Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

Spyros A Papiris,Likurgos Kolilekas,Christina Triantafillidou,Iraklis Tsangaris,Effrosyni D Manali,Charis Roussos,Konstantinos Kagouridis

doi:10.1186/cc9241

Abstract

Idiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected, and IPF acute exacerbations constitute the most devastating complication during its clinical course. IPF exacerbations are subacute/acute, clinically significant deteriorations of unidentifiable cause that usually transform the slow and more or less steady disease decline to the unexpected appearance of acute lung injury/acute respiratory distress syndrome (ALI/ARDS) ending in death. The histological picture is that of diffuse alveolar damage (DAD), which is the tissue counterpart of ARDS, upon usual interstitial pneumonia, which is the tissue equivalent of IPF. ALI/ARDS and acute interstitial pneumonia share with IPF exacerbations the tissue damage pattern of DAD. 'Treatment' with high-dose corticosteroids with or without an immunosuppressant proved ineffective and represents the coup de grace for these patients. Provision of excellent supportive care and the search for and treatment of the 'underlying cause' remain the only options. IPF exacerbations require rapid decisions about when and whether to initiate mechanical support. Admission to an intensive care unit (ICU) is a particular clinical and ethical challenge because of the extremely poor outcome. Transplantation in the ICU setting often presents insurmountable difficulties.

Highlights

Introduction and definitionsIdiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to IPF exacerbations represent acute and clinically significant deteriorations of unidentifiable cause, transforming the slow and more or less steady disease decline [13] to the unexpected appearance of acute lung injury/ acute respiratory distress syndrome (ALI/ARDS) ending in death [6,14]
Even after the exclusion of any identifiable and treatable factor(s) inducing IPF exacerbations, the most important etiologic hypothesis remains that of a clinically occult infection that precipitates an already usual interstitial pneumonia (UIP)-scarred lung into diffuse alveolar damage (DAD) [6]
Toward the intensive care unit An IPF patient is referred to the ICU for severe acute respiratory failure as a consequence of the clinical scenarios that may lead to ventilatory support (Figure 1)

Summary

Introduction

Introduction and definitionsIdiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to IPF exacerbations represent acute and clinically significant deteriorations of unidentifiable cause, transforming the slow and more or less steady disease decline [13] to the unexpected appearance of acute lung injury/ acute respiratory distress syndrome (ALI/ARDS) ending in death [6,14]. After admission to the hospital ward, the patient usually becomes unable to maintain spontaneous breathing within hours or very few days, often not enough time for the extensive work-up required to identify treatable factors of deterioration, and needs ventilatory support and ICU transfer [5,7,8].

Results

Conclusion