Recent innovations in the screening and diagnosis of systemic sclerosis-associated interstitial lung disease

ABSTRACT

Introduction

Interstitial lung disease (ILD) is the leading cause of mortality in patients with systemic sclerosis (SSc). Risk of developing progressive ILD is highest among patients with diffuse cutaneous disease, positive anti-topoisomerase I antibody, and elevated acute phase reactants. With the FDA approval of two medications and a pipeline of novel therapeutics in trials, early recognition and intervention is critical. High-resolution computed tomography of the chest is the current gold standard test for diagnosis of ILD. Yet, it is not offered as a screening tool to all patients due to which ILD can be missed in up to a third of patients. There is a need to develop and validate more innovative screening modalities.

Areas covered

In this review, we provide an overview of screening and diagnosis of SSc-ILD, highlighting the recent innovations particularly the role of soluble serologic, radiomic (quantitative lung imaging, lung ultrasound), and breathomic (exhaled breath analysis) biomarkers in the early detection of SSc-ILD.

Expert opinion

There is remarkable progress in the development of new radiomics and serum biomarkers in diagnosing SSc-ILD. There is an urgent need for conceptualizing and testing composite ILD screening strategies that incorporate these biomarkers.

KEYWORDS:

• Systemic sclerosis
• interstitial lung disease
• screening
• diagnosis
• HRCT
• biomarkers
• lung ultrasound
• oscillometry

Article highlights

• Interstitial lung disease (ILD) is highly prevalent in patients with systemic sclerosis (SSc) and early screening is essential.

• Despite the availability of high-resolution CT-scans of the chest universal baseline screening is not performed routinely.

• Recent interests and innovations in different radiologic evaluation modalities expand the diagnostic options for screening.

• Computer-aided image processing algorithms allow consistency in quantifying aspects of ILD.

• An expanding repertoire of serum or blood-based biomarkers link to different dimensions of the ILD.

Declaration of interest

A Makol reports consultancy fees from Boehringer Ingelheim and Sanofi. V Nagaraja reports consultancy fees from Boehringer Ingelheim. D Khanna reports grant support from Bayer, BMS, Horizon, Immune Tolerance Network, NIH, and Pfizer consultancy fees from AbbVie, Acceleron, Actelion, Amgen, Bayer, Chemomab, Boehringer Ingelheim, CSL Behring, Genentech/Roche, Horizon, Merck, Mitsubishi Tanabe Pharma. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

D Khanna was supported by NIAMS K24 ARAR063120.