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Clinical Rheumatology

, Volume 38, Issue 12, pp 3619–3626 | Cite as

Simplified risk stratification for pulmonary arterial hypertension associated with connective tissue disease

  • Xiaodi Li
  • Xiaoxuan Sun
  • Yingheng Huang
  • Yuanyuan Wang
  • Xiaoman Yang
  • Jingya Wang
  • Ning Zhang
  • Lei Gu
  • Miaojia ZhangEmail author
  • Qiang WangEmail author
Original Article

Abstract

Objective

To explore the long-term prognostic value of a simplified risk assessment strategy based on the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines in Chinese patients with connective tissue disease (CTD) associated with pulmonary arterial hypertension (PAH).

Methods

We identified 50 CTD-PAH patients diagnosed by right heart catheterization. A retrospective chart review was completed to assess their clinical presentation and laboratory test results. A simplified version of the risk stratification model proposed by the 2015 ESC/ERS PH guidelines was applied, which included the WHO functional class, the 6-minute walking distance test, N-terminal pro-B-type natriuretic peptide plasma levels, pericardial effusion, right atrial pressure (RAP), cardiac index (CI), and mixed venous oxygen saturation (SvO2). The risk grades were defined as follows: low risk = at least 3 low-risk variables and no high-risk variables; high risk = at least 2 high-risk variables, including SvO2 or CI; and intermediate risk = when the above definitions of low or high risk were not fulfilled. The study endpoint was 3-year all-cause mortality.

Results

Twenty patients were defined as a low-risk group, while 30 were classified into a combined intermediate-high-risk group at the baseline assessment. All 20 patients in the low-risk group remained in the low-risk group at follow-up, 20 patients in the intermediate-high-risk group were downgraded to the low-risk group, and eight patients remained in the intermediate-high-risk group at the follow-up assessment. Patients in the intermediate-high-risk group exhibited higher 3-year mortality than the low-risk group at baseline (26% vs 14%, P = 0.0384). Compared with patients who remained in the intermediate-high-risk group, patients who were downgraded to the low-risk group showed lower 3-year mortality (P = 0.0281).

Conclusion

A simplified risk stratification model based on the 2015 ESC/ERS PH guidelines helped to identify CTD-PAH patients with poor long-term  prognosis , which was useful in evaluating the severity and treatment response of patients with CTD-PAH.

Key Point

•This study showed that the simplified version of the 2015 ESC/ERS risk stratification model could help identify Chinese CTD-PAH patients with poor prognosis at diagnosis and after treatment initiation.

Keywords

Connective tissue disease Prognosis Pulmonary arterial hypertension Risk stratification 

Abbreviations

PAH

Pulmonary arterial hypertension

CTD

Connective tissue disease

IPAH

Idiopathic pulmonary arterial hypertension

ESC

European Society of Cardiology

ERS

European Respiratory Society

PH

Pulmonary hypertension

FC

Functional class

6MWD

6-minute walking distance

NT-proBNP

N-terminal pro-B-type natriuretic peptide

PVR

Pulmonary vascular resistance

RHC

Right heart catheterization

mPAP

Mean pulmonary arterial pressure

PAWP

Pulmonary arterial wedge pressure

ILD

Interstitial lung disease

HRCT

High-resolution computer tomography

SLE

Systematic lupus erythematosus

ACR

American Rheumatism Association

pSS

Primary Sjogren’s syndrome

SSc

Systemic sclerosis

MCTD

Mixed connective tissue disease

UCTD

Undifferentiated connective tissue disease

SvO2

Mixed venous oxygen saturation

CI

Cardiac index

RAP

Right atrial pressure

Notes

Acknowledgments

Thanks to  all patients involved in  this retrospective study.

Funding

This study was supported by the National Natural Science Foundation of China (NSFC) (81671615, 81701610, and 81302575), a project funded by the Priority Academic Program Development of Jiangsu Higher Education Institutions (PAPD), Young Medical People Project in Jiangsu Province, and Medical Talent of Empowering Medicine through Science and Education Program.

Compliance with ethical standards

This study was approved by the Medical Ethics Committee of the First Affiliated Hospital of Nanjing Medical University and it has been performed in accordance with the ethical standard laid down in the 1964 Declaration of Helsinki.

Disclosures

None.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of RheumatologyThe first affiliated hospital of Nanjing Medical UniversityNanjingChina

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