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.

scholarly journals Correlation between Serum Krebs von den Lungen-6 Levels with Forced Vital Capacity and Modified Rodnan Skin Score of Patients with Restrictive Lung Disease in Diffuse-Type Systemic Sclerosis

2019 ◽  
Vol 11 (2) ◽  
Author(s):  
Herlina Yani ◽  
Sumartini Dewi ◽  
Andri Reza Rahmadi
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Skin Fibrosis ◽  
Diffuse Type ◽  
Skin Score ◽  
Restrictive Lung Disease ◽  
Modified Rodnan Skin Score

Background Pulmonary fibrosis / intersitial lung disease (ILD) in systemic sclerosis (SSc) is a complicated restrictive pulmonary disease and the leading cause of disease-related mortality. Progressive skin fibrosis in diffuse-type SSc (dSSc) is associated with decreased forced vital capacity (FVC). Modified Rodnan Skin Score (mRSS) examination is used as a parameter to assess skin fibrosis, while high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) are used to assess pulmonary fibrosis. The HRCT test remains as the gold standard in diagnosing ILD. However, it costs a lot and is not available in all healthcare facilities. Krebs Von den Lungen-6 (KL-6) is a biomarker to evaluate pulmonary fibrosis. The aim of this study was to analyze the correlation of serum KL-6 levels with FVC and mRSS value of patients with restrictive lung disease in dSSc. Method This was a cross-sectional study that used primary data from dSSc patients who visited rheumatology outpatient clinic in Hasan Sadikin Hospital Bandung, Indonesia, during the period of June-July 2019. History taking, physical examination, mRSS, spirometry, and serum KL-6 levels were performed. Data were analyzed using the Rank Spearman correlation test.  Result There were 27 subjects with the mean age of 42 ± 12 years. Based on FVC (%) restrictive lung disease criteria, the majority of subjects (74.1%) had severe restrictive lung disease and the rest of all subjects (25.9%) were non severe restrictive lung disease. Serum KL-6 levels ranged from 0.545 to 8.138 ng/ml. The results showed that there was no correlation between serum KL-6 levels and FVC values (r = -0.118, p = 0.279) and mRSS (r = 0.101, p = 0.312 ). Conclusion There is no correlation between serum KL-6 levels with FVC and mRSS value of patient with restritive lung disease in diffuse type systemic sclerosis. Keywords : diffuse type systemic sclerosis, Forced Vital Capacity, KL-6, mRSS, restrictive lung disease.      

AB0203 S100A4 Serum Levels Correlate with Disease Activity, Skin Fibrosis and Lung Involvement in Systemic Sclerosis

2014 ◽  
Vol 73 (Suppl 2) ◽  
pp. 871.1-871 ◽  
Author(s):  
M. Tomcik ◽  
L. Andres Cerezo ◽  
S. Skacelova ◽  
M. Komarc ◽  
R. Becvar ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Disease Activity ◽  
Serum Levels ◽  
Skin Fibrosis ◽  
Lung Involvement

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.

Association of HLA Class II Genes with Systemic Sclerosis in Spanish Patients

2009 ◽  
Vol 36 (12) ◽  
pp. 2733-2736 ◽  
Author(s):  
CARMEN P. SIMEÓN ◽  
VICENT FONOLLOSA ◽  
CARLES TOLOSA ◽  
EDUARD PALOU ◽  
ALBERT SELVA ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Fibrosis ◽  
Genetic Susceptibility ◽  
Topoisomerase I ◽  
Chain Reaction ◽  
Clinical Expression ◽  
Caucasian Patients ◽  
Pulmonary Arterial ◽  
Polymerase Chain

Objective.To examine the role of HLA-DRB1 and HLA-DQB1 alleles in the susceptibility to systemic sclerosis (SSc) and its clinical expression in a Spanish population.Methods.One hundred Spanish Caucasian patients with SSc and 130 controls were studied. Molecular HLA-DRB1 and HLA-DQB1 typing was performed by polymerase chain reaction (PCR) sequence-based typing and PCR sequence-specific oligonucleotide.Results.HLA-DRB1*11 was associated with genetic susceptibility to SSc, whereas HLA-DRB1*07 (HLA-DRB1*0701) showed a protective effect. A significant increase in the frequency of the DRB1*1104 allele was observed in patients with anti-topoisomerase I autoantibodies (anti-Topo I) while HLA-DRB1*01 and HLA-DQB1*05 alleles were significantly increased in patients with anti-centromere antibodies (ACA). The HLA-DRB1*11 allele was more frequent in patients with pulmonary fibrosis; however, no significant association with any HLA-DRB1 or DQB1 alleles was identified in patients with pulmonary arterial hypertension.Conclusion.HLA alleles play a role in genetic susceptibility to SSc in Spanish patients. Some alleles are more prevalent in patients with pulmonary fibrosis and in patients with certain SSc-specific autoantibodies (anti-Topo I and ACA).

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