COMMITTED TO PATIENTS

Chiesi partners with Wirral Primary Care Collaborative to advance early detection of undiagnosed COPD

It is estimated that up to 5,000 people are living with undiagnosed COPD on the Wirral, where prevalence is more than 40% higher than the national average1 COPD is a leading cause of emergency hospital admissions, with an average stay of seven days2 Chiesi UK and Ireland and the Wirral Primary Care Collaborative have launched the COMET clinic - a community-based clinic designed to accelerate COPD assessment and diagnosis, reduce system burden and improve patient outcomes COMET Clinic Launch Manchester, UK - Tuesday 6th May – Chiesi UK and Ireland and Wirral Primary Care Collaborative (WPCC) have today announced the launch of COMET (COPD Targeted Management, Early Intervention and Treatment), aimed at improving early diagnosis and management of Chronic Obstructive Pulmonary Disease (COPD) in the Wirral area. The initiative aims to ease pressure on primary care and A&E services by offering early diagnosis and evidence-based interventions. Individuals identified as high risk of COPD are invited to attend a community diagnostic centre (CDC) for spirometry assessment and then a diagnostic clinic for immediate management (if diagnosed). These services will be delivered directly within the community, ensuring timely access to care. Rachel Voller, Advanced Nurse Practitioner at Moreton & Meols PCN, said: “An estimated two million people live with undiagnosed COPD in the UK, with symptoms like breathlessness and chronic cough often mistaken for fatigue or ageing. These delays in diagnosis lead to reduced quality of life for patients, costly emergency hospital admissions and irreversible lung damage. The lack of resources and funding across the UK means diagnostics in COPD, such as spirometry, are not always readily available in primary care. By establishing COMET, we’re equipping the NHS and supporting patients to take control of their lung health, improving early diagnosis and accelerating access to care.” COPD is not only debilitating for patients, but also places a growing burden on the NHS, costing an estimated £1.9 billion annually in England alone.3 The UK continues to have some of the poorest respiratory health outcomes in Europe, with higher mortality rates from COPD than in comparable countries.4 Despite the scale and severity of the disease, COPD can often be over-looked, meaning opportunities for early diagnosis are frequently missed.3 Many cases are identified incidentally, through initiatives such as NHS lung health checks, or during emergency admissions for other conditions, by which point irreversible lung damage may already have occurred. On the Wirral, where COPD prevalence is 2.6% compared to the national average of 1.8%, the COMET initiative aims to address this challenge by proactively identifying those at risk and providing timely access to spirometry and clinical assessment.1 COMET enables those with symptoms and/or CT scan findings consistent with emphysema to be referred for spirometry at local Community Diagnostic Centres before inviting them to the COMET clinic for a full clinical assessment and follow-up. Designed to deliver equitable, community-based care to over 1,000 people, the programme aims to equip patients with the support they need to take control of their lung health while also helping ease the pressure on overstretched GP practices and emergency departments in the Wirral, while identifying feasible approaches that the NHS can embed in the future. Building on Previous Success The partnership builds on insights from FRONTIER, a hospital-based programme in Hull which demonstrated that targeted screening can tackle underdiagnosed COPD by improving access to testing and care. By contrast, COMET brings diagnostic assessment and management into Primary Care for patients taking part in the NHS Lung Cancer Screening Programme on the Wirral, expanding early detection and intervention beyond the hospital setting. Harriet Lewis, Senior Director of Public Affairs and Communications at Chiesi UK and Ireland, comments: “At Chiesi, we believe early action is key to improving outcomes for people living with COPD. COMET has been designed to ensure access to diagnostic services is simple, breaking the cycle of delayed diagnosis and avoidable hospital admissions. By delivering care directly within the community, this partnership is integral to improving access to testing and care, easing NHS pressures and demonstrating how partnerships can drive scalable and sustainable change.” About COMET COPD Targeted Management, Early Intervention and Treatment (COMET) is a collaborative working project by Chiesi UK & Ireland and Wirral Primary Care Collaborative Limited. It aims to enable early COPD diagnosis and timely initiation of evidence-based pharmacological and non-pharmacological interventions to improve outcomes for patients. As part of the project, participants of the NHS Lung Cancer Screening Programme on the Wirral, who are identified as having a high risk of COPD based on symptoms and/or imaging findings are invited to attend a community diagnostic centre (CDC) for spirometry assessment and then a diagnostic clinic for immediate management. COMET will run for 18 months, evaluating the impact of delivering COPD targeted diagnosis and care in a primary care setting as a proof of concept project for potential broader adoption. https://www.chiesi.uk.com/comet About Wirral Primary Care Collaborative (WPCC) Established in 2022, the WPCC was created by the Primary Care Networks (PCNs) and Service Delivery Units (SDUs) of the Wirral to form a single provider entity, representing all General Practices across the region. WPCC enables General Practice to negotiate, collaborate and potentially bid for contracts on the same footing as other healthcare providers such as Wirral University Teaching Hospital NHS Foundation Trust and Community Trust services. The WPCC’s involvement at a system level ensures they are able to be represented and informed in terms of local priorities, and financial constraints, including the significant financial pressures, which are impacting all providers, including General Practice. The role of the WPCC is vital to ensure Primary Care is at the centre of future discussion as the NHS 10-year Plan develops and the landscape shifts to a Neighbourhood Health Service. About Chiesi Group Chiesi is a research-oriented international biopharmaceutical group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company’s mission is to improve people’s quality of life and act responsibly towards both the community and the environment. By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi’s commitment to create shared value for society as a whole is legally binding and central to company-wide decision-making. As a certified B Corp since 2019, we’re part of a global community of businesses that meet high standards of social and environmental impact. The company aims to reach Net-Zero greenhouse gases (GHG) emissions by 2035. With over 85 years of experience, Chiesi is headquartered in Parma (Italy), with 31 affiliates worldwide, and counts more than 7,000 employees. The Group’s research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden. For further information please visit www.chiesi.uk.com. Media contacts Yasmin Ghariani, Chiesi UK Head of External Communications Phone: (+44) 161 488 5555 Email: y.ghariani@chiesi.com References Public Health Profiles: Public health profiles – OHID. Available at: phe.org.uk. Last Accessed April 2025 NHS Health Education England, Respiratory Disease: Applying All Our Health (2021). Available at: Respiratory Disease Report. Last Accessed April 2025 Asthma + Lung UK. Diagnosing the Problem. Right Test, Right Time. Available at Diagnosing the Problem. Last Accessed April 2025 Asthma + Lung UK: Lung conditions kill more people in the UK than anywhere in Western Europe. Press Release. Last Accessed April 2025 UK-CHI-2500481 | May 2025

Chiesi Group delivers on transformational strategy with FY2024 double-digit growth (13% at CER), strong investment in innovation and continued focus on sustainability

HIGHLIGHTS €3.4 billion revenues, up 13% at constant exchange rates (CER) compared to 2023 Record R&D investment of €829 million (24.3% of revenues) Profitability close to 30% for the fifth consecutive year Air franchise: revenues of €1.8 billion, a growth of 7.2% (CER). Fastest growing company product (fixed triple combination inhaler for asthma and COPD patients) hits 35% sales growth Rare disease business unit: revenues of €763 million (up 41%, CER), representing 22% of total Group sales Care franchise: revenues of €818 million, up 6% (CER) Parma (Italy), April 16th, 2025 – Chiesi, an international research-oriented biopharmaceutical group headquartered in Parma, Italy, with commercial affiliates in 31 countries, closed 2024 with strong financial results, reaffirming its trajectory of sustainable, long-term growth with a strong commitment to fostering R&D and innovation.Revenues reached €3,413 million, marking a 13% increase at CER compared to 2023. EBITDA was just over €1 billion, up 12%, with a margin close to 30%.Chiesi Group’s net profit totaled €493 million, and its positive net financial position (cash minus financial liabilities including leasing) reached €535 million. Giuseppe Accogli, CEO of Chiesi Group, commented on the results:“Chiesi Group delivered an impressive performance, making 2024 the third consecutive year of double-digit growth. These results highlight the positive impact of our clear strategy, our innovative, comprehensive and diversified product portfolio, and strong international footprint. Our performance was further accelerated by the successful implementation of the new operating model, designed to drive positive impact for Patients, Planet, People, and Prosperity. This, combined with our outstanding operational excellence, strong internal talent and company culture, gives us confidence that we are firmly on track to meet our ambitious goals of mid- to high-single digit sales growth over the next five years. Our prospects are strong, thanks to the focus on our own existing pipeline with 43 projects, external collaboration push, and significant investments in manufacturing, such as the Biotech Center of Excellence and the newly acquired production site in Nerviano.” GEOGRAPHICAL DISTRIBUTION In 2024, the Group recorded revenue growth across all markets in which it operates. Europe remained the highest contributor, accounting for 64% of total revenues (led by UK, Spain, and France) and registering approximately 10% growth in absolute terms. The Group achieved particularly strong results in the U.S., which is now the largest single-country market by revenue at 20% of total turnover, and with approximately 24% increase in absolute terms. China also delivered an impressive 22% growth at CER. THERAPEUTIC AREAS The Air franchise, which includes products and services for the treatment of respiratory diseases, remained the primary therapeutic area for Chiesi, accounting for €1,832 million - 54% of total revenue. The fixed-triple combination for the treatment of asthma and COPD grew 35% year-on-year. The Carbon-Minimal Inhaler program to reformulate Chiesi's inhaler products with a new, low-carbon propellant, made significant progress. Phase III clinical trials were initiated to support the introduction of Chiesi's respiratory products into the U.S. market. Chiesi also entered into a collaboration with U.S.-based Gossamer Bio to develop a treatment for Pulmonary Arterial Hypertension (PAH) and pulmonary hypertension associated with interstitial lung diseases (PH-ILD). The Care franchise accounted for €818 million - 24% of the Group's revenue . To further strengthen its commitment to supporting the most vulnerable patients, Chiesi continued the collaboration with U.S. biotech company Oak Hill Bio , launched in 2023, with the enrollment of the first patient in a Phase IIb clinical study for a new experimental drug to treat complications of extreme prematurity. The Global Rare Diseases business unit had revenues of €763 million (up 41%), driven by the full integration of Amryt Pharma, acquired in 2023, and the launch of three new products in the U.S. market, following FDA approvals received in the previous year. This represents 22% of revenue (a 4% increase year-on-year) and is one of the Group’s key strategic development areas. R&D, PARTNERSHIPS, AND PRODUCTION INVESTMENTS In 2024, R&D spending reached a record €829 million (24.3% of revenue), an increase from €721 million in the previous year. This growth was primarily driven by Phase III clinical trials for the entry of respiratory products into the U.S. market and the strategic expansion of the pipeline across all therapeutic areas. According to the JRC EU Industrial R&D Investment Scoreboard 2024, Chiesi remained the top pharmaceutical company in Italy for R&D investments and the 11th in Europe. Chiesi also filed 33 new patent applications with the European Patent Office (EPO) in 2024, a testament to the strategic importance of R&D. In September 2024, the Group inaugurated its Biotech Center of Excellence in Parma, with a total investment of €380 million planned between 2024 and 2030, which will also enhance the competitiveness of Italy and Europe in the global biopharmaceutical sector. PEOPLE In 2024, Chiesi Group created over 500 new roles, and increased its workforce by 7.6% workforce compared to the previous year, reaching a total of 7,542 employees globally. The Group also continued to invest in internal talent development, promoting its Human Leadership model and enhancing succession pipelines. Chiesi’s dedication to fostering a positive workplace culture was recognised by Great Place to Work and Fortune magazine, with the company placing on their 25-strong World’s Best Workplaces list - the only European pharmaceutical company and the first-ever Italian company to achieve this. PLANET In 2024, Chiesi made significant strides in its journey to combating climate change and promoting sustainability, with the approval of its greenhouse gas (GHG) emissions reduction targets by the Science Based Targets Initiative (SBTi). Chiesi also increased its EcoVadis rating, confirming the Platinum Medal and ranking among the top 1% of companies worldwide for outstanding performance in Environment, Ethics, Labor and Human Rights, and Sustainable Procurement. The Group has committed to achieving Net Zero Emissions by 2030 for direct emissions (Scopes 1 and 2) and by 2035 for all indirect emissions (Scope 3). OUTLOOK 2025 According to Giuseppe Accogli, CEO of Chiesi Group: “In 2025, Chiesi aims to continue to grow by mid-single digits, and achieve strong cash flow generation and a solid financial position. This will enable us to further boost our future growth, via significant investments in innovation, acceleration in external partnerships and potential acquisitions to expand our portfolio and pipeline across all development stages. Our goal is to never leave any patients behind, and improve their quality of life by providing advanced therapeutic solutions and expanding production capacity to meet increasing product volumes required.” About Chiesi Group Chiesi is a research-oriented international biopharmaceutical group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company's mission is to improve people's quality of life and act responsibly towards both the community and the environment. By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi's commitment to creating shared value for society as a whole is legally binding and central to company-wide decision-making. As a certified B Corp since 2019, we're part of a global community of businesses that meet high standards of social and environmental impact. The company aims to reach Net-Zero greenhouse gases (GHG) emissions by 2035. With over 85 years of experience, Chiesi is headquartered in Parma (Italy), with 31 affiliates worldwide, and counts more than 7,000 employees. The Group's research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden.

Chiesi Study Highlights Challenge of Incorrect Use of Inhalers in the UK

Respiratory conditions affect one in five people in the UK,2 with millions relying on inhalers every day. However, use of an inhaler beyond the number of doses stated on the device can compromise disease control, which is especially concerning in the event of an exacerbation. The UK has one of the highest asthma death rates in Europe - with two-thirds considered preventable.3 Manchester, UK, 20th January 2025 – Chiesi UK & Ireland has published new research in the BMJ Open Respiratory Research, demonstrating that over 50% of UK patients do not know when their inhaler is empty, with many using their device beyond the number of doses available, leading to concerns over patient safety.1 The research involved a weighing study of returned, used inhalers to assess the number of doses remaining, complemented by a survey of almost 200 patients. It uncovered significant gaps in the understanding and practice of inhaler use and disposal among UK patients, signalling that patient education is needed to help people living with respiratory conditions understand when to request a replacement.1 Of patients surveyed, 74.9% reported using a dose counter to understand when their inhaler was empty.1 Other common indicators included when a dose of the medicine was no longer received (24.6%), when the pressurised metered-dose inhaler (pMDI) felt empty on shaking (22.6%), and when pMDIs stopped 'puffing' (19.1%).1 There are many ways patients can be supported to act at the appropriate time including taking note of the time period their inhaler doses are expected to cover, using inhalers with dose counters, or using charts.4 Professor Anna Murphy, Consultant Respiratory Pharmacist and study co-author, commented: "This research shines a light on a concerning reality that patients lack the knowledge to know when to replace their inhaler device. Patients may therefore be at risk of serious exacerbations and, potentially, hospitalisation due to overuse of their inhalers. This is further concerning during the winter season when emergency services are already under significant strain. Tackling this challenge collectively, ensuring patients are educated, is crucial for improving outcomes." Using inhalers incorrectly, or not in line with recommendations from healthcare teams, can lead to suboptimal disease management, posing a risk to overall disease control. Disease control in the UK for respiratory conditions such as asthma and chronic obstructive pulmonary disease (COPD) are lagging behind other countries;5 the UK has one of the highest asthma death rates in Europe, many of which are preventable.3 Dr Andy Whittamore, Clinical Lead at Asthma + Lung UK said: "Many people do not know when their inhaler is empty. This puts people at risk of harm if you are continuing to use your inhaler once it's finished and you are not getting the medication you need. An empty preventer means you may develop a build up of inflammation and develop symptoms or exacerbations. An empty reliever means you cannot treat life-threatening breathlessness. Some inhalers do have dose counters, with some being more accurate than others. "The most important thing someone with a lung condition can do to protect themselves, is to take your inhaler as prescribed with the correct inhaler technique, as well as attending your annual review. If further information is required there are useful resources on the Asthma + Lung UK website https://www.asthmaandlung.org.uk/living-with/inhaler-videos. And if you need advice or support about any breathing symptoms or your lung condition you can give our Helpline team a call on 0300 222 5800 (Monday-Friday, 9am-5pm)." The new study also highlights environmental concerns associated with inhaler disposal. It found that only 27.1% of people dispose of their inhaler appropriately at the pharmacy or as part of a local inhaler recycling scheme.1 Dr Rachel Malone, Head of Medical at Chiesi UK and study author adds: "Respiratory conditions can deeply impact the lives of patients and their families. This research has revealed stark variability in inhaler use, and – in many cases – patients not using inhalers according to device guidance. Looking forward, we must raise awareness of the ways patients can be supported in understanding when their inhaler is running low, so that they can ensure they have the necessary medicine available to maintain disease control or in the event of an exacerbation. It is crucial that patients and care teams are equipped with fundamental knowledge on proper inhaler use and disposal to reduce the likelihood of exacerbations and decrease the carbon footprint associated with inhaler use." Join the Conversation Healthcare professionals are invited to join a webinar on Thursday 27th January to further explore the results of the study and key priorities moving forward. Hosted by study authors Professor Anna Murphy, Consultant Respiratory Pharmacist and Professor Will Carroll, Clinical and Academic Lead in Paediatric Respiratory Medicine, this non-promotional event is organised and funded by Chiesi UK & Ireland. More details can be found on the registration page for the event. Additional high-quality bite-size educational webinars and podcasts on a wide range of topics, intended for healthcare professionals, are also available on the Chiesi UK Medical website. About Chiesi Group Chiesi is a research-oriented international biopharmaceutical group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company's mission is to improve people's quality of life and act responsibly towards both the community and the environment. By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi's commitment to create shared value for society as a whole is legally binding and central to company-wide decision-making. As a certified B Corp since 2019, we're part of a global community of businesses that meet high standards of social and environmental impact. The company aims to reach Net-Zero greenhouse gases (GHG) emissions by 2035. With over 85 years of experience, Chiesi is headquartered in Parma (Italy), with 31 affiliates worldwide, and counts more than 7,000 employees. The Group's research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden. For further information please visit www.chiesi.uk.com. Media contacts Yasmin Ghariani, Chiesi UK Head of External Communications Phone: (+44) 161 488 5555 Email: y.ghariani@chiesi.com References Murphy AC, Carroll W, Gotsell M, et al. (2024).How do patients determine when their inhaler is empty? Insights from an analysis of returned inhalers and a patient survey. BMJ Open Respir Res;11:e002579. doi:10.1136/bmjresp-2024-002579 Public Health England (2019). The 2nd Atlas of variation in risk factors and healthcare for respiratory disease in England: Reducing unwarranted variation to improve health outcomes and value. Available at: https://fingertips.phe.org.uk/profile/respiratory-disease NRAD (2014). Why asthma still kills. The National Review of Asthma Deaths (NRAD). Available at: https://www.hqip.org.uk/wp-content/uploads/2018/04/NRAD-Annual-Report-2014.pdf Staffordshire and Stoke-on-Trent Integrated Care System (2025). Count it out. Available at: https://staffsstokeics.org.uk/~documents/route%3A/download/1067/ Hall, I., Walker, S., & Holgate, S. T. (2022). Respiratory research in the UK: investing for the next 10 years. Thorax, 77(9), 851–853. doi:10.1136/thoraxjnl-2021-218459   UK-CHI-2500048 | January 2025

Great Place To Work® and Fortune Name Chiesi Group No. 23 in 2024 World’s Best Workplaces™ List

The accolade reflects Chiesi's dedication to people's well-being, continuous improvement, and creating a positive work environment Chiesi was also recently ranked in in the Best Workplaces in Europe™ 2024, Best Workplaces for Women™ in Greater China, and UK's Best Workplaces in BioPharma™, among others Manchester, UK, 18th November 2024 – Chiesi Group, an international biopharmaceutical company focused on research, has been named one of the 25 World's Best Workplaces™ in 2024 by Great Place To Work® (GPTW) and Fortune magazine, ranking 23rd on this year's list. The news marks the first time any European pharmaceutical company has achieved the accolade by the global authority on workplace culture. Headquartered in Parma, Italy, Chiesi has 31 offices worldwide and employs over 7,000 people. In the UK, the Chiesi headquarters is in Manchester, with a research and development facility also in Chippenham. "Chiesi aims to be more than just a place of work, we want to be a community where growth, innovation and collaboration thrive," said Kate Lenik, Senior Director, HR & Support Services, UK. "Our success is a collective achievement, built on the dedication and talent of every individual who is part of our team." Great Place To Work®, and Fortune's annual list, selects 25 global organisations that participated in Great Place To Work's employee survey process, with 7.4 million responses received, representing 20 million employees worldwide. To be considered for the list, companies must be identified as outstanding global employers with recognition on at least five Best Workplaces™ lists in Asia, Europe, Latin America, Africa, North America, or Australia during 2023 or early 2024. "We are immensely proud to be the only European pharmaceutical company included in the World's Best Workplaces™ rankings. Being part of such a selective global list of only 25 companies is a source of huge motivation for continuous improvement." said Giuseppe Accogli, Chief Executive Officer, Chiesi Group. "This placement stems from our firm commitment to our people's well-being, as it reflects their direct feedback and contribution in shaping Chiesi into a place where we all enjoy working. I believe this accolade will further increase our sense of pride and belonging to a company fully dedicated to serving patients around the world in everything we do." In addition to this latest recognition, in 2024 Chiesi was also ranked as one of the top 10 Best Workplaces in Europe™. In the UK specifically, Chiesi has been recognised for over a decade in employer and workplace culture awards being named as a UK Great Place to Work™, UK Best Workplace for Wellbeing, UK Best Workplace for Women and Menopause Friendly Employer. About the Fortune World's Best Workplaces™ Great Place To Work selected the World's Best Workplaces by gathering and analysing more than 7.4 million confidential survey responses, representing the experiences of 20 million employees worldwide at Great Place To Work Certified organisations. Organisations are assessed on their efforts to create great workplaces and positively impact people and communities across multiple countries around the world. Companies must have appeared on at least five Best Workplaces lists in Asia, Europe, Latin America, Africa, North America, or Australia during 2023 or early 2024, and have at least 5,000 employees worldwide, with 40% outside of its headquartered country. Read the full methodology. To be considered, all companies use the Great Place To Work Trust Index™ survey. To learn more about the Best Workplaces in Europe™ list click here. About Chiesi Group Chiesi is research-oriented international biopharmaceutical group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company's mission is to improve people's quality of life and act responsibly towards both the community and the environment. By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi's commitment to creating shared value for society as a whole is legally binding and central to company-wide decision-making. As a certified B Corp since 2019, we're part of a global community of businesses that meet high standards of social and environmental impact. The company aims to reach Net-Zero greenhouse gases (GHG) emissions by 2035. With over 85 years of experience, Chiesi is headquartered in Parma (Italy), with 31 affiliates worldwide, and counts more than 7,000 employees. The Group's research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden. For further information please visit www.chiesi.uk.com. UK-CHI-2400939 | November 2024

Filsuvez® (birch bark extract gel) Now Available For Prescribing in Scotland for Epidermolysis Bullosa through SMC Ultra-Orphan Pathway Following Final Approval of the Data Collection Plan by Scottish Government

Filsuvez® (birch bark extract gel) – the first licenced therapy in Europe to treat junctional and dystrophic epidermolysis bullosa (EB) - is now available for prescribing via the Scottish Medicines Consortium’s (SMC) ultra orphan medicines pathway for an initial three-year period whilst further safety and efficacy data is collected. MANCHESTER, UK, 4th November 2024 – Chiesi, the international research-focused biopharmaceutical group, today announced that birch bark extract gel is now available for prescribing as a treatment option for junctional and dystrophic epidermolysis bullosa (EB) in Scotland under the SMC's ultra-orphan medicines framework.1,2 The news comes following approval earlier this year by the SMC and offers a treatment option specifically for partial-thickness wounds associated with junctional and dystrophic forms of EB. The therapy can now be prescribed for eligible patients from 30th October 2024. The availability of birch bark extract gel in Scotland represents an important milestone towards driving equal access to innovative therapies for rare diseases, with the therapy now available across Great Britain. "We are truly delighted that birch bark extract gel is now available for patients 6 months and older living with junctional and dystrophic EB in Scotland; this step is a result of close working between patient organisations, the NHS, the SMC, the Scottish Government and Chiesi. The news is even more meaningful to share as it comes during EB Awareness Week, a week dedicated to increasing awareness of this painful and debilitating genetic skin condition," said David Garzón Lafuente, Head of Rare Diseases, Chiesi UK&I. "Birch bark extract gel offers hope to patients who endure daily challenges from this condition, and we are pleased that the SMC recognises the urgent need for approved therapies." ### About epidermolysis bullosa Epidermolysis bullosa (EB) is a heterogenous group of rare inherited skin disorders caused by mutations in the genes that encode skin anchoring proteins of the dermo-epidermal junction. It is characterised by very fragile skin that is prone to blistering and erosions due to minor trauma or friction, with the resulting wounds usually extending through multiple layers of the skin surface. As well as the high wound burden, there is impaired wound healing and debilitating symptoms such as pain, pruritis, scarring, deformity, and immobility. There are also several systemic complications (for example anaemia, increased risk of infections, osteoporosis, and squamous cell carcinoma). These all can carry considerable morbidity and increased mortality risk.3,4 About Filsuvez® (birch bark extract gel) Birch bark extract is a sterile gel for cutaneous application use for the treatment of partial thickness wounds associated with dystrophic and junctional EB in patients six months and older. Birch bark extract gel is approved by the European Medicines Agency and available across Europe. The Great Britain Summary of Product Characteristics for birch bark extract gel can be found at www.medicines.org.uk/emc/product/13971/smpc. About Chiesi Group Chiesi is a research-oriented international biopharmaceutical group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company's mission is to improve people's quality of life and act responsibly towards both the community and the environment. By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi's commitment to create shared value for society as a whole is legally binding and central to company-wide decision-making. As a certified B Corp since 2019, we're part of a global community of businesses that meet high standards of social and environmental impact. The company aims to reach Net-Zero greenhouse gases (GHG) emissions by 2035. With over 85 years of experience, Chiesi is headquartered in Parma (Italy), with 31 affiliates worldwide, and counts more than 7,000 employees. The Group's research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden. For further information please visit www.chiesi.uk.com. About Chiesi Global Rare Diseases Chiesi Global Rare Diseases is a business unit of the Chiesi Group established to deliver innovative therapies and solutions for people affected by rare diseases. As a family business, Chiesi Group strives to create a world where it is common to have a therapy for all diseases and acts as a force for good, for society and the planet. The goal of the Global Rare Diseases unit is to ensure equal access so as many people as possible can experience their most fulfilling life. The unit collaborates with the rare disease community around the globe to bring voice to underserved people in the health care system. Media Contacts Yasmin Ghariani, Chiesi UK Head of External Communications Phone: (+44) 161 488 5555 Email: y.ghariani@chiesi.com References The Scottish Medicines Consortium (SMC). birch bark extract gel (Filsuvez®): initial assessment. Available at: https://scottishmedicines.org.uk/medicines-advice/birch-bark-extract-filsuvez-uo-pathway-smc2651/ The Scottish Medicines Consortium (SMC). Ultra-orphan medicines for extremely rare conditions. Available at: https://www.scottishmedicines.org.uk/how-we-decide/ultra-orphan-medicines-for-extremely-rare-conditions/ Has C, Bauer JW, Bodemer C, Bolling MC, Bruckner-Tuderman L, Diem A, et al. Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility. Br J Dermatol. 2020 Oct;183(4):614-627. doi: 10.1111/bjd.18921 Pharmaceuticals DAC. Betula pendula Roth, Betula pubescens Ehrh. (as dry extract, refined) (Filsuvez Gel®) Summary of product characteristics. Electronics Medicines Compendium www.medicines.org.uk   UK-CHI-2400902 | November 2024

Chiesi Global Rare Diseases Announces Publication of Results from Phase 3 BRIGHT Study of Pegunigalsidase Alfa in Fabry Disease

Data published in Journal of Inherited Metabolic Disease This article is created and funded by Chiesi PARMA, 29th, 2024 – Chiesi Global Rare Diseases, a business unit of the Chiesi Group established to deliver innovative therapies and solutions for people living with rare diseases, today announced the publication of results from the Chiesi Phase 3 BRIGHT study of pegunigalsidase alfa 2 mg/kg administered every four weeks for 52 weeks in adult patients with Fabry disease who were previously treated with agalsidase alfa or beta administered every two weeks. The data are published in the Journal of Inherited Metabolic Disease.1 "We are delighted to see the publication of these data in the Journal of Inherited Metabolic Disease," said Dr Kamran Iqbal, Head of Medical Affairs (UK and Ireland) - Global Rare Diseases at Chiesi. "While the findings are encouraging, further studies will be important to better understand the implications of this investigational 4 weekly dosing regimen with pegunigalsidase alfa, for suitable patients living with Fabry disease." Pegunigalsidase alfa is a PEGylated α-Gal A enzyme replacement therapy (ERT) that is approved for the treatment of adults with Fabry disease in the United States, European Union and Great Britain at the recommended dose of 1 mg/kg of body weight administered once every two weeks. The regimen of pegunigalsidase alfa 2mg/kg administered every four weeks is investigational and not approved by the EMA or other regulatory authorities. Interactions with regulatory authorities to investigate the feasibility of introducing every four weeks posology as an alternative dosing regimen are underway. A total of 29 patients (23 men, 6 women) completed the Phase 3 open-label, single arm, switchover, 12-month BRIGHT study that was designed to assess the safety and efficacy of pegunigalsidase alfa (2 mg/kg) administered intravenously every four weeks in Fabry patients who had previously been treated with agalsidase alfa (0.2 mg/kg) or agalsidase beta (1 mg/kg) every two weeks for at least three years. The primary endpoint was the number of treatment-emergent adverse events (TEAEs). Other key safety endpoints included occurrence of infusion-related reactions and the development of ADAs directed against pegunigalsidase alfa. Key efficacy endpoints included change in eGFR (calculated using the CKD-EPI equation) and change in plasma concentrations of globotriaosylsphingosine (lyso-Gb3) over the trial period. Additional assessments included patient-reported outcomes and pharmacokinetics. Results from the Phase 3 BRIGHT study showed: There were no new safety concerns among adult patients treated with pegunigalsidase alfa. Overall, 33 (18%) out of a total of 182 TEAEs, reported in 9 (30%) patients in the safety population, were considered treatment-related, and the majority of patients (7/9) with treatment-related TEAEs were male All treatment-related events were mild or moderate in severity, and there were no TEAEs leading to study discontinuation or death 5/30 (16.7%) patients experienced at least one infusion-related reaction, all of which were mild or moderate No patients developed de novo anti-drug antibodies (ADAs) Median estimated glomerular filtration rate (eGFR) change from baseline was -1.9 mL/min/1.73m2/year (-2.4 in males, -0.7 in females) after 12 months of treatment with pegunigalsidase alfa. Plasma lyso-Gb3 concentrations were low and stable in women treated with pegunigalsidase alfa (0/6 ADA-positive), with a slight increase observed in treated men (9/24 ADA-positive) compared to baseline. Pegunigalsidase alfa median plasma concentrations at the end of each 4-week dosing interval were above the lower limit of quantification. "The availability of ERTs for Fabry disease has improved clinical outcomes and disease management for many patients, but there is still room for improvement and a need for additional treatment options. For example, the bi-weekly infusion schedule can often represent a burden for some patients," said Ales Linhart, professor, First Faculty of Medicine, Charles University and General University Hospital in Prague, Czech Republic, author of the publication. "The BRIGHT study results show that 2 mg/kg of pegunigalsidase alfa administered every four weeks is generally well tolerated in adult patients and that this schedule deserves further exploration". "Fabry disease is a complex, life-altering condition that profoundly affects quality of life. While existing treatments have, over the years, enhanced patient autonomy and lightened the load on caregivers, many challenges remain, one of them being the infusion frequency", said Mary Pavlou, President of Fabry International Network. "From a patient's perspective, reducing the frequency of infusions could significantly improve care and enhanced quality of life for both patients and their families, resulting in better health outcomes". About Chiesi Global Rare Diseases Chiesi Global Rare Diseases is a business unit of the Chiesi Group established to deliver innovative therapies and solutions for people affected by rare diseases. As a family business, Chiesi Group strives to create a world where it is common to have a therapy for all diseases and acts as a force for good, for society and the planet. The goal of the Global Rare Diseases unit is to ensure equal access so as many people as possible can experience their most fulfilling life. The unit collaborates with the rare disease community around the globe to bring voice to underserved people in the health care system. About Chiesi Group Chiesi is a research-oriented international biopharmaceutical group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company's mission is to improve people's quality of life and act responsibly towards both the community and the environment. By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi's commitment to create shared value for society as a whole is legally binding and central to company-wide decision-making. As a certified B Corp since 2019, we're part of a global community of businesses that meet high standards of social and environmental impact. The company aims to reach Net-Zero greenhouse gases (GHG) emissions by 2035. With over 85 years of experience, Chiesi is headquartered in Parma (Italy), with 31 affiliates worldwide, and counts more than 7,000 employees. The Group's research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden. For further information please visit www.chiesi.uk.com. References 1. Holida M, Linhart A, Pisani A, Longo N, François Eyskens, Ozlem Goker‐Alpan, et al. A phase III, open‐label clinical trial evaluating pegunigalsidase alfa administered every 4 weeks in adults with Fabry disease previously treated with other enzyme replacement therapies. Journal of Inherited Metabolic Disease. 2024 Oct 9   UK-CHI-2400852 | October 2024