Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is progressive fibrosing interstitial pneumonia of unknown cause, with UIP pattern on HRCT/biopsy, presenting with progressive dyspnea, bibasilar crackles, clubbing, reduced DLCO, and cough. Treatment: antifibrotic therapy (pirfenidone/nintedanib) to slow decline; oxygen for resting hypoxemia; pulmonary rehab; treat comorbidities (GERD/OBM); refer early for lung transplant; treat acute exacerbations (supportive/steroids); avoid smoking/vaccines; monitor progression; palliative care.

Common Symptoms

Progressive dyspnea; bibasilar Velcro crackles; clubbing; reduced DLCO; chronic

Causes and Pathogenesis

Repeated alveolar epithelial injury causes aberrant fibrogenesis and UIP pattern.

Treatment

Antifibrotics; oxygen/rehab; transplant referral; treat exacerbations; palliative care

Preventive Measures

Smoking cessation; treat GERD; vaccines.

Department to Visit

It is recommended to visit the Pulmonology/Thoracic-surgery/Transplant department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.