Interstitial Lung Disease

Our Approach to Treatment

Houston Methodist diagnoses, treats and improve patients’ lives through expert evaluation and advanced management of lung disease.

Our highly skilled staff treats patients with lung disease and has access to the latest technologies. We are committed to providing the best possible care for our patients, no matter their disease stage.

Approved therapies exist for many forms of interstitial lung disease. Patients may qualify for clinical trial participation for experimental therapies to treat interstitial lung disease.

Definition
Interstitial lung disease encompasses hundreds of disorders characterized by lung tissue inflammation, followed by scarring. The interstitium, the area in and around the lung’s small blood vessels and alveoli (air sacs), becomes thickened and damaged, restricting oxygen flow from the air sacs into the bloodstream.

Causes

• Autoimmune or rheumatologic diseases — conditions that cause the body's immune system to attack healthy cells, such as joints and connective tissue (bone and cartilage), and frequently the lungs, including:
  – Dermatomyositis/polymyositis
  – Rheumatoid arthritis
  – Scleroderma
  – Sjogren's syndrome
  – Systemic lupus erythematosus (lupus)
• Infections
  – Fungal
  – Parasitic
  – Viral
  – Tuberculosis
• Occupational and environmental exposures
  – Asbestos, silica, beryllium inhalation
  – Bird dropping inhalation
  – Coal dust inhalation
  – Inhalation of hay dust, mold spores or other agricultural products
  – Inhalation of mold from indoor hot tubs, showers and prior water damage
  – Inhalation of fumes
• Medication and radiation
  – Chemotherapy and immunotherapy drugs
  – Heart medications
  – Antibiotics
• Other causes
  – Idiopathic interstitial pneumonias — lung diseases resulting from unknown causes
  – Pulmonary alveolar proteinosis (PAP) —  a rare lung disease involving buildup of grainy material in the lungs’ air sacs
  – Lymphangioleiomyomatosis (LAM) — a rare lung disease typically affecting childbearing-age women. Muscle cells that line the airways, as well as blood vessels, abnormally multiply
  – Pulmonary Langerhan's cell histiocytosis — a disorder causing cells called histiocytes and eosinophils to multiply in the lungs, often causing scarring. 
  – Pulmonary alveolar microlithiasis (PAM) —a rare, inherited disorder associated with small stone formation in the lung’s air sacs
  – Sarcoidosis — a condition causing abnormal masses or nodules of inflamed tissues to form in certain organs of the body, including the lungs
  
  

Symptoms and exam findings

• Shortness of breath, especially with exertion or exercise
• Dry cough without phlegm
• Weight loss
• Fatigue
• Muscle and joint pain
• Abnormal chest sounds

Diagnostics

• Chest X-rays
• CT scan
• Pulmonary function tests — requires blowing into a spirometer, which measures lung capacity
• Arterial blood gas
• Exercise tests
• Blood tests
• Bronchoscopy 
• Bronchoalveolar lavage — a small amount of sterile saline is injected in the lung and withdrawn. The fluid contains cells that are analyzed under a microscope
• Lung biopsy
  
  

Treatments

• Medications specific to the different kinds of interstitial lung disease  
• Oxygen therapy
• Pulmonary rehabilitation — a program that can include education, exercise, breathing and energy-saving techniques, respiratory therapy evaluation, and nutrition counseling
• Lung transplant
• Whole lung lavage for pulmonary alveolar proteinosis (PAP) after other therapies fail. Sterile fluid is injected into the lung and removed, along with abnormal material