Inhaled Prostacyclin (Ventavis, Iloprost)
|PDE 5 Inhibitors
Stem Cell Transplant
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.
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.
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.
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.
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.
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) 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.
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).
SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, or Volunteer. Donations may also be mailed to:
International Scleroderma Network (ISN)
7455 France Ave So #266
Edina, MN 55435-4702 USA
Email [email protected] to request our Welcome email, or to report bad links or to update this page content.