Vol. 13. Núm. 34.
Páginas 1975-1984 (Mayo 2021)

Actualización
Enfermedad de Behçet

Behçet's disease

G. EspinosaaM. Rodríguez Carballeirab
Doi : https://10.1016/j.med.2021.05.010
Opciones
Resumen

La enfermedad de Behçet es un trastorno inflamatorio crónico recidivante que cursa con brotes y cuya etiología es incierta. Su prevalencia varía según el área geográfica y la etnia, incrementándose desde el Mediterráneo hacia el Este Asiático a lo largo de la antigua «ruta de la seda». La manifestación clínica más característica son las úlceras orales y genitales, seguidas de otras lesiones cutáneas (eritema nudoso, papulopústuas, etc.) y uveítis. Con menos frecuencia se presenta con artritis, afectación gastrointestinal, vascular (arterial y venosa) y del sistema nervioso central. Tras los primeros años desde la presentación, suele evolucionar de forma favorable, pero la clínica vascular y neurológica, aunque menos común, puede ser fatal. La afectación ocular es la principal responsable de daño residual en forma de disminución de la agudeza visual. Suele comenzar en torno a la tercera década de la vida y no muestra un predominio claro de sexo, pero suele ser más grave en el masculino. El tratamiento tiene como objetivo suprimir las exacerbaciones inflamatorias y recurrencias que pueden causar daño orgánico irreversible. Debe individualizarse según el fenotipo y la gravedad de la enfermedad. Se fundamenta en la colchicina para la afectación cutaneomucosa, corticoides, otros inmunosupresores y anticuerpos monoclonales para la clínica más grave.

Palabras clave
Enfermedad de Behçet
Síndromes autoinflamatorios
Vasculitis
Revisión
Abstract

Behçet's disease is a chronic relapsing inflammatory disorder that flares and is of uncertain aetiology. Its prevalence varies by geographic area and ethnicity, increasing from the Mediterranean to East Asia along the ancient «Silk Route». Oral and genital ulcers are the most characteristic clinical manifestations, followed by other skin lesions (erythema nodosum, papulopustular lesions, etc.) and uveitis. Less frequently it presents with arthritis, gastrointestinal, vascular (arterial and venous) and central nervous system involvement. After the first few years from onset, the disease usually progresses favourably, but vascular and neurological symptoms, although less common, can be fatal. Eye involvement is primarily responsible for residual damage in the form of decreased visual acuity. It usually starts around the third decade of life and shows no clear gender predominancy, but is usually more severe in males. The aim of treatment is to suppress inflammatory flare-ups and recurrence that can cause irreversible organ damage. It should be tailored to the phenotype and severity of the disease. It is based on colchicine for involvement of the skin and mucosa, corticosteroids, other immunosuppressants and monoclonal antibodies for more severe disease.

Keywords
Behçet's disease
Autoinflammatory syndromes
Vasculitis
Revision

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