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Clinical picture and treatment of 2212 patients with common variable immunodeficiency
B. Gathmann, N. Mahlaoui, . , L. Gérard, E. Oksenhendler, K. Warnatz, I. Schulze, G. Kindle, TW. Kuijpers, . , RT. van Beem, D. Guzman, S. Workman, P. Soler-Palacín, J. De Gracia, T. Witte, RE. Schmidt, J. Litzman, E. Hlavackova, V. Thon, M....
Language English Country United States
Document type Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't
- MeSH
- Survival Analysis MeSH
- Autoimmunity MeSH
- Common Variable Immunodeficiency complications drug therapy immunology mortality MeSH
- Bronchiectasis pathology MeSH
- Child MeSH
- Adult MeSH
- Immunoglobulins, Intravenous therapeutic use MeSH
- Humans MeSH
- Lymphoproliferative Disorders complications drug therapy immunology mortality MeSH
- Adolescent MeSH
- Delayed Diagnosis MeSH
- Pneumonia complications drug therapy immunology mortality MeSH
- Child, Preschool MeSH
- Retrospective Studies MeSH
- Splenomegaly pathology MeSH
- Age of Onset MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Europe MeSH
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.
Children University Hospital Krakow Poland
Clinic for Immunology and Rheumatology Medical University Hannover Hannover Germany
Department of Immunology Barts Health NHS Trust London United Kingdom
Department of Immunology St James's Hospital Dublin and Trinity College Dublin Dublin Ireland
Department of Infectious Diseases County Hospital Ryhov Jönköping Jönköping Sweden
Department of Pediatric Immunology Ege University Faculty of Medicine Izmir Turkey
Department of Pediatrics University Hospital Dresden Dresden Germany
Department of Pediatrics University Hospital Gasthuisberg Leuven Belgium
Dr v Haunersches Kinderspital Ludwig Maximilians University Munich Germany
Dutch Working Party for Immunodeficiencies Amsterdam The Netherlands
Institute of Child Health Great Ormond Street Hospital London United Kingdom
Medical Department Sanquin Blood Supply Foundation Amsterdam The Netherlands
Medical Science Department CSL Behring West Sussex United Kingdom
Primary Immunodeficiency Clinic Department of Pediatrics Cairo University Cairo Egypt
Research and Clinical Centre for Pediatric Hematology Oncology Immunology Moscow Russia
St Anne's University Hospital Brno Czech Republic
Tallinn Children's Hospital Tallinn Estonia
Translational Centre for Regenerative Medicine University of Leipzig Leipzig Germany
References provided by Crossref.org
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- $a Clinical picture and treatment of 2212 patients with common variable immunodeficiency / $c B. Gathmann, N. Mahlaoui, . , L. Gérard, E. Oksenhendler, K. Warnatz, I. Schulze, G. Kindle, TW. Kuijpers, . , RT. van Beem, D. Guzman, S. Workman, P. Soler-Palacín, J. De Gracia, T. Witte, RE. Schmidt, J. Litzman, E. Hlavackova, V. Thon, M. Borte, S. Borte, D. Kumararatne, C. Feighery, H. Longhurst, M. Helbert, A. Szaflarska, A. Sediva, BH. Belohradsky, A. Jones, U. Baumann, I. Meyts, N. Kutukculer, P. Wågström, NM. Galal, J. Roesler, E. Farmaki, N. Zinovieva, P. Ciznar, E. Papadopoulou-Alataki, K. Bienemann, S. Velbri, Z. Panahloo, B. Grimbacher, . ,
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