Professor Philip Eng provides insights into idiopathic pulmonary fibrosis (IPF), a rare and debilitating lung condition that is often misdiagnosed as other lung conditions or mistaken as merely being signs of ageing.
Idiopathic pulmonary fibrosis (IPF) is a rare disease with non-specific symptoms, which makes it difficult to diagnose. A fact: “idiopathic” means unknown. To increase the awareness of this rare disease, IPF World Week was held from 16 to 24 September 2017, a global awareness campaign that aims to highlight the importance and urgency of recognizing the early signs and symptoms of this fatal irreversible respiratory disease. Prof Eng tells us more about the incidence and potential risk factors associated with IPF as well as recent advancements in treating IPF.
What is a typical day for you as a senior consultant respiratory physician?
I have been in private practice at Mt Elizabeth Hospital for the past nine years after spending 24 years in the public sector at Singapore General Hospital. My typical day starts at 8am, when I go in to see my inpatients and ICU patients. This is followed by outpatient clinics, which takes up most of my day. I then check on my inpatients in the evenings at 5pm before I go home. On Wednesday afternoons, I go to NUS to teach and on Thursdays at lunchtime, I try to make time to attend teaching rounds at Singapore General Hospital.
Let’s talk about lung diseases. IPF World Week was held in September to raise awareness of idiopathic pulmonary fibrosis. Can you tell us more about IPF?
Idiopathic pulmonary fibrosis is a life-threatening condition where 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.
Although survival rates vary in patients, median survival after IPF diagnosis is two to three years in many studies all over the world. It primarily affects patients over the age of 50 and affects more men than women.
What is its prevalence in the Asian region?
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 about 20 patients per 100,000 population with data from US and Europe, I would estimate about 1,000 patients in Singapore.
Asia and the Pacific are currently home to half of the world’s elderly population. With the rapidly ageing population, two-thirds of the elderly population are expected to live in the Asia Pacific region. This puts more people in Asia at risk of having IPF, and furthers the urgency of earlier and accurate diagnosis of IPF.
While IPF is considered a rare disease, why should people pay attention to it?
Early and accurate diagnosis of IPF is important as it impacts a patient’s quality of life and survival. However, IPF is not easy to accurately diagnose because symptoms, such as shortness of breath or cough, are non-specific and often attributed to signs of ageing.
Compounding this fact is that 70 percent of patients with IPF are males and smokers or ex-smokers. About half of IPF patients are initially misdiagnosed as having chronic obstructive pulmonary diseases (COPD), asthma, or congestive heart failure - limiting their ability to seek out proper treatment.
What are the signs and symptoms that healthcare providers can look out for in diagnosing the disease?
Some 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, and sometimes weight loss if the disease is severe. More than 80 percent of IPF patients have a distinct crackling sound that a healthcare professional may be able to hear by examination with a stethoscope.
Can you share any recent advancements in the treatment of idiopathic pulmonary fibrosis?
There have been three major advances in the field of IPF in the past few years.
Firstly, IPF is now recognised as a very distinctive entity with defined clinical and radiological criteria. It is a subset of a broader group of 300 other diseases called interstitial lung disease (ILD).
Secondly, the standard treatment of IPF in the past, namely steroids, have been found to result in negative outcomes and hence is not used anymore.
Thirdly, two drugs, Pirfenidone and Nintedanib have been recently approved for the treatment of IPF as they have been found to be effective in slowing down the decline of lung function. From the INPULSIS Phase III trials with Nintedanib, which involved 1,066 patients across 24 countries, findings suggested that oral Nintedanib (150mg) taken twice daily, slowed disease progression in IPF by reducing the annual decline in forced vital capacity by 50% in patients with IPF over 52 weeks. This was compared to patients taking placebo. Thus this analysis is showing promising results and suggests that Nintedanib may have a long-term effect on slowing disease progression.
You recently attended the European Respiratory Society (ERS) Congress in Italy. Are there any interesting findings you can share from the ERS Congress?
At the IPF Symposium at the ERS in Milan, some of the presenters talked about present research trends. One was an ongoing clinical trial in the use of IPF drugs for another type of ILD due to autoimmune conditions like systemic lupus erythematosus. Another ongoing trial is the use of IPF drugs in a combined fashion. I think that we are at the dawn of a new era for IPF.
This interview was conducted by Lim Guan Yu.
- Nalysnyk L., et al. Incidence and Prevalence of Idiopathic Pulmonary Fibrosis: Review of the Literature. Eur Respir Rev. 2012;21(126):355-361.
About the Author
Professor Philip Eng
Senior Consultant, Respiratory & ICU Medicine
Mount Elizabeth Medical Centre
Professor Philip Eng completed his Masters degree in Medicine, from the National University of Singapore, specializing in Internal Medicine. He went on to pursue advanced training in pulmonary and critical care medicine at the Cleveland Clinic Foundation and the University of California in San Diego, USA. He specializes in the evaluation and treatment of lung cancer, pneumonia, asthma, chronic obstructive pulmonary disease and pleural effusion. Professor Eng also takes a special interest in intensive care management, bronchoscopy and chest radiology