Usual interstitial pneumonia

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.

Etiology

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.

Pathogenesis

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.

Clinical Features

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.

Diagnosis

The diagnosis of UIP can be challenging and often requires a combination of clinical, radiographic, and sometimes pathologic data. High-resolution CT scans of the chest are often used in the diagnostic process.

Treatment

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.

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.