Clinical picture and treatment of 2212 patients with common variable immunodeficiency

Background Common variable immunodeficiency (CVID) is an antibody deficiency with an equal sex distribution and a high variability in clinical presentation. The main features include respiratory tract infections and their associated complications, enteropathy, autoimmunity, and lymphoproliferative d...

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Veröffentlicht in:	JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY 2014-07, Vol.134 (1), p.116-126.e11
Hauptverfasser:	Gathmann, Benjamin, MA, Mahlaoui, Nizar, MD, MSc, MPH, Gérard, Laurence, MD, Oksenhendler, Eric, MD, Warnatz, Klaus, MD, Schulze, Ilka, MD, Kindle, Gerhard, MD, Kuijpers, Taco W., MD, van Beem, Rachel T., PhD, Guzman, David, Lic, Workman, Sarita, RN, MSc, Soler-Palacín, Pere, MD, PhD, De Gracia, Javier, MD, PhD, Witte, Torsten, MD, Schmidt, Reinhold E., MD, Litzman, Jiri, MD, Hlavackova, Eva, MD, Thon, Vojtech, MD, PhD, Borte, Michael, MD, PhD, Borte, Stephan, MD, Kumararatne, Dinakantha, MD, Feighery, Conleth, MD, Longhurst, Hilary, FRCP, PhD, Helbert, Matthew, MD, Szaflarska, Anna, MD, Sediva, Anna, MD, Belohradsky, Bernd H., MD, Jones, Alison, MRCP, PhD, Baumann, Ulrich, MD, PhD, Meyts, Isabelle, MD, PhD, Kutukculer, Necil, MD, Wågström, Per, MD, Galal, Nermeen Mouftah, MD, Roesler, Joachim, MD, Farmaki, Evangelia, MD, PhD, Zinovieva, Natalia, MD, Ciznar, Peter, MD, PhD, Papadopoulou-Alataki, Efimia, MD, PhD, Bienemann, Kirsten, MD, Velbri, Sirje, MD, PhD, Panahloo, Zoya, MD, Grimbacher, Bodo, MD
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Age Age of Onset Allergy and Immunology Autoimmunity Biological and medical sciences Bronchiectasis - pathology Child Child, Preschool Common variable immunodeficiency Common Variable Immunodeficiency - complications Common Variable Immunodeficiency - drug therapy Common Variable Immunodeficiency - immunology Common Variable Immunodeficiency - mortality Delayed Diagnosis enteropathy Europe Female Fundamental and applied biological sciences. Psychology Fundamental immunology granulomas Humans Immunodeficiencies Immunodeficiencies. Immunoglobulinopathies immunoglobulin replacement Immunoglobulins, Intravenous - therapeutic use Immunopathology lymphadenopathy Lymphoma Lymphoproliferative Disorders - complications Lymphoproliferative Disorders - drug therapy Lymphoproliferative Disorders - immunology Lymphoproliferative Disorders - mortality Male Medical sciences Meningitis Multivariate analysis patient self-reported outcomes Patients Pneumonia Pneumonia - complications Pneumonia - drug therapy Pneumonia - immunology Pneumonia - mortality primary antibody deficiency quality of life Retrospective Studies Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Splenomegaly - pathology Studies Survival Analysis treatment
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Zusammenfassung:	Background Common variable immunodeficiency (CVID) is an antibody deficiency with an equal sex distribution and a high variability in clinical presentation. The main features include respiratory tract infections and their associated complications, enteropathy, autoimmunity, and lymphoproliferative disorders. Objective This study analyzes the clinical presentation, association between clinical features, and differences and effects of immunoglobulin treatment in Europe. Methods Data on 2212 patients with CVID from 28 medical centers contributing to the European Society for Immunodeficiencies Database were analyzed retrospectively. Results Early disease onset (
ISSN:	0091-6749 1097-6825
DOI:	10.1016/j.jaci.2013.12.1077