Idiopathic pulmonary fibrosis (IPF)

About

IPF is a chronic, progressive, fibrotic interstitial lung disease with an unknown etiology. The core pathological mechanism is the abnormal repair of alveolar epithelial cells after repeated microdamage, leading to fibroblast activation and excessive accumulation of extracellular matrix. Core dilemmas: ① Pirfenidone and nintedanib can only slow down the rate of decline in lung function (about 50%) and cannot reverse the fibrotic tissue that has been formed;② Lung transplantation is the only possible cure, but donors are scarce, The risk of surgery is high, and lifelong immunosuppression is required after surgery.

Features & Symptoms

• Progressive fibrosis and sclerosis of lung tissue
• Lung function continues to decline
• Progressive dyspnea, dry cough
• Loss of mobility

Treatment Options

Prognosis

The 5-year survival rate after diagnosis is less than 50%, and the terminal stage relies on ventilators

Additional Notes

Notes: Lung transplant evaluation: Indication for lung transplantation should be evaluated as soon as possible after diagnosis, and referral should be made to the transplant center if FVC<80% or DLCO<40%; Anti-fibrotic treatment: Nintedanib and Pirfenidone can delay the decline of lung function and is recommended to start as soon as possible; Prevention of acute exacerbations: Avoid known predisposing factors (such as gastroesophageal reflux, lung infection), the mortality rate after acute exacerbations is significantly increased; lung rehabilitation training: Standardized lung rehabilitation training can improve exercise tolerance and quality of life; oxygen therapy indications: SpO2 <88% at rest or PaO2 <55mmHg should be started.; Study phase: Phase II/III clinical. Prognosis: The 5-year survival rate after diagnosis is less than 50%, and the terminal stage is dependent on ventilators. Study phase: Phase II/III clinical. Prognosis: The 5-year survival rate after diagnosis is less than 50%, and the terminal stage relies on ventilators. Exclusive service commitment: The platform will allocate a dedicated medical team to each patient to assist in dismantling and properly deploying the following precautions throughout the process. When cross-state and cross-border diagnosis and treatment projects or drugs are involved, the platform will make overall arrangements for the entire docking; for disabled and semi-disabled patient groups, the platform will provide rush-free alternative solutions (including remote consultation, door-to-door sampling, direct drug delivery in the cold chain, etc.), to ensure that patients can get the same high-quality diagnosis and treatment resources without having to travel in person. Exclusive service commitment: The platform will allocate a dedicated medical team to each patient to assist in dismantling and properly deploying the following precautions throughout the process. When cross-state and cross-border diagnosis and treatment projects or drugs are involved, the platform will make overall arrangements for the entire docking; for disabled and semi-disabled patient groups, the platform will provide rush-free alternative solutions (including remote consultation, door-to-door sampling, direct drug delivery in the cold chain, etc.), to ensure that patients can get the same high-quality diagnosis and treatment resources without having to travel in person...