Idiopathic Pulmonary Fibrosis
Conditions
Keywords
Diagnosis
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.
Interventions
None listed
Sponsors
Aarhus University Hospital
Nils Hoyer
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Diagnosis of IPF according to international guidelines
Exclusion criteria
* Unable to provide written informed consent * Age below 18 years
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number of patients who fulfil any of the following: disease progression or death | 1 year |
Secondary
| 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 |
| Change in St. George Respiratory Questionnaire symptom scores | 1 year |
| Reduction in diffusion capacity (DLCO) or forced vital capacity (FVC) | 1 year |
| Decrease in walking distance at the 6 minute walking test | 1 year |
Other
| Measure | Time frame | Description |
|---|---|---|
| Diagnostic delays | 1 year | Diagnostic delay subdivided into patient related delays and health care related delays. |
Countries
Denmark
Outcome results
None listed