lungfibros

Lungfibros, or pulmonary fibrosis, is a condition characterized by progressive scarring of the lung interstitium. The scarring thickens the alveolar walls, reducing elasticity and impairing gas exchange. The disease can be idiopathic or linked to other diseases, exposures, or treatments.

Idiopathic pulmonary fibrosis (IPF) is the most common form. Other types include connective tissue disease–associated ILD, hypersensitivity pneumonitis, asbestosis, drug- or radiation-induced fibrosis. Genetic factors may predispose to fibrosis, including telomere-related gene mutations.

Injury and abnormal wound healing drive fibrosis. Histology often shows usual interstitial pneumonia (UIP): patchy, temporally heterogeneous fibrosis with fibroblastic foci; honeycombing may appear in advanced disease.

Symptoms usually begin with exertional breathlessness and a dry cough, with fatigue as the disease progresses. On exam, basal fine crackles and, later, clubbing may be present. Hypoxemia and pulmonary hypertension can develop in advanced stages.

Diagnosis combines clinical assessment, high-resolution CT showing UIP in many cases, and lung function tests revealing a restrictive pattern with reduced DLCO. Lung biopsy is sometimes required when imaging is inconclusive.

Treatment focuses on slowing progression and relieving symptoms. Antifibrotic drugs such as nintedanib or pirfenidone slow FVC decline in IPF. Supportive care includes oxygen therapy, pulmonary rehabilitation, vaccination, and management of complications; lung transplantation may be an option.

Prognosis varies. IPF generally carries a poor prognosis, with median survival around 3 to 5 years after diagnosis, but outcomes differ widely depending on comorbidity and rate of progression.

Research continues to clarify mechanisms, identify biomarkers, and test new therapies.