Author summary Cystic echinococcosis (CE) is a neglected zoonosis caused by infection with the parasiteEchinococcus granulosus sensu lato, naturally transmitted between canids and livestock; CE in humans can be a serious condition. In endemic areas, CE is responsible for significant health and economic losses, but its real burden is difficult to estimate.E.granulosus s.l. is especially present in areas where livestock breeding is practiced, including European Mediterranean and Balkan countries. We carried out a systematic literature review on the epidemiology ofE.granulosus s.l. human and animal infection in this area in 2000-2019. Data were extracted from 79 publications, and referred to Nomenclature of Territorial Units for Statistics (NUTS) levels per year. Average annual incidence rates of human CE ranged from 0.10-7.74/100,000; pooled prevalences ranged from 0.003-64.09% in cattle, 0.004-68.73% in sheep, and 0-31.86% in dogs. Bovine data showed a more similar pattern to human data compared to sheep and dogs. Our results confirm that Italy, Spain, and Eastern Europe are the most affected areas, but data are extremely heterogeneous, geographical coverage very patchy, and human prevalence studies extremely scant. Results also highlight the well-known problem of underreporting ofE.granulosus s.l. infection in both humans and animals. Cystic echinococcosis (CE) is a neglected zoonosis caused by infection with the cestodeEchinococcus granulosus sensu lato. We carried out a systematic literature review onE.granulosus s.l. human and animal (cattle, sheep, dog) infection in European Mediterranean and Balkan countries in 2000-2019, to provide a picture of its recent epidemiology in this endemic area. MEDLINE, EMBASE, Scopus, Google Scholar and Open Grey databases were searched. Included cases were: i) for humans, data from hospital records and imaging studies; ii) for dogs, data from necropsy and coprological studies; iii) for ruminants, cases based on slaughter inspection. The NUTS (Nomenclature of Territorial Units for Statistics) classification was used to categorize extracted data in epidemiological units, defined as data referred to one NUTS2 (basic region) in one year time. Data were then aggregated to NUTS1 level (major regions), calculating the average incidence value of included epidemiological units. For prevalence studies covering different epidemiological units, the pooled prevalence was estimated. Data were extracted from 79 publications, 25 on human infection (covering 437 epidemiological units), and 54 on animal infection (52 epidemiological units for cattle, 35 for sheep and 25 for dogs). At NUTS1 level, average annual incidence rates of human CE ranged from 0.10-7.74/100,000; pooled prevalence values ranged from 0.003-64.09% in cattle, 0.004-68.73% in sheep, and 0-31.86% in dogs. Southern and insular Italy, central Spain, Romania and Bulgaria reported the highest values. Bovine data showed a more similar pattern to human data compared to sheep and dogs. Limitation of evidence included the paucity of human prevalence studies, data heterogeneity, and the patchy geographical coverage, with lack of data especially for the Balkans. Our results confirm Italy, Spain, and Eastern Europe being the most affected areas, but data are extremely heterogeneous, geographical coverage very patchy, and human prevalence studies extremely scant. Results also highlight the notorious problem of underreporting ofE.granulosus s.l. infection in both humans and animals.
