Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group

Abstract

Lewis J Rubin has received consulting income from Actelion and Encysive; and he has served as an investigator for Actelion and Encysive. James R. Seibold, has consultancy relationships and research funding from Actelion, Pfizer, Encysive, FibroGen, Centocor, Bristol-Myers Squibb, Genzyme, Lilly, Gilead and United Therapeutics in the area of potential treatments of scleroderma and its complications. He has received lecture honoraria from Actelion, Pfizer, Encysive and United Therapeutics. His spouse is a full-time employee of Actelion Vibeke Strand has consultancy relationships with Pfizer, FibroGen, and Bristol-Myers Squibb in the area of potential treatments of scleroderma and its complications Daniel E Furst has consultancy relationship, has been a member of scientific advisory board, and/or has received research funding from Abbott, Actelion, Amgen, BMS, BiogenIdec, Centocor, Genentech, Gilead, GSK, Merck, Nitec, Novartis, Roche, UCB, Wyeth and Xoma. He has received lecture honoraria and/or lectured on behalf of Abbott, Actelion, Amgen, BMS, Biogen, Biogenidec, Centocor, Genentech, Gilead, Merck, Nitec and UCB. Oliver Distler has consultancy relationships and/or has received research funding from Actelion, Pfizer, Encysive, FibroGen, Ergonex, NicOx, and Biovitrum in the area of potential treatments of scleroderma and its complications. He has received lecture honoraria from Actelion, Pfizer, Encysive and Ergonex.This study was partially supported by unrestricted educational grants from Actelion,

Bosentan Treatment for Raynaud's Phenomenon and Skin Fibrosis in Patients with Systemic Sclerosis and Pulmonary Arterial Hypertension: An Open-Label, Observational, Retrospective Study

“…The study protocol followed the principles of the Declaration of Helsinki and was approved by the ethic committee of the Recruiting Center. All patients had a diagnosis of SSc-PAH, suspected by clinical signs and symptoms and confirmed by pulmonary function tests, exercise capacity test (6-min walk test) and transthoracic echocardiodoppler for the evaluation of pulmonary artery systolic pressure (PASP) (n.v. ::;35 mmHg) as previously described in details (40). No patient underwent right heart catheterization because transthoracic echocardiodoppler is able to accurately classify patients with chronic heart failure with or without PAH (40), avoiding subjecting the compromised patients to an invasive, potentially dangerous procedure.…”

Scleroderma: Nomenclature, etiology, pathogenesis, prognosis, and treatments: Facts and controversies

“…40 Early intervention in ILD and PAH is thought to improve patient outcomes, and therefore patients with systemic sclerosis should have annual screening for these complications. [41][42][43] Treatment Treatment algorithms for systemic sclerosis and morphea are limited by the rarity of the diseases (which makes conducting trials with enough power to detect differences difficult), the difficulty in assessing disease improvement (validated outcome measures are lacking, which makes conducting therapeutic trials difficult) and the lack of universally used outcome measures (which makes conducting meta-analysis of trials impossible).…”

Screening patients with scleroderma for pulmonary arterial hypertension and implications for other at-risk populations

“…Echocardiography experience is crucial; failure to obtain an estimate of projected systolic pulmonary artery pressure (PAP) has been reported in between 20 and 39% of patients [17,18]. Doppler-derived pressure estimations, while correlating with invasively measured systolic PAP in populations, show poor reliability in individual patients with both under-and overestimation of actual systolic PAP [19][20][21][22].…”