Interstitial Lung Disease (ILD)

Simply Put

The interstitium is a thin, supportive network to the lung air sacs, which contain tiny blood vessels. Interstitial Lung Disease (ILD) is a broad category of more than 100 pulmonary conditions which result in thickening, inflammation, scarring, or edema of the interstitium lung tissues and can affect breathing and absorption of oxygen into the bloodstream.

Potential Causes of ILD

Myositis–related autoimmune diseases such as Polymyositis, Dermatomyositis, Amyopathic Dermatomyositis, Antisynthetase Syndrome and Mixed Connective Tissue Disorder are shown to have an increased risk of ILD, especially those who test positive for the Jo-1 antibody. More than 70% of those who test positive for the Jo-1 antibody will develop ILD.

There are other numerous causes of ILD, but in some cases, the diagnosis is deemed to be “idiopathic;” meaning “no known cause.” The following have been associated with carrying a risk for ILD:

Other Causes of ILD

Other autoimmune diseases such as Lupus, Rheumatoid Arthritis, Scleroderma, and Sjögren’s,
Cigarette smoking
Radiation therapy, specifically in chest area
Bird protein (live pets and feather-containing products)
Mold from indoor hot tubs, showers and prior water damage
Exposure to chemicals and contaminants such as asbestos, coal, cotton, and silica dust

Remember, the causes listed in this section are potential causes and will not always result in ILD.

Medicinal Causes of ILD

Infiximab (immunosuppressant), also known as Remicade
Etanercept (TNF inhibitor used to treat some autoimmune diseases) also known as Embrel
Methotrexate and Cyclophosphamide (chemotherapy and immune modulator) used to treat cancer and some autoimmune diseases
Nitrofurantoin (antibiotic used to treat infections) also known as Macrobid, Macrodantin, other trade names
Sulfasalazine (used to treat rheumatoid arthritis) also known as Azulfidine, Salazopyrin and Sulazine
Sufonamides (sulfa drugs to treat infections)
Bleomycin (cancer treatment)
Amiodarone (antiarrhythmic heart medication) also known as Cordarone, Nexterone, Pacerone
Propranolol (beta blocker) also known as Inderal, Inderide, InnoPran, Hemangeol

Symptoms/Complications of ILD

Difficulty breathing
Progressive shortness of breath
Dry cough
Severe chronic cough, wheezing
Acute pneumonia
Lung infections
Cyanosis (lips, skin or fingernails turn blue from lack of oxygen)
Enlargement of fingernail base
High blood pressure
Heart failure

Diagnosing ILD

Chest x-rays and CT scans can detect some lung conditions. However, because the interstitium contains tiny, thin blood vessels, damage cannot always be detected by these methods.
Echocardiogram (a sonogram for the heart)
Pulmonary Function Tests (Oximetry, Spirometry and Diffusion Capacity tests)
Lung tissue analysis (biopsy of lung tissue using a bronchoscope, a small tube that passes through the mouth or nose into the lungs to obtain a tiny tissue sample)
Surgical cutting of certain muscles to allow for better swallowing
Surgical biopsy (biopsy by extracting lung tissue sample which can obtain a large enough tissue sample to make a more accurate diagnosis and uses camera and video monitor to record condition of the lungs and interstitium)

Treatment for ILD

Corticosteroids and some immunosuppressive drugs have been used successfully to treat various forms of ILD, especially when the cause is autoimmune disease related. Rituxan (Rituximab) and IVIG have shown effectiveness in recent studies, also when autoimmune in nature.

Simply Put

“Simply Put” is a service of Myositis Support and Understanding, to provide overviews of Myositis-related medical and scientific information in understandable language.

MSU volunteers, who have no medical background, read and analyze often-complicated medical information and present it in more simplified terms so that readers have a starting point for further investigation and consultation with healthcare providers. The information provided is not meant to be medical advice of any type.