Nailfold capillaroscopy as a risk factor for pulmonary arterial hypertension in systemic lupus erythematosus patients

Abstract

Background: Pulmonary arterial hypertension (PAH) is a rare and severe complication of systemic lupus erythematosus (SLE). This study aimed to evaluate clinical and laboratory risk factors associated with PAH in SLE patients. Methods: This was a retrospective case-control study in which patients with SLE with PAH (SLE-PAH) confirmed by right heart catheterization (RHC) were compared with SLE patients without PAH. Clinical and demographic variables related to SLE and PAH and nailfold capillaroscopy were evaluated by reviewing the medical records of the patients. Results: Twenty-one patients with SLE-PAH and 44 patients with SLE without PAH matched for sex and disease duration were included. The scleroderma (SD) pattern on nailfold capillaroscopy was more frequently found in patients with SLE-PAH than in those without PAH (56.3% versus 15.9%, respectively, p = 0.002). By univariate analysis, Raynaud's phenomenon, history of abortion, and SD pattern on capillaroscopy were associated with PAH. Arthritis was a protective factor for PAH development. Multivariate analysis showed that the SD pattern on capillaroscopy was the only variable associated with a significantly higher risk of PAH, with an odds ratio of 6.393 (95% confidence interval, 1.530-26.716; p = 0.011). Conclusion: In this study, SD pattern was associated with a 6.3-fold increased risk for PAH development in SLE patients, suggesting that nailfold capillaroscopy might be useful as a screening method to identify SLE patients with a high risk of developing this severe complication.

Nailfold Capillaroscopy in Systemic Sclerosis Patients with and without Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis

“…These findings have later been confirmed by other studies, which additionally reported a significant increase in capillary loop width in idiopathic PAH patients [36,47]. Similar patterns of capillaroscopic abnormalities have been reported in chronic thromboembolic pulmonary hypertension [47] and in connective tissue disease-related PAH beyond SSc [49]. For example, the incidence of scleroderma pattern as well as high vascular deletion…”

“…Similar patterns of capillaroscopic abnormalities have been reported in chronic thromboembolic pulmonary hypertension [47] and in connective tissue disease-related PAH beyond SSc [49]. For example, the incidence of scleroderma pattern as well as high vascular deletion score were established as independent predictors of PAH in systemic lupus erythematous patients [49]. Recently, a cross-sectional study showed that patients with Eisenmenger syndrome had a reduced capillary density, an increased loop width and more abnormal capillaries than ageand sex-matched healthy controls.…”

“…These findings are consistent with the results of our study, suggesting that a higher degree of peripheral nailfold microangiopathy is more common in SSc-PAH, supporting the hypothesis that peripheral microvascular changes may parallel with similar abnormalities in the pulmonary vascular tree. Interestingly, changes in peripheral microcirculation have also been observed in other forms of pulmonary hypertension [35,36,[47][48][49][50]. Hofstee et al suggested that capillary density is significantly reduced in individuals with idiopathic PAH compared to healthy controls and inversely correlated with the severity of idiopathic PAH [35].…”

Peripheral Microangiopathy in Pre-capillary Pulmonary Hypertension: A Nailfold Video Capillaroscopy Prospective Study

“…It has been originally used in the assessment of Raynaud's phenomenon and the diagnosis of systemic sclerosis (SSc) [8], in which NVC changes may serve as an early prognostic marker that determines the risk of developing PAH [9][10][11]. Moreover, few NVC studies demonstrated evidence of peripheral microvascular dysfunction in patients with idiopathic PAH (IPAH), PAH associated with SSc and systemic lupus erythematosus (SLE), suggesting that changes in peripheral microcirculation may parallel pulmonary microangiopathy in patients with PAH [12][13][14][15]. However, no studies have assessed capillary rarefaction across the various types of precapillary PH including CTEPH and its potential correlations with indices of disease severity.…”

Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study

“…It has been originally used in the assessment of Raynaud's phenomenon and the diagnosis of systemic sclerosis (SSc) [8], in which NVC changes may serve as an early prognostic marker that determines the risk of developing pulmonary arterial hypertension (PAH) [9][10][11]. Moreover, a few NVC studies demonstrated evidence of a more severe peripheral microvascular dysfunction in patients with PAH associated with SSc, compared to SSc individuals without PAH, suggesting that changes in peripheral microcirculation may parallel pulmonary microangiopathy in patients with PAH [6,[12][13][14][15]. However, there are no data regarding capillary rarefaction across the various types of precapillary PH including CTEPH, whistle a handful of studies have reported on the presence of peripheral vasculopathy in patients with idiopathic PAH (IPAH) [12,15].…”