About this Research Topic
Systemic sclerosis (SSc) is a rare, chronic, debilitating multisystem connective tissue disorder (CTD) characterized by small-vessel vasculopathy and immune system activation leading to progressive fibrosis of the skin and internal organs.
Lung involvement is a major complication of CTDs, particularly in SSc, and is one of the main causes of morbidity and mortality in this condition. Interstitial lung disease (ILD) can be found in high-resolution CT scans in up to 80% of SSc patients, and up to 40% will develop clinically significant manifestations related to ILD.
Heterogeneity and variability in disease course are the hallmarks of SSc-associated ILD (SSc-ILD). Indeed, some patients will present patterns of slow lung function decline with periods of stability, whereas others will present patterns of rapid, continuous lung function decline.
In recent years, as our understanding of the pathogenesis of this heterogeneous disease has grown, innovative and specific therapies have emerged for the treatment of SSc-ILD. These include the antifibrotic drug nintedanib, as well as biologics such as anti-IL6 tocilizumab and anti-CD20 rituximab.
Several prognostic factors linked to severe progressive SSc-ILD have been identified, such as the diffuse cutaneous subset, anti-Scl70 positivity, and elevated acute phase reactants. However, it remains crucial to identify precise biomarkers that can better categorize subgroups of SSc-ILD patients who would benefit most from early and intensive specific interventions. Specifically, prioritizing biomarkers associated with short-term and long-term rapid decline in lung function, as well as those indicating treatment response to targeted therapies, is imperative.
This Research Topic invites researchers to submit Original Research, Review, Clinical Trial, General Commentary, Hypothesis & Theory, Methods, Mini Review, Brief Research Report, Perspective, Study Protocol, and Systematic Review articles that explore novel biomarkers for identifying patients with potentially progressive and severe SSc-ILD, along with investigating the impact of these biomarkers on initiating early intensive therapy. We also encourage manuscripts focusing on the pathophysiology and prognostic factors of SSc-ILD, as well as novel therapeutic agents in both clinical and pre-clinical settings for SSc.
Lung involvement is a major complication of CTDs, particularly in SSc, and is one of the main causes of morbidity and mortality in this condition. Interstitial lung disease (ILD) can be found in high-resolution CT scans in up to 80% of SSc patients, and up to 40% will develop clinically significant manifestations related to ILD.
Heterogeneity and variability in disease course are the hallmarks of SSc-associated ILD (SSc-ILD). Indeed, some patients will present patterns of slow lung function decline with periods of stability, whereas others will present patterns of rapid, continuous lung function decline.
In recent years, as our understanding of the pathogenesis of this heterogeneous disease has grown, innovative and specific therapies have emerged for the treatment of SSc-ILD. These include the antifibrotic drug nintedanib, as well as biologics such as anti-IL6 tocilizumab and anti-CD20 rituximab.
Several prognostic factors linked to severe progressive SSc-ILD have been identified, such as the diffuse cutaneous subset, anti-Scl70 positivity, and elevated acute phase reactants. However, it remains crucial to identify precise biomarkers that can better categorize subgroups of SSc-ILD patients who would benefit most from early and intensive specific interventions. Specifically, prioritizing biomarkers associated with short-term and long-term rapid decline in lung function, as well as those indicating treatment response to targeted therapies, is imperative.
This Research Topic invites researchers to submit Original Research, Review, Clinical Trial, General Commentary, Hypothesis & Theory, Methods, Mini Review, Brief Research Report, Perspective, Study Protocol, and Systematic Review articles that explore novel biomarkers for identifying patients with potentially progressive and severe SSc-ILD, along with investigating the impact of these biomarkers on initiating early intensive therapy. We also encourage manuscripts focusing on the pathophysiology and prognostic factors of SSc-ILD, as well as novel therapeutic agents in both clinical and pre-clinical settings for SSc.
Keywords: Scleroderma, Lung Disease, biomarkers, therapy
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