Novel Screening Strategies for Scleroderma PAH

Novel Screening Strategies for Scleroderma PAH (Pulmonary Arterial Hypertension)

Status

Completed

Phases

Unknown

Study type

Observational

Source

ClinicalTrials.gov

Registry ID

NCT01959815

Enrollment

156

Registered

2013-10-10

Start date

2013-09-26

Completion date

2019-11-06

Last updated

2021-11-15

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Pulmonary Arterial Hypertension, Scleroderma

Brief summary

Patients with scleroderma can develop heart failure due to high blood pressure in the lungs (a condition called pulmonary arterial hypertension). It is important to find pulmonary arterial hypertension early, so that it can be treated before heart failure develops. However, the tests that we now use to find the earliest form of this disease in scleroderma patients are not good enough. This study will examine whether tests performed during exercise can improve our ability to find early pulmonary arterial hypertension. The study will also try to identify genes that are responsible for the development of pulmonary arterial hypertension.

Interventions

None listed

Study design

Observational model

COHORT

Time perspective

PROSPECTIVE

Eligibility

Sex/Gender

ALL

Age

30 Years to No maximum

Healthy volunteers

Yes

Inclusion criteria

* 30 years or older; * diagnosis of limited or diffuse scleroderma (American College of Rheumatology criteria) * for the high risk group, one of the following features: * resting transthoracic echocardiogram showing elevated right-sided pressures within previous 3 months \[tricuspid regurgitation (TR) jet \>2.8 m/s or evidence of right ventricular dysfunction\] * pulmonary function testing (PFT) showing abnormal diffusing capacity of carbon monoxide (DLCO) not due to significant interstitial lung disease (DLCO\<60% predicted or FVC: DLCO ratio \>1.4)

Exclusion criteria

* Pregnancy * prior diagnosis of pulmonary hypertension * treatment with endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclin analogues * previous diagnosis of obstructive lung disease or pulmonary thromboembolic disease * current smoker * significant valvular disease * resting echocardiogram showing left ventricular ejection fraction\<50% within previous 3 months * resting echocardiogram showing significant (greater than Grade I) diastolic dysfunction * pulmonary emboli (past or present).

Design outcomes

Primary

Measure	Time frame
Development of pulmonary arterial hypertension	Two years after enrollment.

Countries

United States

Outcome results