Pulmonary hypertension (PH) is a rare and incurable disease of the heart and lungs1 that affects people of all ages 2, often developing in early adulthood and predominantly affecting females 3.
In Pulmonary Arterial Hypertenstion (PAH), a specific form of PH, the walls of the arteries become thick and stiff, narrowing the space for blood to pass through, increasing blood pressure and leaving patients struggling to breathe and carry out daily activities. As a consequence, the right side of the heart has to work harder to oxygenate the blood via the lungs. This increased strain can gradually cause the heart to weaken and, if left untreated can ultimately lead to heart failure.
As PAH is a rare disease and its symptoms are somewhat nonspecific, it can be misdiagnosed or diagnosed when the disease is already quite advanced4. In fact, on average patients can wait two years between their onset of symptoms and a diagnosis 5.
Despite significant progress being made in the last decade, one in three patients still die within five years of diagnosis 6. It is because of this that our vision remains to transform PAH into a long-term, manageable condition “giving people with cardiopulmonary disease a normal life” and that one day, we will find a cure.
Our Work in PH
The integration of Actelion into the Janssen Pharmaceutical Companies of Johnson & Johnson in 2017, saw PH established as our sixth disease area strong hold. We aim to build upon the strong heritage of Actelion, where in the past two decades, the prognosis and quality of life for people with PH has significantly improved.
Together, we have a clear strategy to transform lives through closing the diagnosis gap and building an innovative pipeline. Janssen is committed to improving PH patient outcomes for the long haul. This means holistically addressing unmet needs across the patient journey, including faster diagnosis, better monitoring and new treatment approaches, including novel therapeutic devices, digital health solutions and non-invasive diagnostics to aid faster referral to specialist care.
Our Disease Areas of Focus
PAH is a subtype of pulmonary hypertension (PH) and can occur without any identifiable cause, known as Idiopathic PAH. Affecting over 3,000 people in the UK5, PAH causes patients to struggle to breathe and other symptoms include dizziness, fatigue, fainting and chest pain.1
Heritable PAH is a form of PAH that can be inherited, but is relatively uncommon.
In Associated Pulmonary Arterial Hypertension, the PAH is associated with other conditions such as connective tissue diseases (which includes scleroderma and lupus), congenital heart disease and HIV infection.
1. Kiely DG, Elliot CA, Sabroe I, Condliffe R. Pulmonary hypertension: diagnosis and management. Br Med J 2013;346:f2028 doi: 10.1136/bmj.f2028.
2. NHS, Pulmonary Hypertension Overview
3. Pulmonary Hypertension Association, Risk Factors
4. Strange G, et al. 2013. Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: The delay study. Pulmonary Circulation Volume 3:8, p89-94
5. Prins KW, et al. CardiolClin 2016; 34(3):363-743
6. Hoeper MM, et al. Eur Respir J 2017; 50:1700740