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Am J Med Sci ; 367(1): 67-71, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37714269

RESUMO

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder characterized by dense infiltration of IgG4-positive plasma cells in the affected tissue along with characteristic storiform fibrosis that can lead to the development of tumefactive lesions in any organ. CA19-9 is a marker for pancreato-biliary malignancy, however mild to moderate elevation of CA 19-9 can also be observed in IgG4-RD autoimmune pancreatitis (AIP) and sclerosing cholangitis (IgG4-SC). Therefore, it becomes difficult to differentiate between these entities. We describe the case of a 65-year-old male with history of IgG4-RD, presenting with jaundice and abdominal pain. He was found to have a pancreatic mass with significantly elevated IgG4 162 (2-96 mg/dL and CA19-9 levels 2830 (0-35 U/ml). Patient underwent ERCP and biopsy, which ruled out pancreatic cancer and cholangiocarcinoma. He was diagnosed with IgG4-RD autoimmune pancreatitis (AIP) and sclerosing cholangitis. Treatment with steroids and rituximab resulted in significant improvement in the bilirubin and a dramatic decrease in CA19-9 levels.


Assuntos
Doenças Autoimunes , Pancreatite Autoimune , Neoplasias dos Ductos Biliares , Colangite Esclerosante , Doença Relacionada a Imunoglobulina G4 , Pancreatite , Masculino , Humanos , Idoso , Pancreatite Autoimune/diagnóstico , Pancreatite Autoimune/tratamento farmacológico , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/tratamento farmacológico , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/tratamento farmacológico , Imunoglobulina G , Antígeno CA-19-9 , Pancreatite/diagnóstico , Pancreatite/tratamento farmacológico , Diagnóstico Diferencial , Neoplasias dos Ductos Biliares/diagnóstico , Ductos Biliares Intra-Hepáticos/patologia
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