Usual interstitial pneumonia: Difference between revisions

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'''Usual interstitial pneumonia''' ('''UIP''') is a form of [[lung disease]] that results in scarring ([[fibrosis]]) of the lungs. It is a type of [[Interstitial lung disease]] that primarily affects the [[interstitium]], which is the tissue and space around the [[alveoli]] (air sacs), and not directly the alveoli themselves.
{{SI}}
 
{{Infobox medical condition
==Etiology==
| name            = Usual interstitial pneumonia
 
| image          = [[File:CT_scan_in_usual_interstitial_pneumonia_(UIP).jpg|250px]]
The exact cause of UIP is often unknown, and in these cases, it is termed [[idiopathic]]. This idiopathic form of UIP is also known as [[Idiopathic pulmonary fibrosis]] (IPF). However, UIP can also be associated with certain environmental exposures, medications, and medical conditions.  
| caption        = CT scan showing usual interstitial pneumonia
 
| synonyms        = UIP
==Pathogenesis==
| specialty      = [[Pulmonology]]
 
| symptoms        = [[Shortness of breath]], [[dry cough]], [[fatigue (medical)|fatigue]]
In UIP, the normal lung architecture is replaced by patches of fibrotic tissue interspersed with areas of normal lung. This pattern is often described as "temporally heterogeneous" because it suggests an ongoing, unregulated wound-healing process in which fibrosis alternately progresses and regresses, leading to the destruction of the normal lung architecture.
| complications  = [[Pulmonary hypertension]], [[respiratory failure]]
 
| onset          = Typically in adults over 50
==Clinical Features==
| duration        = Chronic
 
| causes          = [[Idiopathic]], [[connective tissue disease]], [[asbestosis]], [[chronic hypersensitivity pneumonitis]]
Patients with UIP typically present with progressive [[dyspnea]] (shortness of breath) and [[cough]]. On physical examination, distinctive "Velcro" crackles can often be heard through a [[stethoscope]].  
| risks          = [[Smoking]], [[environmental exposure]], [[genetic predisposition]]
 
| diagnosis      = [[High-resolution computed tomography|HRCT]], [[lung biopsy]]
==Diagnosis==
| differential    = [[Nonspecific interstitial pneumonia]], [[hypersensitivity pneumonitis]], [[sarcoidosis]]
 
| treatment      = [[Antifibrotic therapy]], [[oxygen therapy]], [[lung transplantation]]
The diagnosis of UIP can be challenging and often requires a combination of clinical, radiographic, and sometimes pathologic data. High-resolution [[Computed tomography|CT]] scans of the chest are often used in the diagnostic process.  
| prognosis      = Variable, often poor
 
| frequency      = Rare
==Treatment==
}}
 
[[File:CT_scan_in_usual_interstitial_pneumonia_(UIP).jpg|left|thumb|CT scan showing usual interstitial pneumonia]]
There is currently no cure for UIP, and treatment is primarily aimed at slowing the progression of the disease and improving quality of life. This often involves the use of medications such as [[corticosteroids]] and other [[immunosuppressive drugs]], as well as supportive measures such as [[oxygen therapy]] and [[pulmonary rehabilitation]].
[[File:UIP_(Usual_interstitial_pneumonia)-CT_scan.jpg|left|thumb|Another CT scan of usual interstitial pneumonia]]
 
[[File:UIPlungbiopsy.jpg|left|thumb|Lung biopsy showing usual interstitial pneumonia]]
==Prognosis==
[[File:Honeycomb_change.jpg|left|thumb|Honeycomb change in usual interstitial pneumonia]]
 
[[File:Fibroblast_focus.jpg|left|thumb|Fibroblast focus in usual interstitial pneumonia]]
The prognosis of UIP is generally poor, with a median survival of only 2-3 years from the time of diagnosis. However, the course of the disease can vary widely from person to person, and some people may live with the disease for many years.
'''Usual interstitial pneumonia''' (UIP) is a specific form of [[lung disease]] characterized by progressive scarring of both lungs. It is the most common form of [[idiopathic pulmonary fibrosis]] (IPF), a type of [[interstitial lung disease]].
 
== Clinical Features ==
[[Category:Respiratory diseases]]
Patients with UIP typically present with [[dyspnea]] (shortness of breath) and a persistent [[dry cough]]. The disease is more common in older adults, particularly those over the age of 50. Physical examination may reveal [[clubbing (medicine)|clubbing]] of the fingers and [[crackles]] on lung auscultation.
[[Category:Interstitial lung disease]]
== Pathology ==
{{medicine-stub}}
The hallmark of UIP is the presence of patchy interstitial fibrosis, which is most pronounced in the subpleural and basal regions of the lungs. Histologically, UIP is characterized by the presence of fibroblastic foci, honeycomb change, and temporal heterogeneity of fibrosis.
== Diagnosis ==
Diagnosis of UIP is typically made through a combination of clinical, radiological, and pathological findings. High-resolution [[computed tomography]] (HRCT) scans of the chest are crucial for diagnosis, often showing a pattern of reticular opacities, traction bronchiectasis, and honeycombing. A lung biopsy may be performed to confirm the diagnosis.
== Treatment ==
There is no cure for UIP, and treatment is primarily supportive. [[Antifibrotic]] medications such as [[pirfenidone]] and [[nintedanib]] may slow disease progression. [[Lung transplantation]] is an option for eligible patients with advanced disease.
== Prognosis ==
The prognosis for patients with UIP is generally poor, with a median survival of 3 to 5 years after diagnosis. The disease course is variable, with some patients experiencing rapid progression and others having a more indolent course.
== See also ==
* [[Idiopathic pulmonary fibrosis]]
* [[Interstitial lung disease]]
* [[Lung transplantation]]
{{Pulmonology}}
[[Category:Interstitial lung diseases]]
[[Category:Pulmonary fibrosis]]
{{stub}}

Latest revision as of 15:29, 13 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Usual interstitial pneumonia
Synonyms UIP
Pronounce N/A
Specialty Pulmonology
Symptoms Shortness of breath, dry cough, fatigue
Complications Pulmonary hypertension, respiratory failure
Onset Typically in adults over 50
Duration Chronic
Types N/A
Causes Idiopathic, connective tissue disease, asbestosis, chronic hypersensitivity pneumonitis
Risks Smoking, environmental exposure, genetic predisposition
Diagnosis HRCT, lung biopsy
Differential diagnosis Nonspecific interstitial pneumonia, hypersensitivity pneumonitis, sarcoidosis
Prevention N/A
Treatment Antifibrotic therapy, oxygen therapy, lung transplantation
Medication N/A
Prognosis Variable, often poor
Frequency Rare
Deaths N/A


CT scan showing usual interstitial pneumonia
Another CT scan of usual interstitial pneumonia
Lung biopsy showing usual interstitial pneumonia
Honeycomb change in usual interstitial pneumonia
Fibroblast focus in usual interstitial pneumonia

Usual interstitial pneumonia (UIP) is a specific form of lung disease characterized by progressive scarring of both lungs. It is the most common form of idiopathic pulmonary fibrosis (IPF), a type of interstitial lung disease.

Clinical Features[edit]

Patients with UIP typically present with dyspnea (shortness of breath) and a persistent dry cough. The disease is more common in older adults, particularly those over the age of 50. Physical examination may reveal clubbing of the fingers and crackles on lung auscultation.

Pathology[edit]

The hallmark of UIP is the presence of patchy interstitial fibrosis, which is most pronounced in the subpleural and basal regions of the lungs. Histologically, UIP is characterized by the presence of fibroblastic foci, honeycomb change, and temporal heterogeneity of fibrosis.

Diagnosis[edit]

Diagnosis of UIP is typically made through a combination of clinical, radiological, and pathological findings. High-resolution computed tomography (HRCT) scans of the chest are crucial for diagnosis, often showing a pattern of reticular opacities, traction bronchiectasis, and honeycombing. A lung biopsy may be performed to confirm the diagnosis.

Treatment[edit]

There is no cure for UIP, and treatment is primarily supportive. Antifibrotic medications such as pirfenidone and nintedanib may slow disease progression. Lung transplantation is an option for eligible patients with advanced disease.

Prognosis[edit]

The prognosis for patients with UIP is generally poor, with a median survival of 3 to 5 years after diagnosis. The disease course is variable, with some patients experiencing rapid progression and others having a more indolent course.

See also[edit]



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