According to a recent article in Healio, Therapeutic Options for Interstitial Lung Disease was one of the topics covered at the Congress of Clinical Rheumatology 2020.

Dr. Elizabeth Volkmann co-director and founder of the UCLA CTD-ILD followed the discussion with her opinion that treatment options may be increasing, along with an increased understanding of disease biology.

What is Interstitial Lung Disease (ILD)?

ILD is a large group of diseases that causes fibrosis (scarring) of the lungs. This in turn creates stiffness that makes breathing difficult. It also interferes with oxygen making its way into the bloodstream. The damage worsens over time and may be irreversible. It affects adults and children.

Dr. Volkmann explained that the disease sometimes occurs after an injury to the lungs that triggers abnormal healing.

The doctor said that the body usually generates the correct amount of tissue to repair the damage. However, if a person has ILD, something must have gone wrong with that process, causing the tissue that surrounds alveoli (tiny air sacs) to thicken and become scarred.

Causes of ILD

There are many factors that may directly cause or increase the risk of ILD. These would include genetics, medical treatments, or even certain medications.

Other causes of ILD include being exposed to hazardous materials and smoking. There is also an increased risk if a person has an autoimmune disease such as rheumatoid arthritis or sarcoidosis.

Symptoms of ILD

Shortness of breath is perhaps the most common ILD symptom. The patient may also have chest discomfort, dry cough, fatigue, and sometimes weight loss.

Unfortunately, when these symptoms appear, the lungs have already been damaged. Therefore, people are encouraged to see their doctors at the first sign of these symptoms.

In severe cases, life-threatening complications such as high blood pressure, respiratory failure, or heart failure may develop.

An important take-away from Dr. Volkmann is to preserve lung function by starting treatment early. That includes smoking cessation.

About Lung Disorders

Dr. Volkmann mentioned several lung diseases beginning with:

• Nonspecific interstitial pneumonia affecting tissue separating alveoli
• Usual interstitial pneumonia involving the scaring of both lungs
• Organizing pneumonia whereby the lungs are inflamed and the alveoli are obstructed

The doctor said that patients with these diseases may be fatigued and may have a persistent cough or shortness of breath (dyspnea). On other hand, some patients may be asymptomatic.

Dr. Volkmann suggested screening using a high-resolution chest CT, blood tests to check for antibodies, and pulmonary function testing. The latter will show how well the lungs are functioning, create baseline levels, and track the progression of the disease.

Additional screening may include checking environmental exposures and the history of the patient’s symptoms.

Biomarkers for ILD can provide value towards assessment and prediction of the disease. The biomarkers may include C-reactive protein. Dr. Volkmann encourages clinicians to work with their pulmonologist and radiologist.

Associated Diseases

Dr. Volkmann discussed several diseases. She began by stating that ILD represents the highest number of deaths associated with systemic sclerosis (scleroderma).

Other common diseases are myositis and organizing pneumonia. Arthritis presents risk factors for myositis-related ILD, especially for older adults. Nonspecific interstitial pneumonia and organizing pneumonia are also common in myositis.

About Treatment Options

Dr. Volkmann said that corticosteroids have not proven to be beneficial in lung diseases. Although steroids may be of benefit in organizing pneumonia, they are not effective in usual interstitial pneumonia or nonspecific interstitial pneumonia.

One drug having relevance in lung disease is mycophenolate, although it is not very effective for arthritis.

Rituximab may be utilized to treat arthritis. It may also be of benefit to improve blood function in rheumatoid arthritis-related ILD.

Dr. Volkmann discussed one other option for CTD-ILD. It is nintedanib which is used to treat the lungs. She explained that since it primarily targets the lungs, the clinicians must treat the connective tissue disease separately.

Dr. Volkmann emphasized that there are three drugs that must be used with caution: TFN, leflunomide, and methotrexate.

Dr. Volkmann left us with one more reminder that to prevent losing lung function, treatment must be started as early as possible.