longfibrose

Longfibrose is a chronic, progressive interstitial lung disease characterized by fibrotic scarring of the lung parenchyma, leading to stiffness of the lungs and impaired gas exchange. In many contexts the term corresponds to pulmonary fibrosis, including idiopathic pulmonary fibrosis (IPF) and other fibrosing interstitial pneumonias.

Etiology and classification: Longfibrose can be idiopathic or secondary to environmental exposures (such as silica or asbestos), certain medications, radiation, or autoimmune diseases. Genetic factors may contribute in some individuals. The most common idiopathic form is idiopathic pulmonary fibrosis, which follows a progressive course.

Pathophysiology and imaging: The disease results from abnormal wound healing after repeated injury to alveolar epithelium, with excessive deposition of collagen and other extracellular matrix. On high-resolution CT imaging, the usual interstitial pneumonia pattern shows subpleural and basal predominance, reticulation, and honeycombing with traction bronchiectasis.

Symptoms and diagnosis: Patients typically experience gradually worsening dyspnea on exertion and a dry cough, along with fatigue and, in some, digital clubbing. Diagnosis relies on clinical assessment, HRCT imaging, and pulmonary function testing showing a restrictive pattern with reduced forced vital capacity and diffusion capacity for carbon monoxide; exclusion of other causes may require bronchoscopy or surgical lung biopsy.

Management and prognosis: There is no cure. Treatments aim to slow progression and manage symptoms, including antifibrotic drugs such as pirfenidone and nintedanib, supplemental oxygen, pulmonary rehabilitation, vaccination, and management of comorbidities. Lung transplantation may be considered in selected patients. The prognosis varies and, for idiopathic forms, median survival after diagnosis is commonly cited as about three to five years.