Epidemiology of European adults with cystic fibrosis

The European Cystic Fibrosis Society Patient Registry (ECFSPR) collects anonymised demographic and clinical data from consenting people with CF in Europe. The aim of this study is to describe the adult population with CF in Europe. We considered patients of 18 years or older in the 2017 data, the latest year of follow-up available. Countries with a coverage below 80% were not considered when reporting figures by country. Percentages are computed for categorical variables; quartiles for numerical variables. For the year 2017, the ECFSPR database contains data of 48,204 patients from 35 countries. 24,491 (51.3%) are adults. The percentage of adults varies from 6.5% in Albania to 62.8% in Sweden. The proportion of adult females varies from 37.1% in North Macedonia to 59.6% in Slovenia. The highest percentage of patients living with lung transplant is 22.7% in Slovenia. Considering patients of 18–29 years, the lowest percentage of chronic Pseudomonas Aeruginosa is 30.6% in France, the highest is 79.4% in Serbia. For chronic Burkholderia cepacia complex species the lowest percentage of infected patients is 2.5% in Italy, the highest 20.6% in Serbia. The lowest percentage of CF related diabetes is 7.3% in Slovak Republic, the highest 34.2% in the Czech Republic. Median of BMI changes from 18.8 in the Russian Federation to 22.4 kg/m2 in Luxembourg. Considering patients not transplanted, we observe a percentage of patients with FEV1% below 40% that varies from 3.0% in Denmark to 28.5% in the Russian Federation. We considered only countries with a coverage of 80% or higher, for a realistic reflection of CF and observed variation among those countries. Data show that the percentage of adult population is higher in North Europe, infections are more frequent in Eastern Europe and growth and lung function are worse in Eastern Europe. Further investigation is required to investigate differences in healthcare systems to plan an adequate development of CF care services for adult CF people.