List of variables to be documented (if available) at scheduled visits
| Variable | Baseline | Follow-up every 6 (±3) months* |
|---|---|---|
| Eligibility criteria Physician-diagnosed IPF based on physician diagnosis | x | |
| Baseline information | ||
| (Socio-)demographic variables: age, gender, race, body mass index; employment status, insurance status | x | |
| (Potential) IPF risk factors Cigarette smoking including pack years; environmental exposure; drug exposure; gastro-oesophageal reflux; genetic factors (family history); other | x | |
| Comorbidities | ||
| Atherothrombotic disease including coronary heart disease including previous myocardial infarction),cerebrovascular disease, peripheral arterial disease; pulmonary hypertension; emphysema, lung cancer, renal insufficiency, other diseases | x | |
| IPF Baseline information on IPF | ||
| First symptoms; date of first diagnosis; if performed, dates and results of HRCT, surgical lung biopsy, bronchoalveolar lavage | x | |
| Symptoms Dyspnoea, cough fatigue, dizziness, chest pain, anxiety, etc UCSD SOBQ33 | x | x |
| Functional assessment Lung function test (VCin, FVC, FEV1, TLC; DLCO; pO2, pCO2)42 6 min walk distance (if performed) CPET (if performed) | x | x |
| Serological evaluation Rheumatoid factor, anticyclic citrullinated peptide and antinuclear antibody titre and pattern† Others, for example, SCL-70, SS-A, SS-B BNP/NT-pro BNP | x | x |
| IPF pharmacological treatment Past/discontinued: drugs by class (eg, steroids yes/no) Current: drugs by class; Participation in IPF trial Anticoagulation | x x | x x |
| Non-pharmacological treatment | x | x |
| Long-term-oxygen-therapy (liquid and/or concentrate) | x | x |
| Physician assessment | ||
| Physician's clinical rating of the probable course of IPF (stable, slow or rapid progression) | x | x |
| Quality of life (facultative: 1–2 times/year) | ||
| EQ-5D | x | x |
| Specific instruments for pulmonary disease SGRQ UCSD SoB | x | x |
| Management of IPF and physician contacts | ||
| Number of physician contacts (own office, other physicians) | x | x |
| Number and type of IPF-related procedures in last 6 months | x | x |
| Clinical events and hospitalisations specifically due to: (acute) worsening of IPF, IPF-exacerbation; manifest pulmonary or cardiovascular complications; lung transplantation; pulmonary rehabilitation | x | x |
| Number of days in hospital or in rehabilitation; work days lost due to IPF | x | x |
| Survival status‡ | x |
*Or extraordinary visit in case of events.
†As recommended in the ATS/ERS guidelines for distinguishing connective tissue disease from IPF.
‡Patients have been asked to provide consent to that they may be located or contacted once they have moved or changed centres.
BNP, brain natriuretic peptide; CPET, cardiopulmonary exercise testing; DLCO2, diffusing capacity of the lung for carbon monoxide; FEV, forced expiratory volume; FVC, forced vital capacity; IPF, idiopathic pulmonary fibrosis; pCO2, partial carbon dioxide; SGRQ, St George Respiratory Questionnaire; UCSD SoB, University of California San Diego Shortness of Breath.