Treatments for Pulmonary Hypertension in Systemic Sclerosis (Scleroderma)

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Video Overview
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.

Treatment of Systemic Sclerosis Complications: What to Use When First-Line Treatment Fails–A Consensus of Systemic Sclerosis Experts. Symptoms treatment recommendations by over 100 scleroderma experts, for scleroderma renal crisis (SRC), digital ulcers (DU), pulmonary hypertension (PAH/PH), reflux, skin involvement, and arthritis. Seminars in Arthritis and Rheumatism Volume 42, Issue 1 , Pages 42-55, August 2012. (Also see Scleroderma Renal Crisis, Digital Ulcers, Reflux, Skin Involvement, Skeletal Involvement, Scleroderma Treatments, and Dr. Janet Pope)

What can be done to treat Pulmonary Arterial Hypertension? Treatment options fall into two main areas: general therapies that are used to reduce symptoms but which do not have a positive impact on the disease progression, and disease-targeted therapies that have been specifically researched in the area of Pulmonary Arterial Hypertension (PAH). Actelion Pharmaceuticals.

Combination Therapy

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.

Combination therapy in the management of pulmonary arterial hypertension (PAH). An optimal combination drug therapy regimen remains debatable and should be customized for individual PAH patients. International Journal of Clinical Practice, 2013 Apr 11.

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)

The potential for inhaled treprostinil in the treatment of pulmonary arterial hypertension (PAH). PAH remains a severe, life-threatening disease in spite of the enormous progress in specific drug therapy over the last decade. Therefore, further improvement of drug therapy will be essential, with clear potential for inhaled treprostinil: a reduction of inhalation frequency and duration would markedly improve quality of life and compliance, and a longer-lasting local prostanoid effect might further enhance the efficacy of inhaled treprostinil. Therapeutic Advances in Respiratory Disease, February 7, 2011.

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)

Effect of Warfarin on Survival in Scleroderma-associated Pulmonary Arterial Hypertension (SSc-PAH) and Idiopathic PAH. Belief Elicitation for Bayesian Priors. This study demonstrates the presence of uncertainty about the effect of warfarin, and provides justification for a clinical trial. Sindhu R. Johnson. Journal of Rheumatology. January 2011, 38 (1).

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