IPF Italian Observational Study (FIBRONET) in Idiopathic Pulmonary Fibrosis

Percentage of participants with IPF symptoms such as cough, fatigue, dizziness, chest pain or any other symptom at 12-month follow up visit. The symptoms in the class 'other' reported upon specific visits were dyspnea, hemoptysis, post-nasal drip, sputum, weight loss, worsening of fatigue and lack of appetite.

Baseline (V1), 3 months (V2), 6 months (V3), 9 months (V4) and 12 months (V5).

In calculating the change from baseline to all follow-up visits (3 months, 6 months, 9 months and 12 months) in lung function: Vital Capacity (VC), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter VC = value of parameter VC at follow up visit - value of parameter VC at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Forced Vital Capacity (FVC), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter FVC = value of parameter FVC at follow up visit - value of parameter FVC at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Forced Vital Capacity (FVC), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter FVC = value of parameter FVC at follow up visit - value of parameter FVC at baseline visit.

A positive value of change indicates a better outcome.

The value of FVC % of predicted is a relevant parameter to understand and classify the severity of the disease at the diagnosis and to follow up patients during the treatment (i.e. annual rate decline of FVC >10% is a predictor of high rate of mortality).

In calculating the change from baseline to follow-up visits in lung function: Forced Expiratory Volume in the 1st second (FEV1), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter FEV1 = value of parameter FEV1 at follow up visit - value of parameter FEV1 at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Total Lung Capacity (TLC), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter TLC = value of parameter TLC at follow up visit - value of parameter TLC at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Diffusion capacity for carbon monoxide (DLCO), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter DLCO = value of parameter DLCO at follow up visit - value of parameter DLCO at baseline visit.

A positive value of change indicates a better outcome.

Values of DLCO with unit = milliliter/minute/millimeter mercury (ml/min/mmHg) were converted to micromole/minute/kilopascal (mmol/min/kPa) according to the following formula:

DLCO (mmol/min/kPa) = DLCO (ml/min/mmHg)/2.986 [46].

In calculating the change from baseline to follow-up visits in lung function: Partial Pressure of Oxygen (PO2), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter PO2 = value of parameter PO2 at follow up visit - value of parameter PO2 at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Partial Pressure of Carbon dioxide (PCO2), only patients with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter PCO2 = value of parameter PCO2 at follow up visit - value of parameter PCO2 at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Oxygen Saturation (SaO2), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter SaO2 = value of parameter SaO2 at follow up visit - value of parameter SaO2 at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Partial Pressure of Oxygen in arterial blood at rest (PaO2), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter PaO2 = value of parameter PaO2 at follow up visit - value of parameter PaO2 at baseline visit.

A positive value of change indicates a better outcome.

In calculating the change from baseline to follow-up visits in lung function: Partial Pressure of Carbon dioxide in arterial blood at rest (PaCO2), only participants with values available at baseline and at follow up were considered. At follow up visit the absolute changes of lung function assessment vs baseline value was calculated as:

Change in parameter PaCO2 = value of parameter PaCO2 at follow up visit - value of parameter PaCO2 at baseline visit.

A positive value of change indicates a better outcome.

Change from baseline to follow-up visits in exercise tolerance was evaluated by means of change in 6 minute walked distance test.

Change versus baseline was calculated as parameter at follow up - parameter at baseline.

A positive value of change indicates a better outcome. The 6-minute walked distance test was carried out using two parameters start of peripheral capillary oxygen saturation (SpO2) and SpO2 at the end of the test. Only participants with values available at baseline and at follow up were considered

IPF enrolled participants were described in terms of socio-demographic variables (e.g. age, gender, race, body mass index, educational degree, and employment status) at baseline.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their gender are presented. The data is provided in baseline section.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their race are presented. The data is provided in baseline section.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their highest education level.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their employment status are presented.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their Body mass index are presented.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their housing situation are presented.

IPF enrolled participants were described in terms of socio-economic variables; number of participants as per their marital status are presented.

IPF enrolled participants were described in terms of potential IPF risk factors; number of participants as per their smoking habits are presented.

IPF enrolled participants were described in terms of potential IPF risk factors; number of participants as per their environmental exposure (such as bricklayer, building material dust, cement dust, chemical gas, coal dust, factory food, marble dust, masonry dust, mold, paint, powdered detergent and textile material) are presented.

IPF enrolled participants were described in terms of potential IPF risk factors; number of participants as per their exposure to drugs associated with IPF are presented.

IPF enrolled participants were described in terms of potential IPF risk factors; number of participants as per their family history for IPF are presented.

Number of participants with ongoing comorbidities (such as gastroesophageal reflux disease, pulmonary hypertension, emphysema, lung cancer, coronary heart disease, depression) are provided. Some participants reported more than one comorbidity at enrollment.

IPF disease severity and manifestation (including lung function, cardiopulmonary exercise testing and/or exercise capacity if available, laboratory values) is measured by the FVC. Percentages are calculated out of the total number of evaluable participants with available FVC of the predicted at baseline.

Several diagnostic approaches were used to detect IPF, the main ones being High Resolution chest Computer Tomography (HRCT), surgical lung biopsy, Bronchoalveolar lavage (BAL), transbronchial biopsy and spirometry.

Number of participants with ≥1 non-pharmacological therapy for IPF ongoing at baseline (visit 1), 3-month (visit 2), 6-month (visit 3) and 12-month (visit 5) follow up visits are presented.

Number of participants who had lung transplantation at baseline, 3-month, 6-month and 12-month follow up visits are presented.

IPF Patients with ≥1 pharmacological therapy for IPF ongoing at baseline, 3-month, 6-month and 12-month follow up visits are presented. The pharmacological therapies used for IPF treatment are Nintedanib and Pirfenidone.

Number of participants with mild, moderate and severe exacerbations during the observation period are presented. An exacerbation was considered occurred during observation period if onset date ≥ date of first IPF diagnosis and onset date ≤ last available visit date (for participants who completed the study) or date of drop out or date of death (for participants who did not complete the study).

Health Related Quality of Life (HRQoL) variation measured with Saint George's Respiratory Questionnaire (SGRQ), developed to measure health in chronic airflow limitation. It is a disease-specific instrument designed to measure health impairment in terms of impact on overall health, daily life, and perceived well-being in participants with obstructive airways disease. Three component scores (symptoms, activity and impacts on daily life) and a total score were calculated, with lower scores corresponding to better health. The Total score is calculated by summing all positive responses in the questionnaire and expressing the result as a percentage of overall impairment where 100 represents worst possible health status and 0 indicates best possible health status.

The quality of life was evaluated by the EuroQol 5-dimension 5-level (EQ-5D-5L) a standardized measure of health status developed by EuroQol Group to provide a simple generic measure of health status for clinical and economic evaluation. EQ-5D-5L was filled in by participants, it was easy from a cognitive point of view, since it took only few minutes for filling. EQ-5D-5L consists of 2 sections: "EQ-5D descriptive system" and EQ visual analogue scale (EQ VAS). The EQ-5D-5L descriptive system comprises the following 5 dimensions: mobility, self-care, usual activities, pain/discomfort and anxiety/depression.

The quality of life was evaluated by the EQ-5D-5L a standardized measure of health status developed by EuroQol Group to provide a simple generic measure of health status for clinical and economic evaluation. EQ-5D-5L consists of 2 sections: "EQ-5D descriptive system" and EQ VAS. The EQ VAS indicate the health status self-assessed by the participants on a visual analogue scale from 0 to 100, where 100 is the "best imaginable health state" and 0 the "worst imaginable health state". It can be used as a quantitative measure of health as judged by participants.

The health care sector-related costs at diagnosis and from diagnosis up to the end of 12-month follow-up according to the INHS point of view, was carried out in a two-step approach:

(i)First of all the resource consumption exclusively related to both IPF, IPF exacerbations and IPF-related adverse events since diagnosis was collected or estimated and then (ii) A monetary value was assigned to the collected or estimated resource consumption.

Health care resource consumption was computed during observational period in terms of number of (inward and day-hospital) hospitalizations and number of Intensive Care Unit (ICU) admissions.