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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

Tracking Information
First Submitted Date  ICMJE October 21, 2018
First Posted Date  ICMJE October 31, 2018
Last Update Posted Date April 2, 2019
Actual Study Start Date  ICMJE September 1, 2018
Estimated Primary Completion Date January 31, 2020   (Final data collection date for primary outcome measure)
Current Primary Outcome Measures  ICMJE
 (submitted: October 29, 2018)
Change in exercise pulmonary vascular resistance (PVR) [ Time Frame: Baseline to 6 months ]
Original Primary Outcome Measures  ICMJE Same as current
Change History Complete list of historical versions of study NCT03726398 on ClinicalTrials.gov Archive Site
Current Secondary Outcome Measures  ICMJE
 (submitted: October 29, 2018)
  • Change in right ventricular pulmonary vascular hemodynamic coupling (RVPA). [ Time Frame: Baseline to 6 months ]
  • Change in maximal oxygen consumption (V02 max). [ Time Frame: Baseline to 6 months ]
  • Change in pulmonary impedance. [ Time Frame: Baseline to 6 months ]
Original Secondary Outcome Measures  ICMJE Same as current
Current Other Pre-specified Outcome Measures Not Provided
Original Other Pre-specified Outcome Measures Not Provided
 
Descriptive Information
Brief Title  ICMJE CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated ILD and PH
Official Title  ICMJE CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study
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.
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.
Study Type  ICMJE Interventional
Study Phase  ICMJE Phase 2
Phase 3
Study Design  ICMJE Intervention Model: Single Group Assignment
Masking: None (Open Label)
Primary Purpose: Prevention
Condition  ICMJE
  • Interstitial Lung Disease
  • Scleroderma
  • Pulmonary Hypertension
Intervention  ICMJE Drug: Opsumit 10 Mg Tablet
Oral tablet taken once daily
Other Name: Macitentan
Study Arms  ICMJE Experimental: Opsumit
Opsumit 10 mg tablet by mouth once daily
Intervention: Drug: Opsumit 10 Mg Tablet
Publications * Not Provided

*   Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
 
Recruitment Information
Recruitment Status  ICMJE Recruiting
Estimated Enrollment  ICMJE
 (submitted: October 29, 2018)
26
Original Estimated Enrollment  ICMJE Same as current
Estimated Study Completion Date  ICMJE December 1, 2020
Estimated Primary Completion Date January 31, 2020   (Final data collection date for primary outcome measure)
Eligibility Criteria  ICMJE

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
Sex/Gender  ICMJE
Sexes Eligible for Study: All
Ages  ICMJE 18 Years and older   (Adult, Older Adult)
Accepts Healthy Volunteers  ICMJE No
Contacts  ICMJE
Contact: Valerie Bloss, MS 520-626-8000 vbloss@email.arizona.edu
Contact: Maria Gordon, MS 520-626-8000 mgordon@email.arizona.edu
Listed Location Countries  ICMJE United States
Removed Location Countries  
 
Administrative Information
NCT Number  ICMJE NCT03726398
Other Study ID Numbers  ICMJE IIS-02801
Has Data Monitoring Committee No
U.S. FDA-regulated Product
Studies a U.S. FDA-regulated Drug Product: Yes
Studies a U.S. FDA-regulated Device Product: No
IPD Sharing Statement  ICMJE Not Provided
Responsible Party Franz Rischard, DO, University of Arizona
Study Sponsor  ICMJE Franz Rischard, DO
Collaborators  ICMJE
  • National Jewish Health
  • University of Pittsburgh
Investigators  ICMJE
Principal Investigator: Franz P. Rischard, DO University of Arizona
PRS Account University of Arizona
Verification Date March 2019

ICMJE     Data element required by the International Committee of Medical Journal Editors and the World Health Organization ICTRP