Find information on medical topics, symptoms, drugs, procedures, news and more, written for the health care professional.

* This is the Professional Version. *

Overview of Interstitial Lung Disease

(Diffuse Parenchymal Lung Diseases)

by Harold R. Collard, MD

Interstitial lung diseases are a heterogeneous group of disorders characterized by alveolar septal thickening, fibroblast proliferation, collagen deposition, and, if the process remains unchecked, pulmonary fibrosis. Interstitial lung diseases can be classified using various criteria (eg, acute vs chronic, granulomatous vs nongranulomatous, known cause vs unknown cause, primary lung disease vs secondary to systemic disease).

Among the numerous possible causes are most connective tissue disorders and occupational lung exposures and many drugs (see Environmental Pulmonary Diseases and Causes of Interstitial Lung Disease). A number of interstitial lung diseases of unknown etiology have characteristic histology, clinical features, or presentation and thus are considered unique diseases, including eosinophilic pulmonary diseases, pulmonary Langerhans cell histiocytosis (granulocytosis), lymphangioleiomyomatosis, pulmonary alveolar proteinosis, and sarcoidosis. In up to 30% of patients who have interstitial lung diseases with no clear cause, the disorders are distinguished primarily by characteristic histopathologic features; these disorders are termed the idiopathic interstitial pneumonias.

Causes of Interstitial Lung Disease

Category

Examples

Connective tissue disorders

Ankylosing spondylitis (rare)

Behçet syndrome (very rare)

Dermatomyositis and polymyositis

Goodpasture syndrome

Mixed connective tissue disease

RA

Sjögren syndrome

SLE

Systemic sclerosis

Undifferentiated connective tissue disease

Drugs

Selected list: Amphotericin B, bleomycin, busulfan, carbamazepine, chlorambucil, cocaine, cyclophosphamide, diphenylhydantoin, flecainide, heroin, melphalan, methadone, methotrexate, methylphenidate, methysergide, mineral oil (via chronic microaspiration), nitrofurantoin, nitrosoureas, procarbazine, silicone (sc injection), tocainide, vinca alkaloids (with mitomycin)

Occupational and environmental exposure

Inorganic (selected): Aluminosis (caused by exposure to metallic aluminum powder), asbestosis, baritosis, berylliosis, coal workers’ pneumoconiosis, exposure to hard metals (eg, cadmium, cobalt, titanium oxide, tungsten, vanadium carbides), radiation fibrosis, siderosis, silicosis, stannosis, talc pneumoconiosis

Organic (selected): Bagassosis, bird fancier’s lung, coffee worker’s lung, farmer’s lung, hot tub lung, humidifier lung, malt worker’s lung, maple bark stripper’s lung, mushroom worker’s lung, tea grower’s lung

Infections

Aspergillosis

Histoplasmosis

Parasitic infection

Mycobacterial infection

Viral infection

Vasculitis

Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome)

Giant cell arteritis (rare)

Granulomatosis with polyangiitis (formerly Wegener granulomatosis)

Microscopic polyangiitis

Polyarteritis nodosa (rare)

Takayasu arteritis (rare)

Idiopathic interstitial pneumonias

Acute interstitial pneumonia

Cryptogenic organizing pneumonia

Desquamative interstitial pneumonia

Idiopathic pulmonary fibrosis

Lymphocytic interstitial pneumonia

Nonspecific interstitial pneumonia

Respiratory bronchiolitis–associated interstitial lung disease

Miscellaneous disorders

Amyloidosis

Chronic aspiration

Eosinophilic pneumonia

Gaucher disease (rare)

Lipoid pneumonia

Lymphangioleiomyomatosis

Microlithiasis

Neurofibromatosis

Niemann-Pick disease (rare)

Pulmonary alveolar proteinosis

Pulmonary Langerhans cell histiocytosis (granulomatosis)

Pulmonary lymphoma

Sarcoidosis

Tuberous sclerosis

Resources In This Article

Drugs Mentioned In This Article

  • Drug Name
    Select Trade
  • FURADANTIN, MACROBID, MACRODANTIN
  • DOLOPHINE
  • CYTOXAN (LYOPHILIZED)
  • No US brand name
  • MITOSOL
  • ALKERAN
  • MYLERAN
  • LEUKERAN
  • MATULANE
  • OTREXUP
  • CONCERTA, RITALIN
  • TEGRETOL

* This is a professional Version *