Pulmonology Book

http://www.fpnotebook.com/

Idiopathic Interstitial Pneumonia

Aka: Idiopathic Interstitial Pneumonia, Idiopathic Pulmonary Fibrosis, Usual Interstitial Pneumonia, Desquamative Interstitial Pneumonia, Respiratory Bronchiolitis, RB-ILD, Acute Interstitial Pneumonia, Hamman-Rich Disease, Nonspecific Interstitial Pneumonia
  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. FingernailClubbing
  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 350:651-6

Acute interstitial pneumonia (C1279945)

Concepts Disease or Syndrome (T047)
ICD9 516.33
SnomedCT 129459004, 236302005, 35037009, 45157009
English Idiopathic fibrosing alveolitis, acute fatal form, Acute interstitial pneumonitis, NOS, Acute interstitial pneum, Acute interstitial pneumonia (disorder), Acute interstitial pneumonitis, Idiopathic pulmonary fibrosis, acute fatal form, Acute interstitial pneumonia [Ambiguous], Acute interstitial pneumonia, AIP, Acute Interstitial Pneumonia, Acute Interstitial Pneumonitis
Spanish Neumonitis intersticial aguda, fibrosis pulmonar idiopática, forma aguda mortal, fibrosis pulmonar idiopática, forma fatal aguda, fibrosis pulmonar idiopática, neumonitis intersticial aguda, neumonía intersticial aguda (concepto no activo), neumonía intersticial aguda (trastorno), neumonía intersticial aguda
French Pneumonie interstitielle aiguë
Dutch acute interstitiële pneumonitis
Portuguese Pneumonite aguda intersticial
German akute interstitielle Pneumonitis
Italian Polmonite interstiziale acuta
Japanese ｷｭｳｾｲｶﾝｼﾂｾｲﾊｲｿﾞｳｴﾝ, 急性間質性肺臓炎
Czech Akutní mezižeberní pneumonitoda
Hungarian Interstitialis acut pneumonitis
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Hamman-Rich syndrome (C0085786)

Concepts Disease or Syndrome (T047)
MSH D011658
ICD10 J84.1
SnomedCT 266410004, 155624004, 196126001, 45157009, 196125002, 196123009, 129459004, 233721005, 236302005
English Idiopathic interstitial fibrosis of lung syndrome, Alveolitides, Fibrosing, Fibrosing Alveolitides, Hamman Rich Syndrome, Hamman-Rich Syndrome, Syndrome, Hamman-Rich, ALVEOLITIS FIBROSING, ALVEOLITIS, CHRONIC DIFFUSE FIBROSING, ALVEOLITIS, CHRONIC DIFFUSE SCLEROSING, ALVEOLITIS, FIBROSING, FIBROSIS, PULMONARY, INTERSTITIAL DIFFUSE, Fibrosing Alveolitis, IDIOPATHIC FIBROSING ALVEOLITIS, Diffuse idiopathic pulmonary fibrosis, Hamman-Rich disease, Idiopathic fibrosing alveolitis, chronic form, DIPF - Diff interstit pul fibr, Diff interstit pulm fibrosis, Idiopath.fibrosing alveol.NOS, Idiopathic fibrosing alveolitis NOS, Idiopath.fibrosing alveolitis, diffuse interstitial pulmonary fibrosis, Idiopath. fibrosing alveolitis (& Hamman-Rich syndrome) (disorder), Alveolitis fibrosing, Idiopathic fibrosing alveolitis NOS (disorder), idiopathic fibrosing alveolitis, usual interstitial pneumonitis, hamman rich syndrome, hamman-rich disease, uip, hamman rich disease, hamman-rich syndrome, fibrosis idiopathic pulmonary, disease hammans rich, fibrosis idiopathic ipf pulmonary, usual interstitial pneumonia, Idiopathic fibrosing alveolitis, Fibrosing alveolitis, Diffuse interstitial pulmonary fibrosis, DIPF - Diffuse interstitial pulmonary fibrosis, Diffuse interstitial pulmonary fibrosis (disorder), Idiopathic fibrosing alveolitis (disorder), Idiopathic interstitial pneumonia, fibrosing; alveolitis, hamman-Rich syndrome, alveolitis; fibrosing, Fibrosing alveolitis-idiopath., Hamman - Rich syndrome, Idiopath. fibrosing alveolitis (& Hamman-Rich syndrome), Idiopath. fibrosing alveolitis, Alveolitis-idiopath. fibrosing, Hamman-Rich syndrome, Alveolitis, Fibrosing, Fibrosing alveolitis (idiopathic), fibrosing alveolitis, Hamman Rich syndrome
Portuguese ALVEOLITE FIBROSANTE, Alveolite fibrosante idiopática, Alveolite fibrosante (idiopática), Alveolite Fibrosante, Alveolite fibrosante, Síndrome de Hamman-Rich
Spanish ALVEOLITIS FIBROSANTE, alveolitis fibrosa idiopática, síndrome de Hamman Rich, Alveolitis fibrosante (idiopática), Alveolitis fibrosante idiopática, Idiopath. fibrosing alveolitis, Hamman-Rich syndrome, Idiopathic fibrosing alveolitis, Fibrosing alveolitis-idiopath., Alveolitis-idiopath. fibrosing, Hamman - Rich syndrome, Idiopath. fibrosing alveolitis (& Hamman-Rich syndrome), Alveolitis Fibrosante, alveolitis fibrosa idiopática (trastorno), alveolitis fibrosante idiopática, SAI (trastorno), alveolitis fibrosante idiopática, SAI, alveolitis fibrosante idiopática, alveolitis fibrosante, enfermedad de Hamman - Rich, fibrosis pulmonar intersticial difusa (trastorno), fibrosis pulmonar intersticial difusa, síndrome de Hamman - Rich, Alveolitis fibrosante, Sindrome de Hamman-Rich, Síndrome de Hamman-Rich
Italian Alveolite fibrosante, Alveolite fibrosante idiopatica, Alveolite fibrosante (idiopatica), Sindrome di Hamman-Rich
Dutch fibroserende longblaasjesontsteking (idiopathisch), idiopathische fibroserende longblaasjesontsteking, syndroom van Hamman-Rich, alveolitis; fibroserend, fibroserend; alveolitis, fibroserende longblaasjesontsteking, Alveolitis, fibroserende, Hamman-Rich-syndroom
French Alvéolite fibrosante idiopathique, Alvéolite fibrosante (idiopathique), Alvéolite fibrosante, ALVEOLITE FIBROSANTE, Fibrose pulmonaire interstitielle diffuse, Maladie d'Hamman-Rich, Syndrome d'Hamman-Rich, Syndrome de Hamman-Rich
German idiopathische fibrosierende Alveolitis, fibrosierende Alveolitis, fibrosierende Alveolitis (idiopathisch), Hamman-Rich Syndrom, Alveolitis, fibrosierende, ALVEOLITIS FIBROSIERENDE, Alveoliltis fibrosierend, Hamman-Rich-Syndrom
Japanese 特発性線維化性胞隔炎, 線維化性胞隔炎, 線維化性胞隔炎（特発性）, ｾﾝｲｶｾｲﾎｳｶｸｴﾝ, ｾﾝｲｶｾｲﾎｳｶｸｴﾝﾄｸﾊﾂｾｲ, ﾄｸﾊﾂｾｲｾﾝｲｶｾｲﾎｳｶｸｴﾝ, ハンマン・リッチ症候群, ﾊﾝﾏﾝﾘｯﾁｼｮｳｺｳｸﾞﾝ
Czech Fibrotizující alveolitida, Hamman-Richův syndrom, Idiopatická fibrotizující alveolitida, Fibrotizující alveolitida (idiopatická), Alveolitida fibrotizující
Hungarian Hamman-Rich-syndroma, Fibrotizáló alveolitis (idiopathiás), Idiopathiás fibrotizáló alveolitis, Fibrotisaló alveolitis, Fibrotizáló alveolitis
Sources
Derived from the NIH UMLS (Unified Medical Language System)


Desquamative interstitial pneumonia (C0238378)

Concepts Disease or Syndrome (T047)
ICD9 516.34, 516.37
SnomedCT 8549006
English PNEUMONIA, DESQUAMATIVE INTERSTITIAL, Desquamative interstitial pneumonia, DIP - Desq interst pneumonitis, Desquam interstit pneumonitis, DIP, PNEUMONIA, DESQUAMATIVE INTERSTITIAL, FAMILIAL, INTERSTITIAL LUNG DISEASE, DESQUAMATIVE, INTERSTITIAL PNEUMONITIS, DESQUAMATIVE, FAMILIAL, PNEUMONITIS, DESQUAMATIVE INTERSTITIAL, FAMILIAL, ILD, DESQUAMATIVE, desquamative interstitial pneumonia, desquamative interstitial pneumonia (diagnosis), desquamative interstitial pneumonia (DIP), Respiratory bronchiolitis interstitial lung disease, Desquamatv interst pneu, Resp brncio interst lung, Desquamative interstitial pneumonitis, DIP - Desquamative interstitial pneumonitis, Desquamative interstitial pneumonia (disorder), Desquamative Interstitial Pneumonia, RBILD, Respiratory Bronchiolitis Interstitial Lung Disease
Spanish enfermedad pulmonar intersticial descamativa (trastorno), enfermedad pulmonar intersticial descamativa, neumonitis intersticial descamativa, neumonía intersticial descamativa (trastorno), neumonía intersticial descamativa
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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