• MeSH
• běžná variabilní imunodeficience diagnóza   etiologie   komplikace   mortalita   patologie   MeSH
• diferenciální diagnóza MeSH
• lidé středního věku MeSH
• lidé MeSH
• mnohočetný myelom * diagnostické zobrazování   farmakoterapie   krev   mortalita   patologie   MeSH
• paraproteinemie krev   MeSH
• sepse diagnóza   farmakoterapie   komplikace   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• kazuistiky 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.

• 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

• MeSH
• běžná variabilní imunodeficience epidemiologie   mortalita   MeSH
• časové faktory MeSH
• dítě MeSH
• dospělí MeSH
• financování organizované MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• morbidita trendy   MeSH
• mortalita trendy   MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• souhrny MeSH

The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients. A total of 7 centers contributed patient data, resulting in the largest cohort yet reported. Patients (334), validated for the diagnosis, were followed for an average of 25.6 years (9461 patient-years). Data were used to define 5 distinct clinical phenotypes: no complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy, and lymphoid malignancy. A total of 83% of patients had only one of these phenotypes. Analysis of mortality showed a considerable reduction in the last 15 years and that different phenotypes were associated with different survival times. Types of complications and clinical phenotypes varied significantly between countries, indicating the need for large, international registries. Ages at onset of symptoms and diagnosis were shown to have a Gaussian distribution, but were not useful predictors of phenotype. The only clinical predictor was polyclonal lymphocytic infiltration, which was associated with a 5-fold increased risk of lymphoid malignancy. There was widespread variation in the levels of serum immunoglobulin isotypes as well as in the percentages and absolute numbers of B cells, confirming the heterogeneity of these conditions. Higher serum IgM and lower circulating CD8 proportions were found to be predictive markers for polyclonal lymphocytic infiltration and autoimmunity, respectively.

• MeSH
• autoimunita MeSH
• běžná variabilní imunodeficience * klasifikace   komplikace   mortalita   patologie   MeSH
• CD8-pozitivní T-lymfocyty imunologie   MeSH
• fenotyp MeSH
• imunoglobulinové izotypy krev   MeSH
• kohortové studie MeSH
• leukemická infiltrace MeSH
• lidé MeSH
• prognóza MeSH
• registrace MeSH
• věk při počátku nemoci MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• multicentrická studie MeSH
• práce podpořená grantem MeSH