Pulmonary Fibrosis and Interstitial Lung Disease: Symptoms and Next Steps

What it involves

MedlinePlus explains that pulmonary fibrosis is a condition in which the tissue deep in the lungs becomes scarred over time, thick and stiff, which makes it hard to catch your breath. Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs, and in most cases the cause cannot be found.

Who this is for

• Adults with a dry cough or shortness of breath that has lasted for weeks or months.
• Patients who have noticed gradual weight loss, aching muscles and joints, or unusual fatigue.
• People with past work around dust, asbestos, silica, mold or fumes who now have breathing symptoms.
• Anyone with a connective tissue disease, such as rheumatoid arthritis or lupus, and a new cough.

What to expect

• Questions about when the cough and breathlessness began, your work history, smoking and your medicines
• A physical exam that includes listening to your lungs and looking at your fingers and nails
• Blood work, which the practice offers, to look for other causes such as autoimmune disease
• A referral for lung function tests, a chest scan or a lung specialist when those are needed

How it tends to show up

The MedlinePlus encyclopedia describes shortness of breath during activity that lasts for months or years and over time may also occur at rest, usually with a dry cough. Chest pain, feeling faint and gradual weight loss can come with it. Because breathlessness has many everyday causes, people often put it down to age. The shortness of breath guide and the persistent cough guide describe the more common explanations a provider checks first, and a cough that will not clear is worth a visit rather than a long wait.

What can lead to scarring

According to MedlinePlus, pulmonary fibrosis can follow environmental pollutants, some medicines, some connective tissue diseases and interstitial lung disease. Breathing in dust or particles causes some types, including asbestosis from asbestos fibers, silicosis from silica dust, farmer's lung from farm dust and black lung from coal dust. Other causes include autoimmune diseases and workplace exposure to molds, gases or fumes. When no cause is found it is called idiopathic pulmonary fibrosis, which the encyclopedia says is more common in people between 60 and 70 and more common in men.

What a provider looks for

Your provider asks about exposure to asbestos or other toxins and whether you have smoked, then examines you. The encyclopedia says the exam may find abnormal breath sounds called crackles that sound like Velcro being pulled apart, clubbing of the fingertips, and a bluish color around the lips or nails with advanced disease. Joint pain and muscle aches raise the question of an autoimmune cause, and the rheumatoid arthritis guide and the lupus guide describe two of them.

Tests that sort it out

MedlinePlus says providers use your history, imaging tests, lung function tests and sometimes a biopsy. The encyclopedia lists a high resolution chest CT scan, chest x-ray, bronchoscopy, blood oxygen measurements, pulmonary function tests, a 6-minute walk test, an echocardiogram and blood tests for autoimmune disease. The practice offers blood work, and an EKG at the Loxahatchee and West Palm Beach offices. Scans, lung function tests and any biopsy are arranged by referral, usually to a lung specialist.

Treatment and living with it

There is no cure, and MedlinePlus says treatment can help with symptoms and quality of life. It lists medicines, oxygen therapy, pulmonary rehabilitation and, for some people, a lung transplant. The encyclopedia names two prescription medicines that slow lung damage without reversing damage that has already happened. These come from a lung specialist, not from a primary care visit. If you or someone at home smokes, stopping matters, and the quitting smoking guide explains how to start. The COPD guide covers a different lung condition with some overlap.

When to be seen and when to go to the ER

Call 911 or go to the ER for severe trouble breathing, blue fingertips or lips, chest pain with sweating, or confusion and sleepiness with hard breathing. The encyclopedia lists breathing that is harder, faster or shallower, needing to lean forward to breathe, fever, dark mucus and frequent headaches as reasons to contact a provider right away. A dry cough or breathlessness that has gone on for weeks needs a regular visit, and the lung cancer screening guide explains a separate test for long-time smokers.

Sources

• MedlinePlus: Pulmonary Fibrosis
• MedlinePlus: Interstitial Lung Diseases
• MedlinePlus Medical Encyclopedia: Idiopathic pulmonary fibrosis