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Arthritis Research and Therapy

, 14:R213

First Online: 05 October 2012Received: 11 June 2012Revised: 09 August 2012Accepted: 05 October 2012

Abstract

IntroductionPulmonary arterial hypertension PAH is a major cause of mortality in connective tissue disease CTD. We sought to quantify survival and determine factors predictive of mortality in a cohort of patients with CTD-associated PAH CTD-PAH in the current era of advanced PAH therapy.

MethodsPatients with right heart catheter proven CTD-PAH were recruited from six specialised PAH treatment centres across Australia and followed prospectively. Using survival methods including Cox proportional hazards regression, we modelled for all-cause mortality. Independent variables included demographic, clinical and hemodynamic data.

ResultsAmong 117 patients 104 94.9% with systemic sclerosis, during 2.6 ± 1.8 mean ± SD years of follow-up from PAH diagnosis, there were 32 27.4% deaths. One-, two- and three-year survivals were 94%, 89% and 73%, respectively. In multiple regression analysis, higher mean right atrial pressure mRAP at diagnosis hazard ratio HR = 1.13, 95% CI: 1.04 to 1.24, P = 0.007, lower baseline six-minute walk distance HR = 0.64, 95% CI: 0.43 to 0.97, P = 0.04, higher baseline World Health Organization functional class HR = 3.42, 95% CI: 1.25 to 9.36, P = 0.04 and presence of a pericardial effusion HR = 3.39, 95% CI: 1.07 to 10.68, P = 0.04 were predictive of mortality. Warfarin HR = 0.20, 95% CI: 0.05 to 0.78, P = 0.02 and combination PAH therapy HR = 0.20, 95% CI: 0.05 to 0.83, P = 0.03 were protective.

ConclusionsIn this cohort of CTD-PAH patients, three-year survival was 73%. Independent therapeutic predictors of survival included warfarin and combination PAH therapy. Our findings suggest that anticoagulation and combination PAH therapy may improve survival in CTD-PAH. This observation merits further evaluation in randomised controlled trials.

KeywordsConnective tissue disease mortality prognosis Abbreviations6MWDsix minute walk distance

95% CI95% confidence interval

CIcardiac index

CTDconnective tissue disease

CTD-PAHconnective tissue disease-associated pulmonary arterial hypertension

DLCOdiffusing capacity of carbon monoxide

DVTdeep vein thrombosis

ERAendothelin receptor antagonists

FVCforced vital capacity

GIgastrointestinal

HRhazard ratio

HRCThigh-resolution computed tomography

ILDinterstitial lung disease

INRInternational Normalised Ratio

IPAHidiopathic pulmonary arterial hypertension

K-M curvesKaplan-Meier curves

MCTDmixed connective tissue disease

mPAPmean pulmonary arterial pressure

mRAPmean right atrial pressure

PAHpulmonary arterial hypertension

PDE5phosphodiesterase type-5 inhibitors

PVRpulmonary vascular resistance

RArheumatoid arthritis

RHCright-heart catheter

SDstandard deviation

SLEsystemic lupus erythematosus

SScsystemic sclerosis

WHO FCWorld Health Organization Functional Class.

Electronic supplementary materialThe online version of this article doi:10.1186-ar4051 contains supplementary material, which is available to authorized users.

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