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Sobanski, Vincent; Giovannelli, Jonathan; Denton, Christopher P.; Coghlan, J G

doi:10.1002/art.39651

Cited by 3 publications

(1 citation statement)

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“…In a recently published registry study from France, the 3- and 5-year overall survival rates after PAH diagnosis were 89.4% and 83.9%, respectively [18]. A UK SLE-PAH cohort study reported a similar result, with an 85% survival rate at 5 years [18, 26]. A Chinese study of 111 SLE-PAH patients described 3- and 5-year survival rates of 81.3% and 61.0%, respectively [27].…”

Section: Discussionmentioning

confidence: 93%

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

et al. 2019

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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%.

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Section: Discussionmentioning

confidence: 93%

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

et al. 2019

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Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus

Hachulla

Jaïs

Cinquetti

et al. 2018

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Pulmonary arterial hypertension associated with primary Sjögren's syndrome: a multicentre cohort study from China

Wang

et al. 2020

Eur Respir J

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ObjectivesPrimary Sjogren's syndrome (pSS) is an important cause of pulmonary arterial hypertension (PAH), which remains insufficiently studied and needs attention. This study aimed to investigate the clinical characteristics, risk factors, prognosis and risk assessment of pSS-PAH.MethodsWe established a multicentre cohort of pSS-PAH diagnosed by right heart catheterisation (RHC). The case-control study was conducted with pSS-non PAH patients as a control group to identify the risk factors for PAH. In the cohort study, survival was calculated, and risk assessment was performed at both baseline and follow-up visits.ResultsIn total, 103 patients with pSS-PAH were enrolled, with 526 pSS-non PAH patients as controls. The presence of anti-SSB (p<0.001, OR=4.095) and anti-U1RNP antibodies (p<0.001, OR=29.518), the age of pSS onset (p<0.001, OR=0.651) and the positivity of corneal staining (p=0.003, OR=0.409) were identified as independent risk factors for PAH. The 1-, 3- and 5-year survival rates were 94.0%, 88.8% and 79.0%, respectively. Cardiac index (p=0.010, HR=0.161), pulmonary vascular resistance (p=0.016, HR=1.105) and Sjogren's syndrome disease damage index (SSDDI, p=0.006, HR=1.570) were identified as potential predictors of death in pSS-PAH. Long-term outcomes were improved in patients in the low-risk category at baseline (p=0.002) and follow-up (p<0.0001).ConclusionThe routine screening of PAH is suggested in pSS patients with early onset and positivity for anti-SSB or anti-U1RNP antibodies. Patients' prognosis might be improved by improving reserved cardiopulmonary function, by achieving a damage-free state and especially by achieving low-risk category, which supports the treat-to-target strategy for pSS-PAH.

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Cited by 3 publications

References 7 publications

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

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

Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus

Pulmonary arterial hypertension associated with primary Sjögren's syndrome: a multicentre cohort study from China

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