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X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis.
Steele, Cathal L; Doré, Matthew; Ammann, Sandra; Loughrey, Maurice; Montero, Angeles; Burns, Siobhan O; Morris, Emma C; Gaspar, Bobby; Gilmour, Kimberly; Bibi, Shahnaz; Shendi, Hiba; Devlin, Lisa; Speckmann, Carsten; Edgar, David M.
Afiliação
  • Steele CL; Regional Immunology Service, Royal Victoria Hospital, The Belfast Trust, Belfast, UK. cathal.steele@nhs.net.
  • Doré M; Regional Immunology Service, Royal Victoria Hospital, The Belfast Trust, Belfast, UK.
  • Ammann S; Centre for Chronic Immunodeficiency, University Medical Centre Freiburg, Freiburg, Germany.
  • Loughrey M; Royal Victoria Hospital, The Belfast Trust, UK and Queen's University Belfast, Belfast, UK.
  • Montero A; Royal Brompton and Harefield Hospitals NHS Foundation Trust, London, UK.
  • Burns SO; Department of Immunology, Royal Free London NHS Foundation Trust, University College London Institute of Immunity and Transplantation, London, UK.
  • Morris EC; Department of Immunology, Royal Free London NHS Foundation Trust, University College London Institute of Immunity and Transplantation, London, UK.
  • Gaspar B; University College London Institute of Child Health, London, UK.
  • Gilmour K; Department of Immunology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
  • Bibi S; Department of Immunology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
  • Shendi H; Regional Immunology Service, Royal Victoria Hospital, The Belfast Trust, Belfast, UK.
  • Devlin L; Regional Immunology Service, Royal Victoria Hospital, The Belfast Trust, Belfast, UK.
  • Speckmann C; Department of Paediatrics and Adolescent Medicine, Germany and Centre for Chronic Immunodeficiency, University Medical Centre Freiburg, Freiburg, Germany.
  • Edgar DM; Regional Immunology Service, Royal Victoria Hospital, The Belfast Trust, Queen's University Belfast, Belfast, UK.
J Clin Immunol ; 36(7): 733-8, 2016 10.
Article em En | MEDLINE | ID: mdl-27492372
ABSTRACT
The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Doenças Pulmonares Intersticiais / Doenças Genéticas Ligadas ao Cromossomo X / Linfo-Histiocitose Hemofagocítica / Hepatite / Transtornos Linfoproliferativos Idioma: En Ano de publicação: 2016 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Doenças Pulmonares Intersticiais / Doenças Genéticas Ligadas ao Cromossomo X / Linfo-Histiocitose Hemofagocítica / Hepatite / Transtornos Linfoproliferativos Idioma: En Ano de publicação: 2016 Tipo de documento: Article