often leads to development of an acute heart failure;
          - pericardis (seldom);
          - in 5-7 weeks from the beginning of fever are found in 25% of patients dilatation and aneurism of coronary arteries in proximal departments, development of a myocardial infarction is possible; formation of aneurisms is possible also in subclavial, elbow, femoral, ileal arteries; mitral or aortal insufficiency forms (seldom).
    * Damage of joints (at 1/3 patients) is shown by arthralgias or polyarthritises of small joints of brushes, feet, knee and ankle joints; usually articulate syndrome proceeds about 3-4 weeks.
    * The gastrointestinal tract disease is shown by abdominal pains, vomiting, diarrhea (not at all patients).
    * In certain cases there are an urethritis, aseptic meningitis.
Diagnostic criteria (Rauch, Hurwitz are offered):

  1. Fever not less than 5 days.
  2. Bilateral reddening of a conjunctiva.
  3. Change of mucous membranes of upper respiratory tracts; reddening of almonds, lips, dryness, hypostasis of lips, "crimson" language.
  4. Changes of peripheral departments of extremities: hypostasis, erythema, skin peeling.
  5. Polymorphic (not vesicular) rash, it is preferential on a trunk.
  6. Cervical lymphadenopathy.

Development of the specified signs should not be connected with any other disease. The diagnosis of a disease of Kawasaki is considered reliable with 5 of 6 criteria.

Etiologies of Kawasaki:

As a possible etiological factor the role of an infectious factor - viruses Epstein-Burra, herpes, retroviruses, rickettsiae is discussed. The great value of genetic predisposition is not excluded. An immunogenetic marker of a disease is HLA BW22 antigen (in Japan). The main mechanism of a course of a disease of Kawasaki is the immuno-complex inflammation (antibodies to an endothelium of arteries are developed). On a membrane of an endothelium there is a Kawasaki antigen with which antibodies interact.

Treatment of the Disease of Kawasaki:

In 2002 accurate recommendations for the purpose of assistance to the British pediatricians in the choice of reasonable maintaining patients with a disease of Kawasaki were developed. These are the instructions based on the available proofs. From the moment of the publication of these recommendations new data on use of VVIG (intravenous immunoglobulin), aspirin and corticosteroids appeared. Bystry recognition of a disease and early treatment are necessary for the purpose of decline in mortality.
It is emphasized as incomplete cases can be subjected to treatment, there are assumptions that they lead to changes of coronary arteries more often, than full cases.
Recommendations are oriented to children about one year because of the peak of incidence at this age, however authors recommend that all incomplete cases were treated, at the discretion of the doctor; children of more advanced age and all incomplete cases also have to be in the field of use of these recommendations.