Evaluation of serum concentrations of the selected cytokines in patients with localized scleroderma

Abstract

A b s t r a c t Introduction: Localized scleroderma is an autoimmune disease primarily affecting the skin. The cause of disease remains unexplained although environmental factors are implicated, which are likely to be responsible for activation of the endothelium and subsequent inflammation leading to excessive synthesis of collagen and extracellular matrix components. Aim: To determine concentrations of interleukin (IL)-27, transforming growth factor (TGF)-β1, TGF-β2, IL-6, and sIL-6R in patients with localized scleroderma compared to controls and to assess the relations between their levels and laboratory markers. Material and methods: The study encompassed 17 females with localized scleroderma (aged 25-67). The control group consisted of 30 age-matched healthy women. The blood was sampled from the basilic vein. Serum levels of cytokines were determined using ELISA. Results: The TGF-β2 levels were found to be significantly lower in patients with localized scleroderma compared to controls. Concentrations of TGF-β1 were decreased in scleroderma patients when compared to controls but without statistical significance. There were no significant differences in serum IL-6, sIL-6R and IL-27 levels between patients and the control group; however, we found a significant positive correlation between the level of sIL-6 and ESR among subjects with localized scleroderma. Conclusions: The findings of decreased serum levels of TGF-β1 and TGF-β2 in patients with localized scleroderma demonstrate a possible association of these cytokines with pathogenesis of the disease. The results suggest also that sIL-6R is likely to be involved in inflammation in patients with localized scleroderma.

Increased expression of latent TGF-β-binding protein 4 affects the fibrotic process in scleroderma by TGF-β/SMAD signaling

“…1 Although LSc and SSc have a number of different disease presentations, both share the common clinical hallmark of skin fibrosis characterized by excessive transforming growth factor-β (TGF-β) activity and extracellular matrix (ECM) production. 2,3 However, underlying mechanisms of this phenomenon are still unclear. 4 Several pro-fibrotic cytokines have been implicated in fibrotic process of scleroderma, including TGF-β, connective tissue growth factor (CTGF), plasminogen activator inhibitor-1 (PAI-1), fibronectin 1 (FN-1), and others.…”

Juvenile Localized Scleroderma with Hyaline Deposits in the Renal Arteriole

“…The underlying pathogenesis of JLS remains unknown, but upregulation of transforming growth factor-β (TGF-β) production is thought to play a prominent role in excessive collagen deposition [ 8 ]. Previous studies gave variable results as to the serum levels of TGF-β in patients with LS [ 9 ], but highly elevated serum levels were reported in children with linear and generalized types of LS [ 10 ]. Furthermore, a recent report demonstrated that ectopic TGF-β induced vascular endothelial growth factor (VEGF) synthesis in normal fibroblasts, and that overexpression of VEGF was seen in activated systemic sclerosis fibroblasts which acquired autocrine TGF-β signaling following chronic inflammation [ 11 ].…”

The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review

“…Less often the process affects deeper tissues (fascia, muscles and bones), leading to deep atrophy and deformations. The excessive synthesis of collagen type I and III and extracellular components is regarded to play the key role in the formation of characteristic skin lesions [2].…”

“…The genetic and environmental factors, as well as microchimerism, are considered to activate keratinocytes to release inflammatory mediators (interleukin-4 (IL-4), IL-6, IL-10, IL-17A, IL-27, interferon-c), which subsequently stimulate lymphocytes, endothelial cells and fibroblasts. Of particular significance, the activation of endothelial cells and lymphocytes provokes fibroblasts to increase collagen synthesis, which clinically manifests as focal skin hardening and active inflammatory border (lilac ring) [1][2][3][4].…”