27 September 2018 - NICE has published draft guidelines rejecting NHS use of CSL Behring’s Respreeza for treating emphysema in adults with severe alpha-1-proteinase inhibitor deficiency.

A1PI deficiency is an incurable, rare and life-limiting genetic disorder in which a lack of the protein A1PI makes the body - especially the lungs - vulnerable to attack from its own infection-fighting enzymes.

Therefore, people with the condition are at high risk of the chronic lung condition emphysema as a result of infections and environmental toxins, including tobacco smoke and pollution.

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