Pulmonary fibrosis (lung scarring)

About Pulmonary fibrosis (lung scarring)

Also known as `interstitial lung disease.` Scarring in the lungs that results in thickening of the lung tissue. 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 & Diagnostics

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.

Common tests: Complete blood count (CBC), Comprehensive metabolic panel (CMP), CT Scan, MRI, X-ray.

Path 3 — Prescription Options

Prescription medications

Conventional treatment summary

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. For additional information contact the National Heart, Lung and Blood Institute Health Information Center at: http://www.nhlbi.nih.gov or call (301)592-8573. American Lung Association: (800)586-4872 National Jewish Medical and Research Center: (800)222-5864

Medical specialties

Pulmonology · Pediatric Pulmonology

This page is educational content, not medical advice. Always consult a qualified clinician for diagnosis and treatment of Pulmonary fibrosis (lung scarring).