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
Improve patient symptoms and quality of life
Delay ILD progression
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
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
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?
Providing palliative/supportive care
- 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.
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.
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.
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.
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.
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.
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.
Roofeh D, Jaafar S, Vummidi D, et al. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31;241–249.
Fischer A, Patel NM, Volkmann ER. Interstitial lung disease in systemic sclerosis: focus on early detection and intervention. OARRR. 2019;11;283–307.
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.
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.
Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.
Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print].
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.
Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.