MD News and Information

Adempas PAH Benefits Extend to SSc

Author: Nancy Walsh
Date Published: July-2016
Source: MedPage Today

Treatment with riociguat (Adempas) increased exercise capacity among patients whose pulmonary arterial hypertension was associated with connective tissue disease (PAH-CTD) in a post-hoc analysis of a phase III clinical trial, researchers reported.

Among PAH-CTD patients randomized to receive riociguat in dosages up to 2.5 mg three times per day, the mean 6-minute walking distance increased by 18 meters at week 12 compared with a decrease of 8 m (about 26 ft.) for those given placebo, for a mean treatment difference of +28 m (95% CI -4 to 61), according to Christopher P. Denton, MD, of University College London, and colleagues.

 

Hand Impairment in Systemic Sclerosis: Various Manifestations and Currently Available Treatment

Author: Amber Young, Rajaie Namas, Carole Dodge, Dinesh Khanna
Date Published: July-2016
Source: Springer Link

Systemic sclerosis (SSc) is an autoimmune disease initially recognized by hand involvement due to characteristic Raynaud’s phenomenon (RP), puffy hands, skin thickening, and contractures resembling claw deformities. SSc contributes to hand impairment through inflammatory arthritis, joint contractures, tendon friction rubs (TFRs), RP, digital ulcers (DU), puffy hands, skin sclerosis, acro-osteolysis, and calcinosis. These manifestations, which often coexist, can contribute to difficulty with occupational activities and activities of daily living (ADL), which can result in impaired quality of life. However, despite this knowledge, most diagnostic and treatment principles in SSc are focused on visceral manifestations due to known associations with morbidity and mortality. Treatment of inflammatory arthritis is symptom based and involves corticosteroids ≤10 mg daily, methotrexate, tumor necrosis factor inhibitors, tocilizumab, and abatacept. Small joint contractures are managed by principles of occupational hand therapy and rarely surgical procedures.

 

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.

 

 
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