The burden of common variable immunodeficiency disorders: A retrospective analysis of the European Society for Immunodeficiency (ESID) registry data

Autor: Odnoletkova, I, Kindle, G, Quinti, I, Grimbacher, B, Knerr, V, Gathmann, B, Ehl, S, Mahlaoui, N, Van Wilder, P, Bogaerts, K, de Vries, E, Farrugia, A, Krishnarajah, S, Mendivil, J, Prior, M, Rübesam, T, Runken, M, in collaboration with the Plasma Protein Therapeutics Association (PPTA) Taskforce
Přispěvatelé: Tranzo, Scientific center for care and wellbeing, Huisarts & Ziekenhuis
Jazyk: angličtina
Rok vydání: 2018
Předmět:
0301 basic medicine
Pediatrics
medicine.medical_specialty
Génétique clinique
Primary immunodeficiency
Primary antibody deficiency
Common variable immunodeficiency
Burden of disease DALY
Health economics
Diagnostic delay
Population
lcsh:Medicine
Pharmacologie
03 medical and health sciences
Life Expectancy
Cost of Illness
medicine
Burden of disease
common variable immunodeficiency
DALY
diagnostic delay
health economics
primary antibody deficiency
primary immunodeficiency
genetics (clinical)
pharmacology (medical)
Humans
Pharmacology (medical)
Registries
Risk factor
education
Genetics (clinical)
Disease burden
Retrospective Studies
education.field_of_study
business.industry
Research
lcsh:R
General Medicine
Sciences bio-médicales et agricoles
medicine.disease
Comorbidity
Europe
030104 developmental biology
Years of potential life lost
Cohort
business
Zdroj: Orphanet Journal of Rare Diseases, 13(1):201. BioMed Central
Orphanet Journal of Rare Diseases
Orphanet journal of rare diseases, 13 (1
Orphanet Journal of Rare Diseases, Vol 13, Iss 1, Pp 1-17 (2018)
ISSN: 1750-1172
Popis: BACKGROUND: Common variable immunodeficiency disorders (CVID) are a group of rare innate disorders characterized by specific antibody deficiency and increased rates of infections, comorbidities and mortality. The burden of CVID in Europe has not been previously estimated. We performed a retrospective analysis of the European Society for Immunodeficiencies (ESID) registry data on the subset of patients classified by their immunologist as CVID and treated between 2004 and 2014. The registered deaths and comorbidities were used to calculate the annual average age-standardized rates of Years of Life Lost to premature death (YLL), Years Lost to Disability (YLD) and Disability Adjusted Life Years (DALY=YLL + YLD). These outcomes were expressed as a rate per 105 of the CVID cohort (the individual disease burden), and of the general population (the societal disease burden). RESULTS: Data of 2700 patients from 23 countries were analysed. Annual comorbidity rates: bronchiectasis, 21.9%; autoimmunity, 23.2%; digestive disorders, 15.6%; solid cancers, 5.5%; lymphoma, 3.8%, exceeded the prevalence in the general population by a factor of 34.0, 7.6, 8.1, 2.4 and 32.6, respectively. The comorbidities of CVID caused 8722 (6069; 12,363) YLD/105 in this cohort, whereas 44% of disability burden was attributable to infections and bronchiectasis. The total individual burden of CVID was 36,785 (33,078, 41,380) DALY/105. With estimated CVID prevalence of ~ 1/ 25,000, the societal burden of CVID ensued 1.5 (1.3, 1.7) DALY/105 of the general population. In exploratory analysis, increased mortality was associated with solid tumor, HR (95% CI): 2.69 (1.10; 6.57) p = 0.030, lymphoma: 5.48 (2.36; 12.71) p
SCOPUS: ar.j
info:eu-repo/semantics/published
Databáze: OpenAIRE
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