RecruitingPhase 2NCT04797286

Sildenafil for Early Pulmonary Vascular Disease in Scleroderma

Sponsor

Johns Hopkins University

Enrollment

30 participants

Start Date

Sep 20, 2021

Study Type

INTERVENTIONAL

Conditions

Scleroderma Mildly Elevated Pulmonary Pressures

Summary

This is a Phase II randomized, double-blind, placebo-controlled trial of sildenafil in men and women with Scleroderma with mildly elevated pulmonary pressures (SSc-MEP) to determine whether sildenafil may be an effective treatment for SSc-MEP.

Eligibility

Min Age: 18 Years

Inclusion Criteria6

Previous documentation of mean pulmonary artery pressure between 21 and 24 mm Hg with a pulmonary capillary wedge pressure (or left ventricular end-diastolic pressure) ≤ 15 mm Hg within six months before study entry.
Diagnosis of SSc according to 2013 American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) classification criteria.
Pulmonary function tests with forced expiratory volume in one second/forced vital capacity (FEV1/FVC) >50% AND either a) total lung capacity (TLC) or forced vital capacity (FVC) > 70% predicted or b) TLC or FVC between 60% and 70% predicted with no more than mild interstitial lung disease on computerized tomography scan of the chest on studies obtained within 6 months of enrollment.
Ventilation perfusion scan or computed tomography with intravenous contrast (CT angiogram) without evidence of chronic thromboembolism at anytime before study entry.
Ability to perform six minute walk testing without significant limitations in musculoskeletal function or coordination.
Informed consent.

Exclusion Criteria11

World Health Organization (WHO) Class IV functional status.
Systolic blood pressure less than 90 mmHg at screening visit prior to enrollment.
Clinically significant untreated sleep apnea.
Left-sided valvular disease (more than moderate mitral valve stenosis or insufficiency or aortic stenosis or insufficiency), pulmonary artery or valve stenosis, or ejection fraction < 45% on most recent echocardiography (within 1 year).
Use of Pulmonary Arterial Hypertension (PAH) therapy (prostacyclin analogues, endothelin-1 receptor antagonists,phosphodiesterase-5 inhibitors, riociguat, selexipag) within the past 3 months.
Hospitalized or acutely ill.
Renal failure (creatinine above 2.0) at screening visit.
Enrollment in a clinical trial or concurrent use of another investigational drug (non FDA approved) or device therapy within 30 days of screening visit.
Age < 18.
Currently pregnant.
Current use of nitrates.

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Interventions

DRUGSildenafil

Sildenafil 20 mg three times a day. This is the approved dose for the treatment of pulmonary arterial hypertension. It is being studied in this trial with a population who has mildly elevated pulmonary pressures.

OTHERPlacebo

Oral pill placebo.