Monitoring and management of ILD progression in CTD-ILDs
Regular monitoring for progression of connective tissue disease-associated interstitial lung diseases (CTD-ILDs) is vital to inform appropriate care1
There is currently no uniformly endorsed definition of the progressive fibrosing phenotype,11 and, in clinical practice, no threshold or rate of decline has been formally accepted12
Once alternative explanations, such as respiratory tract infection, have been excluded and treated, patients with ILDs meeting the following criteria at any point within 24 months despite treatment in clinical practice can be considered to have progressive fibrosis:13
Relative decline of ≥10% in FVC
Relative decline of ≥5–<10% in FVC and relative decline of ≥15% in DLCO
Relative decline of ≥5–<10% in FVC and worsening of respiratory symptoms
Relative decline of ≥5–<10% in FVC and increased extent of fibrosis on HRCT
Worsening of respiratory symptoms and increased extent of fibrosis on HRCT
TIME IS OF THE ESSENCE WHEN PROGRESSIVE FIBROSING ILD IS SUSPECTED9
Delayed detection of progressive pulmonary fibrosis in ILDs can be deadly. Patients have an estimated survival time of 2.5–4 years after detection of progressive fibrosis4,9* Early detection of patients with progressive fibrosing ILD could inform management decisions and patient counselling4
HOW COULD THE TIME BETWEEN ILD DIAGNOSIS AND DETECTION OF THE PROGRESSIVE FIBROSING PHENOTYPE IN YOUR PATIENTS BE SHORTENED?
A patient with CTD-ILD could wait an average of 2.5–3 years from initial ILD symptoms to the diagnosis of progressive fibrosing ILD4*
Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management, 2019, Curr Med Res Opin. Reprinted by permission of the publisher Informa UK Limited trading as Taylor & Francis Ltd.
Patient journey in non-IPF progressive fibrosing ILDs as estimated by an online physician survey*
* Estimates of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of total 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom4
IN CTD-ILDs, ILD MONITORING CAN BE OPTIMIZED BY CROSS-SPECIALTY COLLABORATION1
Multidisciplinary evaluation is the gold standard in the diagnosis of ILD and evaluation of its severity14
In instances when an MDT is challenging to convene for a patient with ILD, multidisciplinary discussion can be conducted virtually or by telephone to enable each specialty group to contribute15
Measurements taken during PFTs should include FVC, DLCO and TLC1
Despite its limitations in identifying ILD in patients with CTD, the 6MWD can be used to monitor ILD progression and provide prognostic information for established CTD-ILD – therefore, a regular assessment of the 6MWD is suggested1
Awareness of patient language can help identify the symptoms of ILD in CTDs16
All patients with SSc-ILD need to be followed up every 3–6 months with PFTs, and repeated HRCT as guided by clinical decision, given the highly variable progression of the disease17,18
Learn more about monitoring your patients with SSc-ILD after initiating treatment
Monitoring for RA-ILD progression with PFTs is recommended at 3–6-monthly intervals, then every 6–12 months if stable19
Monitoring for progression of primary Sjögren's syndrome-ILD using PFTs is recommended every 3–6 months in the first 1–2 years20
Learn more about monitoring for ILD progression in primary Sjögren's syndrome
TREATMENT OF PROGRESSIVE CTD-ILD SHOULD NOT WAIT1,14
A multidisciplinary discussion should be initiated upon diagnosis of ILD in patients with CTD, and during their monitoring, to determine the optimal approach to slow ILD progression12,13
The decision to treat CTD-ILD can be based on whether the patient is clinically impaired by the ILD, whether the ILD is progressive by symptoms, physiology and/or imaging, and what extrathoracic features require therapy1
Proposed algorithm outlining general recommendations for management of ILD in patients with CTD1
*There is currently no consenus on when to initiate treatment of SSc-ILD.
Adapted from: Geerts S,et al. Sacoidosis Vasc Diffuse Lung Dis. 2017;34:326-335
MANAGEMENT GUIDANCE FOR SPECIFIC CTD-ILDs SHOULD SUPERSEDE GENERAL GUIDANCE FOR MANAGEMENT OF CTD-ILDs AS A WHOLE17,20-23
Some examples are presented below:
Treatment initiation in patients with SSc-ILD should aim for prevention of progression to avoid irreversible lung damage17,21,22
How can you identify and optimize treatment of ILD in your patients with SSc-ILD?
Progression and severity of ILD are the main factors to consider when making treatment decisions for RA-ILD23
How can you identify and optimize treatment of ILD progression in your patients with RA-ILD?
Progression and severity of ILD are the main factors to consider when making treatment decisions for primary Sjögren's syndrome-ILD20
How can you identify and optimize treatment of ILD progression in your patients with primary Sjögren's syndrome-ILD?
How else can you manage your patients with CTD-ILDs?
Management of SSc-ILD
Providing palliative/supportive care
- * Estimates of patients with non-IPF ILD that develop progressive fibrosis based on an online survey of total 486 physicians (243 pulmonologists, 203 rheumatologists and 40 internists) from the United States, Japan, France, Germany, Italy, Spain and the United Kingdom4
- † Depending on the overall prognosis and risks of ILD progression.
- 6MWD, six-minute walking distance; BAL, bronchoalveolar lavage; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; CXR, chest X-ray; DLCO, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; HRQoL, health-related quality of life; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; MDD, multidisciplinary discussion; MDT, multidisciplinary team; PFT, pulmonary function test; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc-ILD, systemic sclerosis-associated interstitial lung disease; TLC, total lung capacity.
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