'Complement IPF guidelines with clinical expertise'

When you go to a medical specialist, you expect to hear definite answers on your most pressing questions. Unfortunately, even doctors are only human, and scientific progress is sometimes slower than we would like. Although a lot of progress has been made in idiopathic pulmonary fibrosis (IPF) in the last 15 years, some things still remain unknown. Now, researchers provide guidance for these difficult questions that are asked by patients in daily practice. 

What is IPF?’ Difficult to answer because in many cases a definitive diagnosis of IPF cannot be made.

Argyris Tzouvelekis and colleagues have listed 4 of the most frequently asked but difficult to answer questions they came across in daily practice. The number one frequently asked question in daily practice is: ‘What is IPF?’ Difficult to answer because in many cases a definitive diagnosis of IPF cannot be made. For instance, the guidelines recommend a lung biopsy to establish a definitive diagnosis in ‘possible IPF’ cases, but this is often not desirable because of the risk of complications. The second frequently asked question has a strong emotional component: ‘Why me?’ Also difficult to answer, as it is still unclear what exactly causes IPF. Although some associations with risk factors have been found, this does not prove these risk factors actually cause the disease.

Another challenging question concerns therapy: ‘What treatment do you recommend for me?’ The guideline recommendations are mostly based on trials focussing on patients with mild to moderate IPF. This, however, does not reflect the broader IPF population treated in everyday clinical practice. And what to think of the situation when according to recommendations you need a lung transplant but lung transplantation centers are absent, as is the case in Greece? 

The last question addressed by the researchers is: ‘How long am I going to live?’ This may be the most critical question when you are diagnosed with a disease in which the prognosis is as poor as three to five years. But different IPF groups with different rates of progression can be distinguished. It is, however, not possible to predict whether? your IPF will progress rapidly or not.

Tzouvelekis and colleagues conclude that an important limitation of the current guidelines is that the recommendations do not seem to apply to specific patient subpopulations. They advocate complementing the guidelines with clinical expertise from centers of excellence across the world to provide practical guidance and realistic answers to patients’ questions.

Reference:
Tzouvelekis A, Tzilas V, et al. Diagnostic and prognostic challenges in Idiopathic Pulmonary Fibrosis: A patient's "Q and A" approach. Pulm Pharmacol Ther 2017;42:21-4.