Síndrome de Sjögren en pediatría: diagnóstico y manejo

Article information

Resumen

El síndrome de Sjögren (SS) es una enfermedad autoinmune que compromete las glándulas exocrinas provocando una respuesta inflamatoria que conduce a una hiposecreción de las mismas. Es de rara ocurrencia en la edad pediátrica. Clínicamente se manifiesta como ojo y boca seca, asociados a una gran variedad de síntomas y signos que pueden simular otras enfermedades autoinmunes. Puede ser primario o secundario a otras enfermedades autoinmunes.

Actualmente, se aceptan los criterios europeo-americanos de 2002, de los cuales son necesarios 4 de los 6 criterios y que uno de ellos sea una biopsia de glándula salival positiva o bien Anticuerpos (Ac) anti-Ro/La positivos. El tratamiento abarca 2 aspectos diferentes: por un lado, el tratamiento de la sequedad de ojos y boca (agentes sustitutivos de lágrimas), fármacos agonistas muscarínicos, etc., por otro lado, el abordaje de las manifestaciones extra glandulares (antiinflamatorios no esteroideos, corticoides, agentes modificadores de la enfermedad, agentes citotóxicos e incluso terapias biológicas).

Se describe el caso clínico de una niña de 13 años de edad que cumple con los criterios clínicos, de laboratorio e histopatológicos de síndrome de Sjögren primario.

Palabras clave:

Síndrome Sjögren

glándulas exocrinas

parotiditis recurrente

autoinmune

Summary

Sjögren's syndrome (SS) is an autoimmune disease that involves the exocrine glands causing an inflammatory response that leads to hypo secretion of them. It is a rare disease in childhood. Clinically manifested as eye and dry mouth associated with a variety of symptoms and signs that can mimic other autoimmune diseases. Maybe primary or secondary to other autoimmune diseases.

Currently accepted criteria of European Americans 2002, which requires 4 of the 6 criteria, requiring that one of them is a positive salivary gland biopsy or antibody positive anti-Ro/La. Treatment includes two aspects: on the one hand, treatment of dry eyes and mouth (agents tear substitutes) muscarinic agonist drugs, etc), On the other hand, the approach of extra glandular manifestations (NSAIDs, corticosteroids, disease-modifying agents, including cytotoxic agents and biologic therapy).

We describe a case of a 13-year-old who meets the clinical criteria, laboratory and histopathological for primary Sjögren's syndrome.

Key words:

Sjögren syndrome

exocrine glands

recurrent parotitis

autoimmune

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Los autores declaran no presentar ningún conflicto de interés al momento de la redacción del manuscrito.

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