𝔖 Bobbio Scriptorium
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Is imatinib mesylate a promising drug in systemic sclerosis?

✍ Scribed by P. L. A. van Daele; W. A. Dik; H. B. Thio; P. Th. W. van Hal; J. A. M. van Laar; H. Hooijkaas; P. M. van Hagen


Book ID
101654819
Publisher
John Wiley and Sons
Year
2008
Tongue
English
Weight
114 KB
Volume
58
Category
Article
ISSN
0004-3591

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✦ Synopsis


Abstract

A patient with therapy‐resistant and progressive systemic sclerosis (SSc) with pulmonary involvement who was treated with imatinib mesylate is described herein. Prior to treatment, pulmonary fibroblasts obtained from the patient were cultured and incubated with imatinib mesylate. Preincubation of the fibroblasts for 16 hours with 2.5 μg/ml imatinib mesylate efficiently abrogated platelet‐derived growth factor BB–induced fibroblast proliferation. Furthermore, transforming growth factor β1–induced type I collagen gene transcription was blocked. During treatment, the patient's pulmonary involvement stabilized and her skin tightness improved. To our knowledge, this is the first report of a patient with therapy‐refractory SSc responding to treatment with imatinib mesylate.


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## Abstract Systemic sclerosis (SSc) is an autoimmune disease in which the tyrosine kinases platelet‐derived growth factor receptor (PDGFR) and Abl are hypothesized to contribute to the fibrosis and vasculopathy of the skin and internal organs. Herein we describe 2 patients with early diffuse cutan