What Is Fibrosis? ▼

Fibrosis is the production of excess fibrous tissue that may interfere with the normal function of an organ.

  • Fibrosis that follows a heart attack may lead to a decreased ability to pump blood through the heart.

  • Fibrosis that follows certain kinds of lung diseases (such as asthma and cystic fibrosis) may lead to an inability of the lung to properly inflate and deflate, or even to impaired exchange oxygen with the blood.

  • Fibrosis may lead to disfiguring scars and keloids.

  • Fibrosis within blood vessels may lead to heart attacks, strokes, and kidney failure.

  • Cirrhosis of the liver (a scarring process following alcohol abuse and hepatitis) is a type of fibrosis.

The Global Fibrosis Foundation supports research into the mechanisms of fibrosis. By studying disorders characterized by excessive collagen production and scarring, the GFF believes the basic principles behind fibrosis will be revealed, and ultimately controlled.

Fibrous tissue (chiefly, the protein collagen) is made as part of the normal healing process following tissue injury. In some disorders, the degree of collagen far exceeds what is needed for repair. When this occurs, the excess collagen is called “fibrosis”.

Fibrosis is one possible outcome following injury and inflammation. Understanding the process of fibrosis will lead to therapies that specifically address the “excesses” and will allow physicians to promote healing when it is needed and to reduce fibrosis when it is not. Once fibrosis occurs, it is very difficult to remove or correct.

Nephrogenic Systemic Fibrosis (NSF) ▼

Nephrogenic systemic fibrosis (NSF), known by a variety of names, was first observed in 1997 among patients in a kidney dialysis center. Since the recognition of the disorder, cases of varying severity have been identified around the world.

  • The disease has affected all age groups (children, adults, and the elderly).

  • It tends to occur most commonly in persons in their middle-aged years.

NSF occurs in patients with newly developing, or more commonly, longstanding kidney disease. Many patients are on dialysis.

  • Affected individuals develop progressive hardening of the skin, chiefly of the extremities.

  • The stiffness leads to loss of flexibility, and in severe cases, an inability to walk or use the affected limb.

  • The disorder is generally progressive, but proceeds at varying rates in different individuals.

  • A small number of patients may lose the ability to walk within a few weeks.

While the precise cause of NSF is not known, it is closely linked to the use of contrast dyes used in magnetic resonance imaging (MRI). Because of this close association, the US FDA and other authorities have limited the use of these dyes in patients with kidney disease. The results of these interventions have reduced the number of new cases of NSF around the world, although many persons still live with the disease.

Of interest, the study of NSF has led to new insights into tissue injury and fibrosis, and has provided new tissue culture and animal models that should prove useful in future fibrosis investigations.

Scleroderma (Diffuse: Systemic Sclerosis and Localized: Morphea) ▼

Scleroderma means “hard skin,” and as a disorder has existed for hundreds, if not thousands of years.

  • It affects women four times more commonly than men.

  • Most persons are diagnosed in their middle-aged years.

  • The incidence in the United States is estimated as 1 in 1000 persons.

Scleroderma exists as localized and diffuse forms. Localized scleroderma, termed morphea, generally exists as isolated patches of hard skin, without internal organ involvement. On occasion, areas of involvement are larger and cosmetically deforming.

Diffuse (generalized) scleroderma is termed systemic sclerosis and can affect many organ systems.

Raynaud's phenomenon, a symptom experienced by 80% of systemic sclerosis patients, occurs because of narrowing of the blood vessels of the fingers and toes in response to cold.

Patients also commonly report muscle aches and joint aches. The lungs are commonly involved, although the patient may not experience shortness of breath.

  • Severe cases may lead to heart failure.

  • Some patients experience difficulty swallowing. It is not uncommon for the symptoms to affect the passage of food through the gastrointestinal tract, leading to partial obstruction and poor absorption of nutrients.

  • Kidney failure is a frequent complication, and may lead to the death of the patient.

  • There is no cure for scleroderma.

  • Physicians struggle to treat symptoms.

Idiopathic Pulmonary Fibrosis ▼

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by increased deposition of collagen within the lung tissue. The cause is unknown. It typically effects males more commonly than females, and generally patients over fifty years of age. The average survival following diagnosis is about three years.

While IPF (by definition) has no known cause, IPF-like fibrosis can occur following exposure to inhaled materials (asbestos), medications (chemotherapy), and in the setting of some autoimmune diseases (scleroderma).

A thorough understanding of the processes leading to the fibrosis seen in these latter conditions will likely provide greater insight into the fibrosis of IPF, and the mechanisms of fibrotic processes elsewhere in the body.