Abstract

BackgroundThose affected by advanced fibrotic interstitial lung diseases have limited treatment options and in the terminal stages, the focus of care is on symptom management. However, quantitatively, little is known about symptom prevalence. We aimed to determine the prevalence of symptoms in Progressive Idiopathic Fibrotic Interstitial Lung Disease (PIF-ILD).MethodsSearches on eight electronic databases including MEDLINE for clinical studies between 1966 and 2015 where the target population was adults with PIF-ILD and for whom the prevalence of symptoms had been calculated.ResultsA total of 4086 titles were screened for eligibility criteria; 23 studies were included for analysis. The highest prevalence was that for breathlessness (54–98%) and cough (59–100%) followed by heartburn (25–65%) and depression (10–49%). The heterogeneity of studies limited their comparability, but many of the symptoms present in patients with other end-stage disease were also seen in PIF-ILD.ConclusionsThis is the first quantitative review of symptoms in people with Progressive Idiopathic Fibrotic Interstitial Lung Diseases. Symptoms are common, often multiple and have a comparable prevalence to those experienced in other advanced diseases. Quantification of these data provides valuable information to inform the allocation of resources.

Highlights

  • Those affected by advanced fibrotic interstitial lung diseases have limited treatment options and in the terminal stages, the focus of care is on symptom management

  • The British Thoracic [4] and NICE idiopathic pulmonary fibrosis guidance [5] emphasize the importance of a proactive approach in managing symptoms

  • Overview of included studies Twenty-three articles describing symptoms were selected for this review potentially relevant but excluded studies have been listed separately in Additional file 3 APPENDIX C

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Summary

Introduction

Those affected by advanced fibrotic interstitial lung diseases have limited treatment options and in the terminal stages, the focus of care is on symptom management. We aimed to determine the prevalence of symptoms in Progressive Idiopathic Fibrotic Interstitial Lung Disease (PIF-ILD). A subset of patients with Progressive Idiopathic Fibrotic Interstitial Lung Diseases (PIF-ILD) such as idiopathic pulmonary fibrosis have a short disease trajectory and a similar prognosis to people with lung cancer [1]. It is important to differentiate NSIP from IPF in the early stages when the disease is potentially responsive to therapy [2] .when the. The British Thoracic [4] and NICE idiopathic pulmonary fibrosis guidance [5] emphasize the importance of a proactive approach in managing symptoms

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