Early Diagnosis of Pulmonary Fibrosis - Diagnostic Delay

Early Diagnosis of Pulmonary Fibrosis

Patients with newly diagnosed IPF are investigated for the diagnostic delay before a diagnosis of IPF is made.

Study Overview

• Status: Active, not recruiting
• Conditions: Idiopathic Pulmonary Fibrosis

Detailed Description

Pulmonary fibrosis can be secondary to connective-tissue disease, environmental exposure, or drug toxicity, but it can also appear sporadically without any known cause, i.e. idiopathic interstitial pneumonitis (IIP). Idiopathic pulmonary fibrosis (IPF) is the commonest IIP and usually follows a rapidly progressive course with a short median survival time.

IPF is often diagnosed after a long diagnostic delay, which also affects the prognosis. As new anti-fibrotic treatments have been approved, and awareness of IPF is rising, the diagnostic delay and its implications can be expected to be changing. Also, the new diagnostic guidelines of 2011 could change the diagnostic delay. In order to reduce the diagnostic delay, it is important to investigate the health care utilization and decisions made by healthcare professionals in the period before the final diagnosis is made.

This study will prospectively include all patients at the two centres in Denmark where patients are treated for IPF and has thus a good opportunity to include the majority of incident cases of IPF in Denmark. Patients are included immediately after the diagnosis which reduces recall bias. The database will include both patient reported data and objective data from national registries and patient records. A main focus is the distribution of the diagnostic delay between patient and different health care providers, and the health care utilization by the patients before a diagnosis of IPF is made. Risk factors for a delayed diagnosis are investigated. The importance of the diagnostic delay for the prognosis and the course of the disease will also be investigated.

The database created in this study will also be used for future research in IPF.

• Study Type: Observational
• Enrollment (Estimated): 300

Contacts and Locations

Study Locations

• Denmark
  • Aarhus, Denmark, 8000
    • Aarhus University Hospital
  • Copenhagen
    • Hellerup, Copenhagen, Denmark, 2800
      • Gentofte Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Probability Sample

Study Population

All incident patients with IPF at Gentofte hospital and Aarhus university hospital.

Description

Inclusion Criteria:

• Diagnosis of IPF according to international guidelines

Exclusion Criteria:

• Unable to provide written informed consent
• Age below 18 years

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Other

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Number of patients who fulfil any of the following: disease progression or death	1 year

Secondary Outcome Measures

Outcome Measure	Time Frame
Number of patients who fulfill any of the following: decrease in lung function, reduced walking distance at 6 minutes walking test, increased need for supplementary oxygen, hospitalization	1 year
All-cause and disease-specific mortality	1 year
Number of respiratory and non-respiratory hospitalizations	1 year
Decrease in walking distance at the 6 minute walking test	1 year
Change in St. George Respiratory Questionnaire symptom scores	1 year
Reduction in diffusion capacity (DLCO) or forced vital capacity (FVC)	1 year

Other Outcome Measures

Outcome Measure	Measure Description	Time Frame
Diagnostic delays	Diagnostic delay subdivided into patient related delays and health care related delays.	1 year

Collaborators and Investigators

• Sponsor: Nils Hoyer
• Collaborators: Aarhus University Hospital

Publications and helpful links

General Publications

• Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King TE Jr, Kondoh Y, Myers J, Muller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schunemann HJ; ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824. doi: 10.1164/rccm.2009-040GL.
• Hoyer N, Prior TS, Bendstrup E, Wilcke T, Shaker SB. Risk factors for diagnostic delay in idiopathic pulmonary fibrosis. Respir Res. 2019 May 24;20(1):103. doi: 10.1186/s12931-019-1076-0.

Study record dates

Study Major Dates

• Study Start: March 1, 2016
• Primary Completion (Actual): December 1, 2022
• Study Completion (Estimated): December 1, 2028

Study Registration Dates

• First Submitted: May 10, 2016
• First Submitted That Met QC Criteria: May 12, 2016
• First Posted (Estimated): May 13, 2016

Study Record Updates

• Last Update Posted (Actual): December 22, 2023
• Last Update Submitted That Met QC Criteria: December 21, 2023
• Last Verified: December 1, 2023

More Information

Terms related to this study

• Keywords: Diagnosis
• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Lung Diseases, Interstitial; Fibrosis; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis
• Other Study ID Numbers: DELAY

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED