Has any one heard of usual interstitial pneumonitis is it terminal or not and ho!


Question: What is Usual Interstitial Pneumonia (UIP)?

Usual Interstitial Pneumonia (UIP) is a specific term used by lung pathologists as one of several interstitial pneumonias (the interstitium is a word used for the cellular support structures of the lung). UIP is the most common type of interstitial pneumonia.


IPF is often used as synonymous with UIP because UIP is the most common cellular pattern, and its cause is usually not known. The designation "IPF/UIP" is also commonly used to describe this process of progressive pulmonary fibrosis.


There are several interstitial pneumonias, in the 1960's, Professor Liebow used the term "usual" to describe the usual pattern seen for the interstitial pneumonias. This term continues to be used today or the terms "IPF" or "IPF/UIP" are utilized.


The term "cryptogenic fibrosing alveolitis" is synonymous with UIP. "Cryptogenic" means an unknown cause. "Fibrosing" means progressive or ongoing scar formation of the interstitium and alveoli. "Alveolitis" means inflammation of the alveoli, which are small spherical oxygen exchange units in the lung.

Microscopic Findings: The microscopic findings of UIP show three findings in the lung tissue: (1) Areas of normal lung, (2) Areas of inflammation, and (3) Areas of end-stage, scared, and non-functioning cystic lung with the appearance of a honeycomb. "Architectural derangement" of the lung is seen. "Fibroblastic foci" seen in the areas of inflamed lung establish the diagnosis of UIP. These foci are scar-forming cells called fibroblasts. These cells are rarely seen in
healthy lungs, and their presence in a group (foci) indicates the ongoing scarring process of UIP. Sometimes the area of inflammation has the appearance of cellular organization called BOOP (Bronchiolitis Obliterans Organizing Pneumonia - hypertext to "What's BOOP"). This BOOP process is not the primary disorder but is secondary to the underlying primary disorder of UIP.


The Process: The UIP process is one of gradual replacement of the lung tissue with scar and fibrosis. The cause of this process is not known. The appearance of the "fibroblastic foci" suggests that these fibroblast cells, which are usually beneficial by producing scar to heal an injury, malfunction and continue to live producing scar instead of the normal termination response. Extensive research is being conducted to clarify this reaction so that medications can be developed to stop this abnormal process.


Causes: The cause of UIP is usually not known, and therefore often used interchangeably with IPF (IPF/UIP). There are some known causes of UIP such as certain type of medications, radiation treatment, and hazardous dust exposures. There are also several known general disorders associated with pulmonary fibrosis such as scleroderma and rheumatoid arthritis.


Answers: What is Usual Interstitial Pneumonia (UIP)?

Usual Interstitial Pneumonia (UIP) is a specific term used by lung pathologists as one of several interstitial pneumonias (the interstitium is a word used for the cellular support structures of the lung). UIP is the most common type of interstitial pneumonia.


IPF is often used as synonymous with UIP because UIP is the most common cellular pattern, and its cause is usually not known. The designation "IPF/UIP" is also commonly used to describe this process of progressive pulmonary fibrosis.


There are several interstitial pneumonias, in the 1960's, Professor Liebow used the term "usual" to describe the usual pattern seen for the interstitial pneumonias. This term continues to be used today or the terms "IPF" or "IPF/UIP" are utilized.


The term "cryptogenic fibrosing alveolitis" is synonymous with UIP. "Cryptogenic" means an unknown cause. "Fibrosing" means progressive or ongoing scar formation of the interstitium and alveoli. "Alveolitis" means inflammation of the alveoli, which are small spherical oxygen exchange units in the lung.

Microscopic Findings: The microscopic findings of UIP show three findings in the lung tissue: (1) Areas of normal lung, (2) Areas of inflammation, and (3) Areas of end-stage, scared, and non-functioning cystic lung with the appearance of a honeycomb. "Architectural derangement" of the lung is seen. "Fibroblastic foci" seen in the areas of inflamed lung establish the diagnosis of UIP. These foci are scar-forming cells called fibroblasts. These cells are rarely seen in
healthy lungs, and their presence in a group (foci) indicates the ongoing scarring process of UIP. Sometimes the area of inflammation has the appearance of cellular organization called BOOP (Bronchiolitis Obliterans Organizing Pneumonia - hypertext to "What's BOOP"). This BOOP process is not the primary disorder but is secondary to the underlying primary disorder of UIP.


The Process: The UIP process is one of gradual replacement of the lung tissue with scar and fibrosis. The cause of this process is not known. The appearance of the "fibroblastic foci" suggests that these fibroblast cells, which are usually beneficial by producing scar to heal an injury, malfunction and continue to live producing scar instead of the normal termination response. Extensive research is being conducted to clarify this reaction so that medications can be developed to stop this abnormal process.


Causes: The cause of UIP is usually not known, and therefore often used interchangeably with IPF (IPF/UIP). There are some known causes of UIP such as certain type of medications, radiation treatment, and hazardous dust exposures. There are also several known general disorders associated with pulmonary fibrosis such as scleroderma and rheumatoid arthritis.

Usual interstitial pneumonitis is the most prevalent of the idiopathic interstitial lung diseases. Because it's so common, the terms "usual interstitial pneumonitis" and "idiopathic pulmonary fibrosis" are often used interchangeably. Because usual interstitial pneumonitis develops in patches, some areas of the lungs are normal, others are inflamed and still others are marked by scar tissue. The disease affects more men than women and usually develops in people over 50.

Although the names are nearly identical, pneumonitis is not the same as pneumonia. Pneumonitis is lung inflammation without infection, whereas pneumonia is lung inflammation that results from infection. In addition, pneumonia is generally limited to one or two areas of the lungs, but pneumonitis involves all five lobes



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