Connective Tissue Disease Patients With Pulmonary Hypertension

Last updated: October 15, 2023
Sponsor: The First Affiliated Hospital with Nanjing Medical University
Overall Status: Active - Not Recruiting

Phase

N/A

Condition

Pulmonary Arterial Hypertension

Congestive Heart Failure

Musculoskeletal Diseases

Treatment

No Intervention

Clinical Study ID

NCT05980728
JSPH cohort
  • Ages 18-75
  • All Genders
  • Accepts Healthy Volunteers

Study 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.

Eligibility Criteria

Inclusion

Inclusion Criteria:

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

Exclusion

Exclusion Criteria:

  • 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.

Study Design

Total Participants: 500
Treatment Group(s): 1
Primary Treatment: No Intervention
Phase:
Study Start date:
November 01, 2023
Estimated Completion Date:
September 01, 2030