8 Common Interstitial Lung Diseases and How They're Treated

If you or a loved one has been diagnosed with interstitial lung disease (ILD), you probably have many questions. Read the basics about eight common types of ILD from a trusted physician specializing in diagnosing and treating lung disease.

Interstitial lung diseases encompass a broad group of disorders that affect the lungs, specifically the alveoli (air sacs) and the space around the alveoli (interstitium). These conditions can have various causes, ranging from environmental exposures to autoimmune diseases.

Complications of ILDs include significant lung scarring that make it difficult to breathe normally, reducing the lungs’ ability to produce adequate oxygen for the heart, brain, kidneys, and other vital organs.

Board-certified pulmonologist Dr. Chuan Jiang of Chuan Jiang Medicine in Flushing, New York, specializes in patient-focused, comprehensive care for respiratory conditions such as interstitial lung disease.

Understanding the different types of ILDs and their treatment approaches is crucial for patients and caregivers.

Here, Dr. Jiang and his team share eight common ILDs and how they’re treated:

1. Idiopathic pulmonary fibrosis (IPF)

IPF is one of the most well-known ILDs, characterized by progressive scarring of lung tissue. Treatment options for IPF include medications such as pirfenidone and nintedanib, which can slow down the progression of fibrosis.

Other therapies include supplemental oxygen, pulmonary rehab, exercise, breathing techniques to improve oxygen use, and ongoing education about your condition.

2. Sarcoidosis

Sarcoidosis is an inflammatory disease affecting multiple organs, including the lungs. Treatment may involve corticosteroids to reduce inflammation and other immunosuppressive agents in severe cases. Close monitoring is essential to manage symptoms and prevent complications.

3. Hypersensitivity pneumonitis

This ILD occurs when the lungs become inflamed due to repeated exposure to environmental allergens. Avoiding the triggering agents is a must for managing hypersensitivity pneumonitis.

Dr. Jiang may also prescribe corticosteroids, immunosuppressive medications, and other medicines to reduce inflammation and symptoms.

4. Connective tissue disease-associated ILD (CTD-ILD)

CTD-ILD refers to ILD occurring in association with autoimmune diseases such as rheumatoid arthritis, scleroderma, and lupus.

Treatment focuses on managing the underlying autoimmune condition with corticosteroids, disease-modifying antirheumatic drugs (DMARDs), and biologics. Immunosuppressive therapy may also be necessary to control lung inflammation.

5. Nonspecific interstitial pneumonia (NSIP)

NSIP is characterized by inflammation and scarring of the lung tissue, often with a better prognosis than IPF.

Treatment typically involves corticosteroids and immunosuppressive agents to reduce inflammation and slow disease progression. Dr. Jiang may also prescribe oxygen therapy in advanced cases to relieve symptoms of breathlessness.

6. Cryptogenic organizing pneumonia (COP)

COP is characterized by inflammation and granulation tissue formation in the small airways and alveoli. Corticosteroids are the mainstay of treatment for COP, with most patients experiencing significant improvement within weeks to months of therapy.

However, relapses can occur, requiring prolonged treatment or additional courses of corticosteroids.

7. Lymphangioleiomyomatosis (LAM)

LAM is rare compared to other types, but it’s a progressive ILD that primarily affects women of childbearing age. Treatment options include mTOR inhibitors such as sirolimus and everolimus, which can slow disease progression and improve lung function.

A lung transplant may be considered in advanced cases or when medical therapy is ineffective.

8. Acute interstitial pneumonia (AIP)

AIP is a rapidly progressive form of ILD characterized by severe inflammation and lung injury. Treatment involves supportive care with mechanical ventilation and high-dose corticosteroids to reduce inflammation.

Unfortunately, the prognosis for AIP is often poor, with a high mortality rate despite aggressive management.

Interstitial lung diseases encompass a diverse group of conditions with varying causes and treatment approaches.

Early diagnosis and prompt initiation of appropriate therapy are essential for optimizing outcomes and improving the quality of life for patients with ILDs.

Schedule an evaluation with Dr. Jiang today by calling the office or requesting an appointment online.