Figures
- FIGURE 1
Schematic representation of types of interstitial lung disease (ILD) that may be associated with a progressive fibrosing phenotype. Connective tissue disease (CTD)-ILDs include rheumatoid arthritis-associated ILD, systemic sclerosis-associated ILD, mixed CTD-associated ILD and other autoimmune ILDs. Other ILDs include exposure-related ILDs (asbestosis and silicosis), non-idiopathic pulmonary fibrosis (IPF) idiopathic interstitial pneumonias (desquamative interstitial pneumonia, etc.), and others. g/f PF: genetic and/or familial pulmonary fibrosis; HP: hypersensitivity pneumonitis; iNSIP: idiopathic nonspecific interstitial pneumonia; IPAF: interstitial pneumonia with autoimmune features; uILD: unclassifiable ILD.
- FIGURE 2
Diagnosis of fibrosing interstitial lung diseases (ILDs) that may present a progressive phenotype. BAL: bronchoalveolar lavage; HRCT: high-resolution computed tomography; MDD: multidisciplinary diagnosis; PF-ILD: progressive-fibrosing ILD; PFT: pulmonary function test. Reproduced from [6].
Tables
- TABLE 1
Proposed criteria that may be used in clinical practice to assess disease progression in fibrotic interstitial lung diseases
Lung function Rate of decline in FVC (mL·year−1)
Absolute or relative changes in FVC (mL or % predicted)
Absolute or relative changes in DLCO % predicted
Exercise capacity Absolute change in 6-min walk test distance
Change in oxygen saturation nadir during 6-min walk test
Change in maximal exercise capacity Symptoms and patient-reported outcomes Change in symptoms
Change in everyday life exercise capacity
Questionnaires on shortness of breath, cough, and/or quality of life
Acute worsening Acute exacerbation of fibrosis (idiopathic or triggered)
Non-elective hospitalisation for a respiratory cause
HRCT Change in the extent or texture of fibrotic features on HRCT
Change in quantitative fibrosis scores on HRCT#
Need for supportive care Initiation of ambulatory oxygen therapy at exercise
Initiation of supplemental oxygen therapy at rest, or change in flow of oxygen
Serum biomarkers None validated
Not yet applicable in clinical practice
As these criteria are intended to guide individual decisions in clinical practice, they may differ from end-points used in clinical trials [17]. Most clinicians would make management decisions based on a combination of variables. HRCT: high-resolution computed tomography; FVC: forced vital capacity; DLCO: diffusing capacity of the lung for carbon monoxide. #: not yet routinely available.
- TABLE 2
Definition of disease progression in the main ongoing studies involving patients with progressive fibrosing interstitial lung diseases
Study name and registration number Period considered to assess progression Criteria [Ref.] INBUILD, NCT02999178 24 months prior to screening Relative decline in FVC ≥10% predicted [3] Relative decline in FVC ≥5–<10% predicted combined with increased extent of fibrosis on HRCT Relative decline in FVC ≥5–<10% predicted combined with worsening of respiratory symptoms Worsened respiratory symptoms and increased extent of fibrosis on HRCT only uILD, NCT03099187 6 months prior to screening >5% absolute decline in FVC % predicted [22] Significant symptomatic worsening not due to cardiac, pulmonary (except worsening of underlying uILD), vascular or other causes (as determined by the investigator) RELIEF, DRKS00009822 6 months prior to inclusion Slope calculation of at least three values documenting an annualised decline in FVC % predicted of 5% (absolute) or more despite appropriate conventional therapy [23] One criterion was sufficient for eligibility in each of the respective studies. uILD: unclassifiable interstitial lung disease; FVC: forced vital capacity; HRCT: high-resolution computed tomography.
