Glossary
 

This glossary may be useful to you as you learn more about IPF.

 

Alveoli
Very small air sacs found in the lungs. Click here to see how the alveolar tissue changes with IPF.
ambrisentan (Leitaris)
an endothelin receptor antagonist (ERA) manufactured by Gilead, and currently FDA approved for the treatment of pulmonary arterial hypertension (PAH), an exceedingly prevalent complication for IPF patients as their disease progresses. Currently in Phase III clinical trials as a potential therapy for Pulmonary Fibrosis (January, 2009)
Antifibrotic/antifibrogenic agents
Experimental medications used to suppress the scarring process associated with IPF.
Antioxidants
Experimental medications that may prevent or reverse lung tissue damage caused by IPF.
Azathioprine
A medication commonly prescribed for IPF patients that can suppress the activity of the immune system and reduce inflammation.
bosentan (Tracleer)
an endothelin receptor antagonist (ERA) manufactured by Actelion, Ltd., and currently FDA approved for the treatment of pulmonary arterial hypertension (PAH), and currently in Phase III clinical trials as a potential therapy for Pulmonary Fibrosis (January, 2009)
Bronchoalveolar lavage (BAL)
A diagnostic technique in which fluid is instilled into the lungs and removed for examination.
Clubbing
A buildup of tissue in the fingertips (or sometimes the toes). Clubbing is a sign of advanced IPF. Click here to see what
Collagen
Proteins found in tendons, bones, and connective tissue. Scar tissue found in the lungs of patients with IPF also contains
Computed tomography (CT or CAT scan) or high-resolution computed tomography (HRCT)
A type of x-ray for which a computer is used to construct a three-dimensional image from a series of cross-sectional images. The procedure generates multiple pictures of your lungs in layers (slices) from the top (at your shoulders) to the bottom (just above your waist). This test requires that you lie on a table while the pictures are taken, holding your breath and letting it out as instructed.
Corticosteroids
Medications used to suppress the activity of the immune system and reduce inflammation. The most commonly prescribed corticosteroid is prednisone.
Cyclophosphamide
A medication commonly prescribed for IPF patients that can suppress the activity of the immune system and reduce inflammation.
Cytokines
Molecules produced by the immune system. A cytokine imbalance may be the cause of IPF.
DLCO
Stands for the diffusing capacity of the lung for carbon monoxide (CO), the test used to determine this parameter. DLCO is the extent to which oxygen passes from the air sacs of the lungs into the blood.
Dyspnea
Shortness of breath or labored breathing, usually associated with physical exertion.
Epidemiology
The incidence, distribution, and control of a disease in a population.
Etiology
The cause(s) of a disease.
Familial
Tending to occur in more members of a family than would be expected by chance alone.
Fibrosis
An abnormal scarring of body tissue.
Forced Vital capacity (FVC)
the maximum volume of air that a person can exhale after maximum inhalation. It can also be the maximum volume of air that a person can inhale after maximum exhalation. A person’s vital capacity can be measured by a spirometer which can be a wet or regular spirometer. In combination with other physiological measurements, the vital capacity can help make a diagnosis of underlying lung disease.
Forced Expiratory Volume (FEV or FEV1)
The volume of air that can be forced out taking a deep breath, an important measure of pulmonary function. The forced expiratory volume in the first second is the FEV1.
Histopathology
Tissue changes (e.g., scarring of the lungs) that accompany a disease and are recognized by microscopic examination.
Hypoxia
A deficiency of oxygen in the blood
Hypertension
High blood pressure.
Hypoxemia
A lack of oxygen in the blood.
Hypoxia
A lack of oxygen in the tissues of the body.
Idiopathic
Arising spontaneously or from an obscure or unknown cause.
Idiopathic pulmonary fibrosis (IPF)
A disease characterized by progressive scarring (fibrosis) and deterioration of the lungs.
Interferon gamma-1b
A regulatory cytokine that has antifibrotic and antifibrogenic effects and may regulate macrophage, fibroblast, and mast cell function; inhibit a variety of neutrophil-derived cytokines; and modify the balance of Th1 and Th2 cells in the lung.
Interstitial lung disease (ILD)
A general term for the approximately 200 disorders characterized by inflammation and scarring (fibrosis) of the lungs’ interstitium. IPF is an example of an interstitial lung disease.
Interstitium
The tissue layers between the lungs’ air sacs (alveoli) and blood vessels.
Lung biopsy
A procedure in which a tissue sample is obtained through a flexible tube or by means of a small surgical incision between the ribs.
Lung transplant
Replacement of a lung or lungs with donor organ(s).
Monoclonal antibodies
Experimental medications for the treatment of IPF. They may inhibit “bad” cytokines.
Pathogenesis
The mode of origin or development of a disease.
Pathology
The abnormalities that characterize a particular disease.
Phase I trial
The first phase of drug testing in humans. It usually involves 20 to 100 subjects and focuses on safety.
Phase II trial
The second phase of drug testing in humans. It involves up to several hundred patients, lasts as long as two years, and focuses on safety and effectiveness.
Phase III trial
The third phase of drug testing in humans. It involves several hundred to several thousand patients, often lasts several years, and focuses on safety, dosage, and effectiveness.
Pirfenidone
An orally active small molecule drug manufactured by InterMune, Inc. that inhibits collagen synthesis, down-regulates profibrotic cytokines, inhibits TNF-alpha synthesis and decreases fibroblast proliferation. Recently completed Phase III clinical trial and is being reviewed by FDA during 2009 (January, 2009)
Prednisone
The most commonly prescribed therapy for IPF. Prednisone is a corticosteroid that can suppress the activity of the immune system and reduce inflammation.
Prognosis
The prospect for survival and recovery from a disease.
Pulmonary
Having to do with the lungs.
Pulmonary Arterial Hypertension (PAH)
is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen.PH causes symptoms such as shortness of breath during routine activity (for example, climbing two flights of stairs), tiredness, chest pain, and a racing heartbeat. As the disease worsens, its symptoms may limit all physical activity.
Pulmonary embolism
A blood clot in the lungs.
Pulmonary fibrosis
Thickening and scarring of the lungs, specifically the pulmonary interstitium.
Pulmonary hypertension
High blood pressure in the lungs.
sildenefil (Viagra)
a vasodilator manufactured by Pfizer, Inc., that is currently FDA approved for the treatment of erectile dysfunction, and being investigated as a possible treatment for Pulmonary Fibrosis by several medical centers in independent clinical trials (January, 2009)
spirometer
an instrument for measuring the volume of air entering and leaving the lungs. Used as a preliminary test for early detection of lung disorders
trepostinil (Remodulin)
a vasodilator manufactured by United Therapeutics that is administered by IV pump. Currently FDA approved for the treatment of pulmonary arterial hypertension (PAH), and is currently in Phase I clinical trials as a potential therapy for Pulmonary Fibrosis (January, 2009)
Usual interstitial pneumonia (UIP)
A condition indicated by specific changes in the lung tissue. Finding UIP in a lung biopsy strongly points to a diagnosis of IPF (although other criteria must be met).