Diagnosis of SSc-ILD

High-resolution computed tomography (HRCT) and multidisciplinary care ensure the most accurate diagnosis and assessment of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc)1–3

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  • Baseline screening with HRCT, as part of a wider clinical work-up that includes lung function testing, is necessary for an SSc-ILD diagnosis5

Find out more about how to diagnose SSc-ILD using HRCT

Despite the established utility of PFTs in assessing the progression of ILD in patients with SSc, these tests should not be used in isolation to diagnose as they may not be sensitive enough to detect ILD in a patient with SSc5,6

Staging system for limited vs. extensive lung disease in patients with SSc-ILD7
Staging symptom classifies extensive lung disease as extent of fibrosis being greater than 30% on HRCT

Staging system to stratify patients based on HRCT extent of disease, with FVC% as a recourse in cases where the extent of disease in indeterminate on HRCT.
Reprinted from Annals of the American Thoracic Society, 177(11), Goh et al, Interstitial Lung Disease in Systemic Sclerosis A Simple Staging System, 1248–1254, ©2022.

Discover more about screening, diagnosis and management of SSc-ILD in the clinical management algorithm



Patients may be diagnosed early and more efficiently through multidisciplinary evaluation and collaboration.1

Example of multidisciplinary team for SSc-ILD diagnosis

In instances when an MDT is challenging to convene for a patient with ILD, multidisciplinary discussion can be conducted virtually or by telephone to enable each specialty group to contribute.8

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


Fibrosis may have already progressed to ≥10% of your SSc patient’s lung volume at the moment of diagnosis of their ILD9

Fibrotic ILD affecting ≥10% of lung volume on HRCT is associated with early mortality in patients with SSc9

Detect ILD early in patients with SSc by using HRCT1,5

Download SSc-ILD patient case

Stephanie has SSc-ILD

How can you reach an SSc-ILD diagnosis?

Download SSc-ILD patient case

How can you break the news of an ILD diagnosis to your patients with SSc-ILD?

Breaking bad news is a complex communication task. The SPIKES model involves delivering bad news in clinical practice using six elements 

Once you have reached a diagnosis, how should you manage your patients with SSc-ILD?
  • CTD-ILD, connective tissue disease-associated interstitial lung disease; FVC, forced vital capacity; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; MDT, multidisciplinary team; PFT, pulmonary function test; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease. 

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

  2. Frauenfelder T, Winklehner A, Nguyen TDL, et al. Screening for interstitial lung disease in systemic sclerosis: performance of high-resolution CT with limited number of slices: a prospective study. Ann Rheum Dis. 2014;73:2069–2073.

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

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. Chung JH, Goldin JG. Interpretation of HRCT Scans in the Diagnosis of IPF: Improving Communication Between Pulmonologists and Radiologists. Lung. 2018;196:561–567. 

  9. 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.

  10. 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.

  11. 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.

  12. 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. 

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

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

  15. 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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