Systemic Sclerosis (Scleroderma or SSc) is an uncommon condition that results in hard, thickened areas of skin and sometimes problems with the internal organs and blood vessels. This condition is caused by the immune system attacking the areas under the skin and around internal organs and blood vessels called connective tissue. This causes scarring and thickening in these areas. SSc affects the lungs of about half of the people with the condition. Inflammation and scarring of the lung tissue is called interstitial lung disease (ILD). The most common presenting symptom of ILD is difficult breathing on exertion. Other indicators of ILD may include non-productive cough, fatigue and chest pain. Many patients with SSc become less physically active because of musculoskeletal complaints or the fatiguing nature of the illness. Lung involvement in all its forms in patients with SSc has emerged to be the leading cause of death and disability.
Nintedanib (OFEV®) is a medicine that is being developed for the treatment of Systemic Sclerosis associated interstitial lung disease (SSc-ILD). It acts by targeting the specific mechanisms by which scarring of the lungs occur, reducing progression of the disease. It is given by mouth as capsules. Nintedanib (as OFEV) is already available in the EU for the treatment of idiopathic pulmonary fibrosis (IPF). Because SSc-ILD and IPF share similarities in how the underlying lung scarring, or fibrosis, forms in people with the disease, the development of nintedanib for the treatment of SSc-ILD may address unmet needs. If licensed, nintedanib may offer an additional treatment option (the first licensed) for patients with SSc-ILD.
Benralizumab is a new drug being developed for the treatment of chronic obstructive pulmonary
disease. It is administered by injection under the skin and acts by targeting specific proteins that
causes the airway to narrow. If benralizumab is licensed for use in the UK, it could be a new treatment
option for patients with chronic obstructive pulmonary disease that may improve quality of life and
reduce the number of exacerbations.