Melvyn Rubenfire1, Mark D Huffman2, Sangeetha Krishnan3, James R Seibold4, Elena Schiopu5, Vallerie V McLaughlin3. 1. Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI. Electronic address: mrubenfi@med.umich.edu. 2. Preventive Medicine and Medicine-Cardiology, Northwestern University Feinberg School of Medicine, Chicago, IL. 3. Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI. 4. Scleroderma Research Consultants, Avon, CT. 5. Division of Rheumatology, University of Michigan, Ann Arbor, MI.
Abstract
BACKGROUND: The impact of modern therapy on survival in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) is not clear. We sought to determine associations among commonly used clinical and hemodynamic variables, treatment, and long-term survival in PAH associated with SSc compared with PAH defined as idiopathic, familial, or associated with anorexigens. METHODS: The observation period (1996-2010) included the option for epoprostenol and the availability of oral agents in 2002 (modern era of endothelin antagonists and phosphodiesterase-5 inhibitors). Primary outcome was all-cause mortality. RESULTS: Eighty-three patients had SSc (mean age, 59 years), and 120 had PAH (mean age, 51 years) (P < .0001, > 80% were functional class III or IV in both groups). Compared with PAH, SSc had a lower mean pulmonary artery pressure (48 mm Hg vs 58 mm Hg, P < .0001) and pulmonary vascular resistance (10 resistance units vs 15 resistance units, P < .0001), and a higher cardiac index (2.3 L/min/m2 vs 1.8 L/min/m2, P < .0001). PAH was more often treated with prostacyclin (71% vs 44%, P < .0001), but there were no differences in the use of monotherapy or combination oral therapy. SSc had a twofold-higher mortality over the 14 years. The 5-year survival in the modern era for PAH was 87%, compared with 51% for SSc (P < .001). CONCLUSIONS: Despite an improvement in clinical status, unlike in PAH, mortality in SSc has not improved since the introduction of epoprostenol.
BACKGROUND: The impact of modern therapy on survival in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) is not clear. We sought to determine associations among commonly used clinical and hemodynamic variables, treatment, and long-term survival in PAH associated with SSc compared with PAH defined as idiopathic, familial, or associated with anorexigens. METHODS: The observation period (1996-2010) included the option for epoprostenol and the availability of oral agents in 2002 (modern era of endothelin antagonists and phosphodiesterase-5 inhibitors). Primary outcome was all-cause mortality. RESULTS: Eighty-three patients had SSc (mean age, 59 years), and 120 had PAH (mean age, 51 years) (P &lt; .0001, &gt; 80% were functional class III or IV in both groups). Compared with PAH, SSc had a lower mean pulmonary artery pressure (48 mm Hg vs 58 mm Hg, P &lt; .0001) and pulmonary vascular resistance (10 resistance units vs 15 resistance units, P &lt; .0001), and a higher cardiac index (2.3 L/min/m2 vs 1.8 L/min/m2, P &lt; .0001). PAH was more often treated with prostacyclin (71% vs 44%, P &lt; .0001), but there were no differences in the use of monotherapy or combination oral therapy. SSc had a twofold-higher mortality over the 14 years. The 5-year survival in the modern era for PAH was 87%, compared with 51% for SSc (P &lt; .001). CONCLUSIONS: Despite an improvement in clinical status, unlike in PAH, mortality in SSc has not improved since the introduction of epoprostenol.
Authors: Valentina Mercurio; Monica Mukherjee; Ryan J Tedford; Roham T Zamanian; Rubina M Khair; Takahiro Sato; Omar A Minai; Fernando Torres; Reda E Girgis; Kelly Chin; Rachel Damico; Todd M Kolb; Stephen C Mathai; Paul M Hassoun Journal: Am J Respir Crit Care Med Date: 2018-02-01 Impact factor: 21.405
Authors: Rennie L Rhee; Nicole B Gabler; Sapna Sangani; Amy Praestgaard; Peter A Merkel; Steven M Kawut Journal: Am J Respir Crit Care Med Date: 2015-11-01 Impact factor: 21.405
Authors: Rahul G Argula; Abhijit Karwa; Abigail Lauer; David Gregg; Richard M Silver; Carol Feghali-Bostwick; Lynn M Schanpp; Kim Egbert; Bruce W Usher; Viswanathan Ramakrishnan; Paul M Hassoun; Charlie Strange Journal: Ann Am Thorac Soc Date: 2017-05