EDITORIAL
The Fleischner Society diagnostic criteria for IPF Clinical implications
 
More details
Hide details
1
First Academic Department of Pneumonology, Hospital for Diseases of the Chest, “Sotiria”, Medical School, National and Kapodistrian University of Athens, Athens, Greece
 
 
Corresponding author
Demosthenes Bouros   

First Academic Department of Pneumonology, Hospital for Diseases of the Chest, “Sotiria”, Medical School, National and Kapodistrian University of Athens, Athens, Greece; 152 Messogion Ave., Athens 11527, Greece
 
 
Pneumon 2017;30(3):129-132
 
KEYWORDS
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.
 
REFERENCES (17)
1.
Bouros D. Idiopathic Pulmonary Fibrosis: The dawn of a new era. Pneumon 2014; 27:117-9.
 
2.
Noble PW, Albera C, Bradford WZ. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomized trials. Lancet 2011; 377:1760–9.
 
3.
King TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfe nidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370:2083–92.
 
4.
Richeldi L, du Bois RM, Raghu G, et al INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370:2071–82.
 
5.
Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788-824.
 
6.
Lynch DA, Sverzellati N, Travis WD et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Published online November 15, 2017 http://dx.doi.org/10.1016/S221....
 
7.
Tzilas V, Valeyre D, Tzouvelekis A, Bouros D. Taking a giant step in the diagnosis of idiopathic pulmonary fibrosis. Lancet Respir Med. Published online November 15, 2017 http://dx.doi.org/10.1016/S221....
 
8.
Tzilas V, Tzouvelekis A, Chrysikos S, et al. Diagnosis of Idiopathic Pulmonary Fibrosis “Pragmatic Challenges in Clinical Practice”. Front Med (Lausanne) 2017; 4:151.
 
9.
Brownell R, Moua T, Henry TS, et al. The use of pretest probability increases the value of high-resolution CT in diagnosing usual interstitial pneumonia. Thorax 2017; 72:424-9.
 
10.
Fell CD, Martinez FJ, Liu LX, et al. Clinical predictors of a diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2010; 181:832-7.
 
11.
Salisbury ML, Xia M, Murray S, et al. Predictors of idiopathic pulmonary fibrosis in absence of radiologic honeycombing: A cross sectional analysis in ILD patients undergoing lung tissue sampling. Respir Med 2016;118:88-95.
 
12.
Tzilas V, Bouros D. Usual interstitial pneumonia pattern in the diagnosis of idiopathic pulmonary fibrosis? Lancet Respir Med 2016; 4:770-2
 
13.
Sverzellati N, Wells AU, Tomassetti S, et al. Biopsy-proved idiopathic pulmonary fibrosis: spectrum of nondiagnostic thinsection CT diagnoses. Radiology 2010; 254:957-64.
 
14.
Silva CI, Müller NL, Lynch DA, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008; 246:288-97.
 
15.
Hutchinson JP, McKeever TM, Fogarty AW, et al. Surgical lung biopsy for the diagnosis of interstitial lung disease in England: 1997-2008. Eur Respir J 2016; 48:1453-61.
 
16.
Hutchinson JP, Fogarty AW, McKeever TM, Hubbard RB. InHospital Mortality after Surgical Lung Biopsy for Interstitial Lung Disease in the United States. 2000 to 2011. American journal of respiratory and critical care medicine 2016; 193:1161-7.
 
17.
Tzilas V, Bouros D. Reference centers for interstitial lung diseases. Pneumon 2016; 29:15-7.
 
eISSN:1791-4914
ISSN:1105-848X
Journals System - logo
Scroll to top