scholarly journals Failed Degradation of JunB Contributes to Overproduction of Type I Collagen and Development of Dermal Fibrosis in Patients With Systemic Sclerosis

2014 ◽  
Vol 67 (1) ◽  
pp. 243-253 ◽  
Author(s):  
Markella Ponticos ◽  
Ioannis Papaioannou ◽  
Shiwen Xu ◽  
Alan M. Holmes ◽  
Korsa Khan ◽  
...  
2004 ◽  
Vol 52 ◽  
pp. S286-S287
Author(s):  
A E Postlethwaite ◽  
W K Wong ◽  
J Ingels ◽  
A H Kang ◽  
P Clements ◽  
...  

2000 ◽  
Vol 272 (1) ◽  
pp. 36-40 ◽  
Author(s):  
Ryu-Ichiro Hata ◽  
Jun Akai ◽  
Akinori Kimura ◽  
Osamu Ishikawa ◽  
Masataka Kuwana ◽  
...  

2014 ◽  
Vol 42 (2) ◽  
pp. 236-242 ◽  
Author(s):  
Corrie L. Poelman ◽  
Laura K. Hummers ◽  
Fredrick M. Wigley ◽  
Cynthia Anderson ◽  
Francesco Boin ◽  
...  

Objective.We sought to retrospectively review a single-center experience using intravenous immunoglobulin (IVIG) for the treatment of refractory, active diffuse cutaneous systemic sclerosis (dcSSc).Methods.The mean modified Rodnan Skin score (mRSS) at baseline was compared to the mRSS at 6, 12, 18, and 24 months post-IVIG initiation by the paired Student t test. Changes in mRSS at 6 and 12 months were also compared to data from historical controls of 3 large, negative, multicenter, randomized clinical trials of other medications [D-penicillamine (D-pen), recombinant human relaxin (relaxin), and oral bovine type I collagen (collagen)] and to patients treated with mycophenolate mofetil (MMF) alone using the Student t test.Results.Thirty patients were treated with adjunctive IVIG (2 g/kg/mo) for refractory, active dcSSc. The mean baseline mRSS of our cohort was 29.6 ± 7.2, and this significantly decreased to 24.1 ± 9.6 (n = 29, p = 0.0011) at 6 months, 22.5 ± 10.0 (n = 25, p = 0.0001) at 12 months, 20.6 ± 11.8 (n = 23, p = 0.0001) at 18 months, and 15.3 ± 6.4 (n = 15, p < 0.0001) at 24 months. The mean change in mRSS at 6 months was not significantly different in the IVIG group (−5.3 ± 7.9) compared to the relaxin trial (−4.8 ± 6.99, p = 0.74) or MMF group (−3.4 ± 7.4, p = 0.26); however, at 12 months, the mean change in mRSS was significantly better in the IVIG group (−8 ± 8.3) than in the D-pen (−2.47 ± 8.6, p = 0.005) and collagen (−3.4 ± 7.12, p = 0.005) groups, and was comparable to the group of primary MMF responders (−7.1 ± 9, p = 0.67).Conclusion.Our observational study suggests that IVIG may be an effective adjunctive therapy for active dcSSc in patients failing other therapies.


2006 ◽  
Vol 117 (3) ◽  
pp. 299-306 ◽  
Author(s):  
Thomas M. Chiang ◽  
Hiroshi Takayama ◽  
Arnold E. Postlethwaite

2010 ◽  
Vol 38 (2) ◽  
pp. 289-296 ◽  
Author(s):  
VEERAPONG PHUMETHUM ◽  
SHAHIN JAMAL ◽  
SINDHU R. JOHNSON

Objective.Biologic agents are increasingly used in the rheumatic diseases. Their role in patients with systemic sclerosis (SSc) is uncertain. Our aim was to evaluate the effectiveness and safety of biologic agents in SSc. We review the evidence for the use of biologic agents to improve inflammatory arthritis, disability, and skin score, and we review adverse effects with biologic agents in patients with SSc.Methods.A systematic literature review was performed to identify studies evaluating the use of biologic agents in SSc. Medline, Embase, CINAHL, and Cochrane Database of Systematic Reviews were searched. A standardized abstraction form was used to extract biologic agent, study design, sample size, treatment effect, and adverse effects.Results.A total of 23 studies from 1413 citations were evaluated. Three studies evaluated infliximab, 3 evaluated etanercept, 3 evaluated antithymocyte globulin, 3 evaluated imatinib, 6 evaluated rituximab, and 1 study each evaluated interferon-γ (IFN-γ), IFN-α, relaxin, delipidated, deglycolipidated Mycobacterium vaccae, human anti-transforming growth factor ß1 antibody, and oral type I collagen. Studies of etanercept and infliximab suggest improvements in inflammatory arthritis and Health Assessment Questionnaire Disability Index (HAQ-DI). None of the other biologic agents demonstrated reproducible, statistically significant improvements in joint count, HAQ-DI, or skin score.Conclusion.Anti-tumor necrosis factor-α agents may improve inflammatory arthritis and disability in SSc. The effect on skin score is uncertain. Adequately powered trials are needed to evaluate efficacy, and longitudinal studies are needed to evaluate longterm safety of these agents in SSc.


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