Fibrosis is a natural response of the body to injury, such as a cut or infection, whereby the body produces a tough fibrous (collagen containing) scar in order to repair the damage. In medical terms fibrosis is more often defined pathologically as inappropriate repair that inhibits the normal function of the effected organ. It is the healing process gone awry that can result in organ damage and eventual organ failure. Fibrosis can affect every organ of the body and is associated with diseases and injuries.
Disease and Injuries Where Fibrosis Is A Major Cause of Morbidity and Mortality
Organ Specific Fibrotic Diseases
Lung - idiopathic pulmonary fibrosis (IPF), asthma, COPD
Liver - alcoholic cirrhosis, hepatitis C cirrhosis, primary biliary cirrhosis, sclerosing cholangitis
Kidney - diabetic nephropathy, lupus glomerulosclerosis, GSFS
Heart - heart attack (MI), congestive heart failure (CHF)
Skin - hypertrophic scarring, keloids, nephrogenic fibrosing dermopathy
Eye - retro-orbital fibrosis, post-cataract surgery, post-glaucoma surgery
Bone marrow - idiopathic & drug induced myelofibrosis
Systemic Fibrotic Diseases
Systemic sclerosis (Scleroderma)
Systemic fibrosis (NFD, nephrogenic fibrosing dermopathy; NFS, nephrogenic systemic fibrosis)
Cystic fibrosis
Chronic graft vs. host disease
Atherosclerosis
Injury Associated Fibrotic Diseases
Burn induced skin & soft tissue scarring and contraction
Radiation induce skin & organ scarring post cancer therapeutic radiation treatment
Dialysis related fibrosing dermatopathy
Fibrotic tissue is characterized by the loss of normal tissue architecture as tissue is replaced with scar. This scar tissue is primarily composed of collagen type I, but may also contain collagen type III and IV along with other extracellular matrix proteins. The progressive scaring leads to organ dysfunction which is a common feature of fibrosis in the lung, liver, kidney, and heart and may ultimately result in organ failure.
Fibrosis involves a cascade of normal cellular responses that have gone terribly wrong. Recent research into the cellular and molecular mechanism specifically associated with the production and maintenance of a scar has identified the cells and molecular signals involved. Hope is emerging that drugs will be discovered that can prevent and eventually reverse established fibrosis.