Vol. 13. Núm. 34.
Páginas 1965-1974 (Mayo 2021)

Actualización
Enfermedad de Kawasaki y vasculitis de pequeño vaso

Kawasaki disease and small vessel vasculitis

M. López DuplaA.M. Febrer NafriaZ.J. Santana RodríguezS. Roig Flores
Doi : https://10.1016/j.med.2021.05.009
Opciones
Resumen

La enfermedad de Kawasaki es una vasculitis que afecta a vasos de mediano tamaño, sobre todo las arterias coronarias, y a niños menores de 5 años, y que se acompaña de lesiones mucocutáneas y adenopatías. Están establecidos unos criterios de clasificación de la enfermedad y el tratamiento se fundamenta en que debe ser precoz e indicado cuando se cumplan los criterios establecidos, y se fundamenta en la asociación de inmunoglobulinas y AAS. Las vasculitis de pequeño vaso, de acuerdo con la conferencia de consenso de Chapel Hill, incluyen las vasculitis asociadas a ANCA y las vasculitis por depósito de inmunocomplejos. En este último grupo se incluye la vasculitis IgA, la vasculitis crioglobulinémica, la urticaria vasculitis hipocomplementémica y la vasculitis por anticuerpos antimembrana basal glomerular. El diagnóstico en la mayoría de los casos exige la evidencia de vasculitis de pequeño vaso en la biopsia, pero en el caso de la vasculitis por anticuerpos antimembrana basal glomerular no es imprescindible. La gravedad de las manifestaciones de cada una condiciona el tratamiento. La vasculitis por anticuerpos antimembrana basal exige un tratamiento intenso desde el inicio a base de ciclofosfamida, corticoides y recambio plasmático. En el resto de entidades, la pauta depende de los patrones de gravedad. El pronóstico es también diferente. La vasculitis IgA y la urticaria vasculitis suelen tener mejor pronóstico, y la vasculitis crioglobulinémica y sobre todo la vasculitis por anticuerpos antimembrana basal glomerular lo tienen peor.

Palabras clave
Enfermedad de Kawasaki
Vasculitis IgA
Urticaria vasculitis hipocomplementémica
Vasculitis crioglobulinémica
Vasculitis por anticuerpos antimembrana basal glomerular
Abstract

Kawasaki disease is a vasculitis that affects medium-sized vessels, especially the coronary arteries and children under 5 years of age, and is accompanied by mucocutaneous lesions and adenopathy. There are established criteria for classifying the disease. Treatment should be early and is indicated when these criteria are met based on the combination of immunoglobulins and acetylsalicylic acid. Small vessel vasculitis, according to the International Chapel Hill Consensus Conference includes ANCA-associated vasculitis and immune complex deposition vasculitis. The latter group includes IgA vasculitis, cryoglobulinaemic vasculitis, hypocomplementaemic urticarial vasculitis and anti-glomerular basement membrane antibody vasculitis. Diagnosis in most cases requires evidence of small vessel vasculitis on biopsy, but for anti-glomerular basement membrane antibody vasculitis this is not essential. The severity of the manifestations of each will determine treatment. Anti-glomerular basement membrane vasculitis requires intensive treatment from onset based on cyclophosphamide, corticosteroids, and plasma exchange. In all the other entities the treatment regimen depends on the severity patterns. The prognosis is also different, IgA vasculitis and urticarial vasculitis tend to have a better prognosis, and cryoglobulinaemic vasculitis and especially anti- glomerular basement membrane antibody vasculitis have a worse prognosis.

Keywords
Kawasaki disease
IgA vasculitis
Hypocomplementaemic urticarial vasculitis
Cryoglobulinaemic vasculitis
Anti-glomerular basement membrane vasculitis

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