THE IDENTIFICATION OF SSc-ILD REQUIRES A HIGH LEVEL OF SUSPICION AS NOT ALL PATIENTS DISPLAY RESPIRATORY SYMPTOMS6
The most common initial symptoms of SSc-ILD are fatigue, exertional dyspnea, and dry cough, although early SSc-ILD is often asymptomatic.7 Considering only the presence or severity of respiratory symptoms e.g. exertional dyspnea in defining the prevalence of lung involvement, can result in missed diagnoses of a large minority of SSc-ILD patients with mild interstitial disease.8 SSc patients with ‘severe’ lung disease often develop pulmonary symptoms only after FVC is significantly reduced even if FVC is abnormal at baseline and/or decreasing over time, regardless of whether they are symptomatic.9
Physical examination typically reveals Velcro-like crackles on lung auscultation in addition to the cutaneous findings associated with SSc.10
Most common signs and symptoms of SSc-ILD*
Symptoms of SSc-ILD
Signs of SSc-ILD
* Early SSc-ILD can be asymptomatic.13,15
PATIENTS WITH SSc SHOULD UNDERGO BASELINE LUNG HRCT SCREENING FOR ILD1,2,5
Patients with SSc should receive a thorough clinical assessment including chest imaging with HRCT and PFTs, DLCO and lung auscultation to ensure early identification of ILD and provide baseline measurements to compare with future assessments.1,4,6,13
Screening and diagnosis algorithm for SSc-ILD1
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.
Adapted from Hoffmann-Vold AM, et al. Lancet Rheum.2020;2:e71-e83.
PFTs as the sole screening method for SSc-ILD may cause clinicians to miss a significant number of patients, given the high rate of false-negative results.9,13 PFT as a screening method is limited, by the wide normal ranges of the PFT (e.g. 80–120% of the mean value recorded in age-matched and sex-matched reference populations) which can make it difficult to interpret screening data.14
Pulmonary fibrosis is the leading cause of mortality in SSc, accounting for ~35% of SSc-related deaths15,16
Causes of SSc-related deaths (1997–2001).
Adabted from: Steen VD and Medsger TA. Ann Rheum Dts. 2007;66:940-944 and Tyndall AJ. et al. Ann Rheum Dts. 2010:69(10):1809-1815
Stephanie has SSc-ILD
How can you reach an SSc-ILD diagnosis?
Once you have reached a diagnosis, how should you manage your patients with SSc-ILD?
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; PFT, pulmonary function test; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.
- 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.
- 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.
- Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390;1685–1699.
- Roofeh D, Jaafar S, Vummidi D, et al. Management of systemic sclerosis-associated interstitial lung disease. Curr Opin Rheumatol. 2019;31;241–249.
- 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.
- Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir Res. 2019;20:13.
- Volkmann ER, Tashkin DP. Treatment of systemic sclerosis–related interstitial lung disease: a review of existing and emerging therapies. Annals ATS. 2016;13:2045–2056.
- Wells AU. Interstitial lung disease in systemic sclerosis. La Presse Médicale. 2014;43:e329–e343.
- 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.
- Perelas A, Silver RM, Arrossi AV, et al. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8;304–320.
- Herzog EL, Mathur A, Tager AM, et al. Review: interstitial lung disease associated with systemic sclerosis and idiopathic pulmonary fibrosis: How similar and distinct?: SSc-Associated ILD and IPF. Arthritis Rheumatol. 2014;66;1967–1978.
- 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.
- 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.
- Molberg Ø, Hoffmann-Vold AM. Interstitial lung disease in systemic sclerosis: progress in screening and early diagnosis. Curr Opin Rheumatol. 2016;28:613–618.
- Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66:940–944.
- Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–1815.
- 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.
- 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.
- 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.