IPF: the importance of early identification and a multidisciplinary approach

Ideally, patients with interstitial lung disease (IPF) should be diagnosed and treated as soon as possible. But this is easier said than done when many other lung diseases have similar symptoms as IPF. In their consensus document on the diagnosis and treatment of IPF, Robalo Cordeiro and colleagues provide guidance for this challenge. They stress the importance of raising more awareness of IPF and a timely referral of patients. They highly recommend that experienced pulmonologists, radiologists and pathologists work together in a multidisciplinary team to correctly diagnose and treat the disease.

IPF is a life threatening disease

IPF is a life threatening disease, so early identification and referral of IPF patients is of the utmost importance. Wouldn’t you feel more relaxed if you knew your general practitioner (GP) recognizes IPF-symptoms and refers you to a team of specialists? The Portuguese experts agree: in the consensus document they stress that raising awareness of IPF symptoms is very important among GP’s. Also, patients experiencing IPF-symptoms should be referred to an experienced team of pulmonologists, radiologists and pathologists for the evaluation and correct treatment of the disease.

To make the diagnosis, other known causes of interstitial lung disease must be excluded. One of the procedures that should be performed at diagnosis is a special imaging technique called high resolution computed tomography (HRCT). This technique is used to identify typical patterns of changes of usual interstitial pneumonia (UIP, a relatively common form of interstitial lung disease). The presence of these UIP patterns support the diagnosis of IPF. When there is doubt about the presence of this pattern, a lung biopsy can be taken. But this is not all: next to the standardized HRTC information and histopathology, the consensus document states the multidisciplinary approach demands the use of also all non-standardized data available in individual patients such as bronchoalveolar lavage, serology tests and biomarkers.

Concerning the treatment of IPF, the experts discuss the non-pharmacological treatments, like respiratory rehabilitation, oxygen therapy and lung transplant. They also give an overview of the new drugs that have become available for the treatment of IPF. These two drugs are the first to slow down disease progression. The Portuguese researchers stress that the therapeutic strategy should fit each individual patient. According to them it is more important to take into account the clinical behavior of the disease than the respiratory function parameters.

Reference:
Robalo Cordeiro C, Campos P, Carvalho L, et al. Consensus document for the diagnosis and treatment of idiopathic pulmonary fibrosis: Joint Consensus of Sociedade Portuguesa de Pneumologia, Sociedade Portuguesa de Radiologia e Medicina Nuclear e Sociedade Portuguesa de Anatomia Patológica. Rev Port Pneumol 2016;22:112-22.