Idiopathic pulmonary arterial hypertension is a rare but severe form of pulmonary hypertension. Researchers have now confirmed that smoking plays a causal role in the development of the disease.
Idiopathic pulmonary arterial hypertension (IPAH) is a rare but very severe form of pulmonary hypertension. In this regard, a study group headed by the Department of Pneumology at Hannover Medical School (MHH) has now taken a decisive step forward.
The team, led by Prof. Marius Hoeper, deputy director of the Department of Pulmonology, found that smoking likely plays a causal role in the development of a particularly severe form of IPAH. The study authors came to this conclusion by analyzing data from two pulmonary hypertension registers. The scientific work was published in the journal The Lancet Respiratory Medicine released.
For their study, the researchers used data from the COMPERA pulmonary hypertension register, one of the largest registers of its kind in the world, and the British pulmonary hypertension register ASPIRE.
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Researchers compared three different patient groups
In the study, the team compared three different patient groups. The first group consisted of patients with IPAH who had smoked heavily throughout their lives and in whom the so-called diffusion capacity, a decisive factor for oxygen uptake from the alveoli into the pulmonary vessels, was severely limited. The second group included those affected with a classic form of IPAH without a highly restricted diffusion capacity. The third group consisted of people who had developed pulmonary hypertension as part of a lung disease such as COPD, emphysema or pulmonary fibrosis.
Results help differentiate patients
“We found that the group of smokers with extremely limited diffusion capacity was similar to the group with pulmonary hypertension in the context of a lung disease in almost all important respects,” explains Hoeper. “This affected, among other things, the age and gender distribution, the poor response to drug therapies and life expectancy.” On the other hand, these patients differed significantly from the group of classic IPAH, which was predominantly younger, responded better to drug therapies and had a significantly had higher life expectancy. The researchers concluded that patients with a diagnosis of IPAH and a history of smoking and with severely restricted diffusion capacity should be differentiated from the group with classic IPAH.
More targeted therapies possible in the future
“The results of the study support the hypothesis that smoking can cause direct and serious damage to the pulmonary vessels,” explains Hoeper. The findings also show that this type of damage also occurs in patients who do not have typical smoker’s lungs. On the one hand, the data are relevant because they demonstrate a preventable cause of pulmonary hypertension. On the other hand, they are important because they help doctors to better understand and classify the disease. “The findings will have a major impact on the conduct of future studies and they will help us to be able to provide those affected with better advice and more targeted therapy in the future,” says Hoeper.
This text is based on a press release at the Hannover Medical School. You can find the original publication here and linked in the text.
Image source: Daniel Monteiro, unsplash