Management of SSc-ILD
Treatment initiation in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) should aim for prevention of progression to avoid irreversible lung damage1–3
![Steph hero image](/sites/default/files/2021-11/Hero-Rheums-desktop-1280x495px%20STEPH.jpg)
![Improve patient symptoms and quality
of life](/sites/default/files/2021-11/01-elements-placeholder-card-1-1%403x_34.jpg)
Improve patient symptoms and quality of life
![Delay ILD progression](/sites/default/files/2021-11/01-elements-placeholder-card-1-1%403x_35.jpg)
Delay ILD progression
![Prolong survival](/sites/default/files/2021-11/01-elements-placeholder-card-1-1%403x_36.jpg)
Prolong survival
WHY SHOULD YOU AIM TO PREVENT PROGRESSION IN PATIENTS WITH SSc-ILD?
The current practice of treating patients with SSc-ILD once worsening has occurred is sub-optimal.3 This may lead to missed opportunities to treat patients whose disease is progressing, as these patients might be stable during further follow-up. SSc-ILD patients with evidence of ongoing progression, based on pulmonary function decline or radiographic deterioration, need treatment independent of disease extent.6
Clinical management algorithm for SSc-ILD2
![Algorithm for SSc-ILD screening / diagnosis recommending primary tool HRCT](/sites/default/files/2021-09/desktop-P4-1-R4-3-R4-5%402x.jpg)
This algorithm provides a brief summary of evidence-based European consensus statements, including the supplemental Delphi process, from expert opinion of steering committee for identification and management of SSc-ILD, for use in clinical practice.
Adabted from Hoffmann-Vold AM, et al. Lancet Rheum. 2020;2:e71-e83.
A MULTIDISCIPLINARY TEAM APPROACH OPTIMIZES EVALUATION AND CARE OF PATIENTS WITH SSc-ILD2,7,8
Due to multi-organ involvement in SSc and the complex nature of SSc-ILD, multidisciplinary engagement is key to provide optimal care for patients.8
Example of multidisciplinary team for SSc-ILD management
![P324152](/sites/default/files/2021-11/desktop-P3-2-P4-1-P5-2.jpg)
Guiding optimal clinical management of patients with SSc-ILD can be achieved by incorporating collaboration between rheumatologists, pulmonologists and internists2
What other management considerations should you bear in mind?
![Ongoing monitoring in SSc-ILD](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_11.jpg)
Ongoing monitoring in SSc-ILD
![Scheme representing the approaches available to support patients with ILD.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9%402x_7.png)
Providing palliative/supportive care
![Blurred scheme of NON-IPF ILDs management algorithm.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9%402x_8.png)
Multidisciplinary teams
Footnotes
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CTD-ILD, connective tissue disease-associated interstitial lung disease; DLCO, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.
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Hoffmann-Vold AM, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2020;217455.
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Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheum. 2020;2:e71–e83.
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Distler O, Volkmann ER, Hoffmann-Vold AM, et al. Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease. Expert Rev Clin Immunol. 2019;15:1009–1017.
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Guler, S.A., Winstone, T.A., Murphy, D., et al. Does systemic sclerosis–associated interstitial lung disease burn out? Specific phenotypes of disease progression. Annals ATS. 2018;15;1427–1433.
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Hoffmann-Vold AM, Fretheim H, Halse AK, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200;1258–1266.
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Cappelli S, Bellando Randone S, Camiciottoli G, et al. Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev. 2015;24;411–419.
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Roofeh D, Jaafar S, Vummidi D, et al. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31;241–249.
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Fischer A, Patel NM, Volkmann ER. Interstitial lung disease in systemic sclerosis: focus on early detection and intervention. OARRR. 2019;11;283–307.
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Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020;55;1902026.
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Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203-210.
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Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.
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Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print].
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Sgalla G, Cerri S, Ferrari R, et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res. 2015;2(1):e000065.
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Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.
Resources for patients
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![Life with Pulmonary Fibrosis website logo.](/sites/default/files/2021-07/logo-primary_0.png)
![Scleroderma female patient looking into the window.](/sites/default/files/2021-07/image_0.png)
![More then scleroderma website logo.](/sites/default/files/2021-07/logo-primary_1.png)