Pulmonary Arterial Hypertension Associated With Connective Tissue Disease, Takayasu Arteritis With Pulmonary Artery Involvement
Conditions
Brief summary
The purpose of this study is to identify the clinical features, management pattern and long-term outcomes of patients with pulmonary arteries involvement in Takayasu's arteritis (TAK-PAI).
Detailed description
The present study intends to conduct a prospective analysis of the clinical data in TAK patients in order to: 1. describe the clinical characteristics and current status of multimodal treatment in patients with TAK-PAI, thereby enhancing the understanding of TAK-PAI; 2. to investigate the risk factors for mortality in patients with TAK-PAI, so as to promote early intervention and reduce mortality. 3. explore factors associated with pulmonary hypertention (PH) in patients with TAK-PAI, so as to facilitate the early identification of patients who may develop PH.
Interventions
None listed
Sponsors
China-Japan Friendship Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Hospitalized between Jan 1, 2016 and Dec 31, 2026 * Diagnosed with TAK according to the modified Ishikawa criteria and/or 1990 American College of Rheumatology criteria and/or 2022 ACR/EULAR criteria * Diagnosed with pulmonary artery involvement using computed tomography pulmonary angiography or transcatheter pulmonary angiography * Patients without aortic or primary branch involvement who nonetheless exhibited both clinical features and computed tomography-confirmed evidence of pulmonary artery involvement (PAI), after exclusion of other diseases causing pulmonary artery stenosis or occlusion
Exclusion criteria
* Patients with PAI caused by non-TAK diseases such as other types of vasculitis, fibrosing mediastinitis, pulmonary artery sarcoma, pulmonary sarcoidosis or chronic thromboembolic pulmonary hypertension
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| All-cause death | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026. |
Secondary
| Measure | Time frame |
|---|---|
| Six-minute walk distance | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026 |
| WHO functional class | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026. |
| N-terminal pro-brain natriuretic peptide level | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026 |
Other
| Measure | Time frame | Description |
|---|---|---|
| Mean pulmonary arterial pressure assessed by right heart catheterization | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026. | Hemodynamic parameters assessed by right heart catheterization in patients with pulmonary hypertension. |
| Pulmonary vascular resistance assessed by right heart catheterization | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026. | Hemodynamic parameters assessed by right heart catheterization in patients with pulmonary hypertension. |
| Cardiac output assessed by right heart catheterization | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026. | Hemodynamic parameters assessed by right heart catheterization in patients with pulmonary hypertension. |
| Cardiac index assessed by right heart catheterization | At 3 months, 6 months, and annually thereafter following enrollment, until December 31, 2026. | Hemodynamic parameters assessed by right heart catheterization in patients with pulmonary hypertension. |
Countries
China
Contacts
Primary ContactWanmu Xie, Doctor
Outcome results
None listed