Clinical picture and treatment of 2212 patients with common variable immunodeficiency
Jazyk angličtina Země Spojené státy americké Médium print-electronic
Typ dokumentu časopisecké články, multicentrická studie, práce podpořená grantem
PubMed
24582312
DOI
10.1016/j.jaci.2013.12.1077
PII: S0091-6749(14)00029-3
Knihovny.cz E-zdroje
- Klíčová slova
- Common variable immunodeficiency, autoimmunity, enteropathy, granulomas, immunoglobulin replacement, lymphadenopathy, patient self-reported outcomes, primary antibody deficiency, quality of life, treatment,
- MeSH
- analýza přežití MeSH
- autoimunita MeSH
- běžná variabilní imunodeficience komplikace farmakoterapie imunologie mortalita MeSH
- bronchiektazie patologie MeSH
- dítě MeSH
- dospělí MeSH
- intravenózní imunoglobuliny terapeutické užití MeSH
- lidé MeSH
- lymfoproliferativní nemoci komplikace farmakoterapie imunologie mortalita MeSH
- mladiství MeSH
- opožděná diagnóza MeSH
- pneumonie komplikace farmakoterapie imunologie mortalita MeSH
- předškolní dítě MeSH
- retrospektivní studie MeSH
- splenomegalie patologie MeSH
- věk při počátku nemoci MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Evropa MeSH
- Názvy látek
- intravenózní imunoglobuliny 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
Citace poskytuje Crossref.org
Autoimmunity and Immunodeficiency in Severe SARS-CoV-2 Infection and Prolonged COVID-19
Imaging of Bronchial Pathology in Antibody Deficiency: Data from the European Chest CT Group
