II. Definition

  1. Chronic, nonmalignant, noninfectious diseases
  2. Lower respiratory tract disease
    1. Distal lung parenchyma disease
    2. Alveolar wall inflammation and derangement

III. Causes (Mnemonic: DISCO)

  1. Drugs (See below)
  2. Idiopathic Pulmonary Fibrosis
  3. Sarcoidosis
  4. Collagen Vascular Disease
  5. Occupational Exposure
    1. Asbestos
    2. Fumes and gasses

IV. Causes: Non-Granulomatous Causes

  1. Occupational Exposure
  2. Medications
    1. Antibiotics (e.g. Nitrofurantoin and GFR<30 ml/min)
    2. Chemotherapeutics (e.g. Methotrexate)
  3. Radiation
  4. Aspiration Pneumonia
  5. Post ARDS changes
  6. Idiopathic Pulmonary Fibrosis
  7. Collagen Vascular Disease
  8. Goodpasture's Syndrome
  9. Eosinophilic Pneumonia
  10. Amyloidosis
  11. Graft versus Host disease (Bone Marrow Transplant)

V. Causes: Granulomatous Causes

  1. Hypersensitivity Pneumonitis (Organic dust)
  2. Inorganic dust (Beryllium silica)
  3. Sarcoidosis
  4. Langerhans Cell Granulomatosis
  5. Granulomatous Vasculitis
    1. Wegener's Granulomatosis
    2. Allergic Granulomatosis of Churg-Strauss
    3. Lymphomatoid granulomatosis
  6. Bronchocentric granulomatosis

VI. Demographic Breakdown

  1. Age 20 to 40 years
    1. Sarcoidosis
    2. Collagen Vascular Disease
    3. Eosinophilic Granuloma or Pulmonary Histiocytosis X
    4. Lymphangioleiomyomatosis
  2. Age over 40 years
    1. Idiopathic Pulmonary Fibrosis
  3. Female Predominance
    1. Collagen Vascular Disease
    2. Lymphangioleiomyomatosis
    3. Tuberous Sclerosis
  4. Male Predominance
    1. Pneumoconiosis (Asbestosis)
  5. Tobacco exposure
    1. More likely with Tobacco exposure
      1. Eosinophilic Granuloma or Pulmonary Histiocytosis X
      2. Respiratory Bronchiolitis (Smoker's Bronchiolitis)
      3. Diffuse alveolar Hemorrhage: Goodpasture's Syndrome
    2. Less likely if Tobacco exposure
      1. Idiopathic Pulmonary Fibrosis
      2. Sarcoidosis
      3. Hypersensitivity Pneumonitis

VII. Symptoms

  1. Historical data
    1. Complete occupational and environmental exposures
    2. Complete medication history
  2. Exertional Dyspnea
  3. Chronic Dyspnea
  4. Fatigue
  5. Malaise

VIII. Signs

  1. Inspiratory crackles (rales) at posterior lung bases
  2. Pulmonary Hypertension
  3. Fingernail Clubbing
    1. Suggests Idiopathic Pulmonary Fibrosis
    2. May suggest Bronchogenic Carcinoma

IX. Labs

  1. ACE Level is non-specific and not useful
  2. Erythrocyte Sedimentation Rate (ESR)
    1. Increased
  3. Complete Blood Count (CBC)
    1. Polycythemia (rare)
  4. Arterial Blood Gas (ABG)
    1. Hypoxemia

X. Diagnosis

  1. Pulmonary Function Tests
    1. Restrictive pattern
    2. Reduced Total Lung Capacity
    3. DLCO decreased
  2. Bronchoalveolar Lavage
    1. Alveolar cells reflect type of inflammation

XI. Radiology

  1. Chest XRay
    1. Lung parenchymal change (90%)
    2. Increased Interstitial Markings
    3. Non-resolving infiltrates
  2. Gallium Lung scanning
    1. Diffuse inflammation

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Ontology: Lung Diseases, Interstitial (C0206062)

Definition (MSH) A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.
Definition (MSHCZE) Různorodá skupina plicních onemocnění, která ovlivňují plicní parenchym. Pro tuto skupinu nemocí je charakteristický prvotní zánět PLICNÍCH ALVEOL, který se rozšiřuje na intersticium a vede postupně k difúzní PLICNÍ FIBRÓZE. Intersticiální plicní nemoci jsou klasifikovány podle své etiologie (známých nebo neznámých příčin) a podle radiologicko patologických rysů.
Definition (MEDLINEPLUS)

Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. The inflammation and scarring make it hard to get enough oxygen. The scarring is called pulmonary fibrosis.

Breathing in dust or other particles in the air are responsible for some types of interstitial lung diseases. Specific types include

  • Black lung disease among coal miners, from inhaling coal dust
  • Farmer's lung, from inhaling farm dust
  • Asbestosis, from inhaling asbestos fibers
  • Siderosis, from inhaling iron from mines or welding fumes
  • Silicosis, from inhaling silica dust

Other causes include autoimmune diseases or occupational exposures to molds, gases, or fumes. Some types of interstitial lung disease have no known cause.

Treatment depends on the type of exposure and the stage of the disease. It may involve medicines, oxygen therapy, or a lung transplant in severe cases.

Definition (CSP) heterogeneous group of noninfectious, nonmalignant disorders of the lower respiratory tract, affecting primarily the alveolar wall structures but also often involving the small airways and blood vessels of the lung parenchyma; may lead to respiratory insufficiency and death.
Concepts Disease or Syndrome (T047)
MSH D017563
ICD10 J84.9
SnomedCT 233703007, 64667001
English Interstitial Lung Diseases, Lung Disease, Interstitial, INTERSTITIAL LUNG DISEASE, Interstitial Lung Disease, ILD - Intrstitial lung disease, Interstitial pulmonary disease, unspecified, INTERSTITIAL LUNG DIS, LUNG DIS INTERSTITIAL, ILD, Diffuse Parenchymal Lung Diseases, Lung Diseases, Interstitial [Disease/Finding], Lung Diseases, Interstitial, disease interstitial lung, interstitial disease lung, ild interstitial lung disease, lung interstitial disease, lung diseases interstitial, interstitial lung disease, interstitial lung diseases, interstitial lung disease (diagnosis), Interstitial pulmonary disease, Interstitial lung disease, ILD - Interstitial lung disease, Interstitial lung disease (disorder), lung; disease, interstitial
Italian Malattia polmonare interstiziale, Malattie parenchimali diffuse del polmone, Malattie interstiziali del polmone
Spanish Enfermedades Pulmonares Intersticiales, Enfermedades del Pulmón Intersticiales, PULMON, ENFERMEDAD INTERSTICIAL, Enfermedades Pulmonares Parenquimales Difusas, Neumopatías Intersticiales, enfermedad del intersticio pulmonar, enfermedad pulmonar intersticial (trastorno), enfermedad pulmonar intersticial, Enfermedad pulmonar intersticial
Swedish Lungsjukdomar, interstitiella
Japanese カンシツセイハイシッカン, 肺疾患-間質性, 間質性肺疾患
Finnish Interstitiaaliset keuhkosairaudet
Russian PNEVMONIIA INTERSTITSIAL'NAIA, PNEVMONIT INTERSTITSIAL'NYI, PUL'MONIT INTERSTITSIAL'NYI, LEGKIKH BOLEZNI INTERSTITSIAL'NYE, INTERSTITSIAL'NYE BOLEZNI LEGKIKH, ИНТЕРСТИЦИАЛЬНЫЕ БОЛЕЗНИ ЛЕГКИХ, ЛЕГКИХ БОЛЕЗНИ ИНТЕРСТИЦИАЛЬНЫЕ, ПНЕВМОНИТ ИНТЕРСТИЦИАЛЬНЫЙ, ПНЕВМОНИЯ ИНТЕРСТИЦИАЛЬНАЯ, ПУЛЬМОНИТ ИНТЕРСТИЦИАЛЬНЫЙ
Portuguese DOENCA INTERSTICIAL PULMONAR, Doenças Pulmonares Parenquimatosas Difusas, Doença pulmonar intersticial, Doenças Pulmonares Intersticiais
German Interstitielle Lungenkrankheit, nicht naeher bezeichnet, LUNGENERKRANKUNG INTERSTITIELL, interstitielle Lungenerkrankung, Interstitielle Lungenkrankheiten, Lungenkrankheiten, interstitielle
French PNEUMOPATHIE INTERSTITIELLE DIFFU, Maladies pulmonaires parenchymateuses diffuses, Pneumopathie interstitielle diffuse, Pneumopathies interstitielles, Maladies pulmonaires interstitielles
Czech Intersticiální plicní onemocnění, plíce - nemoci intersticiální, intersticiální nemoci plic, intersticiální plicní procesy, intersticiální plicní nemoci
Korean 상세불명의 사이질성 폐질환
Croatian PLUĆNE BOLESTI, INTERSTICIJSKE
Polish Choroby płuc śródmiąższowe, Zapalenie płuc śródmiąższowe
Hungarian Interstitialis tüdőbetegség
Norwegian ILD, Interstitiell lungesykdom
Dutch long; aandoening, interstitieel, Interstitiële longziekte, niet gespecificeerd, interstitiële longziekte, Interstitiële longziekte, Interstitiële longziekten, Longziekte, interstitiële, Longziekten, interstitiële, Ziekte, long-, interstitiële, Ziekten, long-, interstitiële