Diagnostic value of the reaction at the bacillus calmette-guérin vaccination site in kawasaki disease / Utilidade diagnóstica da reação do sítio de vacinação bacillus calmette-guérin na doença de kawasaki

ABSTRACT Objective: To describe the case of an infant - diagnosed with incomplete Kawasaki disease - who developed BCG scar reactivation. Case description: A 6-month-old patient was admitted to hospital with fever associated with ocular hyperemia, cervical lymphadenopathy, and hyperemic lips, and remained hospitalized for 12 days. The physical examination revealed an inflammatory reaction at the site of the BCG scar, leading to the diagnosis of incomplete Kawasaki disease. The patient was treated with venous immunoglobulin, but presented recurrence of Kawasaki disease, with subsequent onset of coronary artery disease. Comments: BCG scar reactivation is an important finding in countries where the vaccine is routinely given and may be a useful marker for early diagnosis of Kawasaki disease, especially in its incomplete form.

RESUMO Objetivo: Descrever o caso de um lactente - com diagnóstico de Doença de Kawasaki incompleta - que desenvolveu reativação da cicatriz da vacina BCG. Descrição do caso: Um paciente de 6 meses de idade foi admitido no hospital com febre, associada à hiperemia ocular, linfadenomegalia cervical e fissuras labiais, permanecendo hospitalizado por 12 dias. Apresentava, no exame físico, reação inflamatória no local da cicatriz da vacina BCG, tendo sido feito o diagnóstico de Kawasaki incompleto. O paciente foi tratado com imunoglobulina venosa, mas apresentou recorrência da doença, com posterior surgimento de coronariopatia. Comentários: A reativação da BCG é um achado importante na doença de Kawasaki em países onde a vacina é aplicada de forma rotineira e pode ser um marcador útil para o diagnóstico precoce da doença de Kawasaki, principalmente em sua forma incompleta.

Production and Expression of Gro-alpha and RANTES by Peripheral Blood Mononuclear Cells Isolated from Patients with Kawasaki Disease and Measles

We investigated whether the production and gene expression of Gro-alpha and RANTES in Kawasaki disease differ in measles. Forty-two samples from 14 patients in different clinical stages of Kawasaki disease, eight samples from 8 patients in the acute stage of measles and seven samples from 7 healthy children were collected. The present study was performed using ELISA and RT-PCR for the productions and gene expression of the chemokines. The production of Gro-alpha was markedly elevated during the acute stage of measles compared with Kawasaki disease. Moreover, the expression of Gro-alpha was increased in every case of measles, but not in Kawasaki disease. The production of RANTES was elevated in the acute stage of both diseases when compared to the healthy control. However, the plasma RANTES level did not change significantly according to the clinical stages of Kawasaki disease. A correlation between the production and gene expression of RANTES and Gro-alpha was not found in Kawasaki disease. These results suggest that Kawasaki disease differs from measles with regard to Gro-alpha production and expression, but not RANTES. Gro-alpha might play an important role in the acute stage of measles, however not in Kawasaki disease. Further studies are needed to clarify the efficacy of Gro-alpha as a marker in measles.

Enfermedad de Kawasaki. Evaluación inmunológica de 26 casos / Kawasaki disease. Immunological evaluation of 26 cases

La enfermedad de Kawasaki (EK) es una vasculitis aguda febril de la infancia, caracterizada por mútiples signos clínicos y bioquímicos de la inflamación con especial compromiso del corazón. La activación de linfocitos y monocitos/macrófagos y sus produtos solubles secretados, citoquinas , juegan un papel central en la patogénesis de la enfermadad. En este estudio realizamos estudios de laboratorio inmunológico en 26 pacientes con EK. En 22 pacientes en etapa aguda medimos niveles séricos de IgG, IgA, IgM, fracciones del complemento C3 y C4 por Nefelometría, sin encontrar un patrón constante y los anticuerpos FAN y ANCA por imunoflorescencia indirecta fueron negativos. En células mononucleares periféricas de 25 pacientes en etapa aguda encontramos porcentajes variables de CD3, CD4, CD8, CD20, CD56 y DR mediante tinción específica y análisis por cimetría de flujo. El porcentaje de CD25 estuvo elevado en 17/25 casos. Los níveles séricos de TNFalfa medidos poe ELISA en 12 pacientes , fueron bajos. Evaluamos citoquinas intracelulares TNAalfa, IL1beta, IL2, e IFNgamaen células mononucleares periféricas en 15 pacientes en etapas agudas, subagudas y de covalecencia, utilizando tinción específica y análisis por cimetría de flujo, sin encontrar un perfil característico. Dos pacientes mostraron porcentajes elevados de TNFalfa e IL1beta en monocitos durante la etapa de covalecencia, ambos presentaron secuelas coronarias. Es necesario realizar investigación adicioal acerca de este hallazgo. En conclusión, la evaluación inmunulógica mostró un perfil heterógeneo y no se encontró ningún factor de laboratorio en la etapa aguda que sea predictivo de compromiso cardivascular.

Avaliaçäo clinico-laboratorial na doenca de Kawasaki e marcadores da formacao de aneurisma coronariano / Clinical and laboratorial trends in Kawasaki disease and development of coronary artery aneurisms

A Doenca de Kawasaki destaca-se pelas potenciais sequelas cardiovasculares, caracterizadas pela formacao de aneurisma coronariano. O objetivo do presente trabalho e descrever as caracteristicas dos criterios clinicos, das manifestacoes associadas, das alteracoes laboratoriais e das complicacoes cardiovasculares da Doenca de Kawasaki em 36 pacientes, acompanhados no Instituo da Crianca do Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo. As seguintes caracteristicas clinicas estiveram presentes na...

Superantígenos y su rol en las enfermedades infecciosas / The role of superantigens in infectious diseases

Exogenous antigens are presented to T lymphocytes through mechanisms that ensure high recognition specificity. Recently described superantigens in contrast to conventional antigens are particles that follow a different processing and presentation route not binding to a specific region of T lymphocyte receptors. These particles bind to a large number of T lymphocytes, generating a disproportionate and non-specific immune response. Two types of superantigens have been described. Endogenous superantigens, transported in the host genoma, have been involved in clonal depletion and immunological tolerance phenomena. Exogenous superantigens, mainly bacterial toxins, have been involved in several diseases. There is evidence that these antigens participate in diseases such as Kawasaki disease, toxic shock caused by Staphylococcus aureus, rheumatoid arthritis, HIV infection and Streptococcus pyogenes infections

Decreased CD5+ B cells during the acute phase of Kawasaki disease

We investigated the changes of CD5+ B cells in the peripheral blood of 20 Kawasaki disease (KD) patients. The percentage of CD5+ B cells in the total lymphocytes and in the total B cells significantly decreased during the acute phase of KD(p< 0.01), compared to that in the age-matched normal control subjects. After intravenous immunoglobulin(IVIG) treatment, the percentage of CD5+ B cells increased, but was still lower than that in the normal controls(p< 0.01). During the convalescent phase of the disease, the percentage of CD5+ B cells was restored to the normal levels. The levels of CD5+ B cell percentage in the total B cells of the patients with acute febrile disease showed similar levels to age-matched normal controls. The decreased CD5+ B cells in the patients with KD provides an additional abnormal immunological finding during the acute phase of the disease.

Effects of intravenous immune globulin on the peripheral lymphocyte phenotypes in Kawasaki disease

The effect of intravenous immune globulin (IVIG) on the lymphocyte phenotypes in acute Kawasaki disease (KD) was studied in a random trial of IVIG-and-aspirin versus aspirin-alone. Before therapy, patients in each treatment group had an increased percentage of B cells, and a decreased percentage of T cells, CD4 T cells, CD8 T cells and CD5+ B cells. There was no significant difference in immunologic parameters between the two groups measured before therapy. Patients treated with IVIG-and-aspirin had by the fourth day developed a highly-significant increase in T cells, CD4 T cells and CD8 T cells and a decrease in B cells. Despite the decrease of B cells, there were significant increases in CD5+ B cells in both treatment groups. However, the degree of increase in the IVIG-and-aspirin treated group was significantly more noticeable than that in the aspirin-alone treated group. These findings indicate that treatment with IVIG restores the T- and B- cell abnormalities, especially CD5+ B-cell abnormalities found in patients with acute KD.

Effects of intravenous immune globulin on the peripheral lymphocyte phenotypes in Kawasaki disease

The effect of intravenous immune globulin (IVIG) on the lymphocyte phenotypes in acute Kawasaki disease (KD) was studied in a random trial of IVIG-and-aspirin versus aspirin-alone. Before therapy, patients in each treatment group had an increased percentage of B cells, and a decreased percentage of T cells, CD4 T cells, CD8 T cells and CD5+ B cells. There was no significant difference in immunologic parameters between the two groups measured before therapy. Patients treated with IVIG-and-aspirin had by the fourth day developed a highly-significant increase in T cells, CD4 T cells and CD8 T cells and a decrease in B cells. Despite the decrease of B cells, there were significant increases in CD5+ B cells in both treatment groups. However, the degree of increase in the IVIG-and-aspirin treated group was significantly more noticeable than that in the aspirin-alone treated group. These findings indicate that treatment with IVIG restores the T- and B- cell abnormalities, especially CD5+ B-cell abnormalities found in patients with acute KD.

Enfermedad de Kawasaki / Kawasaki disease

La Enfermedad de Kawasaki es una vasculitis multisistémica que afecta principalmente a niños menores de 2 años de edad. Se caracteriza por fiebre prolongada (95%), exantema (92%), alteraciones de la mucosa orofaríngea (90%), cambios en las extremidades (75-90%), inyección conjuntival (88%) y adenopatía cervical (50-75%). El exantema es polimorfo en su presentación clínica, puede ser: maculoso, máculo-papuloso (morbiliforme), urticariano, símil eritema polimorfo, vésicopustuloso, escarlatiniforme o liquemoide; es característica la localización perineal. Las complicaciones más severas son las cardiovasculares, presentes en un 20-25% de los casos