Hi all - I recently found this article, and really want to share it - it centralizes all the basics about FMD - what fibrosis is, how it works and progresses, what might trigger it, the roles infection and our immune systems play, and more.
Fibromuscular dysplasia (FMD) is fibrosis of the artery walls [1] (fibroplasia means fibrosis), and is one of very many fibrotic diseases now epidemic in our world. All fibrotic diseases share some common mechanisms, and “collectively, are the leading cause of morbidity and mortality in North America, Europe, and Japan.” [2] Fibrotic diseases also “represent the largest segment of human disease that remain intractable to drug therapy,” [3] meaning fibrosis is incurable and untreatable.
But treatments for fibrosis are finally on the horizon - one of the main reasons the information in this article is now openly accessible. Until now, there has been no hope except to treat secondary and downstream life threatening symptoms as they occur.
The article is about our underlying disease - fibrosis.
Hope it helps!
…Let me know what you think,
sofi
QUOTE:
Abstract
Fibroproliferative diseases, including the pulmonary fibroses, systemic sclerosis, liver cirrhosis, cardiovascular disease, progressive kidney disease, and macular degeneration, are a leading cause of morbidity and mortality and can affect all tissues and organ systems. Fibrotic tissue remodeling can also influence cancer metastasis and accelerate chronic graft rejection in transplant recipients. Nevertheless, despite its enormous impact on human health, there are currently no approved treatments that directly target the mechanism(s) of fibrosis. The primary goals of this Review series on fibrotic diseases are to discuss some of the major fibroproliferative diseases and to identify the common and unique mechanisms of fibrogenesis that might be exploited in the development of effective antifibrotic therapies.
Introduction
Fibrosis is often defined as a wound-healing response that has gone out of control. Repair of damaged tissues is a fundamental biological process that allows the ordered replacement of dead or damaged cells after injury, a mechanism that is critically important for survival. Damage to tissues can result from various acute or chronic stimuli, including infections, autoimmune reactions, and mechanical injury. The repair process typically involves two distinct stages: a regenerative phase, where injured cells are replaced by cells of the same type, leaving no lasting evidence of damage; and a phase known as fibroplasia, or fibrosis, where connective tissue replaces normal parenchymal tissue. Although initially beneficial, the healing process becomes pathogenic if it continues unchecked, resulting in substantial remodeling of the ECM and formation of permanent scar tissue (Figure 1). In some cases, it might ultimately lead to organ failure and death.
…
The spectrum of diseases that result from chronic tissue damage or out-of-control wound-healing responses are too numerous to list. However, the goal of this Review series on fibrotic diseases is to highlight some of the major fibrotic diseases and to identify common and unique mechanisms of fibrogenesis in the various organ systems affected by these diseases.
END QUOTE
MORE: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1804380
Common and unique mechanisms regulate fibrosis in various fibroproliferative diseases
J Clin Invest. 2007 March 1; 117(3): 524–529.
Thomas A. Wynn
Immunopathogenesis Section, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, Maryland, USA.
ARTICLE CONTENTS
Introduction
The role of chronic infections and innate immunity
Myofibroblasts
The role of ANG II and TGF-β1
ECM remodeling and reversibility of intractable fibrosis
A link between angiogenesis and fibrogenesis
Human embryonic stem cell–based therapies for fibrosis
The challenge ahead: clinical trial design and endpoints
REFERENCES
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"FMD may involve any layer of a visceral artery, and it may be classified on the basis of the primary of involvement of the arterial wall. The classification system includes intimal, medial, or adventitial fibrosis."
Fibromuscular Dysplasia (Visceral Arteries)
http://www.emedicine.com/radio/topic900.htm -
"Collectively, fibrotic disorders are the leading cause of morbidity and mortality in North America, Europe, and Japan."
Combinatorial signaling pathways determine fibroblast proliferation and myofibroblast differentiation
http://www.fasebj.org/cgi/content/full/18/3/469?ck=nck -
"Collectively, fibrotic disorders represent the largest segment of human disease that remain intractable to drug therapy."
Inhibition of TGF-ß-stimulated CTGF gene expression and anchorage-independent growth by cAMP identifies a CTGF-dependent restriction point in the cell cycle
http://www.fasebj.org/cgi/content/full/12/12/1151?ck=nck