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

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  • Male5

  • Older age6

Disease duration
  • Shorter duration7,8

Disease extent
  • >20% on HRCT9

SSc subtype
  • Low baseline FVC1


  • FVC <70% predicted11

  • Declines in FVC of >10% predicted2,12,13

  • Low DLCO <55% predicted10

  • Anti-topoisomerase 1 (and absence of anti-centromere antibody)10,14

  • Elevated C-reactive protein (CRP) level15

  • Elevated IL-6 level16

  • Elevated IL-KL level17

  • Elevated IL-CCL-18 level17

Skin involvement
Reflux/dysphagia symptoms
  • GER1

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



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

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.

The highly variable course of SSc-ILD requires ongoing, regular monitoring to ensure early intervention against ILD progression1–4

All patients with SSc-ILD

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

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

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


Monitoring algorithm for SSc-ILD3
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.

The evaluation of the severity of SSc-ILD should be multidisciplinary with the integration of PFTs, symptoms and HRCT findings22

What management options should you consider for your patients with SSc-ILD?
  • 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. 

  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. Epub ahead of print: doi:10.1136/annrheumdis-2020-217455.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  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. 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. 

  8. 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.

  9. 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.

  10. 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.

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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.

  17. 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.

  18. 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. 

  19. 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.

  20. 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.

  21. 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.

  22. Wells AU. Interstitial lung disease in systemic sclerosis. La Presse Médicale. 2014;43:e329–e343.

  23. 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.

  24. 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. 

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

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

  27. 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. 

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

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