RESUMO
IgG4-related disease is characterized by mass lesions, a dense lymphoplasmacytic infiltrate with immunohistochemical positivity for IgG4, storiform fibrosis and, frequently, elevated serum IgG4 levels. It can be multisystemic; however, myocardial involvement, which is objectively determined by imaging tests, has not been described in the medical literature. We report the case of a man with IgG4-related disease with possible myocardial involvement, detected by cardiac magnetic resonance. This raises the question of a differential diagnosis with other diseases such as sarcoidosis and Fabry disease, the differential diagnosis of which is of great importance due to its therapeutic impact.
Assuntos
Cardiomiopatias/etiologia , Doença Relacionada a Imunoglobulina G4/complicações , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
La enfermedad relacionada con el depósito de IgG4 es una entidad caracterizada por lesiones tumefactas, infiltrado denso linfoplasmocítico con positividad inmunohistoquímica para IgG4, fibrosis estoriforme y, frecuentemente, niveles séricos elevados de IgG4. Puede cursar con afectación multisistémica; sin embargo, la afectación miocárdica, objetivada mediante pruebas de imagen, no ha sido descrita en la literatura médica. Se presenta el caso clínico de un varón afecto de enfermedad relacionada con IgG4 con posible afectación miocárdica, detectada a través de cardiorresonancia magnética, que plantea el diagnóstico diferencial con otras enfermedades como la sarcoidosis y la enfermedad de Fabry, cuyo diagnóstico diferencial es de gran importancia por su repercusión terapéutica
IgG4-related disease is characterized by mass lesions, a dense lymphoplasmacytic infiltrate with immunohistochemical positivity for IgG4, storiform fibrosis and, frequently, elevated serum IgG4 levels. It can be multisystemic; however, myocardial involvement, which is objectively determined by imaging tests, has not been described in the medical literature. We report the case of a man with IgG4-related disease with possible myocardial involvement, detected by cardiac magnetic resonance. This raises the question of a differential diagnosis with other diseases such as sarcoidosis and Fabry disease, the differential diagnosis of which is of great importance due to its therapeutic impact