scholarly journals Increased serum levels and tissue expression of MMP-12 in patients with systemic sclerosis: correlation with severity of skin and pulmonary fibrosis and vascular damage

2012 ◽  
Vol 71 (Suppl 1) ◽  
pp. A48.2-A48
Author(s):  
M Manetti ◽  
S Guiducci ◽  
E Romano ◽  
S Bellando-Randone ◽  
M L Conforti ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Serum Levels ◽  
Tissue Expression ◽  
Vascular Damage

AB0057 Increased IL-35 Serum Levels in Systemic Sclerosis Indicates Association with Pulmonary Fibrosis

2015 ◽  
Vol 74 (Suppl 2) ◽  
pp. 909.2-909
Author(s):  
A.T. Dantas ◽  
S.M.C. Gonçalves ◽  
M.C. Pereira ◽  
R.S.G. Gonçalves ◽  
C.D.L. Marques ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Serum Levels

Elevated Circulating TWEAK Levels in Systemic Sclerosis: Association with Lower Frequency of Pulmonary Fibrosis

2009 ◽  
Vol 36 (8) ◽  
pp. 1657-1662 ◽  
Author(s):  
KOICHI YANABA ◽  
AYUMI YOSHIZAKI ◽  
EIJI MUROI ◽  
TOSHIHIDE HARA ◽  
FUMIHIDE OGAWA ◽  
...  
Keyword(s):  
Longitudinal Study ◽  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Lupus Erythematosus ◽  
Tumor Necrosis ◽  
Vital Capacity ◽  
Protective Factor ◽  
Serum Levels ◽  
Clinical Associations ◽  
Necrosis Factor

Objective.To determine serum levels of tumor necrosis factor-related weak inducer of apoptosis (TWEAK) and its clinical associations in patients with systemic sclerosis (SSc).Methods.Serum TWEAK levels from 70 patients with SSc were examined by ELISA. In a retrospective longitudinal study, sera from 23 patients with SSc were analyzed (followup 0.8–7.2 yrs).Results.Serum TWEAK levels were elevated in patients with SSc (n = 70) compared with healthy controls (n = 31) and patients with systemic lupus erythematosus (n = 22). Among patients with SSc, there were no differences in serum TWEAK levels between limited cutaneous SSc and diffuse cutaneous SSc. Patients with SSc who had elevated TWEAK levels less often had pulmonary fibrosis and decreased vital capacity than those with normal TWEAK levels. In the longitudinal study, SSc patients with inactive pulmonary fibrosis or without pulmonary fibrosis consistently exhibited increased TWEAK levels, while those with active pulmonary fibrosis showed decreased TWEAK levels during the followup period.Conclusion.TWEAK levels were increased in patients with SSc, and associated with a lower frequency of pulmonary fibrosis in patients with SSc. TWEAK could be a protective factor against the development of pulmonary fibrosis in this disease and as such would be a possible therapeutic target.

Elevated Serum Concentrations of Polymorphonuclear Neutrophilic Leukocyte Elastase in Systemic Sclerosis: Association with Pulmonary Fibrosis

2009 ◽  
Vol 36 (1) ◽  
pp. 99-105 ◽  
Author(s):  
TOSHIHIDE HARA ◽  
FUMIHIDE OGAWA ◽  
KOICHI YANABA ◽  
YOHEI IWATA ◽  
EIJI MUROI ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Elevated Serum ◽  
Serum Levels ◽  
Oxidative Stress Marker ◽  
Surfactant Protein D ◽  
Serum Concentrations ◽  
Leukocyte Elastase ◽  
Pmn Elastase ◽  
Pmn Élastase

ObjectiveTo determine the serum concentrations and clinical association of polymorphonuclear neutrophilic leukocyte (PMN) elastase in patients with systemic sclerosis (SSc).MethodsSerum PMN elastase levels from 21 patients with limited cutaneous SSc (lSSc) and 32 with diffuse cutaneous SSc (dSSc) were examined by ELISA.ResultsSerum PMN elastase levels were elevated in patients with SSc, especially dSSc, compared to healthy controls. SSc patients with elevated serum PMN elastase levels had more frequent presence of pulmonary fibrosis, arthritis, contracture of phalanges, and diffuse pigmentation. Anticentromere antibody was detected less frequently in SSc patients with elevated serum PMN elastase levels than in controls. Consistently, serum PMN elastase levels also correlated positively with serum levels of KL-6 and surfactant protein-D, serological markers for pulmonary fibrosis. Serum PMN elastase levels were also associated with levels of serum 8-isoprostane, an oxidative stress marker in SSc.ConclusionSerum PMN elastase levels were elevated in patients with SSc, and it was more prominent in patients with pulmonary fibrosis, suggesting that serum PMN elastase is a novel serological marker for SSc-related pulmonary fibrosis.

Use of Serum Clara Cell 16-kDa (CC16) Levels as a Potential Indicator of Active Pulmonary Fibrosis in Systemic Sclerosis

2011 ◽  
Vol 38 (5) ◽  
pp. 877-884 ◽  
Author(s):  
MINORU HASEGAWA ◽  
MANABU FUJIMOTO ◽  
YASUHITO HAMAGUCHI ◽  
TAKASHI MATSUSHITA ◽  
KATSUMI INOUE ◽  
...  
Keyword(s):  
Longitudinal Study ◽  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Lupus Erythematosus ◽  
Characteristic Curve ◽  
Serum Levels ◽  
Clara Cell ◽  
Inactive Disease ◽  
Surfactant Protein D ◽  
Healthy Controls

Objective.To clarify the clinical significance of concentrations of serum Clara cell 16-kDa protein (CC16; previously denoted CC10) in the diagnosis and monitoring of pulmonary fibrosis (PF) in patients with systemic sclerosis (SSc); and to compare CC16 levels with levels of the current most reliable serum markers for PF, such as Krebs von den Lungen-6 (KL-6) antigen and surfactant protein-D (SP-D).Methods.Serum levels of CC16, KL-6, and SP-D were determined by ELISA in 92 patients with SSc, 20 patients with systemic lupus erythematosus (SLE), and 20 healthy controls. In a retrospective longitudinal study, correlation of serum CC16 levels with the activity of PF was assessed in 16 SSc patients with PF.Results.Although CC16 levels were higher in patients with SSc than in SLE patients or healthy controls, the difference was not significant. Increased serum CC16 levels were associated with involvement of PF, especially active PF, as well as KL-6 and SP-D. Receiver operating characteristic curve analysis revealed that the utility of CC16 is slightly inferior to KL-6, but was comparable with that of SP-D for detecting PF in patients with SSc. In the longitudinal study, serum levels of CC16, KL-6, and SP-D were significantly decreased in the inactive disease phase compared to the active disease phase.Conclusion.CC16 levels can be used as a potential serum biomarker for PF in addition to KL-6 and SP-D in patients with SSc.

scholarly journals IFNL3 genotype is associated with pulmonary fibrosis in patients with systemic sclerosis

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Mayada Metwally ◽  
Khaled Thabet ◽  
Ali Bayoumi ◽  
Mandana Nikpour ◽  
Wendy Stevens ◽  
...  
Keyword(s):  
Risk Factor ◽  
Systemic Sclerosis ◽  
Liver Fibrosis ◽  
Pulmonary Fibrosis ◽  
Genetic Variant ◽  
Serum Levels ◽  
Skin Fibrosis ◽  
Pathophysiological Mechanisms ◽  
Caucasian Patients ◽  
Cutaneous Fibrosis

Abstract Fibrosis across different organs and tissues is likely to share common pathophysiological mechanisms and pathways. Recently, a polymorphism (rs12979860) near the interferon lambda gene (IFNL3) was shown to be associated with fibrosis in liver across multiple disease etiologies. We determined whether this variant is a risk factor for pulmonary fibrosis (PF) and worsening cutaneous fibrosis in systemic sclerosis (SSc). Caucasian patients with SSc (n = 733) were genotyped to test for association with the presence of PF and worsening of skin fibrosis. Serum IFN-λ3 levels from 200 SSc cases were evaluated. An association of the IFNL3 polymorphism with PF was demonstrated (OR: 1.66 (95% CI: 1.142–2.416, p = 0.008). The IFNL3 variant was not a risk factor for worsening of skin fibrosis. Functionally, IFN-λ3 serum levels were higher among subjects with PF compared to those unaffected (P < 0.0001). In conclusion, IFNL3 serum levels and the genetic variant known to be associated with liver fibrosis are similarly linked to PF, but not to worsening of skin fibrosis in SSc. These data highlight both common fibrosis pathways operating between organs, as well as differential effects within the same disease.

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