Rare lung diseases often affect the interstitium, a lace-like network of tissues that extends throughout both lungs. This network provides support to microscopic air sacs where oxygen is absorbed from inhaled air and carbon dioxide is removed and exhaled. Interstitial lung disease (ILD) causes a thickening of this interstitium, which can be caused by scarring (fibrosis), inflammation, or extra fluid. The most common symptom of ILD is shortness of breath, which worsens as the disease progresses; other symptoms include a persistent dry cough, weight loss, and fatigue. The symptoms of ILD interfere with daily life and can be very distressing for both patients and their families. Our focus at Vicore is to develop therapies to help patients who are diagnosed with these life-changing diseases.
There are several forms of ILD, including idiopathic pulmonary fibrosis, pulmonary sarcoidosis, and diffuse systemic sclerosis.
Idiopathic pulmonary fibrosis
The most common type of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF) which is a severe and devastating disease with no known cause. Debilitating symptoms, including breathing difficulties and persistent dry cough, typically appear between the ages of 50 and 70 years and, while the disease is more common in men, the number of cases in women is increasing. It has been estimated that as many as 111,000 people are currently living with IPF in the EU, with around 35,000 new cases diagnosed each year. In the USA, approximately 100,000 people are currently living with IPF, with up to 40,000 new diagnoses per year.
Unfortunately, a diagnosis of IPF is often delayed because the symptoms are similar to other lung diseases. Although IPF is a progressive disease, the rate of progression varies among patients. Sadly, the prognosis for patients diagnosed with IPF is poor, with most patients surviving for only 3-5 years after diagnosis.
Currently, there is no cure for IPF, and only two approved medications are available, pirfenidone and nintedanib. Both medications have been shown to slow the progression of the disease; however, side effects have limited their use. Supportive treatments include oxygen therapy using a portable oxygen tank and immunization against pneumonia and influenza to avoid infection. Patient management plans consist of keeping active, following a healthy diet, and taking plenty of rest. Clearly, new treatments are urgently needed to reduce the significant burden of this disease.