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Sclerosing Cholangitis Related to IgG4: Not Always a Curable Entity.
Martínez-Valle, Fernando; Riveiro-Barciela, Mar; Salcedo, María-Teresa; Merino-Casabiel, Xavier; Fernández-Codina, Andreu; Torres, Inés de; Esteban, Rafael; Buti, María.
Afiliação
  • Martínez-Valle F; Systemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain. Electronic address: mbuti@vhebron.net.
  • Riveiro-Barciela M; Liver Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain.
  • Salcedo MT; Pathology Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
  • Merino-Casabiel X; Abdominal Imaging Unit, Radiology Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
  • Fernández-Codina A; Systemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
  • Torres I; Pathology Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
  • Esteban R; Liver Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain.
  • Buti M; Liver Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain.
Ann Hepatol ; 18(1): 215-219, 2019.
Article em En | MEDLINE | ID: mdl-31113593
IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. One out of three patients with IgG4 sclerosing cholangitis also presents autoimmune pancreatitis, although it can be associated with manifestations in other organs. One of the main features of IgG4-related disease is its good prognosis due to the great response to glucocorticoid therapy. However, relapse of the disease is not uncommon, especially when steroid therapy is decreased or stopped. Rituximab seems to be an effective treatment to achieve remission of the disease. We report the case of a 74 year-old man diagnosed with IgG4-related disease based on increase of serum IgG4 levels, imaging and histopathological findings, with systemic involvement including sclerosing cholangitis. Despite the absence of liver fibrosis at onset, the early use of glucocorticoids and rituximab therapy, the patient presented clinical and analytical deterioration, leading to secondary biliary cirrhosis. In conclusion, this clinical case highlights the importance of prompt diagnosis and therapeutics for sclerosing cholangitis secondary to IgG4-related disease in order to avoid progression of the disease and development of liver cirrhosis, as well as the refractory, aggressive nature of the disease in some cases as this one.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Imunoglobulina G / Colangite Esclerosante / Rituximab / Doença Relacionada a Imunoglobulina G4 / Fígado Tipo de estudo: Diagnostic_studies / Prognostic_studies Limite: Aged / Humans / Male Idioma: En Revista: Ann Hepatol Assunto da revista: GASTROENTEROLOGIA Ano de publicação: 2019 Tipo de documento: Article País de publicação: México
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Imunoglobulina G / Colangite Esclerosante / Rituximab / Doença Relacionada a Imunoglobulina G4 / Fígado Tipo de estudo: Diagnostic_studies / Prognostic_studies Limite: Aged / Humans / Male Idioma: En Revista: Ann Hepatol Assunto da revista: GASTROENTEROLOGIA Ano de publicação: 2019 Tipo de documento: Article País de publicação: México