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Lanadelumab for the prevention of hereditary angioedema attacks: A real-world UK audit.

Dorr, Anthony D; Chopra, Charu; Coulter, Tanya I; Dempster, John; Dziadzio, Magdalena; El-Shanawany, Tariq; Garcez, Tomaz; Gompels, Mark; Herriot, Richard; Jain, Rashmi; Levi, Marcel; Lorenzo, Lorena; Makki, Inas; Mapazire, Elizabeth; Murng, Sai H K; Noorani, Sadia; Savic, Sinisa; Steele, Cathal L; Symons, Christine; Tarzi, Michael; Yong, Patrick F K; Kiani-Alikhan, Sorena.

Allergy ; 78(5): 1369-1371, 2023 05.

Article in English | MEDLINE | ID: mdl-36510404

Subject(s)

Angioedemas, Hereditary , Humans , Angioedemas, Hereditary/drug therapy , Angioedemas, Hereditary/prevention & control , Antibodies, Monoclonal, Humanized , Plasma Kallikrein , United Kingdom/epidemiology , Complement C1 Inhibitor Protein/therapeutic use

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.

J Clin Immunol ; 36(7): 733-8, 2016 10.

Article in English | 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.

Subject(s)

Genetic Diseases, X-Linked/complications , Hepatitis/complications , Lung Diseases, Interstitial/complications , Lymphohistiocytosis, Hemophagocytic/complications , Lymphoproliferative Disorders/complications , Azathioprine/therapeutic use , Biomarkers , Biopsy , Bone Marrow/pathology , Child, Preschool , Female , Genetic Diseases, X-Linked/diagnosis , Genetic Diseases, X-Linked/genetics , Genetic Diseases, X-Linked/therapy , Hepatitis/diagnosis , Hepatitis/therapy , Humans , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Immunophenotyping , Liver/pathology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/drug therapy , Lymph Nodes/pathology , Lymphocytes/immunology , Lymphocytes/metabolism , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/therapy , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/genetics , Lymphoproliferative Disorders/therapy , Male , Mutation , Pedigree , Phenotype , Respiratory Function Tests , Rituximab/therapeutic use , Tomography, X-Ray Computed , X-Linked Inhibitor of Apoptosis Protein/genetics

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