Pulmonary hypertension on systemic sclerosis-lupus erythematosus overlap syndrome

Rachdi, I.; Tougorti, M.; Daoud, Fuheid S.; Aydi, Z.; Zoubeidi, H.; Dhaou, B. Ben; Boussema, F.

doi:10.1016/j.ancard.2018.10.012

Cited by 3 publications

(1 citation statement)

References 21 publications

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“…[ 1 ] Pulmonary arterial hypertension (PAH) is a severe manifestation of SSc and SLE with a prevalence rate ranging, respectively, from 5% to 60% and from 0.5% to 17.5%. [ 3 4 ] Sjögren syndrome (SS) is an autoimmune condition which can be of primary and secondary. [ 5 ] SS can be associated with sialadenitis, which is a chronic autoimmune inflammatory exocrinopathy affecting the salivary and lacrimal glands and may occur as a primary or secondary form.…”

Section: Introductionmentioning

confidence: 99%

A case of progressive systemic sclerosis/lupus overlap syndrome: Presenting with parotid swelling

Ramadoss

Swarnalakshmi

Arunachalam

et al. 2021

J Oral Maxillofac Pathol

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Section: Introductionmentioning

confidence: 99%

A case of progressive systemic sclerosis/lupus overlap syndrome: Presenting with parotid swelling

Ramadoss

Swarnalakshmi

Arunachalam

et al. 2021

J Oral Maxillofac Pathol

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Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment

Mukherjee

Konduri

2021

Comprehensive Physiology

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Clinical characteristics and prognosis of connective tissue disease-associated pulmonary arterial hypertension

Xiang

Dong

et al. 2022

Preprint

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Background Pulmonary arterial hypertension (PAH) is a series of clinicopathological syndromes associated with elevated pulmonary arterial pressure caused by various reasons, and is also the most serious complication of connective tissue disease (CTD). This study aims to evaluate the clinical features and prognostic factors of CTD associated PAH (CTD-PAH) patients in the Chinese Han population, and provide a reference basis for improving their prognosis. Methods A total of 392 individuals with CTD between January 2017 and August 2021 were included in this study. They were divided into two main groups: CTD-PAH and CTD-non-PAH. The independent sample t-test and Mann-Whitney U test were used to compare clinical and laboratory findings. Receiver operating characteristic (ROC) curves were converted into binary variables to determine the critical value. A Kaplan-Meier survival analysis was used to compare the survival rates of the two groups. And an analysis of death risk factors was conducted using Cox regression. Results In this study, SLE was the most common underlying disease among Chinese Han patients with CTD-PAH (51.56%). The level of anti-U1-RNP antibodies was significantly higher in the CTD-PAH group as compared to the CTD-non-PAH group (P = 0.001), whereas the levels of anti-SCL-70 antibodies were lower (P < 0.001). CTD patients with higher anti-U1-RNP antibodies levels were at high risk for PAH (P = 0.021), whereas higher levels of anti-U1-RNP have also been linked to decreased mortality in CTD-PAH (P < 0.001). In addition, the anti-SCL-70 antibody was an independent protective factor for CTD-PAH (P = 0.029). The poor prognosis of CTD-PAH was associated with high RDW (P = 0.010), NLR (P < 0.001), CAR (P = 0.001) and pulmonary infection (P = 0.008). Conclusion Patients with CTD who have higher levels of anti-U1-RNP antibodies are at high risk for PAH, whereas higher levels of anti-U1-RNP antibodies have also been linked to decreased mortality. An increase in the anti-SCL-70 antibody levels is an independent protective factor against CTD-PAH. High CAR, NLR, RDW and pulmonary infection are also significant determinants of total mortality in patients with CTD-PAH.

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Pulmonary hypertension on systemic sclerosis-lupus erythematosus overlap syndrome

Cited by 3 publications

References 21 publications

A case of progressive systemic sclerosis/lupus overlap syndrome: Presenting with parotid swelling

A case of progressive systemic sclerosis/lupus overlap syndrome: Presenting with parotid swelling

Pediatric Pulmonary Hypertension: Definitions, Mechanisms, Diagnosis, and Treatment

Clinical characteristics and prognosis of connective tissue disease-associated pulmonary arterial hypertension

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