Last update 01 Nov 2024

Pulmonary arterial hypertension associated with connective tissue disease

Last update 01 Nov 2024

Basic Info

Synonyms

PAH associated with connective tissue disease, Pulmonary arterial hypertension associated with connective tissue disease, Pulmonary arterial hypertension associated with connective tissue disease (disorder)

+ [1]

Introduction

A form of pulmonary arterial hypertension (PAH) characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of a connective tissue disease.

Drugs associated with Pulmonary arterial hypertension associated with connective tissue disease

XENON XE 129 hyperpolarized

Target-

Mechanism

Magnetic resonance imaging enhancers

Active Org.

Duke University [+4]

Originator Org.

Polarean, Inc.

Active Indication

Contrast agents [+15]

Inactive Indication-

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date23 Dec 2022

Riociguat

Target

sGC

Mechanism

sGC stimulants

Active Org.

Bayer AG [+5]

Originator Org.

Bayer AG

Active Indication

Hypertension, Pulmonary [+9]

Inactive Indication

Coronary Artery Disease [+11]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date08 Oct 2013

Dimethyl fumarate

Target

Nrf2

Mechanism

Nrf2 stimulants

Active Org.

Biogen Netherlands BV [+8]

Originator Org.

Biogen, Inc.

Active Indication

Plaque psoriasis [+4]

Inactive Indication

Flushing [+8]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date27 Mar 2013

Clinical Trials associated with Pulmonary arterial hypertension associated with connective tissue disease

CTR20243649 / RecruitingNot Applicable

波生坦分散片人体生物等效性研究

[Translation] Study on the bioequivalence of bosentan dispersible tablets in healthy volunteers

本试验旨在研究单次空腹和餐后口服江苏天士力帝益药业有限公司研制、生产的波生坦分散片（32 mg（以C27H29N5O6S计））的药代动力学特征；以Actelion Pharmaceuticals Ltd持证、Patheon Inc.生产的波生坦分散片（Tracleer®，32 mg（以C27H29N5O6S计））为参比制剂，比较两制剂中药动学参数Cmax、AUC0-t、AUC0-∞，评价两制剂的人体生物等效性。

[Translation]

This study aimed to investigate the pharmacokinetic characteristics of bosentan dispersible tablets (32 mg (calculated as C27H29N5O6S)) developed and produced by Jiangsu Tasly Diyi Pharmaceutical Co., Ltd. after single oral administration on an empty stomach or after a meal; using bosentan dispersible tablets (Tracleer®, 32 mg (calculated as C27H29N5O6S)) licensed by Actelion Pharmaceuticals Ltd and produced by Patheon Inc. as the reference preparation, to compare the pharmacokinetic parameters Cmax, AUC0-t, and AUC0-∞ of the two preparations, and to evaluate the bioequivalence of the two preparations in humans.

Start Date18 Sep 2024

Sponsor / Collaborator

Jiangsu Tasly Diyi Pharmaceutical Co., Ltd.

NCT06104228 / RecruitingPhase 2IIT

129 Xenon MRI As a Biomarker for Diagnosis and Response to Therapy in Pulmonary Arterial Hypertension (PAH)

The overall study objectives outlined in this study are to derive 129Xe MRI pulmonary vascular biomarker signatures that differentiate common subtypes of PAH and to determine the ability of 129Xe MRI to longitudinally monitor disease progression and response to therapy in PAH, with the aid of additional assessments, such as labs, echocardiography, and six-minute walk distance (6MWD).

Start Date12 Aug 2024

Sponsor / Collaborator

Duke University

[+1]

NCT05462574 / RecruitingNot ApplicableIIT

Clinical and Mechanistic Understanding of Right Ventricular Steatosis in Pulmonary Arterial Hypertension (PAH)

The investigators propose to study the relationship between right ventricle (RV) steatosis and RV function, exercise capacity, and outcomes in humans with pulmonary arterial hypertension (PAH) and to identify potential drivers of lipid accumulation.

Start Date17 Jan 2023

Sponsor / Collaborator

Vanderbilt University Medical Center

[+1]

100 Clinical Results associated with Pulmonary arterial hypertension associated with connective tissue disease

100 Translational Medicine associated with Pulmonary arterial hypertension associated with connective tissue disease

0 Patents (Medical) associated with Pulmonary arterial hypertension associated with connective tissue disease

276

Literatures (Medical) associated with Pulmonary arterial hypertension associated with connective tissue disease

01 Nov 2024·Thrombosis Research

The role of anticoagulation on the long-term survival of patients with pulmonary arterial hypertension: A meta-analysis of 15 cohort studies

Article

Author: Lin, Lan ; Chen, Xiangqi ; Lin, Qiong ; Zhang, Hong ; Zhai, Zhenguo ; Xue, Dan ; Hong, Zhang ; Chen, Xinwang

INTRODUCTIONAnticoagulation was once recommended for patients with pulmonary arterial hypertension (PAH). However, its survival benefit still remained controversial. We performed a meta-analysis to evaluate the effect of anticoagulation on the long-term survival of PAH patients.METHODSThe PubMed, EMBASE, Web of Science, and WanFang electronic database were searched for eligible studies. Pooled hazard ratios (HRs) with 95 % confidence intervals (CIs) were calculated for effect estimate regarding anticoagulation on the survival of PAH patients.RESULTSFifteen cohort studies involving 4266 PAH patients were included. Approximately 45.8 % patients received anticoagulation. The mean follow-up period ranged from 2.1 to 14 years. Anticoagulation had a tendency to, however, did not significantly reduce mortality of PAH patients (HR: 0.86, 95 % CI: 0.73-1.02). In subgroup analysis, anticoagulation decreased the mortality risk as analyzed from retrospective studies (HR: 0.80, 95 % CI: 0.65-0.98), but not prospective studies (HR: 0.95, 95 % CI: 0.70-1.29). For both idiopathic PAH (IPAH) and connective tissue disease associated PAH (CTD-PAH), anticoagulation therapy did not significantly improve the long-term survival rate (HR: 0.83, 95 % CI: 0.65-1.07, and HR: 1.05, 95 % CI: 0.77-1.42, respectively), and this result remained unchanged when pooling data from either retrospective or prospective studies. Further analysis showed that anticoagulation had no advantage in reducing mortality in patients with systemic sclerosis associated PAH, systemic erythematosus lupus related PAH (free of antiphospholipid syndrome), or CTD-PAH of non-specified etiology.CONCLUSIONAnticoagulation may not reduce the long-term mortality of PAH patients, including those with IPAH and CTD-PAH. In the management of PAH, anticoagulants should be prescribed with caution before comprehensive risk to benefit evaluation. Larger and more vigorously designed controlled trials are warranted.

15 Oct 2024·Modern Rheumatology

Patient characteristics, diagnostic testing, and initial treatment profiles of patients with connective tissue disease-associated pulmonary arterial hypertension using a Japanese claims database

Article

Author: Hayashi, Yasuhiro ; Omura, Junichi ; Jinnai, Tatsunori ; Tokushige, Natsuko ; Atsumi, Tatsuya

ABSTRACTObjectivesTo investigate the diagnostic and therapeutic landscape for patients with connective tissue disease (CTD) and CTD-associated pulmonary arterial hypertension (CTD-PAH) in acute-care general hospitals in Japan.MethodsWe conducted a retrospective cohort study by analysing the Medical Data Vision (MDV) database from April 2008 to September 2020. CTD patients who prescribed immunosuppressants were included in cohort 1, and CTD-PAH patients extracted from cohort 1 were included in cohort 2. Patient characteristics, diagnostic screening frequencies for PAH, and initial PAH-specific treatment patterns were assessed.ResultsOverall, 16,648 patients with CTD and 81 patients with CTD-PAH were included in cohorts 1 and 2, respectively. The frequencies of screening tests for PAH, including brain natriuretic peptide (BNP), transthoracic echocardiogram (TTE), and ‘diffusing capacity’ of the ‘lungs for carbon monoxide’ (DLCO), among CTD patients were 0.7, 0.3, and 0.1 tests/person-year, respectively. The most common initial PAH-specific treatment therapy was monotherapy (87.7%), followed by dual therapy (7.4%) and triple therapy (2.5%).ConclusionThis is the first study to describe the patient flow from PAH diagnosis to initial PAH-specific treatment for real-world patients who were followed regularly due to CTD in Japanese clinical practice.

01 Oct 2024·ESC Heart Failure

The prognostic value of right ventricular outflow tract velocity time integral in patients with pulmonary hypertension

Article

Author: Kumral, Zeynep ; Colak, Ayse ; Kis, Mehmet ; Ozpelit, Ebru ; Simsek, Gokcen Omeroglu ; Sezgin, Dilek ; Yildirim, Tuba Demirci ; Akdeniz, Bahri

AbstractAimsRight ventricular (RV) failure is one of the leading causes of death in patients with pulmonary hypertension (PH). Conventional echocardiographic parameters are not included in risk stratification and follow‐up for prognostic assessment due to PH's diverse nature and the RV's complex geometry. RV outflow tract velocity time integral (RVOT VTI) is a simple, non‐invasive estimate of pulmonary flow and an echocardiographic surrogate of RV stroke volume. In this study, we aimed to define the prognostic value of RVOT VTI in PH patients.MethodsSixty‐three subjects with idiopathic PAH (IPAH) (n = 23), connective tissue disease‐associated PAH (CTD‐associated PAH) (n = 19) and chronic thromboembolic pulmonary hypertension (CTEPH) (n = 21) were retrospectively included. A comprehensive two‐dimensional echocardiographic evaluation, including RVOT‐VTI measurement, was performed during the follow‐up and the New York Heart Association functional class (NYHA FC), 6 min walk distance (6MWD) and brain natriuretic peptide (BNP) levels were recorded.ResultsThe median age of the whole cohort was 63 years (52–68), and 47 (74.6%) of the patients were women. The median follow‐up period was 20 months (11–33), and 20 (31.7%) patients died in this period. BNP values were higher [317 (210–641) vs 161 (47–466), P = 0.02], and 6MWD values were lower [197.5 ± 89.5 vs 339 ± 146.3, P < 0.0001] in the non‐survivor group, and the non‐survivor group had a worse NYHA‐FC (P = 0.02). Among echocardiographic data, tricuspid annular plane systolic excursion (TAPSE) (15.4 ± 4.8 vs 18.6 ± 4.2, P = 0.01) and RVOT VTI (11.9 ± 4.1 vs 17.2 ± 4.3, P < 0.0001) values were lower whereas right atrial area (RAA) (26.9 ± 10.1 vs 22.2 ± 7.1, P = 0.04) values were higher in the non‐survivor group. The area under curve of the RVOT VTI for predicting mortality was 0.82 [95% confidence interval (CI) 0.715–0.940, P < 0.0001], and the best cut‐off value was 14.7 cm with a sensitivity of 80% and specificity of 77%. Survival was significantly lower in subjects with RVOT VTI ≤ 14.7 cm (log‐rank P < 0.0001). Survival rates for patients with RVOT VTI ≤ 14.7 cm were 70% at 1 year, 50% at 2 years, %29 at 3 years and 21% at 5 years. The univariate determinants of all‐cause mortality were BNP [hazard ratio (HR) 1.001 (1.001–1.002), P = 0.001], 6MWD [HR 0.994 (0.990–0.999), P = 0.012] and NYHA‐FC III–IV [HR 3.335 (1.103–10.083), P = 0.03], TAPSE [HR 0.838 (0.775–0.929), P = 0.001], RAA [HR 1.072 (1.013–1.135), P = 0.016] and RVOT VTI [HR 0.819 (0.740–0.906), P < 0.0001]. RVOT VTI was found to be the only independent determinant of mortality [HR 0.857 (0.766–0.960), P = 0.008].ConclusionsThe decreased RVOT VTI predicts mortality in patients with PH and each 1 mm decrease in RVOT VTI increases the risk of mortality by 14.3%. This parameter might serve as an additional parameter in the follow‐up of these patients especially when 6MWD and NYHA‐FC could not be determined.

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Pulmonary arterial hypertension associated with connective tissue disease

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