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CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated ILD and PH (CRUSADE)

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ClinicalTrials.gov Identifier: NCT03726398
Recruitment Status : Recruiting
First Posted : October 31, 2018
Last Update Posted : April 2, 2019
Sponsor:
Collaborators:
National Jewish Health
University of Pittsburgh
Information provided by (Responsible Party):
Franz Rischard, DO, University of Arizona

Brief Summary:
Patients with interstitial lung disease (ILD) and scleroderma who develop pulmonary hypertension (PH) do not fit well into the current classification system and treatments for pulmonary hypertension. This study aims to better understand patients with ILD-PH and scleroderma and to determine if treatment with Macitentan is beneficial.

Condition or disease Intervention/treatment Phase
Interstitial Lung Disease Scleroderma Pulmonary Hypertension Drug: Opsumit 10 Mg Tablet Phase 2 Phase 3

Detailed Description:

The investigators aim to use pressure-volume loop derived right ventriculo-vascular coupling, pulmonary impedance, and invasive cardiopulmonary exercise testing (CPET) to:

  1. Comprehensively phenotype patients with scleroderma ILD-PH and pulmonary vascular exercise limitation (PVL) relative to scleroderma ILD-PH without PVL.
  2. Compare the efficacy of chronic Macitentan therapy in improving 1) right ventricular hemodynamics 2) exercise capacity and 3) symptoms in scleroderma ILD-PH patients with and without PVL.

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Study Type : Interventional  (Clinical Trial)
Estimated Enrollment : 26 participants
Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Prevention
Official Title: CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study
Actual Study Start Date : September 1, 2018
Estimated Primary Completion Date : January 31, 2020
Estimated Study Completion Date : December 1, 2020


Arm Intervention/treatment
Experimental: Opsumit
Opsumit 10 mg tablet by mouth once daily
Drug: Opsumit 10 Mg Tablet
Oral tablet taken once daily
Other Name: Macitentan




Primary Outcome Measures :
  1. Change in exercise pulmonary vascular resistance (PVR) [ Time Frame: Baseline to 6 months ]

Secondary Outcome Measures :
  1. Change in right ventricular pulmonary vascular hemodynamic coupling (RVPA). [ Time Frame: Baseline to 6 months ]
  2. Change in maximal oxygen consumption (V02 max). [ Time Frame: Baseline to 6 months ]
  3. Change in pulmonary impedance. [ Time Frame: Baseline to 6 months ]


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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • Patients who have scleroderma ILD will be defined as having a total lung capacity of less than 80% predicted and CT evidence of fibrosis. The degree of fibrosis will be scored by a radiologist using the CT comparative scoring method of Wells et al (13).
  • Pulmonary Hypertension (PH) as defined as resting mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg with a wedge pressure of ≤ 15 mmHg during right heart catheterization.
  • Stable ILD as evident by a stable FEV1 and FVC for 3 months prior to the initiation of the study, and be pulmonary arterial hypertension (PAH)-targeted treatment naïve.

Exclusion Criteria:

  • Patients with a left ventricular ejection fraction <50% or clinical, echocardiographic, and/or catheterization data consistent with heart failure with preserved ejection fraction (HFpEF) and/or moderate-severe aortic or mitral valve abnormality
  • Patients with severe restrictive lung disease (FVC<40% predicted) and/or obstructive lung disease (FEV1 <55% predicted and FEV1/FVC <70%).
  • Patients with radiographic combined pulmonary fibrosis/emphysema (CPFE) will also be excluded if imaging shows predominant emphysema and/or obstruction is moderately severe (FEV1<30%)
  • Patients with a history of pulmonary embolism within the last three months or evidence of chronic pulmonary embolism.
  • Patients with a known contraindication to right heart catheterization.
  • Patients whom have received active or previous pulmonary vasoactive medication within the previous 12 weeks.
  • Patients with a contraindication to exercise testing based on American Heart Association/American College of Cardiology (AHA/ACC) guidelines.
  • PAH associated with significant venous or capillary involvement (PCWP > 15 mmHg), known pulmonary veno-occlusive disease, and pulmonary capillary hemangiomatosis.
  • Persistent pulmonary hypertension of the newborn.
  • Pulmonary Hypertension belonging to groups 2 to 5 of the Venice classification.
  • Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C.
  • Estimated creatinine clearance < 30 mL/min
  • Serum aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT) > 1.5 times the upper limit of normal.
  • Hemoglobin < 75% of the lower limit of the normal range.
  • Systolic blood pressure < 100 mmHg.
  • Acute or chronic physical impairment (other than dyspnea), limiting the ability to comply with study requirements.
  • Pregnant or breast-feeding.
  • Known concomitant life-threatening disease with a life expectancy < 12 months.
  • Body weight < 40 kg.
  • Any condition that prevents compliance with the protocol or adherence to therapy.
  • Treatment with endothelin receptor antagonists (ERAs) within 3 months prior to randomization.
  • Systemic treatment within 4 week prior to randomization with cyclosporine A or tacrolimus, everolimus, sirolimus (calcineurin or mammalian target of rapamycin (mTOR) inhibitors).
  • Treatment with cytochrome P3A (CYP3A) inducers within 4 weeks prior to randomization
  • Known hypersensitivity to drugs of the same class as the study drug, or any of their excipients.
  • Planned treatment, or treatment, with another investigational drug within 1 month prior to randomization

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03726398


Contacts
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Contact: Valerie Bloss, MS 520-626-8000 vbloss@email.arizona.edu
Contact: Maria Gordon, MS 520-626-8000 mgordon@email.arizona.edu

Locations
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United States, Arizona
University of Arizona Recruiting
Tucson, Arizona, United States, 85724
Contact: Valerie Bloss, MS    520-626-8000    vbloss@email.arizona.edu   
Principal Investigator: Franz P. Rischard, MD         
Sponsors and Collaborators
Franz Rischard, DO
National Jewish Health
University of Pittsburgh
Investigators
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Principal Investigator: Franz P. Rischard, DO University of Arizona

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Responsible Party: Franz Rischard, DO, Associate Professor, Medicine, University of Arizona
ClinicalTrials.gov Identifier: NCT03726398     History of Changes
Other Study ID Numbers: IIS-02801
First Posted: October 31, 2018    Key Record Dates
Last Update Posted: April 2, 2019
Last Verified: March 2019

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Studies a U.S. FDA-regulated Drug Product: Yes
Studies a U.S. FDA-regulated Device Product: No
Additional relevant MeSH terms:
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Lung Diseases
Hypertension, Pulmonary
Lung Diseases, Interstitial
Hypertension
Scleroderma, Systemic
Scleroderma, Diffuse
Scleroderma, Localized
Vascular Diseases
Cardiovascular Diseases
Respiratory Tract Diseases
Connective Tissue Diseases
Skin Diseases
Macitentan
Endothelin A Receptor Antagonists
Endothelin Receptor Antagonists
Molecular Mechanisms of Pharmacological Action
Endothelin B Receptor Antagonists