Connective Tissue Disease Patients With Pulmonary Hypertension

NCT05980728 | Not Yet Recruiting

• 1 other identifier: JSPH cohort
• Study Type: observational
• Target: 500
• Locations: 0 countries
• Sites: N/A

Brief Summary

Adult patients with suspected or confirmed Connective Tissue Disease Patients (CTD)With Pulmonary Hypertension(PH)will be recruited. Patients will be approached, consented, have baseline demographics, diagnostics and disease activity measures recorded, and blood taken. The collection of data and biological material will mirror usual clinical practice as far as possible. Subjects will ideally attend further visits at 3, 6 and 12 months to have bloods taken, outcome measures recorded and questionnaires completed.

Conditions

Connective Tissue Diseases; Pulmonary Hypertension

Keywords

prognosis , biomarker, pulmonary hypertension

Outcome Measures

Primary Outcomes (1)

• Death

  number of participants with All cause death

  30 years

Secondary Outcomes (1)

• Clinical worsening

  30 years

Study Arms (3)

Connective Tissue Disease Patients With Pulmonary Hypertension

Connective Tissue Disease Patients With Pulmonary Hypertension

Other: No Intervention

Connective Tissue Disease Patients Without Pulmonary Hypertension

Connective Tissue Disease Patients Without Pulmonary Hypertension

Other: No Intervention

healthy

healthy people

Other: No Intervention

Interventions

No Intervention OTHER

No Intervention

Connective Tissue Disease Patients With Pulmonary Hypertension; Connective Tissue Disease Patients Without Pulmonary Hypertension; healthy

Eligibility Criteria

• Age: 18 Years - 75 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Connective Tissue Disease Patients With Pulmonary Hypertension

You may qualify if:

• Definite diagnosis of CTD included Systemic lupus erythematosus（SLE） diagnosed according to the 2019 The European Alliance of Associations for Rheumatology（EULAR） criteria, primary Sjogren's syndrome (pSS) defined according to the 2016 American College of Rheumatology(ACR) criteria, Systemic Sclerosis(SSc) defined according to the 2013 ACR criteria, mixed connective tissue disease (MCTD) defined by Sharp criteria, and Rheumatoid Arthritis(RA) defined according 2010 ACR criteria of two or more CTD at the same time were defined as having overlap syndrome (OS). Patients who had clinical and serological manifestations suggestive of systemic autoimmune diseases but did not fulfil the classification criteria for CTD were defined as having undifferentiated CTD (UCTD).
• PAH was diagnosed by right heart catheterization was defined as mean Pulmonary Artery Pressure (mPAP)\>20 Millimeters of mercury（mmHg）, Pulmonary Artery Wedge Pressure（PAWP）≤15mmHg, Pulmonary Vascular Resistance（PVR）\>2 Wood

You may not qualify if:

• significant interstitial lung disease or chronic obstructive pulmonary disease;
• left heart disease or congenital heart disease associated with pulmonary hypertension;
• chronic thromboembolic pulmonary hypertension;
• portal hypertension;
• drug or toxin exposure;
• HIV infection;
• any other diseases known to be associated with PAH;
• age\<18 years.

Sponsors & Collaborators

• The First Affiliated Hospital with Nanjing Medical University: lead

Related Publications (3)

• Dai J, Ma L, Zhang Y, Shan L, Li D, Li L, Hu Q, Zhou Z, Sun X, Wang Q, Zhou L. The prognostic value of HFA-PEFF score in connective tissue disease-associated PAH: evidence from a cohort study. BMC Cardiovasc Disord. 2025 Apr 5;25(1):258. doi: 10.1186/s12872-025-04691-y.

  PMID: 40188059 DERIVED

• Sun X, Zhang Y, Liu T, Zhang H, Zu B, Zhou L, Wang Q, Zhang M. Clinical characteristics of primary SS- and overlap SS-associated pulmonary arterial hypertension: a multicentre retrospective study. Rheumatology (Oxford). 2025 Jun 1;64(6):3618-3625. doi: 10.1093/rheumatology/keaf013.

  PMID: 39792031 DERIVED

• Dai J, Liu T, Zhang H, Sun X, Tang Y, Qian W, Zhang Y, Ye H, Shan L, Li L, Du M, Li D, Zhu Y, Ma K, Liu L, Wang Q, Zhou L. Fragmented QRS complex could predict all-cause mortality in patients with connective tissue disease-associated pulmonary arterial hypertension. Rheumatology (Oxford). 2025 Feb 1;64(2):789-797. doi: 10.1093/rheumatology/keae084.

  PMID: 38323656 DERIVED

Biospecimen

Retention: SAMPLES WITH DNA

DNA RNA Peripheral blood mononuclear cells, Serum, Plasma, Urine,Stool

MeSH Terms

Conditions

Connective Tissue Diseases; Hypertension, Pulmonary

Condition Hierarchy (Ancestors)

Skin and Connective Tissue Diseases; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases

Study Officials

• Qiang Wang, MD

  The First Affiliated Hospital with Nanjing Medical University

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Qiang Wang, MD

CONTACT

+862568307588; jerrytortoise@163.com

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 20 Years
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: July 3, 2023
• First Posted: August 8, 2023
• Study Start: November 1, 2023
• Primary Completion (Estimated): September 1, 2027
• Study Completion (Estimated): September 1, 2030
• Last Updated: October 17, 2023

Record last verified: 2023-10

Data Sharing

• IPD Sharing: Will not share