Abstract
Systemic sclerosis (SSc) is a rare autoimmune disease, characterized by vasculopathy and fibrosis of the skin and internal organs. This disease is still considered incurable and is associated with a high risk of mortality, which is related to fibrotic events. An early diagnosis is useful for preventing complications, and targeted therapies reduce disease progression and ameliorate patients’ quality of life. Nevertheless, there are no validated biomarkers for early diagnosis with predictive prognostic value. Exosomes are membrane vesicles, transporting proteins and nucleic acids that may be delivered to target cells, which influences cellular behavior. They play important roles in cell–cell communication, both in physiological and pathological conditions, and may be useful as circulating biomarkers. Recent evidences suggest a role for these microvesicles in the three main aspects related to the pathogenesis of SSc (immunity, vascular damage, and fibrosis). Moreover, exosomes are of particular interest in the field of nano-delivery and are used as biological carriers. In this review, we report the latest information concerning SSc pathogenesis, clinical aspects of SSc, and current approaches to the treatment of SSc. Furthermore, we indicate a possible role of exosomes in SSc pathogenesis and suggest their potential use as diagnostic and prognostic biomarkers, as well as therapeutic tools.
Highlights
Systemic sclerosis (SSc) is a rare systemic autoimmune disease with an unknown etiology, and it is characterized by a progressive fibrotic process that affects the skin, microvasculature, and numerous internal organs
We indicate a possible role of exosomes in SSc pathogenesis and suggest their potential use as diagnostic and prognostic biomarkers, as well as therapeutic tools
In SSc patients, lung disease is significantly heterogeneous. It is possible identify two major pathological processes, as follows: The vasculopathy of medium and small pulmonary vessels, which cause pulmonary arterial hypertension (PAH) and interstitial fibrosis [2]. It seems that an inflammatory phase, named alveolitis, precedes lung fibrosis, which can start as unusual interstitial pneumoniae (NSIP), considered a potential reversible or stoppable phase, and evolve into usual interstitial pneumoniae (UIP), with an irreversible honeycombing-like appearance, leading to restrictive lung syndrome and end-stage lung disease [70]
Summary
Systemic sclerosis (SSc) is a rare systemic autoimmune disease with an unknown etiology, and it is characterized by a progressive fibrotic process that affects the skin, microvasculature, and numerous internal organs. Despite intensive investigations aimed at the identification of outcome measures for SSc, no validated biomarkers exist for the early diagnosis and assessment of SSc disease activity with a predictive prognostic value. In this context, the discovery of biomarkers that identify patient subgroups is of great scientific interest. A large body of evidence suggests that miRNAs and proteins are selectively packaged in exosomes that differ in terms of their physiological and pathological conditions These vesicles are studied as potential biomarkers for diagnostic purposes, monitoring the disease evolution and treatment responses. We highlight a potential pathogenic role of exosomes in SSc and their possible use for specific drug delivery
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