Fibrosing CTD-ILDs with a progressive phenotype
Up to 4 in 10 patients with connective tissue disease-associated interstitial lung diseases (CTD-ILDs) can develop a progressive fibrosing phenotype1–5
PATIENTS WITH CTD-ILDs ARE AT RISK OF DEVELOPING A PROGRESSIVE FIBROSING PHENOTYPE, REGARDLESS OF THE UNDERLYING CTD2,6–8
The progressive fibrosing phenotype is defined by the presence of:6–9
In progressive fibrosing ILD, pulmonary fibrosis continues to develop independent of the originating disease (SSc, RA, primary Sjögren’s syndrome, etc.) or trigger (inflammation, exposure, etc.), along common pathogenic pathways6,9–11
PREVALENCE OF THE PROGRESSIVE FIBROSING PHENOTYPE IN PATIENTS WITH CTD-ILDs
It is estimated that 16%–40% of patients with CTD-ILDs develop a progressive fibrosing phenotype – based on a systematic review of the published literature and a physician survey1–5
Proportion of patients with a progressive phenotype, %
Reference / source
Rheumatoid arthritis-associated interstitial lung disease (RA-ILD)
Systemic sclerosis-associated interstitial lung disease (SSc-ILD)
Polymyositis- and dermatomyositis-associated interstitial lung disease (PM/DM-ILD)
|Primary Sjögren’s syndrome-associated interstitial lung disease (primary Sjögren’s syndrome-ILD)||24||
Systemic lupus erythematosus-associated interstitial lung disease (SLE-ILD)
Mixed connective tissue disease-associated interstitial lung disease (MCTD-ILD)
* Estimates of the percentage of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of total 486 physicians (243 pulmonologists, 203 rhuematologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom. Sourced percentages (24%) for primary Sjögren’s syndrome-ILD, SLE-ILD and MCTD-ILD were from the same ‘Other CTD-ILDs’ group.
THE BURDEN OF PROGRESSIVE FIBROSING CTD-ILD CAN SUBSTANTIALLY REDUCE A PATIENT'S HEALTH-RELATED QUALITY OF LIFE9
Cough and dyspnea adversely affect the everyday lives of patients with CTD-ILDs15–17
Patients with progressive CTD-ILDs suffer from emotional distress that may include feelings of depression, anxiety and loss of independence18,19
Loss of independence
PROGRESSIVE CTD-ILDs HAVE A WIDE RANGE OF POTENTIAL IMPACTS ON PATIENTS7,18
Patients with progressive CTD-ILDs report almost universal symptoms of anxiety and/or depression18
Progressive fibrosing ILD is characterized by progression of pulmonary fibrosis, worsening of symptoms, decline in lung function and deterioration in health-related quality of life7
Factors that reflect progression of ILDs
Adapted from: Kolb M, Vašáková M. Respir Res. 2019;20(1).
Declines in lung function shown by PFTs are associated with increased mortality in patients with CTD-ILDs14,20–22
UNCOVER THE FULL IMPACT OF PROGRESSIVE FIBROSING CTD-ILDs
See the impact of progressive fibrosing ILD across a range of CTD-ILDs
Patients with interstitial lung diseases face an unpredictable journey. There are over 200 types of ILDs, of which Idiopathic pulmonary fibrosis, or IPF, is the most common fibrosing type. Around 1 in 5 patients with differing ILD diagnoses may develop a ‘progressive fibrosing phenotype’. Progressive fibrosing ILDs are believed to progress via common pathobiological fibrotic pathways, independent of the original trigger. These progressive fibrosing ILDs are characterised by self-sustaining, diffuse and irreversible fibrosis, worsening respiratory symptoms and declining lung function. The progressive and permanent fibrotic lung damage can place an overwhelming burden on patient’s quality of life and is associated with high mortality. No treatments are currently approved for progressive fibrosing ILDs, other than IPF. Thus, there is a high unmet need for effective treatments that slow the disease progression in these patients. Boehringer Ingelheim has a strong commitment to scientific research. The INBUILD® clinical trial is the first and largest phase III global trial to include patients across the broad spectrum of ILDs that develop the ‘progressive fibrosing phenotype’, into a single trial. So together, we hope to help patients with progressive fibrosing ILDs find new ways forward.
The first step to slowing progressive CTD-ILDs is identification of ILD
CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; DLCO, diffusing capacity of the lung for carbon monoxide; DM-ILD, dermatomyositis-associated interstitial lung disease; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; MCTD-ILD, mixed connective tissue disease-associated interstitial lung disease; PFT, pulmonary function test; PM-ILD, polymyositis-associated interstitial lung disease; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SLE-ILD, systemic lupus erythematosus associated interstitial lung disease; SSc-ILD, systemic sclerosis-associated interstitial lung disease.
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