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DIAGNOSIS AND MONITORING OF SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE (SSc-ILD) Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc) 1 53% of those with DIFFUSE CUTANEOUS SSc (n=1349) 35% of patients with LIMITED CUTANEOUS SSc (n=2101) REFERENCES 1. Cottin V, Brown KK. Respir Res. 2019;20(1):13. 2. Walker UA, et al. Ann Rheum Dis. 2007;66:754-763. 3. Winstone TA, et al. Chest. 2014;146(2):422-436. 4. Tyndall AJ, et al. Ann Rheum Dis. 2010;69(10):1809-1815. 5. Steen VD, Medsger TA. Ann Rheum Dis. 2007;66:940-944. 6. Jaeger VK, et al. PLoS ONE. 2016;11(10): e0163894. 7. Solomon JJ, et al. Eur Respir Rev. 2013;22(127):6-19. 8. Chowaniec M, et al. Reumatologia. 2018;56(4):249-254. 9. Asano Y, et al. J Dermatol. 2018;45:633-691. 10. Roofeh D, et al. Curr Opin Rheumatol. 2019;31(3):241-249. 11. Suliman YA, et al. Arthritis Rheumatol. 2015;67(12):3256-3261. dcSSc, diffuse cutaneous systemic sclerosis; DLco, diffusing capacity of the lungs for carbon monoxide; EUSTAR MEDS, European Scleroderma Trials and Research group Minimal Essential Data Set; FVC, forced vital capapcity; HCP, healthcare professional; HRCT, high resolution computed tomography; ILD, interstitial lung disease; lcSSc, limited cutaneous systemic sclerosis; PFT, pulmonary function test; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease. © Boehringer Ingelheim International GmbH. All rights reserved. August 2019 PC-CRP-100899 The gold-standard diagnostic tool for ILD. 8 HRCT detects interstitial changes in up to 90% of patients 7 However, only two-thirds of SSc specialists routinely perform chest HRCT at SSc diagnosis 10 PFTs include: 8 • Forced vital capacity (FVC) • Diffusing capacity of the lung for carbon monoxide (DL CO ) Both FVC and DL CO are predictors of mortality in pulmonary fibrosis but shouldn’t be used as the sole screening method given the high false-negative rate (62.5% for FVC alone) 8,11 SCREEN EARLY AND REGULARLY TO DETECT SSc-ILD FROM ITS OUTSET 8 Screening should involve a careful review of symptoms, physical examination, complete PFTs with lung volumes, and lung imaging with HRCT (with prone images), and may include functional measures like the 6-minute walk test 1,10 Due to the variable and unpredictable nature of pulmonary fibrosis, vigilant ongoing monitoring should be conducted, with HRCT repeated upon worsening of either PFT or respiratory symptoms 1,8,10 The absence of pulmonary symptoms does not exclude lung fibrosis in patients with normal FVC, as 53% of patients with normal FVC values showed significant ILD on HRCT 11 VIGILANCE AND PROACTIVITY ARE CRITICAL Identifying pulmonary fibrosis in your patients as early as possible may help to reduce their burden of disease, and the risk of early mortality 1 ILD appears early in the course of SSc 6 In the EUSTAR MEDS database, ILD was found in: 2 ILD is the leading cause of mortality in SSc, responsible for ~35% of SSc-related deaths 3-5 PFT HRCT Screening with HRCT for the presence of ILD is recommended at baseline for all patients with a diagnosis of SSc 1,8,9 PULMONARY FIBROSIS GASTROINTESTINAL HEART MULTI-ORGAN OTHER RENAL CRISIS PULMONARY ARTERIAL HYPERTENSION 35% For most patients with SSc, pulmonary involvement is often evident WITHIN 3 YEARS OF DIAGNOSIS 7
