Focus on Idiopathic Pulmonary Fibrosis: Interview with Dr Low Su Ying at the ERS Congress 2017

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While attending the European Respiratory Congress at Milan in September 2017, I, Laxmi Iyer from www.biotechin.asia, interviewed Dr. Low Su Ying, Senior Consultant, Department of Respiratory & Critical Care Medicine of Singapore General Hospital about IPF. Dr Low is one of the few experts on idiopathic pulmonary fibrosis (IPF) in Singapore, and during our talk, she shared local insights on IPF, including factors such as diagnosis/misdiagnosis and treatment.

The diagram illustrates the lung scarring with the progression of IPF. Photo Credit: PocketDoktor

Dr Low, can you tell us more about your specialty area of care?

I am a Respiratory Physician working in Singapore General Hospital (SGH), and my work involves general respiratory and also critical care medicine. My specific interest is in diffuse parenchymal lung diseases, which include idiopathic pulmonary fibrosis (IPF). 

What is idiopathic pulmonary fibrosis or IPF?

Idiopathic is a medical term for “Doctors don’t know at this point in time”. Essentially the medical community currently does not know the exact cause of the pulmonary fibrosis, hence the word “idiopathic”. Perhaps in 5-10 years’ time, we will know more about the disease, its etiology and pathogenesis, and we may then be able to elucidate the cause.

Overall it is a devastating disease with a median survival of 2.5 to 3 years from the time of diagnosis. It tends to present insidiously, starting with a mild cough and some breathlessness. As the symptoms progress and worsen, patients then seek medical attention, by which time the disease is fairly advanced when the diagnosis of IPF is made. With increasing public awareness of this condition, patients may seek advice earlier, and the diagnosis could then be made earlier.

What is the prevalence rate in Singapore?

There is no data on the incidence and prevalence of IPF in Singapore. If we extrapolate from international data, the incidence of IPF is estimated at 5 to 15 per 100,000 and prevalence of 15 to 20 per 100,000.

What are some of the symptoms presented by patients with IPF?

Patients with IPF present with a cough, which does not resolve associated with shortness of breath. Patients are generally of an older age group, more common in males, and there is an association with smoking. As the disease progresses, patients may lose weight, and the symptoms of cough and breathlessness will worsen and become more debilitating.

Is the diagnosis simple?

The diagnosis of IPF can only be made after excluding other known causes of pulmonary fibrosis (which are many). After taking a detailed clinical history and performing a thorough physical examination, initial investigations would include chest radiograph and pulmonary function testing. Further evaluation would entail specific blood tests and CT scan of the lungs. Some patients may require more invasive investigations such as bronchoscopy and lung biopsies.

What is the general awareness of the disease from the physician’s as well as the patient’s side?

From the physician point of view, there is increasing awareness. From the patient’s perspective, as this is a rare disease, they might not have heard much about it as compared to other more commonly occurring lung conditions such as asthma, chronic obstructive pulmonary disease (COPD), pneumonia, and tuberculosis (TB). But in the era of the Internet, the public is well adept in performing online searches once they have been given the diagnosis of IPF.

The current treatment for IPF as per the information available online is Nintedanib or Pirfenidone?

I would like to take a step back and look at the overall treatment of IPF. The first most important thing is to ensure that a correct diagnosis has been made. The managing physician needs to exclude known causes of pulmonary fibrosis such as those related to connective tissue disease, drug treatment, environmental or occupational exposure, for which the treatment is very different. Once the diagnosis of IPF has been confidently made, then we need to consider both pharmacological and non-pharmacological treatment options.

Non-pharmacological treatment – as the name suggests it involves no drugs.

  1. Need to evaluate whether the patient is hypoxic (i.e. does not have enough oxygen in the blood), and if so need to consider renting or buying supplemental oxygen therapy to help alleviate their symptoms of breathlessness.
  2. Referral for pulmonary rehabilitation, which comprises a comprehensive physiotherapy exercise program that includes strengthening exercises, breathing techniques, nutritional advice and other strategies that would help to improve exercise tolerance and quality of life.
  3. Smoking cessation as smoking is a risk factor and has a strong association with IPF.
  4. Vaccinations (influenza and pneumococcal) to reduce the risk of contracting these respiratory infections.

Pharmacological treatment – currently comprises anti-fibrotic drugs (as pulmonary fibrosis is driven by a fibrotic process). There are currently two different anti-fibrotic drugs available that have been approved by the United States Food and Drug Administration (FDA):

  1. Nintedanib
  2. Pirfenidone

Studies have shown both the drugs have equal clinical efficacy with regards to slowing the rate of disease progression by about 50% per year. These drugs do not reverse the established fibrosis nor halt the disease progression. They just slow down the rate of decline.

As such it is essential, prior to starting therapy with these drugs, that managing physicians emphasize to patients that it is not going to make them feel better as it has no impact on their symptoms, or stop the disease from further progression, but will hopefully slow the rate of their lung function decline over time, which translates to their degree of breathlessness.

Lung transplantation should also be considered early in this disease, but will depend on the availability of organs/donors as well as the suitability of the patient.

One doctor almost compared it with lung cancer, stating that IPF is terminal. What are your thoughts?

IPF is a progressive disease but there are different clinical phenotypes. Some patients have a very aggressive form of pulmonary fibrosis and pass on quickly within months, whilst about 20% of patients with IPF have a 10-year survival. So yes, the disease is unfortunately terminal but the only question is how fast.

What are some of the highlights from the ERS Congress 2017 with respect to IPF?

The major breakthrough with anti-fibrotic drugs for the treatment of IPF occurred in 2014 when both nintedanib and pirfenidone were approved for use by US FDA. We now also have the long-term tolerability and safety data for both these drugs. With more drugs undergoing Phase 2 trials, the prospective of newer pharmacological treatment options for IPF remains exciting.

Any other message related to awareness of IPF?

If you have respiratory symptoms that won’t go away, please do visit a physician for evaluation. In case it does not get better, your primary physician may consider referring you early to a respiratory specialist for a more thorough and comprehensive work-up to reach a correct diagnosis expediently.

For further resources on IPF, please refer to: www.lifewithipf.com