Clinical trial NCT02772549: Early Diagnosis of Pulmonary Fibrosis - Diagnostic Delay
Official title: Early Diagnosis of Pulmonary Fibrosis
Study type: OBSERVATIONAL
Overall status: ACTIVE_NOT_RECRUITING
Conditions: Idiopathic Pulmonary Fibrosis
Keywords: Diagnosis
Lead sponsor: University Hospital, Gentofte, Copenhagen
Sponsor class: OTHER
Collaborators: name: Aarhus University Hospital
First posted: 2016-05-13
Enrollment: 300
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: OTHER
Sampling method: PROBABILITY_SAMPLE
Study population: All incident patients with IPF at Gentofte hospital and Aarhus university hospital.

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

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.

Eligibility criteria: Inclusion Criteria:

* Diagnosis of IPF according to international guidelines

Exclusion Criteria:

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

Primary outcomes:
measure: Number of patients who fulfil any of the following: disease progression or death
timeFrame: 1 year
Secondary outcomes:
measure: 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
timeFrame: 1 year
measure: All-cause and disease-specific mortality
timeFrame: 1 year
measure: Number of respiratory and non-respiratory hospitalizations
timeFrame: 1 year
measure: Decrease in walking distance at the 6 minute walking test
timeFrame: 1 year
measure: Change in St. George Respiratory Questionnaire symptom scores
timeFrame: 1 year
measure: Reduction in diffusion capacity (DLCO) or forced vital capacity (FVC)
timeFrame: 1 year
Other outcomes:
measure: Diagnostic delays
timeFrame: 1 year
Locations:
city: Hellerup
country: Denmark
facility: Gentofte Hospital
state: Copenhagen
city: Aarhus
country: Denmark
facility: Aarhus University Hospital
Study officials:
References:
citation: 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.
citation: 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.