RESUMO
Inflammatory pseudotumor (IPT) of the liver is a rare benign tumor of unknown origin, it has the appearance of a malignant tumor but has a benign histology and clinical course. We report a case of a 63-year-old man diagnosed of IPT of the liver and followed for 10 years. During the clinical course, he developed a secondary Budd-Chiari syndrome, with a successful response to a transjugular intrahepatic portosystemic shunt over a 5-year follow-up period.
Assuntos
Síndrome de Budd-Chiari/etiologia , Granuloma de Células Plasmáticas/complicações , Hepatopatias/complicações , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de TempoRESUMO
El pseudotumor inflamatorio (PTI) hepático es una entidad rara, de origen desconocido y aunque puede tener apariencia maligna, su naturaleza es benigna. Presentamos el caso de un varón de 63 años diagnosticado de PTI hepático en seguimiento durante 10 años. En su evolución desarrolló un síndrome de Budd-Chiari, con buena respuesta tras la colocación de una derivación porto-sistémica transyugular, a los cinco años (AU)
Inflammatory pseudotumor (IPT) of the liver is a rare benign tumor of unknown origin, it has the appearance of a malignant tumor but has a benign histology and clinical course. We report a case of a 63-yearold man diagnosed of IPT of the liver and followed for 10 years. During the clinical course, he developed a secondary Budd-Chiari syndrome, with a successful response to a transjugular intrahepatic portosystemic shunt over a 5-year follow-up period (AU)
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Colangiocarcinoma/complicações , Colangiocarcinoma/diagnóstico , Laparotomia/métodos , Laparotomia , Biópsia/métodos , Síndrome de Budd-Chiari/fisiopatologia , Síndrome de Budd-Chiari/cirurgia , Síndrome de Budd-Chiari , Imunoglobulinas/análise , Imunoglobulinas/isolamento & purificação , Imageamento por Ressonância Magnética/instrumentação , Imageamento por Ressonância Magnética/métodosRESUMO
Primary sclerosing cholangitis is an infrequent extraintestinal manifestation of ulcerative colitis. Damage to bile ducts is irreversible and medical therapies to prevent progression of the disease are usually ineffective. We describe a patient with long-standing ulcerative colitis, which was refractory to corticosteroid therapy who developed primary sclerosing cholangitis (biochemical stage II/IV) in the course of his pancolitis. Treatment with infliximab (5 mg/kg as an induction dose followed by maintenance doses every two months) was indicated because of steroid-dependent disease associated to primary sclerosing cholangitis as well as sacroiliitis and uveitis and previous episode of severe azathioprine-related hepatic toxicity. At present, after two years of follow-up, the patient is asymptomatic with normal liver tests and complete resumption of daily life activities. This case draws attention to the usefulness of anti-tumor necrosis factor-alpha therapy for the management of primary sclerosing cholangitis as extraintestinal manifestation of inflammatory bowel disease.
