Usual interstitial pneumonia

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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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

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.

See also

• Idiopathic pulmonary fibrosis
• Interstitial lung disease
• Lung transplantation