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July - September 2017:
Volume 30, Issue 3

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Pneumon 2017; 30(3):129-132
The Fleischner Society diagnostic criteria for IPF - Clinical implications
Authors Information
1: First Academic Department of Pneumonology, Hospital for Diseases of the Chest, “Sotiria”, Medical School, National and Kapodistrian University of Athens, Athens, Greece
Abstract

Idiopathic Pulmonary Fibrosis is a debilitating, relentlessly progressing disease with a median survival of about 3-5 years. The introduction of antifibrotic agents, pirfenidone and nintedanib marked the beginning of a new era in the management of IPF1. These two agents have been tested only in the context of IPF2-4. This means that the precise diagnosis of IPF is not just an academic exercise but has direct clinical implications.

The latest guidelines for the diagnosis of IPF by ATS/ERS/JRS/ALAT date back to 20115. According to them, the presence of a definite UIP pattern (presence of honeycombing in a predominantly peripheral bibasilar distribution) after exclusion of alternative diagnoses, is considered diagnostic of UIP/IPF obviating the need for surgical lung biopsy (SLB). Thus, honeycombing was a prerequisite in order to avoid tissue based diagnosis. When the patient presented with a possible UIP pattern (presence of traction bronchiectasis/bronchiolectasis in a predominantly peripheral bibasilar distribution but without honeycombing) and inconsistent with UIP pattern, surgical lung biopsy was advised in the diagnostic algorithm in order to establish diagnosis.

It is important to note that back then; there was no approved therapy for IPF. The main focus was to create clinical trial based guidelines in order to ensure the formation of a well characterized population of patients to enroll in clinical trials. The arrival of pirfenidone and nintedanib has created the need for new guidelines. The recently published diagnostic guidelines by the Fleischner Society6 represent a major step forward. They incorporate findings during the last decade and are clinical practice oriented7. Several
points are worth mentioning that have direct implications for clinical practice.