Description Also known as "...
Description
Also known as "interstitial lung disease". Scarring in the lungs results in thickening of the lung tissue between air sacs. Pulmonary fibrosis is different from emphysema in a smoker because emphysema causes lung tissue to thin. Pulmonary fibrosis interferes with lung function and causes low oxygen in the blood. Many conditions cause gradual scar formation (fibrosis) in the lungs including: hypersensitivity reactions (a severe type of allergic reaction), sarcoidosis, Wegener's granulomatosis, lupus, rheumatoid arthritis, infections, environmental agents (asbestos, silica, exposure to certain gases), exposure to ionizing radiation (such as radiation therapy to treat cancer), and certain medications (nitrofurantoin, methotrexate). Often no cause of fibrosis is identified. This may be called "idiopathic pulmonary fibrosis".
Symptoms
Chest pain (occasionally), cough (usually dry), exercise intolerance, shortness of breath, fatigue.
Tests
Common tests used for diagnosis and treatment
Workup:
A history and physical exam will be performed. The following test may be performed to help establish the diagnosis: bronchoscopy with transbronchial lung biopsy, pulmonary function tests, surgical lung biopsy. Blood tests to diagnose the following autoimmune diseases may be done: rheumatoid arthritis, lupus, and/or scleroderma.
Tests:
Complete blood count (CBC), Comprehensive metabolic panel (CMP), CT Scan, Magnetic resonance imaging (MRI), X-ray
Other Specific Tests: Bronchoscopy with transbronchial lung biopsy, pulmonary function tests, surgical lung biopsy
Specialists:
Pulmonology, Pediatric Pulmonology
Workup:
A history and physical exam will be performed. The following test may be performed to help establish the diagnosis: bronchoscopy with transbronchial lung biopsy, pulmonary function tests, surgical lung biopsy. Blood tests to diagnose the following autoimmune diseases may be done: rheumatoid arthritis, lupus, and/or scleroderma.
Tests:
Complete blood count (CBC), Comprehensive metabolic panel (CMP), CT Scan, Magnetic resonance imaging (MRI), X-ray
Other Specific Tests: Bronchoscopy with transbronchial lung biopsy, pulmonary function tests, surgical lung biopsy
Specialists:
Pulmonology, Pediatric Pulmonology
Treatment
There is no cure for pulmonary fibrosis. Treatment is aimed at slowing down the scarring process and preventing hypoxia (low oxygen levels). Medications such as corticosteroids and immunosuppressive medications (cyclophosphamide, azathioprine, methotrexate, penicillamine, cyclosporine) may be given to reduce the inflammation and scarring, but these treatments are not very effective. Oxygen is administered for low blood oxygen levels. Treatment of acid reflux with medicines and surgery may help some patients. Lung transplant may be needed for some patients.