The Role of Lung Ultrasound in Early Diagnosis of Systemic Sclerosis-Related Interstitial Lung Disease
Abstract
Interstitial lung disease (ILD) is a major extra-articular manifestation of systemic sclerosis (SSc), significantly contributing to morbidity and mortality. Early detection and close monitoring of patients at high risk of progression are critical for establishing the need for targeted treatment with immunomodulatory and antifibrotic drugs that have the potential to alter the course of the disease. Although the predictive value of high-resolution computed tomography (HRCT) is undeniable, it increases the risk of cancer in SSc-ILD patients with a propensity for developing cancer due to medication overuse and radiation exposure. Lung ultrasonography (LUS) is an intriguing alternative technology in this context, providing a non-invasive and non-radiating evaluation that can be performed prior to other imaging techniques, such as a CT scan, to detect interstitial abnormalities within the subpleural space. Vertical artifacts such as B lines and pleural line modifications have a strong link with the presence of ILD on HRCT and the extent and severity of the disease, with sensitivity and negative predictive values of up to 100%. In the past decades, LUS has been shown to be a cost-effective and repeatable procedure when combined with pulmonary function tests as a primary assessment, limiting the frequency of HRCT application and minimizing radiation exposure that contributes to the probability of cancer development and progression. In this review, we have provided an etiological background for pulmonary involvement in SSc, screening modalities for ILD in SSc, and a thorough description of LUS findings and scoring systems as a point-of-care diagnostic tool in SSc-ILD.
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6. Rongioletti F, Ferreli C, Atzori L, et al. Scleroderma with an update about clinico-pathological correlation. G Ital Dermatol Venereol. 2018;153(2):208–15.
7. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685–99.
8. Perelas A, Arrossi AV, Highland KB. Pulmonary manifestations of systemic sclerosis and mixed connective tissue disease. Clin Chest Med. 2019;40(3):501–18.
9. Chung JH, Walker CM, Hobbs S. Imaging features of systemic sclerosis-associated interstitial lung disease. J Vis Exp. 2020;(1):1–21.
10. Bastos AL, Corrêa RA, Ferreira GA. Tomography patterns of lung disease in systemic sclerosis. Radiol Bras. 2016;49(5):316–21.
11. Fan Y, Bender S, Shi W, et al. Incidence and prevalence of systemic sclerosis and systemic sclerosis with interstitial lung disease in the United States. 2020;26:1–9.
12. Bairkdar M, Rossides M, Westerlind H, et al. Incidence and prevalence of systemic sclerosis globally: A comprehensive systematic review and meta-analysis. Rheumatology (Oxford). 2021;1–13.
13. Spagnolo P, Distler O, Ryerson CJ, et al. Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs). Ann Rheum Dis. 2021;80(2):143–50.
14. Calle Botero E, Abril A. Interstitial lung involvement in systemic sclerosis. Rev Colomb Reumatol. 2020;27(1):36–43.
15. Mackintosh JA, Stainer A, Barnett JL, et al. Systemic sclerosis associated interstitial lung disease: A comprehensive overview. Semin Respir Crit Care Med. 2019;40(2):208–26.
16. Fischer A, Patel NM, Volkmann ER. Interstitial lung disease in systemic sclerosis: Focus on early detection and intervention. 2019;283–307.
17. Doskaliuk B, Zaiats L, Yatsyshyn R, et al. Pulmonary involvement in systemic sclerosis: Exploring cellular, genetic and epigenetic mechanisms. Rheumatol Int. 2020.
18. Pattanaik D, Brown M, Postlethwaite BC, et al. Pathogenesis of systemic sclerosis. Front Immunol. 2015;6:.
19. De Luca G, Bosello SL, Berardi G, et al. Tumour-associated antigens in systemic sclerosis patients with interstitial lung disease: Association with lung involvement and cancer risk. Rheumatology (Oxford). 2015;54(11):1991–9.
20. Volkmann ER. Natural history of systemic sclerosis–related interstitial lung disease: How to identify a progressive fibrosing phenotype. 2019.
21. Rubio-Rivas M, Homs NA, Cuartero D, et al. The prevalence and incidence rate of pulmonary arterial hypertension in systemic sclerosis: Systematic review and meta-analysis. Autoimmun Rev. 2020;19(12):102713.
22. Tsai C, Hsieh S, Wu T, et al. Pathogenic roles of autoantibodies and aberrant epigenetic regulation of immune and connective tissue cells in the tissue fibrosis of patients with systemic sclerosis. 1–25.
23. Mirsaeidi M, Barletta P, Glassberg MK. Systemic sclerosis associated interstitial lung disease: New directions in disease management. 2019;6(4):4–13.
24. Young A, Vummidi D, Visovatti S, et al. Prevalence, treatment, and outcomes of coexistent pulmonary hypertension and interstitial lung disease in systemic sclerosis. 2019;71(8):1339–49.
25. Bruni C, De Luca G, Lazzaroni M, et al. Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review. Eur J Intern Med. 2020;0–1.
26. Jiang Y, Turk MA, Pope JE, et al. Factors associated with pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). Autoimmun Rev. 2020;19(4):102602.
27. Scallan CJ, Collins BF, Raghu G. Systemic sclerosis-associated interstitial lung disease. Chest. 2020;157(2):487–95.
28. Perelas A, Silver RM, Arrossi AV, et al. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8(3):304–20.
29. Volkmann ER, Fischer A. Update on morbidity and mortality in systemic sclerosis–related interstitial lung disease. J Scleroderma Relat Disord. 2020;5(2):90–105.
30. Bergamasco A, Hartmann N, Wallace L, et al. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol. 2019;11:257–73.
31. Disease IL. HHS Public Access. 2021;6(1):11–20.
32. Elhai M, Meune C, Boubaya M, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis. 2017;76(11):1897–905.
33. Hunninghake GM, Goldin JG, Kadoch MA, et al. Detection and early referral of patients with interstitial lung abnormalities: An expert survey initiative. Chest. 2022;161(2):470–82.
34. Becker M, Graf N, Sauter R, et al. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: A European Scleroderma Trials and Research (EUSTAR) analysis. Rheumatology (Oxford). 2019;58(7):1242–8.
35. 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(10):1009–17.
36. Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020;55(3):2000810.
37. 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. 2021;80(2):219–27.
38. Picano E, Semelka R, Ravenel J, et al. Rheumatological diseases and cancer: The hidden variable of radiation exposure. Ann Rheum Dis. 2014;73(11):2065–8.
39. Peng H, Wu X, Wen Y, et al. Association between systemic sclerosis and risk of lung cancer: Results from a pool of cohort studies and Mendelian randomization analysis. Autoimmun Rev. 2020;19(11):102633.
40. Partl R, Regitnig P, Lukasiak K, et al. Incidence of morphea following adjuvant irradiation of the breast in 2,268 patients. Breast Care (Basel). 2020;15(3):246–52.
41. Bennardo L, Passante M, Cameli N, et al. Skin manifestations after ionizing radiation exposure: A systematic review. Bioengineering (Basel). 2021;8(7):1–13.
42. Maria ATJ, Partouche L, Goulabchand R, et al. Intriguing relationships between cancer and systemic sclerosis: Role of the immune system and other contributors. Front Immunol. 2019;10:1–14.
43. 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. 2021;80(2):219–27.
44. Roofeh D, Jaafar S, Vummidi D, et al. HHS Public Access. 2020;31(3):241–9.
45. Vanaken L, Landini N, Lenaerts J, et al. Progressive lung fibrosis and mortality can occur in early systemic sclerosis patients without pulmonary abnormalities at baseline assessment. Clin Rheumatol. 2020;39(10):3393–400.
46. Mecoli CA, Adler BL, Yang Q, et al. Cancer in systemic sclerosis: Analysis of antibodies against components of the Th/To complex. Arthritis Rheumatol. 2021;73(2):315–23.
47. Gigante A, Rossi Fanelli F, Lucci S, et al. Lung ultrasound in systemic sclerosis: Correlation with high-resolution computed tomography, pulmonary function tests and clinical variables of disease. Intern Emerg Med. 2016;11(2):213–7.
48. Ananyeva LP. Interstitial lung disease associated with systemic sclerosis. Nauchno-Prakt Revmatol. 2017;55(1):87–95.
49. Ferro F, Sedie AD, Sedie AD. The use of ultrasound for assessing interstitial lung involvement in connective tissue diseases. Rheumatology (Oxford). 2018;57(2):165–70.
50. Allanore Y, Bendstrup E, Bruni C, et al. The need for a holistic approach for SSc-ILD – achievements and ambiguity in a devastating disease. Rheumatology (Oxford). 2020;59(0):1–13.
51. Distler O, Assassi S, Cottin V, et al. Predictors of progression in systemic sclerosis patients with interstitial lung disease. Eur Respir J. 2020;55(3):2000810.
52. Kaenmuang P, Navasakulpong A. Short-term lung function changes and predictors of progressive systemic sclerosis-related interstitial lung disease. Clin Respir J. 2020;14(4):312–20.
53. Hoffmann-Vold A, Molberg Ø. Detection, screening, and classification of interstitial lung disease in patients with systemic sclerosis. Curr Opin Rheumatol. 2020;32(6):497–504.
54. Ferrazza AM, Gigante A, Gasperini ML, et al. Assessment of interstitial lung disease in systemic sclerosis using the quantitative CT algorithm CALIPER. Clin Exp Rheumatol. 2020;38(Suppl 125):123–9.
55. Chung JH, Goldin JG. Interpretation of HRCT scans in the diagnosis of IPF: improving communication between pulmonologists and radiologists. Lung. 2018;196(5):561–7.
56. Allanore Y, Elhai M. Circulating lung biomarkers in idiopathic pulmonary fibrosis and interstitial lung diseases associated with connective tissue diseases: where do we stand? Curr Opin Rheumatol. 2020;32(5):480–91.
57. Castelino FV. Detection and management of interstitial lung diseases associated with connective tissue diseases. Rheumatology (Oxford). 2021;60(0):1–10.
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| Files | ||
| Issue | Vol 8, No 2 (2025) | |
| Section | Review Article | |
| DOI | https://doi.org/10.18502/igj.v8i2.18001 | |
| Keywords | ||
| Interstitial Lung Disease High-Resolution Computed Tomography Lung Ultrasound Systemic Sclerosis Scleroderma | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Hosseini N, Pourriyahi H, Asnnaashari K. The Role of Lung Ultrasound in Early Diagnosis of Systemic Sclerosis-Related Interstitial Lung Disease. Immunol Genet J. 2025;8(2):213-225.
