Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Mar 27;21(1):82.
doi: 10.1186/s13075-019-1868-0.

Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study

Hung-An Chen  1   2 , Tsai-Ching Hsu  3 , Su-Ching Yang  4 , Chia-Tse Weng  5 , Chun-Hsin Wu  5 , Chien-Yao Sun  5 , Chun-Yu Lin  6
Affiliations

Incidence and survival impact of pulmonary arterial hypertension among patients with systemic lupus erythematosus: a nationwide cohort study

Hung-An Chen et al. Arthritis Res Ther. .

Abstract

Background: No population-based study has investigated the cumulative incidence of pulmonary arterial hypertension (PAH) in patients with newly diagnosed systemic lupus erythematosus (SLE) or the survival impact of PAH in this population.

Method: We used a nationwide database in Taiwan and enrolled incident SLE patients between January 1, 2000, and December 31, 2013. The cumulative incidence of PAH in the SLE patients and the survival of these patients were estimated by the Kaplan-Meier method. Potential predictors of the development of PAH were determined using a Cox proportional hazards regression model.

Results: Of 15,783 SLE patients, 336 (2.13%) developed PAH. The average interval from SLE diagnosis to PAH diagnosis was 3.66 years (standard deviation [SD] 3.36, range 0.1 to 13.0 years). Seventy percent of the patients developed PAH within 5 years after SLE onset. The 3- and 5-year cumulative incidence of PAH were 1.2% and 1.8%, respectively. Systemic hypertension was an independent predictor of PAH occurrence among the SLE patients (adjusted hazard ratio 2.27, 95% confidence interval 1.59-2.97). The 1-, 3-, and 5-year survival rates of SLE patients following the diagnosis of PAH were 87.7%, 76.8%, and 70.1%, respectively.

Conclusions: PAH is a rare complication of SLE and the majority of PAH cases occur within the first 5 years following SLE diagnosis. Systemic hypertension may be a risk factor for PAH development in the SLE population. The overall 5-year survival rate after PAH diagnosis was 70.1%.

Keywords: Incidence; Prognosis; Pulmonary arterial hypertension; Systemic lupus erythematosus.

PubMed Disclaimer

Conflict of interest statement

Ethics approval and consent to participate

The Institutional Review Board of National Cheng Kung University Hospital approved this study (A-EX-104-045). Informed consent was not required because the datasets were devoid of personally identifiable information.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Number of patients with different intervals from systemic lupus erythematosus diagnosis to pulmonary arterial hypertension diagnosis
Fig. 2
Fig. 2
Cumulative incidence of pulmonary arterial hypertension in patients with new diagnosis of systemic lupus erythematosus. a Whole cohort. b Comparison of patients based on the presence of systemic hypertension (HTN)
Fig. 3
Fig. 3
Survival probability of patients with systemic lupus erythematosus after the diagnosis of pulmonary arterial hypertension
See this image and copyright information in PMC

References

    1. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) Eur Respir J. 2015;46:903–975. doi: 10.1183/13993003.01032-2015. - DOI - PubMed
    1. McGoon MD, Miller DP. REVEAL: a contemporary US pulmonary arterial hypertension registry. Eur Respir Rev. 2012;21:8–18. doi: 10.1183/09059180.00008211. - DOI - PMC - PubMed
    1. 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;179:151–157. doi: 10.1164/rccm.200806-953OC. - DOI - PubMed
    1. Schreiber BE, Connolly MJ, Coghlan JG. Pulmonary hypertension in systemic lupus erythematosus. Best Pract Res Clin Rheumatol. 2013;27(3):425–434. doi: 10.1016/j.berh.2013.07.011. - DOI - PubMed
    1. Jing ZC, Xu XQ, Han ZY, et al. Registry and survival study in Chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest. 2007;132:373–379. doi: 10.1378/chest.06-2913. - DOI - PubMed

MeSH terms