Ongoing monitoring in SSc-ILD
The highly variable course of systemic sclerosis-associated interstitial lung disease (SSc-ILD) requires ongoing, regular monitoring to ensure early intervention against disease progression1–4
![Steph hero image](/sites/default/files/2021-11/Hero-Rheums-desktop-1280x495px%20STEPH.jpg)
RISK FACTORS FOR ILD PROGRESSION IN SSc-ILD
Sex |
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Age |
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Disease duration |
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Disease extent |
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SSc subtype |
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FVC |
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DLCO |
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Serological |
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Skin involvement |
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Reflux/dysphagia symptoms |
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The main risk factors for the progression of ILD are male sex, dcSSc subtype, presence of anti-topoisomerase I antibodies, an FVC of <70%, and the extent of fibrosis at baseline.19 In a 2020 EUSTAR database analysis, FVC, presence of reflux/dysphagia symptoms and mRSS at baseline were predictive for significant ILD progression in the first 12±3 months.1
MONITORING FOR ILD PROGRESSION IN SSc-ILD
SSc-ILD shows heterogenous rates of progression but is fundamentally progressive over the long term.1,20
SSc-ILD shows highly variable patterns of progression over a 5-year period1
![SSc-ILD shows highly variable patterns of progression over a 5-year period](/sites/default/files/2021-11/r4-6-desktop-01.jpg)
Variable patterns of disease course for SSc-ILD patients in the EUSTAR database based on magnitude of FVC changes (% predicted) in individual patients from baseline to the end of the 5-year follow-up period.
Adabted from Hoffmann-Vold A-M, et al. Ann Rheum Dis 2020. Epub ahead of print dol: 10.1138/annrheumdis-2020-217455.
![All patients with SSc-ILD](/sites/default/files/2021-11/01-elements-placeholder-card-1-1%403x_37.jpg)
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 disease3,4
![OmSSCILDKF2](/sites/default/files/2021-11/01-elements-placeholder-card-1-1%403x_38.jpg)
The percentage predicted FVC value, DLCO, extent of fibrosis as seen on HRCT and exercise-induced blood oxygen saturation can indicate ILD progression in patients with SSc-ILD3
![OmSSCILDKF3](/sites/default/files/2021-11/01-elements-placeholder-card-1-1%403x_39.jpg)
HRCT should be performed in all patients with SSc-ILD in the case of abnormal PFT results and/or if progression of symptoms is suspected6,21
HOW CAN YOU CARRY OUT REGULAR ONGOING MONITORING OF ILD IN YOUR PATIENTS WITH SSc-ILD?
Monitoring algorithm for SSc-ILD3
![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.
What management options should you consider for your patients with SSc-ILD?
![Management of SSc-ILD](/sites/default/files/2021-11/01-elements-placeholder-card-16-9%403x_10.jpg)
Management of 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
![card image](/sites/default/files/2021-11/01-elements-placeholder-card-16-9_0.jpg)
Multidisciplinary teams
Footnotes
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CCL-18, chemokine (C-C motif) ligand-18; CRP, C-reactive protein; CTD-ILD, connective tissue disease-associated interstitial lung disease; dcSSc, diffuse cutaneous systemic sclerosis; DLCO, diffusing capacity of the lung for carbon monoxide; EUSTAR, European League Against Rheumatism Scleroderma Trials and Research; FVC, forced vital capacity; GER, gastroesophageal reflux; HRCT, high-resolution computed tomography; IL-6, interleukin-6; ILD, interstitial lung disease; KL-6, Krebs von den Lungen-6; mRSS, modified Rodnan skin score; PFT, pulmonary function test; scl-70, scleroderma 70 kDa; 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. Epub ahead of print: doi:10.1136/annrheumdis-2020-217455.
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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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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, 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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Winstone T, Assayag D, Wilcox P, et al. Predictors of mortality and progression in scleroderma-associated interstitial lung disease. A systematic review. CHEST. 2014;146:422-436.
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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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Khanna D, Tseng C, Farmani N, et al. Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group. Arthritis Rheum. 2011;63:3078–3085.
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Asano Y, Jinnin M, Kawaguchi Y, et al. Diagnostic criteria, severity classification and guidelines of systemic sclerosis: Guideline of SSc. J Dermatol. 2018;45:633–691.
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Moore OA, Goh N, Corte T, et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology. 2013;52:155–160.
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Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long–term survival in systemic sclerosis. Arthritis Rheumatol. 2014;66: 1625–1635.
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Goh NS, Desai SR., Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;177:1248–1254.
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Maher TM, Mayes MD, Kreuter M, et al. Effect of nintedanib on lung function in patients with systemic sclerosis-associated interstitial lung disease: further analyses of the SENSCIS trial. Arthritis Rheumatol. 2020 Nov 3. doi:10.1002/art.41576. Online ahead of print.
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Volkmann, Elizabeth R, Tashkin DP, et al. Short-term progression of interstitial lung disease in systemic sclerosis predicts long-term survival in two independent clinical trial cohorts. Ann Rheum Dis. 2019a;78:122–130.
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Khanna D, Tashkin DP, Denton CP, et al. Etiology, risk factors, and biomarkers in systemic sclerosis with interstitial lung disease. Am J Respir Crit Care Med. 2020;201:650–660.
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Liu X, Mayes MD, Pedroza, C. et al. Does C-reactive protein predict the long-term progression of interstitial lung disease and survival in patients with early systemic sclerosis? Arthritis Care Res (Hoboken). 2013;65(8):1375–1380.
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De Lauretis A, Sestini P, Pantelidis P, et al. Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis. J Rheumatol. 2013;40(4):435–446.
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Volkmann, Elizabeth R, Tashkin DP, et al. Progression of interstitial lung disease in systemic sclerosis: the importance of pneumoproteins Krebs von den Lungen 6 and CCL18. Arthritis Rheumatol. 2019b;71:2059–2067.
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Wu W, Jordan S, Becker MO, et al. Prediction of progression of interstitial lung disease in patients with systemic sclerosis: the SPAR model. Ann Rheum Dis. 2018;77:1326–1332.
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Hoffmann-Vold A, Aaløkken TM, Lund MB, et al. Predictive Value of Serial High-Resolution Computed Tomography Analyses and Concurrent Lung Function Tests in Systemic Sclerosis. Arthritis Rheumatol. 2015;67:2205–2212.
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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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Chowaniec M, Skoczyńska M, Sokolik R, et al. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56:249–254.
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Wells AU. Interstitial lung disease in systemic sclerosis. La Presse Médicale. 2014;43:e329–e343.
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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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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)