UAB Medicine

Interstitial Lung Disease and Pulmonary Fibrosis - Overview

A cough that won’t go away and shortness of breath may be the first indication of Interstitial Lung Disease (ILD), which makes up about 150 chronic lung disorders. All of the disorders under this classification result in inflammation and fibrosis (scarring) in the space between the lungs’ air sacs (the interstitium). The most common type is Idiopathic Pulmonary Fibrosis (IPF), which causes scarring and loss of lung function. People diagnosed with IPF have only a 50% survival rate after three years following diagnosis.

Patients often experience a persistent, dry cough associated with gradual decrease in the ability to breathe, so much so that it becomes harder to perform regular daily activities.

Conditions Treated and Services Performed
ILD is a generic term that could account for more than 150 diseases. Some of them are:
  • Acute interstitial pneumonia
  • Alveolar proteinosis
  • Asbestosis
  • Berylliosis
  • Coal worker's pneumoconiosis (black lung disease)
  • Connective tissue disease-associated interstitial lung disease
  • Desquamative Interstitial Pneumonia (DIP)
  • Farmer’s lung
  • Hamman-Rich syndrome
  • Hard-metal pneumoconiosis
  • Honeycomb lung
  • Hypersensitivity Pneumonitis (HSP)
  • Idiopathic Pulmonary Fibrosis
  • Interstitial lung disease
  • Systemic lupus erythematosus (Lupus lung)
  • Lymphocytic Interstitial Pneumonia (LIP)
  • Mixed connective tissue disease
  • Nonspecific Interstitial Pneumonia (NSIP)
  • Pigeon breeder’s disease
  • Pneumoconiosis
  • Polymyositis, dermatomyositis
  • Pulmonary alveolar microlithiasis
  • Pulmonary phospholipoproteinosis
  • Pulmonary eosinophilic granuloma (EG), histiocytosis X (HX), or Langerhans cell        granulomatosis
  • Pulmonary fibrosis
  • Respiratory bronchiolitis
  • Respiratory Bronchiolitis-associated Interstitial Lung Disease (RB-ILD)
  • Rheumatoid lung
  • Sarcoidosis
  • Scleroderma lung
  • Shortness of breath
  • Silicosis
  • Smoker’s bronchiolitis
  • Usual Interstitial Pneumonia (UIP) 
Many ILDs are chronic conditions without a cure. However, UAB can offer many treatments to improve a patient’s quality of life, such as oxygen and pulmonary rehabilitation. Lung transplantation may be an option for some patients suffering from these disorders and is the only treatment shown to actually increase survival of patients with Idiopathic Pulmonary Fibrosis.
  • Cardio Pulmonary Exercise Testing (CPET)
  • Chest x-ray
  • High-Resolution CT of the Chest
  • Bronchoscopy  
  • Surgical Lung Biopsy/Video-Assisted Thoracoscopic lung biopsy