Usual interstitial pneumonia: Difference between revisions

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Usual Interstitial Pneumonia

Lung biopsy showing 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

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

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.

Related Pages

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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.
+== Usual Interstitial Pneumonia ==
-==Etiology==
+[[File:CT_scan_in_usual_interstitial_pneumonia_(UIP).jpg|thumb|CT scan showing usual interstitial pneumonia]]
+[[File:UIP_(Usual_interstitial_pneumonia)-CT_scan.jpg|thumb|Another CT scan of usual interstitial pneumonia]]
+[[File:UIPlungbiopsy.jpg|thumb|Lung biopsy showing usual interstitial pneumonia]]
+[[File:Honeycomb_change.jpg|thumb|Honeycomb change in usual interstitial pneumonia]]
+[[File:Fibroblast_focus.jpg|thumb|Fibroblast focus in usual interstitial pneumonia]]
-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.
+'''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]].
-==Pathogenesis==
+== Clinical Features ==
+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.
-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.
+== Pathology ==
+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.
-==Clinical Features==
+== 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.
-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]].
+== 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.
-==Diagnosis==
+== 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.
-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.
+== Related Pages ==
+* [[Idiopathic pulmonary fibrosis]]
+* [[Interstitial lung disease]]
+* [[Lung transplantation]]
-==Treatment==
+{{Pulmonology}}
-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]].
+[[Category:Interstitial lung diseases]]
+[[Category:Pulmonary fibrosis]]
-==Prognosis==
-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.
-[[Category:Respiratory diseases]]
-[[Category:Interstitial lung disease]]
-{{medicine-stub}}