Microvascular and Fibrosis Imaging Study

Duke University63 enrolled

Overview

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 Type

OBSERVATIONAL

Enrollment

Conditions

Autoimmune Diseases Sickle Cell Disease Chronic Graft Versus Host Disease

Eligibility

Sex: ALLMin age: 18 YearsHealthy volunteers:

Medical Language ↔ Plain English

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.

Outcomes

Primary Outcomes

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.

Time frame: 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.