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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

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SSc-ILD SHOWS HETEROGENOUS RATES OF PROGRESSION BUT IS FUNDAMENTALLY PROGRESSIVE OVER THE LONG TERM1,4

The aim of initiating treatment in patients with SSc-ILD should be to: 2

Improve patient symptoms and quality
of life

Improve patient symptoms and quality of life
Delay ILD progression

Delay ILD progression
Prolong survival

Prolong survival

Fibrosis may have already progressed to ≥10% of your SSc patient’s lung volume at the moment of diagnosis of their ILD5

Fibrotic ILD affecting ≥10% of lung volume on HRCT is associated with early mortality in patients with SSc5

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

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
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Guiding optimal clinical management of patients with SSc-ILD can be achieved by incorporating collaboration between rheumatologists, pulmonologists and internists2

Learn more about how to proactively collaborate to optimise ILD care through a multidisciplinary team approach

Don’t delay initiating treatment in your patients with SSc-ILD – early treatment is important to achieve a better prognosis6

What other management considerations should you bear in mind?
Footnotes
  • 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.

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. Roofeh D, Jaafar S, Vummidi D, et al. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31;241–249.

  8. Fischer A, Patel NM, Volkmann ER. Interstitial lung disease in systemic sclerosis: focus on early detection and intervention. OARRR. 2019;11;283–307.

  9. 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.

  10. 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. 

  11. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980. 

  12. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print]. 

  13. 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.

  14. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.

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