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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
immunology and microbiology
Clinical picture and treatment of 2212 patients with common variable immunodeficiency
Journal of Allergy and Clinical Immunology, Volume 134, No. 1, Year 2014
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Description
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 (<10 years) was very frequent in our cohort (33.7%), especially in male subjects (39.8%). Male subjects with early-onset CVID were more prone to pneumonia and less prone to other complications suggesting a distinct disease entity. The diagnostic delay of CVID ranges between 4 and 5 years in many countries and is particularly high in subjects with early-onset CVID. Enteropathy, autoimmunity, granulomas, and splenomegaly formed a set of interrelated features, whereas bronchiectasis was not associated with any other clinical feature. Patient survival in this cohort was associated with age at onset and age at diagnosis only. There were different treatment strategies in Europe, with considerable differences in immunoglobulin dosing, ranging from 130 up to 750 mg/kg/mo. Patients with very low trough levels of less than 4 g/L had poor clinical outcomes, whereas higher trough levels were associated with a reduced frequency of serious bacterial infections. Conclusion Patients with CVID are being managed differently throughout Europe, affecting various outcome measures. Clinically, CVID is a truly variable antibody deficiency syndrome. © 2014 American Academy of Allergy, Asthma and Immunology.
Authors & Co-Authors
Gathmann, Benjamin
Germany, Freiburg Im Breisgau
Universitätsklinikum Freiburg
Mahlaoui, Nizar
France, Paris
Hôpital Necker Enfants Malades
France, Paris
Université Paris Cité
Gérard, Laurence
France, Paris
Hôpital Saint-louis
France, Paris
Centre de Référence Des Déficits Immunitaires Héréditaires Ceredih
France
Defi Study Group
Oksenhendler, Éric
France, Paris
Hôpital Saint-louis
Warnatz, Klaus F.
Germany, Freiburg Im Breisgau
Universitätsklinikum Freiburg
Schulze, Ilka
Germany, Freiburg Im Breisgau
Universitätsklinikum Freiburg
Kindle, Gerhard R.
Germany, Freiburg Im Breisgau
Universitätsklinikum Freiburg
Kuijpers, T. W.
Netherlands, Amsterdam
Dutch Working Party for Immunodeficiencies Wid
van Beem, Rachel T.
Netherlands, Amsterdam
Stichting Sanquin Bloedvoorziening
Guzman, David
United Kingdom, London
The Royal Free Hospital
Workman, Sarita
United Kingdom, London
The Royal Free Hospital
Soler-Palacín, P.
Spain, Barcelona
Vall D'hebron Institut de Recerca
De Gracia, Javier
Spain, Barcelona
Hospital Universitari Vall D'hebron
Witte, Torsten
Germany, Hannover
Hannover Medical School
Schmidt, Reinhold Ernst
Germany, Hannover
Hannover Medical School
Litzman, Jiří
Czech Republic, Brno
Fakultní Nemocnice u Sv. Anny V Brně
Hlaváčková, Eva O.
Czech Republic, Brno
Fakultní Nemocnice u Sv. Anny V Brně
Thon, Vojtech
Czech Republic, Brno
Masaryk University
Borte, Michael
Germany, Leipzig
Universität Leipzig
Borte, Stephan
Germany, Leipzig
Universität Leipzig
Kumararatne, Dinakantha S.
United Kingdom, Cambridge
Cambridge University Hospitals Nhs Foundation Trust
Feighery, Conleth F.
Ireland, Dublin
St James's Hospital
Longhurst, Hilary J.
United Kingdom, London
Barts Health Nhs Trust
Helbert, Matthew
United Kingdom, Manchester
Manchester University Nhs Foundation Trust
Szaflarska, Anna
Poland, Krakow
Szpital Uniwersytecki w Krakowie
Šedivá, Anná
Czech Republic, Prague
Fakultní Nemocnice V Motole
Belohradsky, Bernd H.
Germany, Munich
Klinikum Der Universität München
Jones, Alison M.
United Kingdom, London
Great Ormond Street Hospital for Children Nhs Foundation Trust
Baumann, Ulrich H.
Germany, Hannover
Hannover Medical School
Meyts, Isabelle
Belgium, Leuven
Ku Leuven– University Hospital Leuven
Kütükçüler, Necil
Turkey, Izmir
Ege Üniversitesi
Wågström, Per
Sweden, Jonkoping
County Hospital Ryhov
Galal, Nermeen Mouftah
Egypt, Cairo
Faculty of Medicine
Roesler, Joachim R.
Germany, Dresden
Universitätsklinikum Carl Gustav Carus Dresden
Farmaki, Evangelia
Greece, Thessaloniki
Aristotle University of Thessaloniki
Zinovieva, Natalia
Russian Federation, Moscow
Oncology
Čižnár, Peter
Slovakia, Bratislava
Univerzita Komenského V Bratislave
Papadopoulou-Alataki, Efimia
Greece, Thessaloniki
Aristotle University of Thessaloniki
Bienemann, Kirsten
Germany, Dusseldorf
Heinrich-heine-universität Düsseldorf Medizinische Fakultät
Velbri, Sirje
Estonia, Tallinn
Tallinn Children's Hospital
Panahloo, Zoya
United Kingdom
Medical Science Department
Grimbacher, Bodo
Germany, Freiburg Im Breisgau
Universitätsklinikum Freiburg
United Kingdom, London
The Royal Free Hospital
Statistics
Citations: 485
Authors: 42
Affiliations: 34
Identifiers
Doi:
10.1016/j.jaci.2013.12.1077
ISSN:
00916749
e-ISSN:
10976825
Research Areas
Health System And Policy
Study Design
Cohort Study
Participants Gender
Male