Drugs are available for the treatment of IPF
Idiopathic pulmonary fibrosis (IPF) is a serious lung disease that primarily affects patients over the age of 50 and affects more men than women. While IPF is considered a rare disease – it affects approximately three million people worldwide – an ageing Asian population means that more and more men in Asia will be at risk of having IPF. Furthermore, with IPF being difficult to diagnose, those who have the condition could simply attribute it to signs of ‘growing old’. In conjunction with IPF World Week, Prof Philip Eng, Senior Consultant, Respiratory & ICU Medicine at Mount Elizabeth Medical Centre, Singapore recently spoke to Priyanka Bajpai of BioSpectrum Asia about the deadly disease and latest advancements in its treatments. Prof Eng has published more than 120 peer-reviewed articles and books. He also sits on several editorial boards such as the Singapore Allergy & Asthma Newsletter, MIMS Respiratory Guide and Journal of Bronchology.
Can you briefly explain what IPF is, and the symptoms and signs to look out for? How common is this condition?
Idiopathic pulmonary fibrosis (IPF) is a serious and life-threatening condition in which the lungs become thickened, stiff, and scarred over a period of time. The lungs then progressively lose their ability to take in and transfer oxygen into the bloodstream, decreasing the amount of oxygen, the lungs can supply to major organs of the body.
IPF affects approximately three million patients worldwide. There are no accurate statistics of prevalence of this disease in Singapore or most of Asia. Based on the estimated prevalence of 20 patients per 100,000 population from data from US and Europe, I would estimate there would be about 1,000 patients in Singapore. Symptoms of IPF include breathlessness, dry and persistent coughing which goes on for months. Clinical examination shows clubbing (widening and rounding) of the tips of fingers and toes. Sometimes weight loss occurs if the disease is severe. More than 80 per cent of IPF patients have a distinct crackling sound that a HCP may be able to hear by examination with a stethoscope.
What causes IPF?
IPF is defined as an idiopathic disease (i.e. of unknown cause). Before one makes the diagnosis of IPF, it is important to exclude certain other causes, for example environmental toxins, certain drugs and other connective tissue diseases like SLE.
Who is more likely to be affected by IPF, and are there preventive measures that one can take?
IPF generally affects males over the age of 65. More than 70 per cent of patients with IPF are males and smokers or ex-smokers. A person suffering from gastroesophageal reflux disease (GERD) is at a higher risk of having IPF. Unfortunately, there is nothing one can do to prevent the disease. The important message is for one who has persistent symptoms like shortness of breath or cough to go for medical evaluation, rather than attribute it to the normal ageing process.
What is the treatment for IPF? Are there any recent new developments?
Generally, patients get progressively more short of breath with time and will need long-term oxygen in the long run. Once they are end stage, lung transplantation should be considered if the patient is fit. Two new drugs have been recently approved for the treatment of IPF – Pirfenidone and Nintenanib. Studies have shown them to be effective in slowing down the decline of lung function.
What is the general prognosis for IPF? Has it improved because of the advancements above?
Although survival rates vary by patient, median survival after IPF diagnosis is two to three years in many studies all over the world. It is too early to say if the new drugs will make a major impact in overall survival of these patients, although it is very promising.
Prof Philip Eng Senior Consultant, Respiratory & ICU Medicine at Mount Elizabeth Medical Centre, Singapore