MD News and Information

Actemra may reduce scleroderma skin thickening

Author: Ara Freeman
Date Published: June-2016
Source: Dermatology News Digital Network

The interleukin-6 inhibitor tocilizumab (Actemra) substantially reduced skin thickening in patients with systemic sclerosis in the “proof-of concept” faSScinate trial, the results of which have now been published in the Lancet. The mean change in the primary endpoint of a modified Rodnan skin score at 24 weeks was –3.92 in the tocilizumab group and –1.22 in the placebo group, with an overall difference of –2.70 (95% confidence interval, –5.85-0.45; P = .0915). The effect was sustained at 48 weeks’ follow-up (–6.33 vs. –2.77; 95% CI, –7.2-0.12; P = .0579).

 

Clinical characteristics and predictors of gangrene in patients with systemic sclerosis and digital ulcers in the Digital Ulcer Outcome Registry: a prospective, observational cohort

Author: Daniel Rosenberg, Barbara Schwierin and Marco Matucci-Cerinic Yannick Allanore, Christopher P Denton, Thomas Krieg, Peter Cornelisse
Date Published: June-2016
Source: Annals of the Rheumatic Diseases

Digital vasculopathy in systemic sclerosis (SSc) consists of a spectrum of Raynaud’s phenomenon (RP), digital ulcers (DUs), critical digital ischaemia and escalation to gangrene. The complications of severe digital vasculopathy often require hospital-based management with intravenous therapies and surgery.1–3 Although gangrene is not infrequent in the clinic, data on the prevalence and implications of gangrene in patients with SSc are scarce.

 

Potential beneficial role for endothelin in scleroderma vasculopathy: inhibition of endothelial apoptosis by type B endothelin-receptor signaling

Author: Yongqing Wang, Nezam Altorok, Bashar Kahaleh
Date Published: June-2016
Source: Scleroderma Journal

Microvascular endothelial cell (MVEC) apoptosis is considered to be a key event in the pathogenesis of systemic sclerosis (SSc), an increased expression of endothelin-1 (ET1) is also well recognized in the disease. ET1 is thought to exert deleterious effects on the vasculature by virtue of its known vasospastic, proliferative and fibrotic effects, yet ET1 can act as a survival factor for a variety of cells, including MVEC. The aim of this study is to investigate if ET1 signaling protects SSc-MVECs from apoptosis.

 

iBio’s Endostatin-Related Peptides for Fibrosis, Scleroderma Issued US Patent

Author: Magdalena Kegel
Date Published: June-2016
Source: Scleroderma News

The pharmaceutical company iBio, which focuses on the clinical development of drugs for systemic sclerosis and other fibrotic diseases, was recently issued a U.S. patent protecting the company’s drug development attempts using endostatin-related peptides for the treatment of fibrosis.

According to a press release, the patent, US 9,365,616, titled “Use of Endostatin Peptides for the Treatment of Fibrosis,” complements earlier U.S. patents 8,507,441 and 8,716,232 covering this line of drug development. The patent covers both the composition of the drugs and the methods for which endostatin-related peptides might be used.

 

An Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Disease: the LOTUSS Trial

Author: Dinesh Khanna, Carlo Albera, Aryeh Fischer, Nader Khalidi, Ganesh Raghu, Lorinda Chung, Dan Chen, Elena Schiopu, Margit Tagliaferri, James R. Seiboldand Eduard Gorina
Date Published: June-2016
Source: The Journal of Rheumatology

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) shares a number of clinical features and pathogenic mechanisms with idiopathic pulmonary fibrosis (IPF). This study was designed to evaluate the tolerability of the IPF treatment pirfenidone in SSc-ILD. The known gastrointestinal, skin, and liver adverse events (AE) of pirfenidone are of importance given the involvement of these organs in SSc.

 

Scleroderma Patients and Lung Transplantation

Author: John Hansen-Flaschen, MD
Date Published: June-2016
Source: ATS News

Two studies published in the June issue of Annals of the American Thoracic Society look at scleroderma and lung transplantation. Patients with advanced lung disease due to systemic sclerosis (SSC) have long been considered poor candidates for lung transplantation. However, in a retrospective study of lung transplant outcomes over an eight-year period at the University of Pittsburgh, Maria M. Crespo. MD, and colleagues found that one- and five-year survival in patients with scleroderma are similar to pulmonary fibrosis. The results, they wrote, “indicate that lung transplant is a reasonable treatment option in selected patients with scleroderma.”

 
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