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Sclerosis, Volume 2, Issue 4 (December 2024) – 2 articles

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12 pages, 2053 KiB  
Review
Insights and Future Perspectives in Calcinosis Cutis Associated with Systemic Sclerosis
by Luna Lazar, Mette Mogensen, Mikael Ploug Boesen and Anne Braae Olesen
Sclerosis 2024, 2(4), 302-313; https://doi.org/10.3390/sclerosis2040019 - 10 Oct 2024
Viewed by 270
Abstract
Introduction: Calcinosis cutis (CC), the pathological deposition of calcium salts in the skin, is a frequent and challenging complication of systemic sclerosis (SSc). Despite its high prevalence, the underlying pathophysiology remains poorly understood, complicating treatment strategies. Material and Methods: This narrative review synthesizes [...] Read more.
Introduction: Calcinosis cutis (CC), the pathological deposition of calcium salts in the skin, is a frequent and challenging complication of systemic sclerosis (SSc). Despite its high prevalence, the underlying pathophysiology remains poorly understood, complicating treatment strategies. Material and Methods: This narrative review synthesizes the literature on CC in the context of SSc. The current understanding and treatment of CC in SSc is reviewed, focusing on the role of hypoxia in its pathogenesis and the therapeutic potential of sodium thiosulfate (STS). Results and Discussion: Research indicates a potential link between hypoxia and the development of CC in SSc, shedding light on novel pathogenic mechanisms. Additionally, promising results from treatments such as STS spurs interest in conducting larger, randomized controlled trials to validate these findings. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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Review
A Narrative Review of Therapeutic Options in Systemic Sclerosis Associated Interstitial Lung Disease
by Robert Harrington, Patricia Harkins and Richard Conway
Sclerosis 2024, 2(4), 288-301; https://doi.org/10.3390/sclerosis2040018 - 30 Sep 2024
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Abstract
Background: Interstitial lung disease (ILD) has replaced scleroderma renal crisis as the leading cause of mortality in systemic sclerosis (SSc), with a 10-year mortality of 40%. There have been well-powered randomised control trials (RCTs) demonstrating the effect of cyclophosphamide (CYC), mycophenolic acid (MMF), [...] Read more.
Background: Interstitial lung disease (ILD) has replaced scleroderma renal crisis as the leading cause of mortality in systemic sclerosis (SSc), with a 10-year mortality of 40%. There have been well-powered randomised control trials (RCTs) demonstrating the effect of cyclophosphamide (CYC), mycophenolic acid (MMF), nintedanib and tocilizumab (TCZ) in SSc-ILD but a paucity of sufficiently powered studies investigating other agents in the disease. Methods: This is a narrative review which examines the existing evidence for immunosuppressive treatments, transplant and adjunctive therapies in SSc-ILD by reviewing the key landmark trials in the last two decades. Results: MMF for 2 years is as effective as oral CYC for 1 year. Rituximab (RTX) is non-inferior to CYC. TCZ appears to have a beneficial effective regardless of the extent of lung involvement. Conclusions: There is now a strong evidence base supporting the use of MMF as the first line option in SSc-ILD. RTX, CYC and TCZ are viable therapeutic options if there is ILD progression on MMF. Anti-fibrotic and pulmonary arterial (PAH) treatments likely add long-term synergistic benefits. There remains a role for lung transplantation in select patients. Full article
(This article belongs to the Special Issue Recent Advances in Understanding Systemic Sclerosis)
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