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10 March 2015 EMA/COMP/42154/2005 Rev.2 Committee for Orphan Medicinal Products Ciclosporin (inhalation use) for treatment of graft rejection after lung transplantation First publication 1 July 2005 Rev.1: transfer of sponsorship 30 November 2007 Rev.2: sponsor s change of address 10 March 2015 Disclaimer Please note that revisions to the Public Summary of Opinion are purely administrative updates. Therefore, the scientific content of the document reflects the outcome of the Committee for Orphan Medicinal Products (COMP) at the time of designation and is not updated after first publication. On 10 March 2005, orphan designation (EU/3/05/265) was granted by the European Commission to Chiron Corporation Ltd, United Kingdom, for ciclosporin for inhalation use for the treatment of graft rejection after lung transplantation The sponsorship was transferred to Right Track Regulatory Limited, United Kingdom, in September 2007. What is graft rejection in lung transplantation? Lung transplantation is performed when, because of underlying conditions, the lungs are so damaged that one or both need to be replaced. It is most commonly performed in pulmonary emphysema, idiopathic pulmonary fibrosis, pulmonary hypertension and cystic fibrosis. Rejection is the process by which the organ recipient's defence (immune) system recognises, becomes sensitised against, and attempts to eliminate the foreign donor organ. It has been observed that at least one episode of rejection is common within the first year after a transplant. More than 80% of people who receive a lung transplant develop some symptoms of rejection within a month of transplantation. Symptoms include fever, shortness of breath, and weakness due to the non-optimal functioning of the transplanted lungs. Scar tissue may form in the small airways and gradually block them, possibly indicating gradual rejection. 30 Churchill Place Canary Wharf London E14 5EU United Kingdom Telephone +44 (0)20 3660 6000 Facsimile +44 (0)20 3660 5555 Send a question via our website www.ema.europa.eu/contact An agency of the European Union European Medicines Agency, 2015. Reproduction is authorised provided the source is acknowledged.

The condition can be chronically debilitating and life threatening due to the high incidence of severe infections and graft rejection. What is the estimated number of patients affected by the condition? At the time of designation, graft rejection after lung transplantation affected approximately 0.05 in 10,000 people in the European Union (EU). This was equivalent to a total of around 2,300 people *, and is below the ceiling for orphan designation, which is 5 people in 10,000. This is based on the information provided by the sponsor and the knowledge of the Committee for Orphan Medicinal Products (COMP). What treatments are available? In order to preserve the new lung(s) in the recipient s body, a suppression of the patient s defence potential (immunosuppression) is induced after lung transplantation. The immunosuppressive agents already authorized for the use in the treatment of graft rejection are ciclosporin (intravenous administration) and corticosteroids. Other therapies that can be used are other immunosuppressive drugs such as methotrexate, or other methods that decrease the number of immune cells of the recipient, such as photopheresis and antilymphocyte antibodies. Inhaled ciclosporin might be of potential significant benefit for the treatment of graft rejection after lung transplantation due to its new route of administration. The assumption will have to be confirmed at the time of marketing authorisation. This will be necessary to maintain the orphan status. How is this medicine expected to work? Ciclosporin for inhalation use is an immunosuppressive drug used to prevent the body from rejecting transplanted organs, as it suppresses the body s natural reaction to the transplanted organ and is a component of most of the post transplant regimens. Ciclosporin acts by inhibiting some special defence cells called lymphocyte T cells. It inhibits also the production of some special substances called lymphokines, which play a role in the defence reactions of the body. Ciclosporin for inhalation is meant to be used to treat patients who develop a rejection against their lung transplants by delivering the medicinal product directly to the lungs. What is the stage of development of this medicine? The effects of ciclosporin for inhalation use was evaluated in experimental models. At the time of submission of the application for orphan designation, clinical trials in patients with graft rejection in lung transplantation were completed. Ciclosporin for inhalation use was not marketed anywhere worldwide for graft rejection in lung transplantation at the time of submission. Orphan designation of ciclosporin for inhalation use was granted in the United States for prophylaxis of acute rejection in patients requiring allogenic lung transplant and for treatment of refractory acute graft rejection in patients requiring allogenic lung transplant * Disclaimer: For the purpose of the designation, the number of patients affected by the condition is estimated and assessed on the basis of data from the European Union (EU 25), Norway, Iceland and Liechtenstein. At the time of designation, this represented a population of 466,600,000 (Eurostat 2005). EMA/COMP/42154/2005 Page 2/5

In accordance with Regulation (EC) No 141/2000 of 16 December 1999, the COMP adopted a positive opinion on 3 February 2005 recommending the granting of this designation. Opinions on orphan medicinal product designations are based on the following three criteria: the seriousness of the condition; the existence of alternative methods of diagnosis, prevention or treatment; either the rarity of the condition (affecting not more than 5 in 10,000 people in the EU) or insufficient returns on investment. Designated orphan medicinal products are products that are still under investigation and are considered for orphan designation on the basis of potential activity. An orphan designation is not a marketing authorisation. As a consequence, demonstration of quality, safety and efficacy is necessary before a product can be granted a marketing authorisation. For more information Sponsor s contact details: Right Track Regulatory Limited 2nd Floor Titan Court 3 Bishop Square Hatfield AL10 9NA United Kingdom Tel. +44 (0)1707 226 025 E-mail: info@rtregulatory.co.uk For contact details of patients organisations whose activities are targeted at rare diseases see: Orphanet, a database containing information on rare diseases, which includes a directory of patients organisations registered in Europe; European Organisation for Rare Diseases (EURORDIS), a non-governmental alliance of patient organisations and individuals active in the field of rare diseases. EMA/COMP/42154/2005 Page 3/5

Translations of the active ingredient and indication in all official EU languages 1, Norwegian and Icelandic Language Active ingredient Indication English Ciclosporin (inhalation use) Treatment of graft rejection after lung transplantation Bulgarian Циклоспорин (за инхалаторно Лечение на отхвърляне на присадката след приложение) белодробна трансплантация. Czech Ciclosporin (k inhalaci) Léčba rejekce transplantátu plic Danish Ciclosporin (til inhalation) Behandling af afstødning af transplantat efter lungetransplantation Dutch Ciclosporine (inhalatie) Behandeling van afstotingsreacties na longtransplantatie Estonian Tsüklosporiin (inhalatsiooniks) Kopsu transplantatsiooni järgse äratõukereaktsiooni ravi Finnish Siklosporiini (inhalaatioon) Keuhkosiirrännäisten hylkimisreaktioiden hoito French Ciclosporine (voie inhalée) Traitement du rejet du greffon après transplantation pulmonaire German Ciclosporin (zur Inhalation) Behandlung von Abstoßungsreaktion nach Lungentransplantation Greek Κυκλοσπορίνη (Xρήση δια ειδπνοής) Θεραπεία κατά της απόρριψης μοσχεύματος μετά από μεταμόσχευση πνεύμονα Hungarian Cyclosporin (inhalációs alkalmazásra) A kilökődési reakció kezelése tüdőtranszplantáció esetén Italian Ciclosporina (uso inalatorio) Trattamento del rigetto a seguito di trapianto del polmone Latvian Ciklosporīns (inhalācijām) Transplantāta atgrūšanas ārstēšana pēc plaušu transplantācijas Lithuanian Ciklosporinas (inhaliacijoms) Transplantanto atmetimo reakcijos gydymas po plaučių transplantacijos Maltese Ciclosporin (għal biex jinġibed Kura ta riġettar ta l-organu wara trapjant talpulmun man-nifs) Polish Cyklosporyna Płyn (do inhalacji parowej) Leczenie odrzucenia przeszczepu po transplantacji płuca Portuguese Cyklosporyna (inhalacja) Tratamento da rejeição do enxerto após transplante do pulmão Romanian Ciclosporină (administrare Tratamentul rejetului de grefă după transplantul inhalatorie) pulmonar Slovak Ciclosporina (via inalatoria) Liečba odmietnutia štepu po transplantácii pľúc. Slovenian Cyklosporín (inhalácia) Zdravljenje zavrnitve presadka pri trasplantaciji pljuč Spanish Ciklosporin (za inhalacijo) Tratamiento del rechazo del injerto tras el transplante de pulmón Swedish Ciclosporina (vía inhalatoria) Behandling av avstötning av lungtransplantat 1 At the time of designation EMA/COMP/42154/2005 Page 4/5

Language Active ingredient Indication Norwegian Ciklosporin (til inhalasjon) Behandling av rejeksjon av lungetransplantat Icelandic Cíklósporín (Innöndunargufa, lausn) Meðferð gegn líffærahöfnun eftir lungnaígræðslu EMA/COMP/42154/2005 Page 5/5