Detection and classification of systemic sclerosis-related interstitial lung disease: a review

Abstract

Purpose of review Systemic sclerosis (SSc) is a heterogeneous disease with a variable disease course. Interstitial lung disease (ILD) is one of the leading causes of morbidity and mortality in patients with SSc. The present review highlights recent advances in the classification, diagnosis, and early detection of SSc-associated ILD (SSc-ILD). Recent findings Risk stratification through measurement of disease extent on high-resolution computed tomography (HRCT) of the chest, longitudinal declines in pulmonary function tests (PFTs), and mortality prediction models have formed the basis for classifying clinically significant ILD. HRCT may be preferred over PFTs for screening, as PFTs lack sensitivity and have a high false-negative rate. Novel imaging modalities and biomarkers hold promise as adjunct methods for assessing the presence and severity of SSc-ILD, and predicting risk for progressive disease. Further validation is required prior to their use in clinical settings. Summary Classification of SSc-ILD has shifted to a personalized approach that considers an individual patient's probability of progressive disease through identification of risk factors, measurement of disease extent on HRCT, longitudinal declines in PFTs, and mortality prediction models. There remains an unmet need to develop screening guidelines for SSc-ILD.

High-Resolution Computed Tomography: Lights and Shadows in Improving Care for SSc-ILD Patients

“…Moreover, the SSc fibroblasts also acquire the ability to selectively respond to pro-fibrotic cytokines and growth factors, persistently producing excessive amounts of extracellular matrix. Thus, various types of individually activated cells interact with one other and co-ordinately cause damage at the levels of the different organs, such as in the lung [8][9][10]. In this scenario, although major advances have been made in the identification and prognostic evaluation of SSc-ILD, the pathogenesis of interstitial lung disease (ILD) in systemic sclerosis (SSc-ILD) remains an open question and accurate epidemiological risk factors are still scanty [1][2][3][4].…”

“…However, there is a high prevalence of lung involvement in SSc early diagnosis, making for the necessity of a tailored approach which takes into consideration the patient's probability of progressive disease, due to its considerable impact on prognosis [2,3]. Indeed, despite recent advances in treatment, 40-75% of SSc patients have reduced pulmonary function and ILD, which is usually observed within the first 3 years from diagnosis and is currently the major cause of death in SSc patients (70-90%) [3][4][5][6][7][8][9][10]. The European Scleroderma Trials and Research group (EUSTAR) studied 3,656 SSc patients, reporting that 53% of diffuse cutaneous SSc cases had ILD, as did 35% of cases with limited cutaneous SSc [11][12][13].…”

High-Resolution Computed Tomography and Lung Ultrasound in Patients with Systemic Sclerosis: Which One to Choose?

“…Systemic sclerosis (SSc) is an autoimmune disease that affects multiple organ systems, including the lungs [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 ]. Interstitial lung disease (ILD) is a common manifestation in systemic sclerosis (SSc) and, despite continuous advances in treatment, remains the major cause of death in SSc patients [ 1 , 2 , 3 , 4 ].…”

“…Interstitial lung disease (ILD) is a common manifestation in systemic sclerosis (SSc) and, despite continuous advances in treatment, remains the major cause of death in SSc patients [ 1 , 2 , 3 , 4 ]. It is of utmost importance to detect ILD in its very early stages so as to be able to choose the correct treatment regimen [ 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 ]. Imaging plays a central role in the management of systemic sclerosis-associated interstitial lung disease (SSc-ILD), and chest high-resolution computed tomography (HRCT) is currently considered the ‘gold standard’ for establishing an ILD diagnosis [ 14 , 15 , 16 , 17 , 18 ].…”

“…However, HRCT is unsuitable for frequent screening of the lung parenchymal modifications due to high costs and the risks involved in ionizing radiation (particularly in young women with an early SSc diagnosis) [ 14 , 15 , 16 , 17 , 18 ]. Several authors have recently proposed lung ultrasound (LUS) as an imaging technique for the assessment of SSc-ILD even in its early stages, as LUS has a very high negative predictive value [ 11 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ]. Moreover, scoring systems have been proposed to quantify the severity of SSc-ILD, and recent research reports the predictive value of LUS [ 11 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ].…”