Novel Therapeutic Strategies in the Treatment of Systemic Sclerosis

Abstract

Systemic sclerosis is a connective tissue disease of unknown origin and with an unpredictable course, with both cutaneous and internal organ manifestations. Despite the enormous progress in rheumatology and clinical immunology, the background of this disease is largely unknown, and no specific therapy exists. The therapeutic approach aims to treat and preserve the function of internal organs, and this approach is commonly referred to as organ-based treatment. However, in modern times, data from other branches of medicine may offer insight into how to treat disease-related complications, making it possible to find new drugs to treat this disease. In this review, we present therapeutic options aiming to stop the progression of fibrotic processes, restore the aberrant immune response, stop improper signalling from proinflammatory cytokines, and halt the production of disease-related autoantibodies.

Immunogenetics of Systemic Sclerosis

“…The discovery of the non-receptor tyrosine kinase family (Janus kinases-JAK), a group of intracellular enzymes essential for the proper functioning and activation of class I and class II receptor families translated to the discovery of Janus kinase inhibitors, a group of drugs that have demonstrated their safety and efficacy in several diseases with an autoimmune background. Apart from their well-known efficacy in rheumatoid arthritis [68], their position in the treatment of several autoimmune diseases, including systemic sclerosis, has been intensively tested [69,70].…”

Multidisciplinary Aproach: A Goal For The Management Of Complications In Systemic Scleroderma. Literature Review And Case Scenario

“…Systemic sclerosis (also known as scleroderma) is a chronic fibrosing autoimmune disease with both skin and multisystem organ involvement [1,2]. Scleroderma has the highest mortality among all rheumatic diseases [3].…”

“…Fibrosis of internal organs is a hint for systemic sclerosis, moreover associated with interstitial lung disease (SSc-ILD) is a complex process involving inflammation, alveolar epithelial damage, and activation of resident fibroblasts resulting in thickening of the lung interstitium [2,4,5].…”

A Multidisciplinary Approach as a Goal for the Management of Complications in Systemic Scleroderma: A Literature Review and Case Scenario

“…Systemic sclerosis (also known as scleroderma) is a rare chronic fibrosing autoimmune disease with both skin and multisystem organ involvement [1,2]. Scleroderma has the highest mortality among all rheumatic diseases [3].…”

“…Fibrosis of the internal organs is a hint of systemic sclerosis, and when also associated with interstitial lung disease (SSc-ILD), it is a complex process involving inflammation, alveolar epithelial damage, and activation of resident fibroblasts, resulting in the thickening of the lung interstitium [2,4,5]. In this paper, we have conducted a case-based literature review of pulmonary fibrosis related to systemic scleroderma, because the increased risk of developing pulmonary fibrosis and pulmonary artery hypertension is among the leading causes of SSc-related deaths.…”