What are the other Names for this Condition? (Also known as/Synonyms)

• Chronic Fibrous Pneumonia
• Idiopathic Interstitial Pneumonia (IIP)
• UIP (Usual Interstitial Pneumonia)

What is Usual Interstitial Pneumonia? (Definition/Background Information)

• Usual Interstitial Pneumonia (UIP) is a progressive condition in which there is an increased scarring of the lung tissue. Specifically, the disease involves damage to the interstitium, which renders protection to the air sacs
• Usual Interstitial Pneumonia is known to commonly affect elderly individuals. Smoking is reported to exacerbate the condition. A family history of UIP, exposure to harmful chemicals, smoking, etc., are some predisposing factors to developing Usual Interstitial Pneumonia
• Although the exact cause of Usual Interstitial Pneumonia is still under investigation, some supposed causes include smoking, inhalation of harmful chemicals, and certain autoimmune disorders. In some cases, certain inherited genes have been implicated
• Breathing difficulties, cough, and blue-tinged skin are the most characteristic symptoms of Usual Interstitial Pneumonia. The condition may be diagnosed through a physical exam, medical (family) history, chest x-ray, and lung function tests
• The treatment options could include the use of steroids, medications to suppress the immune system, and smoking cessation. In severe cases, lung transplantation may be advised
• Usual Interstitial Pneumonia is a progressive disease and complications, such as pulmonary embolism or cor pulmonale, can occur, making the outcome undesirable or unpredictable

Who gets Usual Interstitial Pneumonia? (Age and Sex Distribution)

• Usual Interstitial Pneumonia occurs in individuals older than 50 years of age. The elderly population is more likely to develop the condition
• Both men and women are at risk; however, the number of cases may be more in men due to the type of risk factors involved (smoking, exposure to harmful chemicals, etc.)
• UIP is more commonly seen among Caucasians than other races or ethnic groups

What are the Risk Factors for Usual Interstitial Pneumonia? (Predisposing Factors)

The risk factors associated with Usual Interstitial Pneumonia include:

• A family history of the condition
• Usual Interstitial Pneumonia is especially more predominant among smokers
• Working in an environment that causes exposure to harmful chemicals and dust
• Scleroderma
• Rheumatoid arthritis
• Asbestosis
• Chronic nitrofurantoin toxicity

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Usual Interstitial Pneumonia? (Etiology)

The exact cause for Usual Interstitial Pneumonia is still under investigation. However,

• Researchers believe that the condition could be caused by autoimmune diseases, such as those involving the connective tissue, or even systemic sclerosis
• Some studies have indicated that certain genes to be involved and that the condition may be inherited
• Inhalation of harmful chemicals is believed to be the most common cause, since it involves inhalation of hazardous substances into the body. Hence, smoking is a very important risk factor

What are the Signs and Symptoms of Usual Interstitial Pneumonia?

The following are some known signs and symptoms of Usual Interstitial Pneumonia:

• Cough: It results from the lack of oxygen that is being distributed from the alveoli, as it is inflamed
• Difficulty breathing
• More effort has to be exerted to perform normal breathing
• Feeling of weakness and fatigue
• Noticeable changes in the color of the skin: The skin will appear cyanotic (blue), due to a lack of oxygen being supplied in the body from the lungs

How is Usual Interstitial Pneumonia Diagnosed?

Usual Interstitial Pneumonia could be diagnosed by:

• Performing a physical examination, by listening for abnormal sounds within the lungs
• A chest x-ray to identify any signs of inflammation or presence of any form of infection within the lungs
• Assessment of lung function
• Bronchoalveolar lavage: During this procedure, a fluid is injected into the airways and is then sucked out. This allows a physician to determine, if any particular cell type is accumulated in the airways
• High resolution CT scans of the lungs

A surgical biopsy may be necessary for confirming the diagnosis. An open biopsy of the lung is the preferred biopsy method.

• A tissue biopsy of the lung is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
• Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
• Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely electron microscopic studies to assist in the diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Usual Interstitial Pneumonia?

• The complications associated with Usual Interstitial Pneumonia lead to severe respiratory failure, which can lead to death
• Some individuals with UIP may develop other lung-related conditions, such as pulmonary embolism, cor pulmonale, or even pneumothorax

How is Usual Interstitial Pneumonia Treated?

Many individuals with Usual Interstitial Pneumonia do not respond to any treatment. The lung scarring continues to occur.

• Individuals with UIP are generally treated with steroids
• If they do not respond to these medications, then other forms of medications are prescribed, such as immunosuppressant drugs
• Smoking cessation is always advised, so that further progression of the disease can be avoided
• Lung transplant may be recommended, depending on the severity of the condition, in some cases

How can Usual Interstitial Pneumonia be Prevented?

The following measures are advised for Usual Interstitial Pneumonia prevention:

• Cessation of smoking
• Avoiding inhalation of hazardous contaminants
• Genetic counseling: Individuals with family history of lung-associated disease should see a physician to assess, if they have genetically inherited the disease. If that is the case, counseling may help understand the risks of having children with the condition, when planning for a family

In many cases, there are no definitive methods to prevent Usual Interstitial Pneumonia.

What is the Prognosis of Usual Interstitial Pneumonia? (Outcomes/Resolutions)

• The prognosis for Usual Interstitial Pneumonia is poor
• The scarring of the lung is progressive and the survival rate for the condition after diagnosis is around 6-13 years
• Sometimes, the scarring occurs rapidly, which is called ‘accelerated UIP’

Additional and Relevant Useful Information for Usual Interstitial Pneumonia:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/healthy-lungs/