Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus

Hachulla, É.; Jaïs, Xavier; Cinquetti, G.; Clerson, Pierre; Rottat, Laurence; Launay, David; Cottin, Vincent; Habib, Gilbert; Prévôt, Grégoire; Chabanne, Céline; Foïs, Eléna; Amoura, Zahir; Mouthon, Luc; Guern, Véronique Le; Montani, David; Simonneau, Gérald; Humbert, Marc; Sobanski, Vincent; Sitbon, Olivier

doi:10.1016/j.chest.2017.08.014

Cited by 81 publications

(84 citation statements)

References 24 publications

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“…A single‐center retrospective study in Japan found cumulative rates free of PAH‐related death were best in patients with a simultaneous diagnosis of PAH and CTD who were treated initially with a combination of glucocorticoids and immunosuppressants, and suggested that patients with PAH and CTD, including SLE, should receive immunosuppressive treatment regimens to achieve better short‐ and long‐term outcomes . Results from the French Pulmonary Hypertension Registry showed a trend for better survival in patients who were receiving hydroxychloroquine at the time of PAH diagnosis, which is similar to our results. Considering the histopathological similarity between PAH and proliferative glomerulonephritis in SLE patients, a combination of glucocorticoids and CYC may be a reasonable and preferable treatment regimen for SLE‐associated PAH .…”

Section: Discussionsupporting

confidence: 86%

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Zhang

Qian

et al. 2019

Int J of Rheum Dis

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Aim: Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)-confirmed SLE-associated PAH and identify risk factors for PAH in SLE patients. Methods: A multicenter, cross-sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry. Baseline data for patients with SLEassociated PAH and SLE patients without PAH were collected and compared. Risk factors for PAH among patients with SLE were identified.

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Section: Discussionsupporting

confidence: 86%

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Zhang

Qian

et al. 2019

Int J of Rheum Dis

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“…However, the availability of advanced therapies of prostanoids, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors has improved outcomes, with a recent registry study showing a 3-year survival rate of SLE-PAH of 89.4%. 32 Outcomes are more favorable compared with systemic sclerosis-associated PAH which has a 3-year survival of only 47%. 33 In SLE, it is generally recommended to give immunosuppression as well as specific PAH therapy to give optimal long-term outcome.…”

Section: Pulmonary Arterial Hypertensionmentioning

confidence: 99%

Pulmonary Complications of Systemic Lupus Erythematosus

Hannah

D’Cruz

2019

Semin Respir Crit Care Med

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Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterized by the production of pathogenic autoantibodies and immune complexes and is responsible for significant morbidity and mortality through a wide range of clinical manifestations which can affect almost any organ. Pulmonary involvement is prevalent and seen in 50 to 70% of SLE patients and may even be the presenting feature in 4 to 5% of patients. By 10 years postdiagnosis, 12% will have accumulated an element of permanent lung damage. Pulmonary complications are broad and include pleural disease, interstitial lung disease (ILD), vasculitis, pulmonary embolism, pulmonary hypertension, large airway disease, shrinking lung syndrome, and infection. Conditions can range mostly from asymptomatic, for example, in mild cases of pleural effusion or obstructive airway disease, to life-threatening disease, for example, in acute lupus pneumonitis or diffuse alveolar hemorrhage. ILD and pulmonary hypertension are both frequently seen in other autoimmune rheumatic diseases such as systemic sclerosis; however, in SLE, they tend to be milder and have a comparatively favorable prognosis. Although collectively pulmonary involvement in SLE is common, the heterogeneity of SLE and rareness of individual complications make clinical trials difficult and treatment is usually based on case series reports and anecdotal experience with various immunosuppressive agents. Some of these immunosuppressive agents such as azathioprine, methotrexate, and cyclophosphamide have also been linked with drug-induced lung injury.

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“…6 Recently, a French registry indicated a high prevalence of anti-SSA/Ro and anti-SSB/La antibodies in SLE patients with PAH compared to those without PAH. 10 In a multicenter cohort, anti-centromere antibody, usually detected in the limited cutaneous subset of SSc, was significantly more prevalent in SSc patients with PAH than those without PAH. 4 3 | PATHOGENESIS…”

Section: Epidemiologymentioning

confidence: 94%

“…Anti‐U1RNP antibody has been shown to be an independent predictor of PAH development among SLE patients and was the most prevalent autoantibody, followed by anti‐SSA/Ro antibody, detected in a Japanese cohort of CTD‐PAH . Recently, a French registry indicated a high prevalence of anti‐SSA/Ro and anti‐SSB/La antibodies in SLE patients with PAH compared to those without PAH . In a multicenter cohort, anti‐centromere antibody, usually detected in the limited cutaneous subset of SSc, was significantly more prevalent in SSc patients with PAH than those without PAH …”

Section: Epidemiologymentioning

confidence: 98%

Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects

Kato

Atsumi

2017

Eur J Clin Investigation

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Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired. K E Y W O R D Sconnective tissue diseases, pulmonary arterial hypertension, pulmonary hypertension, systemic lupus erythematosus, systemic sclerosis | INTRODUCTIONPulmonary hypertension (PH) is an increased blood pressure in pulmonary arteries and affects the right side of the heart, being diagnosed when the blood pressure in the circulation of the lung measured by right heart catheterization (RHC) is 25 mm Hg or greater. PH occurs as a consequence of remodelling and constriction of the pulmonary arteries and arterioles, called pulmonary arterial hypertension (PAH) and classified as group 1 PH; impaired left heart function and subsequent pulmonary vascular congestion, group 2 PH; hypoxia-mediated pulmonary vasoconstriction in the presence of lung diseases, group 3 PH; occlusion of the pulmonary vascular bed caused by thromboembolism, group 4 PH; or unclear multifactorial mechanisms, group 5 PH.1 Of these 5 forms of PH, PAH is of particular clinical significance because of its high mortality if left untreated and the recent advances in specific therapy including endothelin receptor antagonists (ERA), phosphodiesterase type 5 inhibitors (PDE5i), a soluble guanylate cyclase stimulator, prostacyclin analogues and a prostacyclin receptor agonist. Connective tissue diseases (CTD) are the major cause of PAH, occupying around one-fourth of the total PAH population. 2,3 PAH associated with CTD (CTD-PAH), compared with idiopathic PAH (IPAH), has some unique clinical characteristics, such as less favourable outcome, venous and cardiac involvement and response to immunosuppression. This review focuses on these CTD-PAH-specific aspects and discusses how to efficiently detect and diagnose PAH in CTD patients and to effectively treat CTD-PAH.

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Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus

Abstract: Patients with SLE-PAH have an overall 5-year survival rate of 83.9% after the PAH diagnosis. Anti-SSA/SSB antibodies may be a risk factor for PAH, and the presence of anti-U1-RNP antibodies appears to be a protective factor regarding survival.

Cited by 81 publications

References 24 publications

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Pulmonary arterial hypertension in systemic lupus erythematosus based on a CSTAR‐PAH study: Baseline characteristics and risk factors

Pulmonary Complications of Systemic Lupus Erythematosus

Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects

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