Microvascular and Fibrosis Imaging Study

Mechanistic Study: Non-Invasive Cutaneous Microvascular and Fibrosis Imaging of Patients With Systemic Sclerosis, Sickle Cell Disease and Chronic Graft-Versus-Host Disease (GVHD) Compared to Healthy Subjects

In this study, Laser Doppler Flowmetry (LDF), Laser Doppler Imaging (LDI), Orthogonal Polarization Spectral Imaging (OPSI), Nail fold video capillaroscopy (NVC) and Optical Coherence Tomography (OCT) will be used to assess differences in microvascular function and density of oral mucosa and skin in subjects with 1) autoimmune diseases with cutaneous involvement: systemic sclerosis (SSc), morphea, dermatomyositis, cutaneous lupus and vasculitis, 2) sickle cell disease (SCD) and 3) chronic graft-versus-host disease (GVHD) compared to healthy subjects. The microvascular changes will be compared to overall treatment response in patients with scleroderma and chronic GVHD as assessments will be made before and after the patients start treatment for their diseases and determine if these imaging techniques provide valuable and reproducible data when assessing a patient's response to treatment for those diseases. In addition, the application of Acoustic Radiation Force Impulse (ARFI) in determining cutaneous thickness in patients with SSc, GVHD and morphea will be evaluated.

The investigators hypothesize that the vascular and dermal structures are altered in patients with autoimmune disease, SCD and chronic GVHD. In addition, they hypothesize that imaging modalities such as LDF, LDI, OCT, NVC, OPSI and ARFI can quantify such structural alterations and can be used to 1) detect early disease activity, 2) quantify and assess response to therapy and 3) quantify and correlate with overall disease activity.

Study Overview

• Status: Completed
• Conditions: Autoimmune Diseases; Chronic Graft Versus Host Disease; Sickle Cell Disease
• Intervention / Treatment: Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)

• Study Type: Observational
• Enrollment (Actual): 63

Contacts and Locations

Study Locations

• United States
  • North Carolina
    • Durham, North Carolina, United States, 27710
      • Duke University Medical Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patient population at Duke University Medical Center and healthy controls

Description

Inclusion Criteria:

• Subjects above the age of 18 who are capable of giving informed consent with one of the following conditions: Scleroderma, Graft vs Host Disease, Sickle Cell Disease, Morphea, Connective Tissue Disease with cutaneous manifestations: cutaneous lupus, dermatomyositis, vasculitis, patients who are undergoing HSCT, or patients with a diagnosis of hematologic and non-hematologic malignancies without GVHD
• Control patients include normal healthy controls above the age of 18 who are capable of giving informed consent.

Exclusion Criteria:

• Patients who experience discomfort, will be prone to experience discomfort or cannot tolerate the position required for the imaging studies.
• Subjects unable to provide informed consent.
• Smokers, patients unable to tolerate caffeine avoidance for the day of the study.

Study Plan

How is the study designed?

Number of groups / cohorts

6

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Systemic Sclerosis; Patients with SSc will have imaging studies performed at baseline and at 3, 6, 9, 12, 15, 18, 21 and 24 months.	Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)
GVHD; Patients with GVHD will have imaging studies performed at baseline and at 3, 6, 9, 12, 18 and 24 months.	Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)
Undergoing HSCT; Patients who are about to undergo HSCT will have imaging studies performed at 1 week pre-transplantation, day 40 and 80 post transplantation and at 3 months, 6 months, 12 months 18 months and 24 months post-transplant.	Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)
Controls; Healthy Controls and Controls with hematologic and solid organ malignancies and dermatitis will have imaging studies performed at a single point in time.	Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)
Sickle cell disease; Patients with SCD will have imaging studies performed at baseline and at 3, 6, 9, 12, 15, 18, 21 and 24 months.	Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)
Cutaneous fibrosing disorder; Patients with active cutaneous fibrosing disorder will have imaging studies performed at a single point in time.	Other: Acoustic Radiation Force Impulse (ARFI); Other: Laser Doppler Flowmetry (LDF); Other: Laser Doppler Perfusion Imaging (LDI); Other: Optical Coherence Tomography (OCT); Other: Orthogonal Polarization Spectral Imaging (OPSI); Other: Nail fold video capillaroscopy (NVC)

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Microvascular density in oral mucosa and skin	Microvascular density as evaluated using LDF, LDI, OPSI, OCT, NVC and AFRI, will be compared between subjects with and without disease.	over 24 months
Microvascular Perfusion in oral mucosa and skin	Microvascular perfusion as evaluated using LDF, LDI, OPSI, OCT, NVC, and AFRI, will be compared between subjects with and without disease.	over 24 months

Collaborators and Investigators

• Sponsor: Duke University
• Investigators: Principal Investigator: Keith Sullivan, MD, Duke University; Principal Investigator: Adela Cardones, MD, Duke University

Study record dates

Study Major Dates

• Study Start (Actual): February 1, 2013
• Primary Completion (Actual): February 1, 2018
• Study Completion (Actual): February 1, 2018

Study Registration Dates

• First Submitted: December 26, 2012
• First Submitted That Met QC Criteria: December 26, 2012
• First Posted (Estimate): January 1, 2013

Study Record Updates

• Last Update Posted (Actual): September 11, 2020
• Last Update Submitted That Met QC Criteria: September 10, 2020
• Last Verified: May 1, 2020

More Information

Terms related to this study

• Keywords: Dermatomyositis; Sickle Cell Disease; Vasculitis; Scleroderma; Graft versus Host Disease; Systemic sclerosis; Morphea; Cutaneous lupus; Autoimmune diseases with cutaneous involvement
• Additional Relevant MeSH Terms: Immune System Diseases; Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell; Autoimmune Diseases; Graft vs Host Disease
• Other Study ID Numbers: Pro00039256