Drugs Classes Available for Treatment

Prostacyclin Analogs and IP Receptor Agonists

Epoprostenol: The main concern with this drug is its half-life of approximately 5 minutes. It is also limited by its intravenous infusion administration, which leads to the discontinuation of treatment in many patients. As a result, patients are at risk for serious side effects such as rebound worsening of symptoms, possibly within a short period of time; right heart failure; or death.


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Treprostinil, iloprost, and selexipag: These agents have longer half-lives and are available in subcutaneous, inhaled, and oral formulations. Side effects associated with these drugs include jaw pain, diarrhea, headache, and facial flushing.

Endothelin Receptor Antagonists

Bosentan, ambrisentan, and macitentan: All are orally administered and have been approved by the US Food and Drug Administration (FDA). They are similar in efficacy and response rates. Main side effects include liver function test abnormalities, anemia, and teratogenicity.

Phosphodiesterase Type 5 Inhibitors (PDE-5I) and Soluble Guanylate Cyclase (sGC) Stimulator

Sildenafil, tadalafil, and riociguat: These have been approved by the FDA in oral and intravenous formatulations. Side effects include headache, muscle pain, facial flushing, nausea, pain (arms, legs, and back), nasal congestion, and syncope.

“In vasoreactive patients, we still use calcium channel blockers as the first line of therapy,” Dr Minkin said. “However, follow closely for a response. If it is absent, proceed to medications targeting PAH. If the response is not as high as anticipated, sequential combination therapies are advised with early referral for surgical intervention such as atrial septostomy as a bridge for eventual lung transplantation.”

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References

  1. Minkin R. Pulmonary arterial hypertension in systemic sclerosis. Presentation at: ACR/ARHP 2017 Annual Meeting; November 3-8, 2017; San Diego, CA. Session 3S017.
  2. Coghlan JG, Denton CP, Grünig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum. 2014;73(7):1340-1349.
  3. Ruiter G, Lanser IJ, de Man FS, et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford). 2014;53(2):285-292.