Connective Tissue Disease Patients With Pulmonary Hypertension

N/ANot Yet RecruitingNCT05980728

The First Affiliated Hospital with Nanjing Medical University500 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

500

Conditions

Connective Tissue Diseases Pulmonary Hypertension

Interventions

No InterventionOTHER

No Intervention

Eligibility

Medical Language ↔ Plain English

Inclusion Criteria: * 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 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.

Outcomes

Primary Outcomes

Death

number of participants with All cause death

Time frame: 30 years

Secondary Outcomes

Clinical worsening

number of participants with Hospitalization for worsening pulmonary arterial hypertension(PAH),lung or heart/lung transplant) or unsatisfactory long-term clinical response (the 6-minute walk distance decreased \> 15% from baseline and World Health Organization(WHO) functional class III/IV symptoms assessed at two clinic visits separated by \> 6 months after sufficient PAH targeted drug treatment.