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Lorinda Chung, MD; Juliana Liu, MSN; Lori S. Parsons, BS; Paul M. Hassoun, MD, FCCP; Michael D. McGoon, MD, FCCP; David B. Badesch, MD, FCCP; Dave P. Miller, MS; Mark R. Nicolls, MD; Roham T. Zamanian, MD
Author and Funding Information

From the Division of Immunology and Rheumatology (Dr Chung), and the Division of Pulmonary and Critical Care Medicine (Ms Liu and Drs Nicolls and Zamanian), Stanford University; the Veterans Affairs Palo Alto Health Care System (Drs Chung and Nicolls); ICON Clinical Research (Ms Parsons and Mr Miller); the Division of Pulmonary and Critical Care Medicine (Dr Hassoun), Johns Hopkins University; the Division of Cardiology (Dr McGoon), Mayo Clinic; the Division of Pulmonary and Critical Care Medicine (Dr Badesch), University of Colorado; and the Vera Moulton Wall Center for Pulmonary Vascular Disease (Ms Liu and Drs Nicolls and Zamanian).

Correspondence to: Lorinda Chung, MD, 3801 Miranda Ave, VA Palo Alto Health Care System, Palo Alto, CA 94304; e-mail: shauwei@stanford.edu


Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Chung has received research support funding from Gilead Sciences and United Therapeutics Corp and has served on the speakers’ bureau for Actelion Pharmaceuticals (< $10,000 per year). Ms Liu has served as a consultant to Gilead Sciences and United Therapeutics Corp and participated in advisory board meetings for Actelion Pharmaceuticals. Ms Parsons and Mr Miller are employees of ICON Clinical Research, a company that receives research funding from Actelion and other pharmaceutical companies. Dr Hassoun has received research funding support from Actelion/CoTherix Inc and is on the Advisory Board for Novartis. Dr McGoon has received research funding from Gilead and Medtronic Inc. He has served on steering committees for Gilead and Lung Rx Inc and participated on clinical end point committees in studies sponsored by Actelion. He is on a Data Safety Monitoring Board for a study sponsored by Gilead. Dr McGoon has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Badesch has received honoraria for service on steering committees and/or advisory boards for Actelion/CoTherix, Gilead/Myogen Inc, Encysive Pharmaceuticals, Pfizer Inc, GlaxoSmithKline, Lung Rx Inc, United Therapeutics, Eli Lilly and Company/ICOS, Biogen Idec Inc, mondoBIOTECH AG/mondoGEN AG, and Bayer. Dr Badesch has received grants from Actelion/CoTherix, Gilead/Myogen Inc, Encysive Pharmaceuticals, Pfizer Inc, United Therapeutics, Lung Rx Inc, Eli Lilly and Company/ICOS, Bayer, Novartis, and National Institutes of Health/National Heart, Lung, and Blood Institute. Dr Badesch has received honoraria for his service on the REVEAL Steering Committee, which is supported by Actelion. Dr Zamanian has received research funding support through the Entelligence-Actelion career development research grant ($75,000) and has served as a consultant to United Therapeutics and Gilead pharmaceuticals. Dr Nicolls has reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;139(6):1548-1549. doi:10.1378/chest.11-0341
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To the Editor:

We appreciate the review of our article1 by Dr Condliffe and colleagues. We agree that unadjusted outcomes of incident and prevalent populations of patients with pulmonary arterial hypertension (PAH) differ.2 Therefore, it is paramount that target populations are explicitly described when reporting data obtained from large registries. REVEAL (the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) was designed to observe and characterize a patient population encountered in daily clinical practice; thus, consecutive patients from 55 US PAH Centers were enrolled. These included (1) patients with established disease who previously underwent right-sided heart catheterization (RHC) and were receiving PAH-specific therapies at the time of enrollment, and (2) newly diagnosed patients who had recently undergone diagnostic RHC. In our report, we estimated 1-year survival from the date of enrollment for established and newly diagnosed patients with connective tissue disease associated-PAH (CTD-APAH).1 Survival differed by type of CTD: patients with systemic sclerosis (SSc) had the poorest survival (82%), followed by those with systemic lupus erythematosus (94%), mixed connective tissue disease (88%), and rheumatoid arthritis (96%).1 Although these estimates are valid and generalizable to patients with CTD-APAH seen in clinical practice, we agree that they are not generalizable to the much smaller clinical population of patients with CTD with exclusively incident PAH.

We concur that analyzing an incident population of patients with PAH is important, particularly to compare with other cohorts of incident patients. We analyzed the population of patients newly diagnosed with CTD-APAH (n =235) enrolled in REVEAL and recently reported their outcomes.3 As expected, unadjusted survival in our newly diagnosed population was worse than in the overall cohort. One- and 3-year survival estimates for the CTD-APAH groups were: SSc (78% and 54%), systemic lupus erythematosus (92% and 87%), mixed connective tissue disease (84% and 84%), and rheumatoid arthritis (88% and 61%).3 Our findings for the patients with SSc-APAH are consistent with those observed in the UK cohort of incident patients (1- and 3-year survival of 78% and 47%),4 although slightly worse than those reported from a US single-center cohort of patients with incident SSc-APAH (1- and 3-year survival of 85% and 67%).5

We contend that survival analyses of combined prevalent and incident PAH cases provide an accurate assessment of patients seen in clinical practice. This type of assessment permits the prediction of outcomes for a clinically relevant cross section of patients with PAH and explicitly allows for the comparison of outcomes between incident and prevalent cohorts. We believe that excluding prevalent cases from future registries would sacrifice abundant clinically relevant information.

Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;1386:1383-1394. [CrossRef] [PubMed]
 
Humbert M, Sitbon O, Yaïci A, et al; French Pulmonary Arterial Hypertension Network French Pulmonary Arterial Hypertension Network Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;363:549-555. [CrossRef] [PubMed]
 
Chung L, Parsons L, Hassoun PM, et al. Outcomes in a newly diagnosed population of connective tissue disease-associated pulmonary arterial hypertension patients from the REVEAL registry. Arthritis Rheum. 2010;62suppl 10:S512-S513
 
Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;1792:151-157. [CrossRef] [PubMed]
 
Campo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010;1822:252-260. [CrossRef] [PubMed]
 

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References

Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;1386:1383-1394. [CrossRef] [PubMed]
 
Humbert M, Sitbon O, Yaïci A, et al; French Pulmonary Arterial Hypertension Network French Pulmonary Arterial Hypertension Network Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;363:549-555. [CrossRef] [PubMed]
 
Chung L, Parsons L, Hassoun PM, et al. Outcomes in a newly diagnosed population of connective tissue disease-associated pulmonary arterial hypertension patients from the REVEAL registry. Arthritis Rheum. 2010;62suppl 10:S512-S513
 
Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;1792:151-157. [CrossRef] [PubMed]
 
Campo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010;1822:252-260. [CrossRef] [PubMed]
 
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