Researchers Identify Predictive Factors for Pulmonary Hypertension in Scleroderma Patients

Patients with a form of scleroderma called systemic sclerosis are particularly susceptible to developing high blood pressure in their heart and lungs, also known as pulmonary hypertension. A new study published in the European Respiratory Journal and reported on by Scleroderma News, found that measuring a patient’s heart blood pressure, and capacity for gas exchange, can help to predict the development of pulmonary hypertension.

Scleroderma is a rare condition that causes skin to become hard and thick. It is caused when the body’s immune system doesn’t recognise connective tissue and begins to attack it, causing internal scarring. Systemic sclerosis is a particularly severe form of scleroderma, in which the blood vessels and internal organs are also affected. It usually first occurs in women aged between 30 and 50. When internal organs are affected patients can suffer serious side effects, one of which is pulmonary hypertension (PH). This occurs when there is high blood pressure in blood vessels in the lungs and can lead to heart damage, and even heart failure, if left untreated.

Pulmonary hypertension is the primary cause of death in people with systemic sclerosis, affecting around 12% of people. Only half of systemic sclerosis patients with pulmonary hypertension survive three years or more after diagnosis. This is partly due to the nature of the disease, which often does not show symptoms until it has already reached a late stage. There is therefore a strong need for better screening systems to detect pulmonary hypertension early on. Current methods for detecting the disease, such as the six-minute walking test and MRI, each have disadvantages and will not suit all patients. This recent study, carried out by an international team of researchers, suggests new methods for early diagnosis of pulmonary hypertension.

The researchers found that pulmonary hypertension could be predicted by two factors; patient’s levels of gas exchange, and heart blood pressure. They carried out a three-year study of 96 systemic sclerosis patients, testing their heart blood pressure at the beginning, and, for 71 of the patients, also at the end of the study. They found that pulmonary hypertension had a higher occurrence rate in patients whose heart blood pressure was already raised; suggesting that the increase occurs gradually over time and patients can be identified as at risk before their heart blood pressure reaches levels considered to be pulmonary hypertension.

Furthermore, it was also found that one-quarter of patients who had low levels of gas exchange developed pulmonary hypertension within three years. This suggests that patient’s gas exchange levels could be monitored, and those judged to be at risk of pulmonary hypertension could recieve more thorough check-ups.

The results of this study suggest it may be possible to profile systemic sclerosis patients likely to develop pulmonary hypertension early on, thereby increasing the probability that the disease will be detected and treated at an early stage. This could have a significant effect on the health of patients with systemic sclerosis.


Anna Hewitt

Anna Hewitt

Anna is from Cambridge, England and recently finished her undergraduate degree, where she specialised in Biological Anthropology. She has an interest in medicine and enjoys writing. In her spare time she likes to cook, hike, and hang out with cats.

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