What are the other Names for this Condition? (Also known as/Synonyms)

• Idiopathic Pulmonary Fibrosis (IPF)
• ILD (Interstitial Lung Disease)
• Interstitial Pneumonia

What is Interstitial Lung Disease? (Definition/Background Information)

• Interstitial Lung Disease (ILD) is a group of progressive lung disorders characterized by the thickening of the interstitium - the connective tissue which provides structural support to many components of the lung
• Interstitial Lung Disease can be caused by certain autoimmune conditions and/or chronic exposure to airway irritants. The condition can result in shortness of breath and persistent dry cough, and severe complications if left untreated
• Interstitial Lung Disease is a blanket term for many related lung disorders. Hence, the treatment options may vary depending on the etiology/cause, severity of signs and symptoms, and the associated complications
• The scarring and inflammation of the interstitium is generally irreversible and makes it difficult for efficient oxygen and carbon dioxide exchange to occur. The prognosis of Interstitial Lung Disease depends on its severity among other factors

Who gets Interstitial Lung Disease? (Age and Sex Distribution)

• Although all age groups may be affected, middle and older age adults are at an increased risk for Interstitial Lung Disease (ILD)
  • Those who work in construction, farming, or mining industries are at an increased risk for ILD due to increased exposure to airway irritants
  • Increased risk seen in those with connective tissue diseases such as lupus, rheumatoid arthritis, scleroderma, dermatomyositis, and many other conditions
• Both males and females are affected, and no gender preference is noted
• The condition is observed worldwide. All racial and ethnic groups may be affected

What are the Risk Factors for Interstitial Lung Disease? (Predisposing Factors)

The risk factors for Interstitial Lung Disease may include:

• Chronic exposure to airborne toxins and particulates present in the construction, farming, and mining sectors. These include asbestos, coal dust, grain dust, silica dust, animal droppings, mold exposure, bird proteins, etc.
• Certain autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, dermatomyositis, Sjogren’s syndrome, etc.
• Inherited surfactant deficiencies which increase the risk of many chronic lung disorders that may present as Interstitial Lung Disease
• Cancer treatment: Radiation therapy to the chest and administration of certain chemotherapeutic drugs
• Use of certain medications, such as amiodarone, methotrexate, and nitrofurantoin, among others

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Interstitial Lung Disease? (Etiology)

Interstitial Lung Disease (ILD) is caused by damage to tissues around the alveoli that ultimately leads to inadequate oxygen supply in the body. Conditions that cause damage to the lung tissues include:

• Smoking
• Long-term exposure to airborne toxins
• Radiation therapy and chemotherapy for lung cancer, which can lead to lung damage
• Certain autoimmune disorders such as rheumatoid arthritis, sarcoidosis, and scleroderma

In some cases, the cause of ILD is unknown (idiopathic).

What are the Signs and Symptoms of Interstitial Lung Disease?

The signs and symptoms of Interstitial Lung Disease primarily include:

• Chronic dry cough
• Shortness of breath and tachypnea (fast breathing)
• Fatigue
• Weight loss

How is Interstitial Lung Disease Diagnosed?

Interstitial Lung Disease is diagnosed on the basis of the following information:

• Complete physical examination
• Evaluation of medical history, including one’s occupation and medication history
• Assessment of the presenting signs and symptoms
• Lung function assessment via pulmonary function test (PFT) and 6 minute walk test
• Laboratory and imaging tests such as chest X-ray or CT chest (high resolution)
• Lung biopsy, if required - however, this has become less necessary with high resolution chest CT (HRCT)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Interstitial Lung Disease?

The complications of Interstitial Lung Disease may include:

• Respiratory failure
• Pneumonia
• Bronchiolitis
• Pulmonary hypertension

How is Interstitial Lung Disease Treated?

Typically, there is no cure for Interstitial Lung Disease (ILD), as the lung scarring is generally irreversible. Various forms of ILD require different treatments depending on the cause and sudden exacerbations. These include:

• Antibiotics to treat pneumonia
• Corticosteroids to reduce inflammation in the lungs
• Oxygen administration to relieve shortness of breath and improve low oxygen levels in the blood
• Diuretics, such as furosemide, for fluid overload (pulmonary edema)
• Anticoagulation to treat blood clots
• Pulmonary rehabilitation to improve walking capacity and breathing
• Treatment of gastroesophageal reflux disease (GERD) to prevent aspirations into the lungs
• Antifibrotic therapy, such as pirfenidone or nintedanib, has been approved for Idiopathic Pulmonary Fibrosis (IPF)
• Nintedanib has been approved for the use of Interstitial Lung Disease related to scleroderma and related to progressive fibrosing ILD

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

How can Interstitial Lung Disease be Prevented?

At the present time, there are no effective methods or guidelines available for the prevention of Interstitial Lung Disease. However, the following steps may be considered to lower one’s risk for the same:

• Seeking medical attention at the onset of signs and symptoms may help avoid life-threatening complications. Early treatment may prevent progression of the disease
• Minimizing exposure to pathogens and airborne pollutants
• Adhering to the treatment regimen given by the healthcare provider is imperative to the recovery and relief of symptoms

Active research is currently being performed to explore the possibilities for treatment and prevention of inherited autoimmune disorders that may contribute to Interstitial Lung Disease.

What is the Prognosis of Interstitial Lung Disease? (Outcomes/Resolutions)

• The prognosis of Interstitial Lung Disease is dependent upon the etiology, the affected individual’s age, genetics, the severity of signs and symptoms and associated complications, and progression of the condition
• Interstitial Lung Disease form a group of chronic and progressive disorders that can result in severe deterioration of health without prompt medical treatment 
• Many individuals have shown an improvement in managing/stabilizing the condition over time with adequate management of the condition

Additional and Relevant Useful Information for Interstitial Lung Disease:

The following link is a useful resource for more information on rare disorders:

https://www.dovemed.com/diseases-conditions/rare-disorders/