وصف
Pulmonary fibrosis is diagnosed through a combination of medical history, imaging studies (like high-resolution CT scans), pulmonary function tests, and sometimes lung biopsies. Treatment often involves medications such as corticosteroids, immunosuppressants (like azathioprine or mycophenolate), and antifibrotic drugs (such as pirfenidone or nintedanib) to slow disease progression and manage symptoms.Supplemental oxygen therapy is commonly prescribed to relieve shortness of breath and improve oxygen levels in the blood. This program combines exercise training, education, and breathing techniques to improve overall physical condition and quality of life. Patients with pulmonary fibrosis should receive vaccinations against influenza and pneumococcal pneumonia to reduce the risk of respiratory infections. In severe cases, lung transplantation may be considered for eligible patients to improve survival and quality of life.
Treatments like nutritional support, psychological counseling, and palliative care can provide comprehensive support and symptom management.
ملخص
- Dr. Subna discusses progressive pulmonary fibrosis (PPF), highlighting its increasing prevalence post-COVID and the challenges in determining which patients require treatment versus observation. The talk will cover the definition, management, and crucial considerations for these patients, especially given the significant advancements in interstitial lung disease (ILD) treatment over the last decade.
- ILD encompasses a variety of lung diseases, including idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), respiratory bronchiolitis-associated ILD, and cryptogenic organizing pneumonia. Autoimmune-related ILDs, hypersensitivity pneumonitis, sarcoidosis, and rare conditions like lymphangioleiomyomatosis also contribute to the spectrum of interstitial lung diseases.
- PPF is diagnosed when patients with ILD (excluding IPF) and radiological evidence of pulmonary fibrosis exhibit at least two of three criteria: worsening respiratory symptoms, physiological evidence of disease progression (e.g., FVC decline >5% or DLCO decline >10% annually), and HRCT evidence of disease progression (e.g., increased reticulation, honeycombing, or volume loss).
- Diseases other than IPF known to cause progressive fibrosis and be involved in PPF include various idiopathic interstitial pneumonias (IIPs) like NSIP, fibroelastosis, and unclassifiable ILDs, as well as organizing pneumonia (OP), lymphoid interstitial pneumonia (LIP), and autoimmune-related ILDs associated with conditions like rheumatoid arthritis, systemic sclerosis, and vasculitis.
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