scholarly journals Pulmonary arterial hypertension in interferonophaties: a case report and a review of the literature

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401986983 ◽  
Author(s):  
A. Trombetta ◽  
S. Ghirardo ◽  
S. Pastore ◽  
A. Tesser ◽  
E. Piscianz ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Kinase Inhibitors ◽  
Janus Kinase ◽  
Type I ◽  
Systemic Lupus ◽  
Janus Kinase Inhibitors ◽  
Pulmonary Arterial

Background Pulmonary arterial hypertension consists in an increase of mean pulmonary arterial pressure (PAPm ≥ 25 mmHg), and may lead to right ventricular failure. Pulmonary arterial hypertension can arise in several disorders, encompassing inflammatory conditions and connective tissue diseases. The occurrence of pulmonary arterial hypertension has recently been reported in monogenic interferonopathies and in systemic lupus erythematosus, highlighting the pathogenic role of type I interferons and paving the way to therapies aimed at inhibiting interferon signaling. Case We describe a 17-year-old boy with DNase II deficiency, presenting a clinical picture with significant overlap with systemic lupus erythematosus. During treatment with the Janus kinase inhibitor ruxolitinib, he developed pulmonary arterial hypertension, raising the question whether it could represent a sign of insufficient disease control or a drug-related adverse event. The disease even worsened after drug withdrawal, but rapidly improved after starting the drug again at higher dosage. Summary and conclusion Pulmonary arterial hypertension can complicate type I interferonopathies. We propose that ruxolitinib was beneficial in this case, but the wider role of Janus kinase inhibitors for the treatment of pulmonary arterial hypertension is not clear. For this reason, a strict cardiologic evaluation must be part of the standard care of subjects with interferonopathies, especially when Janus kinase inhibitors are prescribed.

scholarly journals The Role of Conjunctival Microvasculation Combined with Echocardiography in Evaluating Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Jiangbiao Xiong ◽  
Shujiao Yu ◽  
Ren Liu ◽  
Xia Fang ◽  
Rui Wu
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Walking Distance ◽  
Flow Index ◽  
Systemic Lupus ◽  
Microvascular Changes ◽  
Pulmonary Arterial

Objective. To explore the role of conjunctival microvasculation combined with echocardiography in evaluating the prognosis of pulmonary arterial hypertension in systemic lupus erythematosus (SLE-PAH). Methods. We prospectively compared the conjunctival microvascular changes in 17 SLE-PAH patients and 34 SLE patients without PAH in our hospital from January 2020 to December 2020, and we observed the characteristics of conjunctival microvascular changes in SLE-PAH patients. We analyzed the correlation between the corresponding conjunctival microvascular changes and cardiopulmonary function and evaluated the predictive value of the vessel density (VD) and the microvascular flow index (MFI) of conjunctival microvasculation combined with echocardiography in SLE-PAH. Results. Compared with SLE patients without PAH, the ischemic areas in conjunctival microvasculation were significantly increased in SLE-PAH patients. The VD and MFI of conjunctival microvasculation are significantly correlated with N-terminal prohormone of brain natriuretic peptide and 6-minute walking distance. Combined with the VD and MFI, it can improve the accuracy of echocardiography in assessing the risk of death due to SLE-PAH (94.1% vs. 82.2%). Conclusion. The ischemic area, VD, and MFI of conjunctival microvasculation in SLE-PAH patients can indicate the occurrence of severe SLE-PAH and improve the accuracy of echocardiography in evaluating the prognosis of SLE-PAH.

scholarly journals SAT0240 THE PROGNOSIS OF TWO DISTINCT CLINICAL PHENOTYPES OF SLE-PAH

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1063.2-1063
Author(s):  
J. Wang ◽  
Y. Feng ◽  
Y. Lei ◽  
X. Zhang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Systemic Lupus ◽  
Clinical Phenotypes ◽  
Weighted Score ◽  
Pulmonary Arterial

Background:Based on the characteristics of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH), Sunet alhas put forward a scoring system to distinguish two clinical phenotypes as vasculitic and vasculopathic subtypes[1]. A weighted score ≥2 suggested a vasculitic subtype by combining two factors: The time interval between SLE and PAH diagnosis <2 years and ≥2 years were 1 and 0 point; SLE Disease Activity Index (SLEDAI) >9, 5-9 and <5 were 2, 1, 0 point, respectively. While the vasculitic subtype seemed to have poorer prognosis in Sun’s research, other study has shown controversial result[2].Objectives:To find out the prognosis of two distinct clinical phenotypes of SLE-PAH.Methods:Between 2008 and 2019, a SLE-PAH cohort confirmed by right heart catheterization (RHC) from Guangdong Provincial People’s Hospital was included. Other groups of pulmonary hypertension were excluded. Based on the scoring system, patients were divided into vasculitic (weighted score≥2) and vasculopathic subtypes (weighted score<2). The endpoint was PAH-related mortality. Survival status were confirmed by clinic follow-up data or phone call.Results:A total of 53 SLE-PAH patients were enrolled. The cases of vasculitic and vasculopathic subtype were 14 and 39, respectively. Ten endpoint events occurred. Eight attributed to PAH and the cause could not be traced in two which were still included in study. The pooled 1-, 3-, 5-year survival rates were 85.7%, 78.6%, 65.5% in vasculitic subtype, and 93.9%, 87.5%, 87.5% in vasculopathic subtype, respectively. Kaplan-Meier analysis showed vasculitic subtype tended to have a poorer prognosis than vasculopathic subtype (p=0.16, HR 2.4, 95%CI 0.5-13.8, figure 1).Figure 1.Survival curves for patients with systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) in two distinct subtypes. RHC, Right Heart Catheterization.Conclusion:The prognosis of the two phenotypes of SLE-PAH was statistically indifferent while the vasculitic subtype showed a trend of worse prognosis. Further studies are needed.References:[1]F. Sun, Y. Lei, W. Wu, L. Guo, K. Wang, Z. Chen, W. Xu, X. Wang, T. Li, X. Zhang, S. Ye, Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus, Ann Rheum Dis 78(1) (2019) 148-150.[2]J. Qian, M. Li, J. Zhao, Q. Wang, Z. Tian, X. Zeng, Inflammation in SLE-PAH: good news or not?, Ann Rheum Dis (2018).0:1–2. doi:10.1136/annrheumdis-2018-214605Disclosure of Interests:None declared

Pulmonary Arterial Hypertension Responsive to Immunosuppressive Therapy in Systemic Lupus Erythematosus

Lupus ◽  
1993 ◽  
Vol 2 (6) ◽  
pp. 367-369 ◽  
Author(s):  
Sergio Morelli ◽  
Marcello Giordano ◽  
Paolo De Marzio ◽  
Roberta Priori ◽  
Alessandro Sgreccia ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Pulmonary Arterial

scholarly journals Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction

2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Atiya Dhala
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Connective Tissue Diseases ◽  
Anticardiolipin Antibody ◽  
Current Status ◽  
Systemic Lupus ◽  
Right Heart ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is commonly associated with connective tissue diseases (CTDs) including systemic sclerosis and systemic lupus erythematosus (SLE). The prevalence of PAH in SLE is estimated to be 0.5% to 17.5%. The pathophysiology of PAH involves multiple mechanisms from vasculitis andin-situthrombosis to interstitial pulmonary fibrosis which increases pulmonary vascular resistance, potentially leading to right heart failure. Immune and inflammatory mechanisms may play a significant role in the pathogenesis or progression of PAH in patients with CTDs, establishing a role for anti-inflammatory and immunosuppressive therapies. The leading predictors of PAH in SLE are Raynaud phenomenon, anti-U1RNP antibody, and anticardiolipin antibody positivity. The first-line of diagnostic testing for patients with suspected SLE-associated PAH (SLE-aPAH) involves obtaining a Doppler echocardiogram. Once the diagnosis is confirmed by right heart catheterization, SLE-aPAH patients are generally treated with oxygen, anticoagulants, and vasodilators. Although the prognosis and therapeutic responsiveness of these patients have improved with the addition of intensive immunosuppressive therapies, these treatments are still largely unproven. Recent data put the one-year survival rate for SLE-aPAH patients at 94%. Pregnant women are most at risk of dying due to undiagnosed SLE-aPAH, and screening should be considered essential in this population.

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