Annual report of the Scientific Network on BSE-TSE 2015

TECHNICAL REPORT APPROVED: 10 December 2015 PUBLISHED: 11 December 2015 Annual report of the Scientific Network on BSE-TSE 2015 Abstract European Food Safety Authority The EFSA Scientific Network on bovine spongiform encephalopathies and other transmissible spongiform encephalopathies (BSE-TSE) held its 10th meeting on 7 and 8 October 2015 in Parma. The meeting served as an opportunity to exchange scientific information on BSE-TSE related issues among EU Member States, countries from the European Free Trade Association (EFTA), EFSA, the European Commission and ad hoc participants. In this occasion, ad hoc representation included the World Animal Health Organisation (OIE), a scientific expert on genetic breeding for resistance to Classical scrapie in goats, and two scientific experts on human variant Creutzfeldt-Jakob disease (vcjd). The topics discussed included an update on BSE-TSE-related activities at national and international level and results from recent TSE-related research projects and risk assessments in several EU countries. In addition, the investigations of recent Classical BSE cases, the monitoring and control of Classical scrapie in small ruminants, and the distribution and trends of vcjd cases in humans were also discussed. European Food Safety Authority, 2015 Key words: BSE, meeting, network, TSE Requestor: EFSA Question number: EFSA-Q-2015-00738 Correspondence: biocontam@efsa.europa.eu www.efsa.europa.eu/publications EFSA Supporting publication 2015:EN-915

Acknowledgements: EFSA wishes to thank the members of the Scientific Network on BSE-TSE: Austrian Agency for Health and Food Safety (Austria), Veterinary and Agrochemical Research Centre (Belgium and Luxembourg), Bulgarian Food Safety Agency (Bulgaria), Croatian Veterinary Institute and Faculty of Veterinary Medicine - University of Zagreb (Croatia), Veterinary Service Cyprus (Cyprus), State Veterinary Institute Jihlava (the Czech Republic), Technical University of Denmark - National Veterinary Institute (Denmark), Animal Health, Welfare and Feedingstuffs Department - Veterinary and Food Board (Estonia), Finnish Food Safety Authority - EVIRA (Finland), Agence Nationale de Sécurité Sanitaire (France), Friedrich Loeffler Institute - FLI and Bundesinstitut fur Risikobewertung - BfR (Germany), Ministry of Rural Development and Food (Greece), National Food Chain Safety Office - Animal Health and Animal Welfare Directorate Unit of Epidemiology (Hungary), Department of Agriculture, Food and the Marine (Ireland), Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d Aosta (Italy), Food and Veterinary Service (Latvia), State Food and Veterinary Service (Lithuania), Officer for Risk Assessment and Research - Netherlands Food and Consumer Products Safety Authority - Ministry of Economic Affairs (the Netherlands), Department of Virology - National Veterinary Research Institute (Poland), Ministry of Agriculture (Portugal), National Sanitary Veterinary and Food Safety Authority (Romania), State Veterinary and Food Administration of the Slovak Republic (Slovakia), Veterinary Administration of the Republic of Slovenia - Sector for Food Safety, Feed and Veterinary Medicinal Products (Slovenia), Subdirección General de Sanidad e Higiene Animal y Trazabilidad, Ministerio de Agricultura, Alimentación y Medio Ambiente - MAGRAMA (Spain), National Veterinary Institute - Department of Disease Control and Epidemiology (Sweden), Food Standards Agency (United Kingdom), Norwegian Veterinary Institute (Norway), and Federal Food Safety and Veterinary Office - FSVO (Switzerland) for the preparatory work on this output, and the observers of the Scientific Network on BSE-TSE: the European Commission, the World Animal Health Organisation (OIE), Dr. Jan Langeveld, Dr. Maurizio Pocchiari and Dr. Maria Puopolo for the support provided to this scientific output. Suggested citation: EFSA (European Food Safety Authority), 2015. Annual report of the Scientific Network on BSE-TSE 2015. EFSA Supporting publication 2015:EN-915. 9 pp. European Food Safety Authority, 2015 Reproduction is authorised provided the source is acknowledged. www.efsa.europa.eu/publications 2 EFSA Supporting publication 2015:EN-915

Summary Establishing a system of Networks of organisations operating in the fields within EFSA s mission is among the tasks of EFSA, according to its founding regulation (Regulation (EC) No 178/2002), in order to facilitate a scientific cooperation framework by the coordination of activities, the exchange of information, the development and implementation of joint projects, the exchange of expertise and best practices. Additionally, the EFSA Science Strategy 2012 2016 set the objective of developing, together with Member States competent authorities, multi-annual work programmes focused on filling data gaps and setting priorities for data collections. To implement the above provisions various Networks were established. The Scientific Network on bovine spongiform encephalopathies and other transmissible spongiform encephalopathies (BSE-TSE) was launched in 2006. The BSE-TSE Network had its first meeting in 2006, and, following this, one meeting per year has been held. The BSE-TSE Network is currently composed as follows: Network Members representing 27 EU MSs (Malta has not appointed a representative to this Network) and countries from the European Free Trade Association (EFTA). The European Commission Directorate-General of Health and Consumers and of Research and Innovation are also Observers of the Network. The 10th meeting of the Network was held on 7 8 October 2015 in Parma. In this occasion, ad hoc representation included the World Animal Health Organisation (OIE), a scientific expert on genetic breeding for resistance to Classical scrapie in goats, and two scientific experts on human variant Creutzfeldt-Jakob disease (vcjd). The topics discussed included an update on BSE-TSE-related activities at national and international level, results from recent TSE-related research projects and risk assessments in several EU countries, the investigations of recent Classical BSE cases, the monitoring and control of Classical scrapie in small ruminants, and the distribution and trends of vcjd cases in humans. www.efsa.europa.eu/publications 3 EFSA Supporting publication 2015:EN-915

Table of contents Abstract... 1 Summary... 3 1. Introduction... 5 1.1. Background and Terms of Reference as provided by EFSA... 5 2. Annual meeting 2015... 6 2.1. Follow-up from annual meeting 2014... 6 2.2. Update on Network Members and EFSA s activities in the field of TSE since the annual meeting 2014... 6 2.3. Results from search and safeguarding of TSE-resistant goats... 7 2.4. Update on UK/EU-RL activities and research projects... 7 2.5. Investigation on the origin of a case of Classical BSE in Ireland... 7 2.6. Investigation on the existence of spontaneous cases of Classical BSE... 7 2.7. Recent ANSES activities on TSE... 7 2.8. Practical problems and challenges with the design of TSE surveillance in small ruminants... 8 2.9. Distribution and trends of vcjd and associated risk factors... 8 2.10. Update on the OIE activities in the field of TSE... 8 2.11. Update on the activities of the European Commission in the field of TSE... 8 2.12. Future EFSA involvement in drafting the Annual report on TSE monitoring in the EU... 8 2.13. Information on a Workshop on TSE in Italy... 9 3. Planned Network activities for 2016... 9 References... 9 Abbreviations... 9 www.efsa.europa.eu/publications 4 EFSA Supporting publication 2015:EN-915

1. Introduction 1.1. Background and Terms of Reference as provided by EFSA Art. 23 (g) of the EFSA founding regulation 1 stipulates that EFSA shall establish a system of Networks of organisations operating in the fields within its mission and be responsible for their operation. Furthermore, Art. 23 (e) and Art. 33 provide for collection, collation, analysis and reporting on scientific and technical data in the fields within the Authority s mission. The aim of such networking, as defined in Art. 36, is to facilitate a scientific cooperation framework by the coordination of activities, the exchange of information, the development and implementation of joint projects, the exchange of expertise and best practices. Additionally, since in order to obtain data of adequate quality it is essential that data collections are planned over the medium to longer term, the EFSA Science Strategy 2012 2016 2 set the objective of developing, together with the Member States competent authorities, multi-annual work programmes focused on filling data gaps and setting priorities for data collections. To implement the above provisions of the founding regulation various Networks were established. In 2006 the Network on Microbiological Risk Assessment and the Network on bovine spongiform encephalopathies and other transmissible spongiform encephalopathies (BSE-TSE) convened, strengthening over time the scientific cooperation on issues of concern, anticipating and reducing the duplication of activities and hence avoiding divergence of opinions. All these ongoing initiatives were supported by consultative processes, such as the review 3 of the EFSA s Strategy for cooperation and networking with Member States. 4 In result EFSA identified four priority areas for the cooperation, notably: (i) exchange of scientific data and information, (ii) sharing of the risk assessment practices, (iii) harmonisation of risk assessment methodologies, and (iv) cooperation and coherence in communication. Meanwhile, the Management Board adopted a Decision 5 governing the establishment and operation of EFSA Networks. In 2013 the Network mandates expired. Owing to a need for a strong specific cooperation platform between EFSA and the Member States to provide advice, steer the process and help establishing a common vision, EFSA Advisory Forum at their meeting in December 2013 made recommendations with regard to the terms of reference of the Networks. Therefore it is now opportune to renew the mandates of the Networks operating in the remits of the Risk Assessment and Scientific Assistance (RASA) units. The main overall goals of the Scientific Network on BSE-TSE are: to improve dialogue among participants; to build mutual understanding of risk assessment principles; to enhance knowledge on and confidence in the scientific assessments carried out in the EU; and to provide increased transparency in the current process among Member States and EFSA. The Network also aims to raise the harmonisation level of the risk assessments developed in the European Union (EU). The Scientific Network strengthens the scientific cooperation on BSE-TSE. It aims at anticipating and reducing the duplication of activities and hence avoiding divergence of opinions. The Network is a privileged environment to share data and methodologies facilitating harmonisation of assessment practices and to assist in anticipating emerging risks in the EU. 1 Regulation (EC) No 178/2002 of the European Parliament and the Council of 28 January 2002 laying down the general principles and requirements of food law, establishing the European Food Safety Authority and laying down procedures in matters of food safety. OJ L 31, 1.2.2002, p.1 24. 2 EFSA Science Strategy 2012-2016. Available online: http://www.efsa.europa.eu/en/corporate/pub/sciencestrategy12.htm 3 Interim Review of the Strategy for Cooperation and Networking between EU Member States and EFSA. Available online: http://www.efsa.europa.eu/en/keydocs/docs/msstrategyreview.pdf 4 MB 19.12.2006 6a Strategy for cooperation and networking. Available online: http://www.efsa.europa.eu/en/keydocs/docs/ networksoperation.pdf 5 MB 18 03 10 item 7 doc 6 Management Board Decision concerning the establishment and operation of European Networks of scientific organisations operating in the fields within the Authority s mission. Available online: http://www.efsa.europa.eu/ en/scdocs/doc/panelnetworksrop.pdf www.efsa.europa.eu/publications 5 EFSA Supporting publication 2015:EN-915

The specific objectives of the Scientific Network on BSE-TSE are: a. identifying common themes and areas for mutual collaboration; b. identifying and avoiding duplication and divergence of opinion; c. identification of experts in specific areas and on special issues; d. sharing of data availability and quality; e. strengthening cooperation amongst risk assessors and risk managers; f. exchanging information between EFSA, Member States and other stakeholder; g. strengthening communication between EFSA and the EU Member States and among risk assessors, risk managers and stakeholders; h. focusing attention on and streamlining of common research needs; i. identifying potential emerging risks when addressing current issues. EFSA may entrust to the Network certain tasks, in particular preparatory work for scientific opinions, scientific and technical assistance, and collection of data. 2. Annual meeting 2015 The Scientific Network on BSE-TSE was launched in 2006. The BSE-TSE Network had its first meeting in 2006, and, following this, one meeting per year has been held. The 10th meeting of the EFSA Scientific Network on BSE-TSE was held on 7 8 October 2015 in Parma ( annual meeting 2015 ). The annual meeting 2015 was attended by representatives of 22 EU Member States (Austria, Belgium, Bulgaria, Croatia, Cyprus, the Czech Republic, Estonia, Finland, France, Germany, Greece, Hungary, Ireland, Italy, Luxembourg, the Netherlands, Poland, Romania, Slovakia, Slovenia, Spain and the United Kingdom) and by the representative of one EFTA country (Norway). The European Commission (Directorate-General Health and Consumers (DG SANTE)) was also present at the meeting. Further ad hoc attendees to this meeting included: Dr. Derek Belton (World Organisation for Animal Health (OIE)), Dr. Jan Langeveld, Dr. Maurizio Pocchiari and Dr. Maria Puopolo. Apologies were received from Network Members from Denmark, Latvia, Lithuania, Portugal, Sweden and Switzerland. Malta has not appointed a representative to this EFSA Network. Topics discussed during the meeting are summarised in the following sections. 2.1. Follow-up from annual meeting 2014 During the 2014 Network meeting, the Network expressed an interest to strengthen the collaboration of the Network with the OIE and other international organisations/agencies, and to consider the invitation of representatives from Third Countries in order to have insights on BSE-TSE related issues from a non-eu perspective (EFSA, 2014). In reply to these suggestions, OIE participation was confirmed for the 2015 Network meeting, and EFSA has taken contacts with the European Centre for Disease Prevention and Control (ECDC), inviting representatives from the ECDC former EuroCJD Network for participation in the 2015 Network meeting. Invitation of representatives from Third Countries will be considered in future meetings. 2.2. Update on Network Members and EFSA s activities in the field of TSE since the annual meeting 2014 The Members of the Network provided an update on the situation of classical and atypical TSE in their countries and summarised to the group the scientific activities in the field of TSE, including risk assessment activities, which had been undertaken in their respective countries since the previous Network meeting. Austria, the Czech Republic and France reported that the EFSA TSE infectivity (TSEi) Model presented during the 2014 Network Meeting, and provided by EFSA to the three countries upon request, was asked and possibly used for study/research purposes. www.efsa.europa.eu/publications 6 EFSA Supporting publication 2015:EN-915

The Secretariat of the EFSA Unit on Biological Hazards and Contaminants (BIOCONTAM) presented the EFSA activities in the field of TSE already completed and ongoing since the 2014 Network meeting. Completed activities include one Scientific Opinion of the Panel on Biological Hazards (BIOHAZ) on the zoonotic potential of ovine scrapie prions (EFSA BIOHAZ Panel, 2015). Ongoing activities include two mandates for scientific and technical assistance and a procurement activity: a) the evaluation of applications of Denmark, Finland and Sweden to be recognised as having a negligible risk status for classical scrapie; b) the revision of the BSE monitoring regime in Croatia; c) procurement activity on an experimental study on the infectivity of sheep embryos. 2.3. Results from search and safeguarding of TSE-resistant goats Dr. Jan Langeveld presented to the Network an overview on the results of several research projects carried out in the EU over 13 years, focusing on the work conducted in the last few years by the Goat-TSE-FREE consortium in relation to genetic resistance to TSE in goats. He presented the different goat genotypes identified in several EU countries and the association of genotype distribution and occurrence of scrapie. He presented results from experimental challenges of goats of different genotypes with different TSE agents and from the use of several mouse models to study TSE infectivity in goats. Dr. Jan Langeveld concluded that, based on current scientific evidence, the K222 allele and some other polymorphisms of the prion protein gene have shown to confer resistance to TSE in goats. The representative of Cyprus indicated that the studies on genetic resistance to classical scrapie in goats in Cyprus, presented to the 2014 Network meeting, have been progressing and that final results will be reported soon to the European Commission. 2.4. Update on UK/EU-RL activities and research projects The representative of the United Kingdom (UK) presented to the Network an update of the results of 2015 TSE surveillance in the UK, also including a recent case of classical BSE in a cow, currently under investigation. She presented results of studies on atypical BSE transmission to sheep and of studies investigating the presence of prion accumulation in human appendices. She also updated the Network on the activities of the EU Reference Laboratory (EU-RL) for TSE, including results from studies on the diagnostic sensitivity of rapid tests for the detection of TSE in goats. 2.5. Investigation on the origin of a case of Classical BSE in Ireland The representative of Ireland updated the Network on the classical BSE case detected in a 5-year-old cow in June 2015 in Ireland. He presented the results of the epidemiological investigation aimed at detecting the origin of the case, which has not produced so far evidence to support horizontal transmission of classical BSE to the cow following to environmental exposure or feed, vertical transmission or iatrogenic transmission. 2.6. Investigation on the existence of spontaneous cases of Classical BSE The representatives of Poland and Germany presented to the Network the results of joint studies on classical BSE isolates from cattle and comparison with atypical BSE cases using biochemical characterisation and mouse bioassay. 2.7. Recent ANSES activities on TSE The representative of France updated the Network on the most recent TSE risk assessments carried out by The French Agency for Food, Environmental and Occupational Health & Safety (ANSES). In particular, he presented the results of an assessment of the surveillance programmes for TSE in small ruminants in France, of two assessments of the human TSE transmission risks linked to a possible change of the list of specified risk material, and of one assessment of the risks linked to the use of ruminant fat in animal feed. www.efsa.europa.eu/publications 7 EFSA Supporting publication 2015:EN-915

2.8. Practical problems and challenges with the design of TSE surveillance in small ruminants The representative of Croatia updated the Network on the TSE surveillance in small ruminants in his country. Surveillance in small ruminants started in Croatia in 2002 and the number of animals tested increased only in 2010 2015. Despite almost 11,000 tests in sheep and 3,000 tests in goats, classical scrapie has never been detected, and only two cases of atypical scrapie (in 2013 and 2015) have been detected. Also considering the epidemiological situation in neighbouring countries, he concluded that it seems surprising that classical scrapie has not been detected in Croatia so far. A discussion followed on relevant issues that Croatia may consider when studying the situation and designing and implementing a surveillance programme aimed at maximising the chances to detect classical scrapie if present, including: a) investigation on genotypes of the small ruminant population; b) implementation of risk-based active surveillance (e.g. focused on fallen stock); c) use of incentives to encourage reporting and presentation of animals for testing; d) consideration to breeding for genetic resistance. 2.9. Distribution and trends of vcjd and associated risk factors Dr. Maurizio Pocchiari, representative of the former EuroCJD Network, managed by the ECDC, presented to the Network a recent analysis on the distribution and trends of human vcjd cases. He discussed vcjd case definition, clinical and diagnostic features, including differences between EU countries, and risk factors for vcjd development. Dr. Pocchiari recommended continued surveillance of all forms of CJD in the EU, also to allow a better study and comparison of human and animal TSE epidemiological data. 2.10. Update on the OIE activities in the field of TSE Dr. Derek Belton, representative of the OIE, explained to the Network the procedure for setting OIE standards and provided an update on the recent activities carried out at the OIE in relation to TSE. In 2015 the OIE Terrestrial Animal Health Code was updated to specifically exclude atypical BSE from the OIE process of official BSE risk status recognition as a condition believed to occur spontaneously in all cattle populations at a very low rate. 2.11. Update on the activities of the European Commission in the field of TSE The representative of the European Commission (DG SANTE) updated the Network on the recent TSErelated risk management activities in the European Commission, as a follow-up of the TSE Road Map 2, in relation to feed ban, specified risk material, TSE surveillance in cattle and small ruminants, and other measures on scrapie eradication and intra-eu trade. The European Commission is planning to send EFSA new mandates in relation to genetic resistance to classical scrapie in goats and an update on a past EFSA risk assessment of the BSE risk in processed animal proteins. Representatives of Member States and of the European Commission discussed recent developments of the TSE surveillance and control measures implemented in the EU and their possible impact. 2.12. Future EFSA involvement in drafting the Annual report on TSE monitoring in the EU The EFSA BIOCONTAM Secretariat updated the Network on the future transfer from the European Commission to EFSA of the responsibility for the preparation of the annual report on animal TSE surveillance in the EU (as of 2016, with respect to the analysis of 2015 data), and related data collection (as of 2017, with respect to 2016 data). In relation to 2015, no changes are expected for Member States on the collection and transmission of data to the European Commission, while in future years EFSA will reflect on the opportunity to adjust the requirements and the system for data collection. www.efsa.europa.eu/publications 8 EFSA Supporting publication 2015:EN-915

2.13. Information on a Workshop on TSE in Italy The representatives of Italy provided to the Network information with respect to a Workshop on TSE that is going to take place on 10 December 2015 in Turin (Italy), organised by the Italian National Reference Centre for TSE, and intended for EU Candidate and Potential Candidate Countries. 3. Planned Network activities for 2016 Network members were invited to provide, also after the meeting, suggestions for improvement of the functioning of the Network and possible issues for future discussion in the Network. The next meeting of the EFSA Network on BSE-TSE will be organised during the third quarter of 2016. References EFSA (European Food Safety Authority), 2014. Annual report of the Scientific Network on BSE-TSE 2013. EFSA supporting publication 2014:EN-714, 9 pp. EFSA BIOHAZ Panel (EFSA Panel on Biological Hazards), 2015. Scientific Opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions. EFSA Journal, 2015;13(8):4197, 58 pp. doi:10.2903/j.efsa.2015.4197 Abbreviations ANSES The French Agency for Food, Environmental and Occupational Health & Safety BIOCONTAM EFSA Unit on Biological Hazards and Contaminants BIOHAZ Biological Hazards BSE Bovine spongiform encephalopathy DG SANTE Directorate-General Health and Food Safety ECDC European Centre for Disease Prevention and Control EFSA European Food Safety Authority EFTA European Free Trade Association EU European Union EU-RL European Union Reference Laboratory OIE World Health Organization RASA Risk Assessment and Scientific Assistance TSE Transmissible spongiform encephalopathies TSEi TSE Infectivity (model) UK United Kingdom vcjd Variant Creutzfeldt-Jakob disease www.efsa.europa.eu/publications 9 EFSA Supporting publication 2015:EN-915