Emerging horizons in pulmonary hypertension,
Scleroderma Society AGM/Conference in Oct 02

Dr. Dev Mukerjee, MRCP,

Clinical Research Fellow, Royal Free Hospital, London.

Introduction

One of the commonly asked questions is "What is pulmonary hypertension?" Well, it sounds like a lung disease, but it’s a heart disease. This is because this condition involves the arteries that take blood from the right side of the heart to the lungs. Narrowing of these small arteries, due to an as yet unknown cause, leads to build up of back pressure onto the right side of the heart. Consequently, the heart has to work harder than usual, causing breathlessness on exertion which is the commonest symptom. So, this condition is different to "hypertension" which is due to high blood pressure in the circulation and is treated in a completely different way.

Why is pulmonary hypertension important?

It is important because it can lead to increased breathlessness and fatigue and so we need to diagnose and treat this condition early to prevent the right side of the heart from getting damaged progressively. There are several possible causes of breathlessness in scleroderma. It is important to identify the route of the problem, as the treatments for this condition are very precise and also very expensive.

What is my risk of having pulmonary hypertension?

It is fortunately rare. 1 in 10 people with scleroderma may be affected, but often this is mild and does not progress. For some reason people with the Limited form of Scleroderma have a higher chance of developing it. Also, it can come on several years after a person gets scleroderma. This is why screening for this condition on a regular yearly basis is so important.

How can we treat this condition?

New treatments are being developed that benefit people with high pressures. Initially, it involves thinning the blood out with Warfarin. After that specific drugs can be used to reduce the narrowing of the pulmonary arteries. Obviously, different drugs suit different people and we have to choose the most suitable drug in each individual. The treatment depends on test results of the ECG, echo, lung function tests and a cardiac catheter test which directly measures the pressure in the arteries. The last test is important as it is the most accurate and helps the doctor in deciding on the precise treatment. At present, there is no specific blood test that tells us whether an individual is at higher risk of getting pulmonary hypertension. This is why we need to do these heart and lung tests and carefully investigate all scleroderma patients who develop breathlessness.

Newer treatments

Over the last year there have been exciting new treatments that have been developed to treat this condition. We have a choice of oral treatment (as well as Warfarin). This includes a drug called Bosentan and an oral form of the drug Iloprost. They all help open up the pulmonary arteries and have been shown to be beneficial in reducing breathlessness. All these new drugs have been trialled at the Royal Free Hospital. There is ongoing research to develop other treatments at present. Another drug which is undergoing trials is Viagra which acts by improving blood flow to the lungs (its action on other parts of the body! is well known). Also, Iloprost can be given by different routes, using a pump with a needle placed under the skin where the drug is constantly delivered over 24 hours. Another is giving Iloprost via a nebuliser (like an inhaler) which can be taken several times a day to keep the pressures down and help the breathing. Clearly the field of treatments is currently changing rapidly as newer drugs are being developed to combat this condition.

Note: - The PHA UK (Pulmonary Hypertension Association) is a very helpful and informative support group, membership is free, for more information contact PHA (UK) PO Box 2760, Lewes, Sussex, BN8 4WA, Tel: 0800 389 8156 or email: pha.uk@hotmail.com Web Site: www.pha-uk.com .