A Unifying Hypothesis for Scleroderma: Identifying a Target Cell for Scleroderma
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : William M. Mahoney Jr & Jo Nadine Fleming & Stephen M. Schwartz
- چاپ و سال / کشور: 2010
Description
We propose that a recent change in the conception of the role of type 1 interferon and the identification of adventitial stem cells suggests a unifying hypothesis for scleroderma. This hypothesis begins with vasospasm. Vasospasm is fully reversible unless, as proposed here, the resulting ischemia leads to apoptosis and activation of type 1 interferon. The interferon, we propose, initiates immune amplification, including characteristic scleroderma-specific antibodies. We propose that the interferon also acts on adventitial stem cells, producing myofibroblasts, rarefaction, and intimal hyperplasia—three morphologic changes that characterize this disease. Regulator of G-protein signaling 5 (RGS5), a regulator of vasoactive G-protein–coupled receptors, is a cell type–specific marker of pericytes and scleroderma myofibroblasts. RGS5 may provide a key link between initial hyperplasia and fibrosis in this disease
Curr Rheumatol Rep (2011) 13:28–36 DOI 10.1007/s11926-010-0152-8 Published online: 23 December 2010
