ReviewPulmonary hypertension in systemic lupus erythematosus: prevalence, predictors and diagnostic strategy
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.
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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
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PH, and particularly PAH, is not a major clinical problem in patients with SLE.
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Screening echocardiograms of asymptomatic lupus patients without a clinical suspicion of PH is not recommended.
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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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