Heart-Lung Treatments

Module: Scleroderma: Basic Overview

Elaine A. Furst, RN, MA, Janet L. Poole, Ph.D., OTR/L, Cindy Mendelson, PhD, RN, Dinesh Khanna, MD, MS

Several useful treatments are now available to treat pulmonary fibrosis and pulmonary hypertension caused by scleroderma.

  1. Exercise and smoking cessation are recommended for all people with scleroderma.
  2. High-dose cyclophosphamide (brand name, Cytoxan®) taken by mouth or monthly intravenous infusion, or daily mycophenolate mofetil has been shown to help slow or stop the progression of pulmonary fibrosis. However, not every patient with lung fibrosis needs these medications.
  3. Drugs taken by mouth for pulmonary arterial hypertension include bosentan (brand name, Tracleer®), sildenafil (brand name, Revatio®), ambrisentan (brand name, Letairis®), tadalafil (brand name, Adcirca®), and macitentan (brand name, Opsumit).
  4. Epoprostenil (brand name, Flolan®) and treprostinil (brand name, Remodulin®) given by constant intravenous infusion has been useful, although difficult to give over a long time. Treprostinil is also available as an oral medication (Orenitram®) or inhaled form (Tyvaso®). Another medication, iloprost (brand name, Ventavis®) can be inhaled and, as a result, has a much lower risk of complications than intravenous therapies.
  5. Recently, 2 new oral medications have been approved by the FDA for PAH. These include riociguat (brand name Adempas®) and selexipag (brand name Uptravi®).
  6. Lung transplantation may be a possible treatment for severe pulmonary hypertension and lung fibrosis if treatment of the underlying disorder fails.