Elsevier

Autoimmunity Reviews

Volume 12, Issue 3, January 2013, Pages 410-415
Autoimmunity Reviews

Review
Pulmonary hypertension in systemic lupus erythematosus: prevalence, predictors and diagnostic strategy

https://doi.org/10.1016/j.autrev.2012.07.010Get rights and content

Abstract

Objectives

To investigate the prevalence and predictors of pulmonary hypertension (PH) in patients with systemic lupus erythematosus (SLE) and to validate a diagnostic strategy.

Methods

245 patients with SLE entered a screening program. Possible PH was defined as two consecutive systolic pulmonary arterial pressure (PAP) values  40 mm Hg by echocardiography. The subsequent diagnostic procedure, including right heart catheterization if needed, confirmed or excluded the diagnosis of PH secondary to cardiopulmonary disease or SLE-related pulmonary arterial hypertension (PAH). Independent predictors of PH were identified by multivariant multiple linear or logistic regression models. The sensitivity (S), specificity (SP), positive (PPV) and negative predictive values (NPV) were calculated for different screening cutoff values.

Results

88% patients were women. The mean (SD) age at the time of enrolment was 45 (16) years. 12 cases of PH were detected, all secondary, with a resulting prevalence of 5%. Two consecutive echocardiographic PAP measurements  40 mm Hg performed best as the cutoff point for screening (S 100%, SP 97%, PPV 70, NPV 100), as compared with single PAP measurements  30 mm Hg or ≥ 40 mm Hg The age at the time of enrolment was the only variable independently associated with PAP values (p = 0.0001), with the SLICC damage index score showing a borderline association (p = 0.08). Only the age at the time of enrolment showed an independent association with PH (OR 1.10, 95% CI 1.06–1.17).

Conclusion

We found a low prevalence of PH. Screening echocardiograms in asymptomatic lupus patients are thus not recommended. Two consecutive PAP values  40 mm Hg by echocardiogram is the best screening cutoff for starting investigations in SLE patients with suspected PH.

Introduction

Pulmonary hypertension (PH) is characterized by the progressive increase in pulmonary vascular resistance, eventually leading to right ventricular failure. It is defined as a resting mean pulmonary arterial pressure (mPAP)  25 mm Hg measured by right heart catheterization. For the diagnosis of the subclass known as pulmonary arterial hypertension (PAH), a measured pulmonary arterial wedge pressure < 15 mm Hg is required [1], [2]. Transthoracic Doppler echocardiography is the most widely used screening test for PH in the presence of clinical suspicion and/or predisposing conditions [2], despite the fact that echocardiography can miss asymptomatic patients with early mild PH [3]. Right heart catheterization is considered the definitive diagnostic method [2], [3].

The clinical classification of PH was last updated in 2008 [3], with PAH associated with systemic autoimmune diseases being included within the Group I. Indeed, PAH is a recognized complication of systemic sclerosis, and, much less frequently, systemic lupus erythematosus (SLE) [4], [5]. In addition, PH in SLE can be secondary to chronic thromboembolic disease or cardiopulmonary complications [3], [5].

PH has been identified as a predictor of morbidity and mortality in SLE [6], [7]. Several variables have been proposed as potential risk factors for PH: Raynaud's phenomenon [6], [8], [9], [10], antiphospholipid antibodies [11], [12], antiU1-RNP antibodies [8], [13] and disease activity [8], [14]. The prevalence of PH is variable across the different lupus cohorts, ranging from less than 2% [15] to 43% [16]. Such discordant results may actually reflect the varying definitions of PH and the differences in the diagnostic protocols. In fact, there are no standardized guidelines for the screening of PH in SLE.

Section snippets

Study design and objectives

This cross-sectional study has the main objective of establishing the prevalence of PH and PAH in an observational longitudinal cohort of SLE patients. The secondary objectives were to identify potential predictors for PH and to validate a screening program to detect PH in lupus patients.

Patients

The Lupus-Cruces cohort is a longitudinal observational cohort joining SLE patients fulfilling the 1997 classification criteria of the American College of Rheumatology [17]. At enrolment, all patients signed an

Clinical characteristics of the cohort

Two hundred forty five patients were included in the study (Table 1). Eighty eight percent were women, Caucasians accounting for 99.2% of the cohort. The mean age (SD) at SLE diagnosis was 34 (15) years. The mean time of follow up to the time of enrolment in the screening program was 10.7 (8) years. Previous SLE manifestations and treatments received are shown in Table 1.

Prevalence of PH

The estimated PAP (SD) obtained in the first echocardiography was 32.4 (7.4) mm Hg. One hundred twenty four patients (50%) had

Discussion

PH can complicate the course of SLE. Chronic cardiopulmonary conditions can increase PAP, and PAH, i.e., without concomitant cardiac or respiratory underlying disease, can also happen in SLE [20].

The availability of echocardiography has facilitated the screening of PH, being a non-invasive, no harming and reproducible technique. However, the values obtained by echocardiogram in individual patients can overestimate the real PAP values [21]. On the other hand, mild cases of PH can be overlooked

Take-home messages

  • PH, and particularly PAH, is not a major clinical problem in patients with SLE.

  • Screening echocardiograms of asymptomatic lupus patients without a clinical suspicion of PH is not recommended.

  • Two consecutive PAP values  40 mm Hg estimated by echocardiogram are the best initial procedure to decide further testing for the diagnosis of PH.

Contributorship statement

Conception and design: GR-I, MG, M-VE, CA.

Analysis and interpretation of data: GR-I, MG, IV.

Drafting the article or revising it critically for important intellectual content: All authors.

Final approval of the version to be published: All authors.

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