Treatments for Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Video Overview
ASK1 inhibitor
Combination Therapies
Endothelin Receptors
Inhaled Prostacyclin (Ventavis, Iloprost)
Heart/Lung Transplants
Flolan (Epoprostenol)
PDE 5 Inhibitors
Sildenafil (Revatio)
Tadalafil (ADCIRCA)
Vardenafil (Levitra)
Stem Cell Transplant
Treprostinil (TYVASO)
Uptravi (Selexipag)
Warfarin (Coumadin)

Video Overview

Overview of Treatments for Pulmonary Hypertension Video
Video: Overview of Treatments for Pulmonary Hypertension

Presented by Amanda Thorpe
*More Sclero Videos*

Pulmonary hypertension secondary to systemic scleroderma can have a poor outcome, if untreated. With treatment the quality of life may improve and disease progression may slow down.

Treatment of pulmonary hypertension in systemic scleroderma is similar to that in idiopathic pulmonary hypertension, however systemic scleroderma causes many complications when it comes to managing treatment such as skin, renal and gastrointestinal involvement caused by scleroderma.

A multidisciplinary approach is vital, and preferably one coordinated by a registered scleroderma expert. (Also see What is Scleroderma?, What is Pulmonary Hypertension?, and Scleroderma Experts)

Treatments for pulmonary hypertension (PH) include bosentan (Tracleer), Letairis (Ambrisentan), Macitentan/Opsumit, lung transplants, oxygen therapy, sildenafil citrate (Revatio, Viagra), stem cell transplants, and warfarin (Coumadin).

Current Approaches to the Treatment of Systemic–Sclerosis–Associated Pulmonary Arterial Hypertension (SSc–PAH). This review describes the current management of SSc–PAH with an emphasis on the impact of the different organ involvements in the prognosis and treatment response. PubMed, Curr Rheumatol Rep, 2016 Jan;18(2):10.

Apoptosis Signal-regulating Kinase 1 inhibitor (ASK1)

Gilead Announces Top-Line Phase 2 Results for GS-4997 (Selonsertib) in Nonalcoholic Steatohepatitis (NASH), Pulmonary Arterial Hypertension (PAH) and Diabetic Kidney Disease (DKD) GS-4997 demonstrated anti-fibrotic activity in an open-label Phase 2 clinical trial that included 72 patients with NASH and moderate to severe (F2-F3) liver fibrosis, who received treatment with GS-4997., 10/26/2016. (Also see Clinical Trials)

Combination Therapy

Case Report: Systemic scleroderma–related interstitial pneumonia associated with borderline pulmonary arterial hypertension. The clinical condition improved dramatically with a combination therapy of an antifibrotic agent, pirfenidone for 12 months, and the dual endothelin receptor antagonist, macitentan, with prednisolone. PubMed, BMJ Case Rep.

Use of Coils and a Pulmonary Vasodilator to Reduce Pulmonary Hypertension in a Patient with Interstitial Pneumonia and Scleroderma. The improvement of symptoms following use of a supplementary pulmonary vasodilator provides the hope that the chosen treatment could be a viable alternative approach for other similar cases. PubMed, Intern Med, 2015;54(21):2721–6.

PDE 5 Inhibitors

Phosphodiesterase type 5 inhibitors (PDE 5) are used in the treatment of pulmonary arterial hypertension, often as part of combination treatment. There are three main brands of PDE 5: sildenafil (Revatio® and Viagra®), tadalafil ADCIRCA® and Cialis®, and vardenafil (Levitra®). This class of drugs is also used to treat erectile dysfunction, in lower dosages. ISN.

Treprostinil (TYVASO)

Extended–release oral treprostinil in the management of pulmonary arterial hypertension (PAH): clinical evidence and experience. Ongoing clinical trials will determine the long–term effects of higher doses of oral treprostinil on clinical outcomes. PubMed, Ther Adv Respir Dis, 2018 Jan-Dec;12:1753466618766490.

Uptravi® (Selexipag)

Uptravi by Actelion is an oral medication for PAHUptravi® (selexipag) is an oral IP prostacyclin receptor agonist marketed by Actelion Pharmaceuticals US, Inc., which was approved by the US FDA for the treatment of pulmonary arterial hypertension in December 2015. Common side effects include headache, diarrhea, jaw pain, nausea, muscle pain (myalgia), vomiting, pain in an extremity, and flushing. ISN.

Warfarin (Coumadin)

Prospects for improving outcomes in systemic sclerosis–related pulmonary hypertension.. The role of anti-coagulation for SSc–PAH has been the subject of a recent meta–analysis of nine observational studies that suggests it may confer a survival benefit, but to date, there have been no randomised controlled trials to confirm this. PubMed, Intern Med J, 2015 Mar;45(3):248-54.

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