Guest post co-written by Maria Padilla, MD, Professor of Pulmonary, Critical Care, and Sleep Medicine Divison at the Icahn School of Medicine at Mount Sinai, and Aditi Mathur, MD, Assistant Professor of Pulmonary, Critical Care, and Sleep Medicine Division at the Icahn School of Medicine at Mount Sinai.
“So, I have been told I have ILD” what does this mean? How do I live with it? What do I do next?
Nothing can be more surprising than to be told that you have interstitial lung disease as an explanation for your cough, shortness of breath or abnormal radiograph of the chest. You know what asthma, bronchitis, pneumonia, lung cancer or emphysema are, but for many, this may be the first time that you are hearing the term “interstitial lung disease”. This is a group of mostly chronic illnesses that involve the lung tissue in a widespread manner and cast abnormalities on your chest x-ray or CAT scan, a more detailed form of imaging of lung disease. These abnormalities are the result of or response of the lung to injury. The lungs can be injured by matters that we inhale, ingest, carry in our blood stream, receive externally as form of treatment (radiation) or by genetic or autoimmune factors. The injury is often progressive and gradually impairs your ability to carry out the activities you enjoy or even the activities of daily living.
Let’s talk about Interstitial lung disease :
- What is Interstitial Lung Disease?
Interstitial Lung Disease is a rare condition where you develop inflammation, scarring, or both in your lungs. When you get a skin injury, you get redness, swelling and sometimes a visible scar. This can also happen in your lungs and if it is extensive enough, it can cause shortness of breath.
- Why do I have Interstitial Lung Disease?
There are many different subtypes of Interstitial Lung Disease. Sometimes, there is no identifiable cause of ILD and it is termed “idiopathic.” Sometimes, ILD is associated with:
- Autoimmune diseases such as Rheumatoid Arthritis, Lupus, Myositis, Scleroderma, Sjogren’s Syndrome
- Medications such as Amiodarone, Nitrofurantoin, Methotrexate, Bleomycin and other treatments such as radiation
- Chemical exposures such as asbestos, chemicals and fumes used in the cosmetic industry, car repair, cleaning solutions and many more
- Close contact with birds (canaries, parakeets, parrots, pigeons)
- Familial or genetic predisposition – Hermansky Pudlak Syndrome
- Smoking increases your risk of developing certain ILD’s but not all smokers develop ILD
- Acid reflux can make ILD worse and is very commonly associated with ILD
- How is ILD diagnosed?
Diagnosis of ILD requires a review of your medical history, lung function tests, blood tests, a CT scan of your chest, and sometimes a lung biopsy. Your pulmonologist thoroughly reviews all this information and then comes up with a diagnosis. It can sometimes take a month to have all the information needed to determine the cause of your ILD.
- How is ILD treated?
Treatment for ILD often depends on the cause of the ILD. For example, if your ILD is caused by bird exposure, then elimination of contact with the birds would be recommended. Promptly discontinuing medications that are linked to ILD would also be recommended. Some types of ILD are difficult to treat, particularly when they are caught late. Two common types of ILD treatment are anti-fibrotic therapies ( Pirfenidone and Nintedanib) and immunomodulatory therapies such as Prednisone, Mycophenolate and Azathioprine. Nintedanib and Pirfenidone are anti-fibrotic agents approved only for the treatment of Idiopathic Pulmonary Fibrosis (IPF), a fibrosing form of interstitial lung disease . Immunomodulatory agents are used when inflammation is the predominant manifestation of the interstitial lung disease. . All of these medications have side effects and require strict monitoring by your primary care doctor and your pulmonologist. When your pulmonologist makes a decision to treat you with any of these medications, it is because he or she believes the benefits of these medications outweigh the risks.
- What else do I need to know about my ILD?
ILD is associated with a number of co-morbidities (associated illnesses) including acid reflux, sleep apnea, pulmonary hypertension, and depression. These co-morbidities can make your ILD symptoms worse and thus we work together to treat these issues as well.
- How often should I see my pulmonologist if I have ILD?
You should see your pulmonologist every 3 months. If you are doing well and on a stable dose of medications, then we sometimes will see you every 6 months. If you do not feel well or have very aggressive disease, then we may see you every 4 weeks. Always have your blood work appropriately monitored and sent to your doctor’s office for review. Follow up with your doctor to insure that test results have been received, reviewed and any necessary adjustment made to your regimen. Keep your medical records and CT scans in your possession at all times. Always talk to your doctor before stopping a medication.
It is important that you partner with your pulmonologist and/or primary physician in the care of your interstitial lung disease. These diseases are treatable and together we can find the best treatment for your illness in order to improve or resolve it.