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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Molecular framework for response to imatinib mesylate in systemic sclerosis
Lorinda Chung1, David F Fiorentino, Maya J Benbarak
1Stanford University, Stanford, California, and VA Palo Alto Health Care System, Palo Alto, California.
Arthritis and Rheumatism
|January 31, 2009
Summary
Systemic sclerosis (SSc) treatment with imatinib mesylate reduced skin sclerosis in two patients. This suggests platelet-derived growth factor receptor (PDGFR) and Abl tyrosine kinases are key in SSc and targeted by imatinib.
Area of Science:
- Immunology
- Oncology
- Dermatology
Background:
- Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis and vasculopathy.
- Tyrosine kinases, including platelet-derived growth factor receptor (PDGFR) and Abl, are implicated in SSc pathogenesis.
Observation:
- Two patients with early diffuse cutaneous SSc (dcSSc) received imatinib mesylate therapy.
- Skin biopsy analyses showed reduced phosphorylated PDGFRbeta and Abl after imatinib treatment.
Findings:
- Imatinib therapy led to decreased cutaneous sclerosis in the observed dcSSc patients.
- Gene expression profiling identified an imatinib-responsive signature specific to dcSSc.
- PDGFRbeta and Abl signaling pathways appear critical and synergistic in SSc development.
Implications:
- Imatinib may target a dysregulated gene expression program in dcSSc.
- Targeting PDGFRbeta and Abl offers a potential therapeutic strategy for systemic sclerosis.
- Further research into imatinib's role in SSc is warranted.
