The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

Abstract

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases. @ERSpublicationsThe strengths and limitations of IPF management by a multidisciplinary team are highlighted in two patient cases http://ow.ly/H04Xe

European IPF Patient Charter: unmet needs and a call to action for healthcare policymakers

“…Similar needs have been identified in other studies in IPF. For example, the difficulties faced by HCPs in reliably diagnosing IPF [13,[18][19][20], the need for improved multidisciplinary care [21][22][23], the need for improved access to treatment [24], the need for improved awareness of clinical guidelines [18,25] and the need for improved palliative care [26]. This initiative also emphasised the need for improved disease awareness and information, consistent with two recent PG-informed reports from the British Lung Foundation [14,27,28].…”

Safety and Diagnostic Yield of Transbronchial Lung Cryobiopsy in Diffuse Parenchymal Lung Diseases: A Comparative Study versus Video-Assisted Thoracoscopic Lung Biopsy and a Systematic Review of the Literature

“…Diagnosis of DPLDs is a dynamic multidisciplinary process, requiring close communication between clinicians, radiologists, and pathologists [33,34]. SLB is recommended in a large patient subgroup for diagnostic confirmation, in particular in the diagnosis of IPF, in the absence of a UIP pattern on HRCT [2].…”

Transbronchial Cryobiopsies for the Diagnosis of Diffuse Parenchymal Lung Diseases: Expert Statement from the Cryobiopsy Working Group on Safety and Utility and a Call for Standardization of the Procedure

“…While a broad spectrum of DPLD exists, approximately 30% of these patients have idiopathic pulmonary fibrosis (IPF), a progressive fibrotic disease with variable clinical course but, in general, poor survival [2]. The current approach to the diagnosis and management of DPLD includes a multidisciplinary discussion (MDD) undertaken by clinicians, radiologists, and pathologists [3]. Qualitatively and quantitatively adequate lung biopsies are essential contributors to the diagnosis when highresolution computed tomography (HRCT) findings as well as clinical and laboratory data are insufficient.…”