Sickle Cell Disease Clinical Trial
Official title:
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.
n/a
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