Fibrosis

Original Editor - Lucinda hampton

Top Contributors - Lucinda hampton and Vidya Acharya  

Introduction

The term fibrosis relates the development of fibrous connective tissue as a way to repair tissue following injury or damage. Pathophysiologic fibrosis, essentially an excessive accumulation of extracellular matrix components (particularly collagen), is the final result of a cascade of events following tissue injury via inflammation, and resulting in permanent scar formation.

Fibrosis may refer to the connective tissue deposition that occurs as part of normal healing or to the excess tissue deposition that occurs as a pathological process. Fibrosis can affect all tissues of the body, and left unrestrained, can result in organ failure and death. It is caused by a process that stems from wound healing that has gone awry. Fibrogenesis is becoming increasingly recognized as a major cause of morbidity and death in most chronic inflammatory diseases, with a dearth of treatment strategies available to target the pathogenesis of fibrosis.

Main Types of Fibrosis

Fibrosis is the excessive accumulation of fibrous connective tissue (parts of the extracellular matrix like collagen and fibronectin) in and around inflamed or damaged tissue, which can lead to permanent scarring, organ malfunction and, ultimately, death. This can be seen in as seen in end-stage liver disease, kidney disease, idiopathic pulmonary fibrosis, liver cirrhosis and heart failure (cardiac fibrosis).

Fibrosis is also a major pathological characteristic of many chronic autoimmune diseases (eg scleroderma, rheumatoid arthritis, Crohn’s disease, ulcerative colitis, myelofibrosis and systemic lupus erythematosus).

Pulmonary fibrosis, occurs in 3 distinct entities: Replacement fibrosis, as occurs in response to lung damage caused by infarction or an infection); focal fibrosis, as occurs in a response to irritation by inhaled substances that are inhaled eg silica or asbestos; diffuse parenchymal lung disease (DPLD).

Skeletal muscle injuries may cause fibrosis. With minor injuries the muscles are able to regenerate completely and recover their usual capabilities. However, in severe injuries, the injured muscle cannot recover to a functional level because of the formation of fibrous scar tissue.

Fibrosis also impacts tumor invasion and metastasis, chronic graft rejection and the pathogenesis of many progressive myopathies. Fibrogenesis is becoming increasingly recognized as a major cause of morbidity and death in most chronic inflammatory diseases, with a dearth of treatment strategies available to target the pathogenesis of fibrosis.

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