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World J Respirol. Mar 28, 2016; 6(1): 14-23
Published online Mar 28, 2016. doi: 10.5320/wjr.v6.i1.14
Prognostic scoring systems for clinical course and survival in idiopathic pulmonary fibrosis
Ermanno Puxeddu, Paola Rogliani
Ermanno Puxeddu, Paola Rogliani, Respiratory Medicine, Department of Systems Medicine, University of Rome “Tor Vergata”, 00133 Rome, Italy
Author contributions: Both Puxeddu E and Rogliani P performed literature review and wrote the paper.
Conflict-of-interest statement: Rogliani P and Puxeddu E have no conflict of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Paola Rogliani, MD, Respiratory Medicine, Department of Systems Medicine, University of Rome “Tor Vergata”, Via Montpellier 1, 00133 Rome, Italy. paola.rogliani@uniroma2.it
Telephone: +39-6-20903616
Received: September 23, 2015
Peer-review started: October 3, 2015
First decision: November 24, 2015
Revised: December 22, 2015
Accepted: January 29, 2016
Article in press: January 31, 2016
Published online: March 28, 2016
Processing time: 184 Days and 10.1 Hours
Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common and rapidly fatal among idiopathic interstitial pneumonias. Its clinical course is variable. A significant fraction of the population of patients display a slow disease course and can remain stable for years, while other patients show a rapid progressive course and may die within few months from diagnosis. For these reasons estimating prognosis of IPF patients is extremely difficult and has important clinical repercussions on optimal patients management including patients referral for lung transplantation. Several studies have tried to address this key point in the course of the two last decades analyzing different clinical, functional, radiological and biological variables. The purpose of this review is to assess relevant studies published on this subject and to examine the variety of prognostic predictors proposed along with staging systems.

Keywords: Idiopathic pulmonary fibrosis; Prognosis; Survival; Scoring systems

Core tip: Idiopathic pulmonary fibrosis (IPF) is the most common and rapidly lethal among interstitial lung disease. Its clinical course is highly variable and estimating prognosis of patients with IPF is extremely difficult with important impacts on the best clinical management of patients, including the referral of patients for lung transplantation. In this review article we evaluate relevant studies published on this subject and examine the variety of proposed prognostic predictors along with staging systems.