MD News and Information

Advanced Echo Detection of Heart Disease in Scleroderma

Author: Johns Hopkins Rheumatology
Date Published: July-2016
Source: Johns Hopkins Medicine

A new imaging technique reveals evidence of heart dysfunction in Scleroderma patients with undiagnosed heart disease. In a team effort between the Johns Hopkins Divisions of Rheumatology, Cardiology, and Pulmonary/Critical Care Medicine, led by Monica Mukherjee, M.D. and Ami A. Shah, M.D., researchers coupled traditional echocardiography (echo) with a new technique called “speckle-tracking” to reveal the presence of right heart dysfunction that was not detected by traditional methods. This new method may identify patients with Scleroderma who are at high risk for developing disability and even death due to severe heart disease.


Systemic sclerosis: The need for structured care

Author: Kathleen Morrisroe, Tracy Frech, Janine Schniering, Britta Maurer, Mandana Nikpour
Date Published: July-2016
Source: Clinical Rheumatology

Autoimmune connective tissue diseases (CTDs) have a propensity to affect multiple organ systems as well as physical function, quality of life, and survival. Their clinical heterogeneity, multisystem involvement, and low worldwide prevalence present challenges for researchers to establish a study design to help better understand the course and outcomes of CTDs.

Systemic sclerosis (SSc) is a notable example of a CTD, wherein longitudinal cohort studies (LCS) have enabled us to elucidate disease manifestations, disease course, and risk and prognostic factors for clinically important outcomes, by embedding research in clinical practice. Nevertheless, further efforts are needed to better understand SSc especially with regard to recognizing organ involvement early, developing new therapies, optimizing the use of existing therapies, and defining treatment targets.


Raynaud’s Symptoms in SSc Seen to Ease with Iloprost and Bosentan Treatment

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

Iloprost (brand name, Ventavis) in combination with bosentan (Tracleer) can improve blood flow in microscopic vessels called capillaries in the hands and feet of patients with systemic sclerosis, where poor capillary function often gives rise to Raynaud’s phenomenon.

The study, “Effect of treatment with iloprost with or without bosentan on nailfold videocapillaroscopic alterations in patients with systemic sclerosis,“ recently published in the journal Modern Rheumatology, inspected capillaries at the nail fold of the finger using video microscopy to evaluate if the two drugs improve the workings of these blood vessels, potentially easing Raynaud’s symptoms.


Actelion initiates Phase III study to evaluate macitentan (Opsumit) in children with PAH

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

Actelion Ltd (ATLN.VX) announced today that it will be initiating a Phase III study to evaluate the effect of macitentan on delaying disease progression in children with PAH using a pediatric formulation of macitentan (Opsumit).

TOMORROW (pediaTric use Of Macitentan tO delay disease pRogRessiOn in PAH Worldwide) is a multicenter, controlled, randomized, open-label event-driven study to assess the efficacy, safety and pharmacokinetics of macitentan versus standard of care in children with PAH.



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.

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