RESUMO
La enfermedad relacionada con IgG4 (ER-IgG4) es un proceso patológico que ha unificado un gran número de enfermedades consideradas como propias de distintos órganos cuya etiopatogenia se desconocía. Su diagnóstico se establece mediante criterios histológicos, radiológicos y serológicos. El tratamiento se basa en corticoides durante tiempo prolongado, reservándose el uso de rituximab para casos refractarios. Es extremadamente inusual que esta entidad asiente solo en cabeza y cuello sin afectación sistémica, por lo que hay pocos casos como el que presentamos descritos en la literatura. Exponemos el caso de una paciente con una tumoración orbitaria con afectación de senos paranasales, diagnosticada de ER-IgG4 nasosinusal. La precisión del diagnóstico propició el inicio precoz de tratamiento corticoideo. En la actualidad la paciente se encuentra asintomática
IgG4 related disease (igG4-RD) is a pathological process which integrates a large number of diseases of unknown pathogenesis, considered as being exclusive to many different organs. Diagnosis is established through histological, radiological and serological criteria. Treatment is based on long term corticosteroids; rituximab being used only in refractory cases. It is unusual for this entity to be found exclusively in the head and neck, without systemic involvement; there are only a few reported cases to date. We present a case of a nasosinusal IgG4-RD orbital tumor with paranasal sinus involvement. The accurate diagnosis made early onset corticosteroid treatment possible and the patient is currently asymptomatic
Assuntos
Humanos , Feminino , Idoso , Doença Relacionada a Imunoglobulina G4/patologia , Neoplasias dos Seios Paranasais/patologia , Neoplasias Orbitárias/patologia , Fibrose/patologia , Invasividade Neoplásica/patologia , Achados IncidentaisRESUMO
IgG4 related disease (igG4-RD) is a pathological process which integrates a large number of diseases of unknown pathogenesis, considered as being exclusive to many different organs. Diagnosis is established through histological, radiological and serological criteria. Treatment is based on long term corticosteroids; rituximab being used only in refractory cases. It is unusual for this entity to be found exclusively in the head and neck, without systemic involvement; there are only a few reported cases to date. We present a case of a nasosinusal IgG4-RD orbital tumor with paranasal sinus involvement. The accurate diagnosis made early onset corticosteroid treatment possible and the patient is currently asymptomatic.
