The DOI matches its Crossref record; 25 of the 25 references it cites were found in a registry or an index.
Common variable immunodeficiency disorders: division into distinct clinical phenotypes
Abstract
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.
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Cites
Common Variable Immunodeficiency: Clinical and Immunological Features of 248 Patients
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Psoriasis: epidemiology, clinical features, and quality of life
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The EUROclass trial: defining subgroups in common variable immunodeficiency
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Self-citationHomozygous loss of ICOS is associated with adult-onset common variable immunodeficiency
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Self-citationAn Antibody-Deficiency Syndrome Due to Mutations in the CD19 Gene
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Self-citationLong-Term Follow-Up and Outcome of a Large Cohort of Patients with Common Variable Immunodeficiency
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Immune adaptations that maintain homeostasis with the intestinal microbiota
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International Consensus Document (ICON): Common Variable Immunodeficiency Disorders
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Gut Microbiota–Host Interactions in Inborn Errors of Immunity
Castagnoli, R., Pala, F., Bosticardo, M., Licari, A., Delmonte, O. M., Villa, A., Marseglia, G. L., & Notarangelo, L. D. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22031416 · Cited by 34
Metadata
- Authors
- Chapel, H., Lucas, M., Lee, M., Bjorkander, J., Webster, D., Grimbacher, B., Fieschi, C., Thon, V., Abedi, M. R., & Hammarstrom, L.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2008-07-15
- Volume
- 112
- Issue
- 2
- Pages
- 277-286
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2035451300
- Cited by
- 835
- References
- 25
- FWCI
- 12.84
- Type
- journal article
- Language
- en
- Open access
- closed
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