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Investigating Constitutive and Cell-Driven Fibroblast Activation in Pediatric Morphea

May 11, 2020 By Katherine Devenport

Fellow

Laila Abbas
UT Southwestern Medical Center

Mentor

Heidi Jacobe, MD, MSCS
UT Southwestern Medical Center

Overview

Morphea, or localized scleroderma, is a neglected autoimmune disease of the skin and underlying tissue that produces permanent disability due to joint contracture and soft tissue loss. Approximately half of patients have disease onset in childhood, and pediatric morphea is associated with significant morbidity, with reported complications including loss of range of motion, joint deformity, facial disfigurement, and neurologic manifestations. Morphea has remitting and relapsing activity, marked by inflammation and fibrosis, and damage which produces atrophy. The overarching hypothesis of this project is that fibroblasts are constitutively activated in morphea and that both T cells and macrophages will promote activation of fibroblasts.

Aim 1: Determine constitutive expression of pro-fibrotic and inflammatory markers in fibroblasts derived from morphea lesions as compared to matched control specimens.

Aim 2: Co-cultures will be performed with combinations of healthy and morphea fibroblasts along with healthy and morphea derived T cells and macrophages to determine if these cells can potentiate fibroblast activation.

Status

This project was funded through a 2020 PeDRA Research Fellowship Grant.

Filed Under: Connective Tissue & Autoimmune

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