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Published on 8 December 2014

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SMC accepts Adempas® (riociguat) to treat rare respiratory disease in Scotland

Bayer HealthCare welcomes the announcement from the Scottish Medicines Consortium (SMC) that the first specifically licenced treatment for chronic thromboembolic pulmonary hypertension (CTEPH) – Adempas® (riociguat) – will be made available for restricted use in Scotland. (1)

Bayer HealthCare welcomes the announcement from the Scottish Medicines Consortium (SMC) that the first specifically licenced treatment for chronic thromboembolic pulmonary hypertension (CTEPH) – Adempas® (riociguat) – will be made available for restricted use in Scotland. (1)

Adempas is accepted by the SMC for the treatment of adult patients with World Health Organisation (WHO) functional class II to III* with “…inoperable CTEPH, persistent or recurrent CTEPH after surgical treatment and to improve exercise capacity for patients in whom a PDE5 inhibitor is inappropriate, not tolerated, or ineffective. It is restricted to prescribing by specialists in the Scottish Pulmonary Vascular Unit.” (1)

CTEPH causes severe narrowing of blood vessels in the lungs leading to high blood pressure in the pulmonary arteries (leading from the heart to the lungs), giving rise to ‘pulmonary hypertension’ (PH). The heart has to work very hard to push blood through the narrowed blood vessels to be infused with oxygen in the lungs. The resulting symptoms can include breathlessness and fatigue upon minimal physical activity, chest pain and dizziness. Untreated, the pulmonary hypertension associated with CTEPH progressively worsens and eventually leads to heart failure and premature death. (2,3)

Adempas is the first licenced treatment to stimulate soluble guanylate cyclase (sGC), an enzyme that catalyses the synthesis of a signalling molecule called cyclic guanosine monophosphate (cGMP).

Within cells cGMP plays an important role in regulating processes that influence vascular tone, proliferation of cells, fibrosis and inflammation. (4)

The SMC Advice states that, following a full submission considered under the end of life/orphan medicine process: “Riociguat demonstrated significant improvement compared with placebo in exercise capacity, in terms of 6-minute walk distance, in patients with inoperable CTEPH or persistent or recurrent pulmonary hypertension after pulmonary endarterectomy.” (1)

Pulmonary endarterectomy is the standard and potentially curative treatment for CTEPH. It is a highly specialised surgical procedure in which the blood vessels of the lungs are cleared of diseased scar tissue that develops from accumulated blood clots. (2) However, CTEPH is inoperable in a substantial proportion of patients5 and up to 10–15% of patients who undergo pulmonary endarterectomy have persistent or recurrent PH following surgery. (6) Riociguat can now be considered in these two groups of patients. (1)

Dr Martin Johnson, Consultant Physician at Scotland’s only specialist pulmonary vascular unit located at the Golden Jubilee National Hospital in Glasgow, commented: “We are very excited to have access to a proven medical treatment for patients with CTEPH. The data from the clinical studies are compelling and our own experience of this medication through the pre-licensing trials has been very positive. We are especially pleased that Scotland is the first area within the UK to have access to this medication.” (4,7)

Ongoing commitment towards research and making new treatments available for rare and complex diseases like CTEPH is so important to those who suffer from them. So we are very positive about the SMC’s decision to accept riociguat for restricted use in Scotland,” said Dr Iain Armstrong, Chairman of the Pulmonary Hypertension Association UK.

*The WHO classification reflects the severity of the disease on physical activity; Class I (no limitation in physical activity) Class II (some limitation), Class III (severe limitation) and Class IV (symptoms at rest and limited physical activity). (7)

References:

  1. Scottish Medicines Consortium. Riociguat (Adempas) Advice. Available at: www.scottishmedicines.org.uk Last accessed: December 2014.
  2. McNeil K and Dunning J. Chronic thromoembolic pulmonary hypertension (CTEPH), Heart 2007;93(9):1152–1158.
  3. Rosenkranz S. Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol 2007;96(8):527-541.
  4.  Adempas® Summary of Product Characteristics. Available at https://www.medicines.org.uk/emc/medicine/28739 Last accessed: November 2014.
  5. Condliff R et al. Improved outcomes in medically and surgically treated chronic thromoembolic pulmonary hypertension. Am J Respir Crit Care Med. 2008;177(10):1122-7.
  6. Rahnavardi M et al. Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension: a systematic review. Ann Thorac Cardiovasc Surg. 2011;17(5):435–445.
  7. Galie N et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30(20):2493-2537.European Medicines Agency.


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