Post on 05-Oct-2020
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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 patients7
However, only two-thirds of SSc specialists routinely perform chest
HRCT at SSc diagnosis10
PFTs include:8
• Forced vital capacity (FVC)• Diffusing capacity of the lung for carbon
monoxide (DLCO)
Both FVC and DLCO are predictors of mortality in pulmonary � brosis 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 OUTSET8
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 test1,10
Due to the variable and unpredictable nature of pulmonary � brosis, vigilant
ongoing monitoring should be conducted, with HRCT repeated upon worsening of either PFT or respiratory symptoms1,8,10
The absence of pulmonary symptoms does not exclude lung � brosis in patients with normal FVC, as 53% of patients with normal FVC values showed signi� cant ILD on HRCT11
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 mortality1
ILD appears early in the course of SSc6
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 deaths3-5
PFTHRCT
Screening with HRCT for the presence of ILD is recommended at baseline for all patients with a diagnosis of SSc1,8,9
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 YEARSOF DIAGNOSIS7