Pulmonology Book

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Idiopathic Interstitial PneumoniaAka: Idiopathic Pulmonary Fibrosis, Usual Interstitial Pneumonia, Desquamative Interstitial Pneumonia, Respiratory Bronchiolitis, RB-ILD, Acute Interstitial Pneumonia, Hamman-Rich Disease, Nonspecific Interstitial Pneumonia

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  1. Epidemiology
    1. Prevalence
      1. Men: 29 per 100,000
      2. Women: 26 per 100,000
    2. Ages: 40-70 years
  2. Types of Idiopathic Interstitial Pneumonia
    1. Usual Interstitial Pneumonia (UIP)
      1. Previously known as Idiopathic Pulmonary Fibrosis
      2. Associated conditions
        1. Collagen vascular disease
        2. Drug Reaction (Nitrofurantoin)
        3. Pneumoconioses (Asbestosis)
        4. Idiopathic Pulmonary Fibrosis (IPF)
      3. Imaging appearance: Honey combing
      4. Poor response to Corticosteroids
      5. Recovery not possible
      6. Mortality 68% with mean survival 5-6 years
    2. Desquamative Interstitial Pneumonia (DIP)
      1. Imaging appearance: Ground Glass Appearance
      2. Good response to Corticosteroids
      3. Mortality 27% with mean survival 12 years
    3. Respiratory Bronchiolitis (RB-ILD)
      1. Imaging appearance: Ground Glass Appearance
      2. Good response to Corticosteroids
      3. Mortality 0%
    4. Acute Interstitial Pneumonia (AIP)
      1. Also known as Hamman-Rich Disease
      2. Acute, severe Interstitial Lung Disease
      3. Poor response to Corticosteroids
      4. Mortality 62% with mean survival 1-2 months
    5. Nonspecific Interstitial Pneumonia (NSIP)
      1. Subacute onset
      2. Good response to Corticosteroids
      3. Mortality 11% with mean survival 17 months
  3. Symptoms
    1. Non-productive cough for 1 to 3 years
    2. Increasing breathlessness
  4. Signs
    1. Bilateral basilar crackles
    2. Fingernail Clubbing
  5. Associated Conditions
    1. Associated autoimmune disease in 30%
  6. Radiology
    1. Chest XRay (Normal in 16% of IPF patients)
      1. Diffuse reticular pattern
      2. Predominately lower zones
    2. CT Chest
      1. Linear opacities, honeycombing
      2. Ground glass attenuation
  7. Diagnosis
    1. Pulmonary Function Testing
      1. Restrictive disease
      2. Low Lung Volumes
      3. Impaired gas exchange (diminished DLCO)
    2. Transbronchial lung biopsy or Bronchoalveolar lavage
      1. Will not confirm IPF
      2. Rules out other causes of Interstitial Lung Disease
        1. Sarcoidosis
        2. Cryptococcus
        3. Lung Cancer
  8. Differential Diagnosis
    1. See also Interstitial Lung Disease
    2. Other causes of fibrosing alveolitis
    3. Occupational exposure etiologies
  9. Management
    1. Corticosteroid Therapy
      1. Efficacy
        1. Corticosteroid Responders: 10-15%
        2. Indicators of responders
          1. Young women
          2. Ground glass appearance on CT Chest
          3. Active inflammation on lung biopsy
      2. Adverse Effects
        1. Serious complications in 26%
    2. Cytotoxic Drugs
      1. Available preparations
        1. Azathioprine
        2. Cyclophosphamide
      2. Indications
        1. Second line drugs
        2. Used in combination with Corticosteroids
  10. Prognosis
    1. Poor (recently improved on combination therapy)
  11. Resources
    1. Pulmonary Fibrosis Association
      1. http://www.pulmonaryfibrosisassn.com/
    2. Pulmonary Fibrosis Foundation
      1. http://www.pulmonaryfibrosis.org
  12. References
    1. Chan-Yeung (1997) Lancet 350:651

Hamman-Rich syndrome (C0085786)

Definition (CSP)chronic inflammation and progressive fibrosis of the pulmonary alveolar walls with steady progressive dyspnea; finally, resulting in death from lack of oxygen or right heart failure.
ConceptsDisease or Syndrome (T047)
ICD9516.3, 516.3
MSHD011658
EnglishALVEOLITIS FIBROSING, Alveolitis-idiopath. fibrosing, CFA - Cryptogenic fibrosing alveolitis, Cryptogenic fibrosing alveolitis, Diffuse idiopathic pulmonary fibrosis, Diffuse interstitial pulmonary fibrosis, DIPF - Diffuse interstitial pulmonary fibrosis, FIBROCYSTIC PULMONARY DYSPLASIA, Fibrosing Alveolitides, Fibrosing alveolitis, Fibrosing alveolitis-idiopath., Hamman - Rich syndrome, Hamman Rich syndrome, HAMMAN-RICH DISEASE, Hamman-Rich Syndrome, IDIO FIBROS ALVEOLITIS, Idiopath. fibrosing alveolitis, Idiopathic fibrosing alveolitis, Idiopathic interstitial pneumonia, idiopathic pulmonary fibrosis, IPF, IPF - Idiopathic pulmonary fibrosis
Spanishalveolitis fibrosa idiopatica, alveolitis fibrosante, alveolitis fibrosante idiopatica, enfermedad de Hamman - Rich, fibrosis pulmonar intersticial difusa, sindrome de Hamman - Rich, sindrome de Hamman Rich
Parent Conceptspulmonary fibrosis/granuloma (C0178818), Other alveolar and parietoalveolar pneumonopathy (C0859814), Pulmonary Fibrosis (C0034069), Pneumonitis, Interstitial (C0206061), Pneumonia (C0032285), Hamman-Rich syndrome (C0085786), Lung Diseases, Interstitial (C0206062), Alveolar pneumopathy (C0264502), Chronic fibrosis of lung (C0264526), Ambiguous concept (C1274012), Chronic inflammatory disorder (C1290886)
SourcesCOSTAR, CSP, CST, DXP, ICD9CM, MSH, MTH, MTHICD9, NCI, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Desquamative interstitial pneumonia (C0238378)

ConceptsDisease or Syndrome (T047)
ICD9516.8
EnglishDesquamative interstitial pneumonia, Desquamative interstitial pneumonitis, DIP, DIP - Desquamative interstitial pneumonitis, RBILD, Respiratory Bronchiolitis Interstitial Lung Disease
Spanishenfermedad pulmonar intersticial descamativa, neumonia intersticial descamativa, neumonitis intersticial descamativa
Parent ConceptsHamman-Rich syndrome (C0085786), Pneumonitis, Interstitial (C0206061)
SourcesDXP, MTH, MTHICD9, NCI, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)


Acute interstitial pneumonia (C1279945)

ConceptsDisease or Syndrome (T047)
EnglishAcute interstitial pneumonia, Acute interstitial pneumonitis, AIP
Spanishfibrosis pulmonar idiopatica, neumonia intersticial aguda, neumonitis intersticial aguda
Parent ConceptsHamman-Rich syndrome (C0085786), Pneumonitis, Interstitial (C0206061), Alveolar pneumopathy (C0264502), Acute inflammatory disease (C1290885), Ambiguous concept (C1274012)
SourcesMTH, NCI, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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