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

• Published in: Orphanet journal of rare diseases 2018-11, Vol.13 (1), p.201-201, Article 201
• Main Authors: Odnoletkova, Irina, Kindle, Gerhard, Quinti, Isabella, Grimbacher, Bodo, Knerr, Viviane, Gathmann, Benjamin, Ehl, Stephan, Mahlaoui, Nizar, Van Wilder, Philippe, Bogaerts, Kris, de Vries, Esther
• Format: Article
• Language: English
• Subjects: Age; Autoimmunity; Bacterial infections; Bronchiectasis; Burden of disease; Common variable immunodeficiency; Common Variable Immunodeficiency - economics; Common Variable Immunodeficiency - epidemiology; Comorbidity; Cost of Illness; DALY; Death; Defects; Diagnosis; Europe - epidemiology; Health economics; Health risk assessment; Humans; Immunoglobulins; Immunologic deficiency syndromes; Infections; Life Expectancy; Lung diseases; Lymphoma; Medical diagnosis; Medical research; Mortality; Patient outcomes; Patients; Population; Primary antibody deficiency; Primary immunodeficiency; Quality of life; Questionnaires; Rare diseases; Registries; Retrospective Studies; Solid tumors
• ISSN: 1750-1172; 1750-1172
• DOI: 10.1186/s13023-018-0941-0

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 10 of the CVID cohort (the individual disease burden), and of the general population (the societal disease burden). 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/10 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/10 . With estimated CVID prevalence of ~ 1/ 25,000, the societal burden of CVID ensued 1.5 (1.3, 1.7) DALY/10 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