Novel Imaging Strategies in Systemic Sclerosis
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SCLERODERMA (J VARGA, SECTION EDITOR)
Novel Imaging Strategies in Systemic Sclerosis Molly Wolf 1,2 & Sydney B. Montesi 1,2
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Imaging modalities such as computed tomography, ultrasound, magnetic resonance imaging, and molecular imaging are being used to evaluate for disease in systemic sclerosis (SSc) patients. Here, we review novel imaging strategies to detect organ and vascular complications of SSc and novel imaging techniques for assessing interstitial lung disease and pulmonary hypertension in other conditions that may have further applicability to SSc. Recent Findings Imaging techniques can be used to identify disease in the lungs, pulmonary vascular system, heart, skin, vascular tissue, and gastrointestinal tract of SSc patients. These show promise in detecting early disease, many without the use of ionizing radiation. Summary Novel imaging techniques in patients with SSc can be used to detect disease in multiple susceptible organs. These imaging strategies have potential for early disease detection, as well as potential for incorporation into clinical trials to accelerate the development of SSc therapies. Keywords Systemic sclerosis . Scleroderma . Computed tomography . Ultrasound . Magnetic resonance imaging . Molecular imaging
Introduction Systemic sclerosis (SSc), often termed scleroderma, is a chronic heterogenous immune-mediated connective tissue disease which confers a high morbidity and mortality. The hallmarks of SSc include vascular dysfunction and deposition of extracellular collagen, leading to progressive skin and organ fibrosis and in some cases organ failure [1]. Multisystem disease involvement is common including pulmonary (pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD)), cardiac (cardiac fibrosis), skin (sclerosis), vascular (Raynaud’s phenomenon), and gastrointestinal (esophageal dysmotility) manifestations.
This article is part of Topical Collection on Scleroderma * Sydney B. Montesi [email protected] Molly Wolf [email protected] 1
2
SSc is typically divided into two disease phenotypes, limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc), differentiated by patterns of organ involvement. However, there is marked clinical disease heterogeneity and increasing recognition of additional SSc disease phenotypes [2]. The variability in clinical presentation makes diagnosis difficult and often delayed, especially given the rarity of the disease. Once diagnosed, screening and monitoring for organ involvement is challenging, and in many cases, organ involvement cannot be detected until permanent damage has occurred. Lastly, effective treatments for the systemic complications of SSc remain a large unmet need. Here, we will review novel imaging techniques being developed to diagnose and monitor disease activity in SSc, sorted by the organ system involved (Table 1). We will highlight promising imaging strategies for assessment of
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