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.
Nintedanib is already licensed for treating idiopathic pulmonary fibrosis which is a subtype of ILDs. Nintedanib acts by blocking specific enzymes and pathways that lead to the development of blood vessels within lung cells involved in the scarring process. This inhibits further growth of the scarring tissues resulting in slowing of the disease progression. This action has been shown to be similar in patients with idiopathic pulmonary fibrosis. If licensed, nintedanib may provide the first novel therapy for PF-ILD where limited treatment options currently exist.