Objectives | Endpoints | |
---|---|---|
Primary | To compare various PPF groups and treatment strategies in terms of characteristics and outcomes (lung function, survival, quality of life, other) | Time to progression of disease (any one of the following): Decrease of FVC > 10% predicted within one year Decrease Dlco > 15% predicted plus initiation of LTOT or permanent increase of oxygen flow when LTOT is established within one year Decrease of 6-MWD > 50 m within one year Hospitalization due to respiratory decompensation Death of any cause Change of treatment strategy (stop of anti-inflammatory or antifibrotic therapy, initiation of new anti-inflammatory or antifibrotic therapy or a combination of both) |
Secondary | To assess drug utilization | Treatment strategy (immunosuppression, antifibrotic therapy) Dose and dosing schedule Duration of treatment (persistence) Switches between treatments Phases without drug treatment (with reasons) Reasons for drug discontinuation |
1. To determine risk factors for progression of disease 2. To determine factors for treatment success or failure, respectively, defined as clinical, functional, and radiographic stabilization (i.e. absence of deterioration in each of the categories) and change of treatment strategy (i.e. stop/start of antifibrotic therapy; stop/start of immunosuppressive therapy) | Survival Clinical symptoms Dyspnea Cough Lung function Annual DLCO decline Annual FVC decline 6-min walk distance QoL scores over time Radiographic course: fibrosis on HRCT Therapy escalation Clinical events (exacerbations, hospitalisations) Analyses stratified according to different diagnosis groups | |
3. To determine potential biomarkers, which are routinely measured in many centers, to differentiate inflammatory driven from fibrosis driven progress | Laboratory parameters: CRP LDH Differential blood cell count (lymphocytes, neutrophils, eosinophils, monocytes), BAL differential cell count (as available) | |
To assess safety | Adverse Events Adverse Drug Reactions |