Pulmonology Book

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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
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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. 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

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)


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)


Usual interstitial pneumonitis (C1800706)

Definition (MSH) A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.
Definition (NCI) Chronic and progressive fibrosis of the lung parenchyma of unknown cause.
Definition (NCI) A disease in which the alveoli (tiny air sacs at the end of the bronchioles in the lungs) are overgrown with fibrous tissue. The cause of the disease is unknown and it gets worse over time. Symptoms include difficult, painful breathing and shortness of breath.
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.
Concepts Disease or Syndrome (T047)
MSH D054990
ICD10 J84.1
SnomedCT 233721005, 28168000, 196123009, 237121003, 196125002
English FIBROSIS, IDIOPATHIC PULMONARY, PNEUMONITIS, USUAL INTERSTITIAL, CFA - Crypt fibros alveolitis, Cryptogen fibrosing alveolitis, IPF - Idiopath pulm fibrosis, UIP - Usual inter pneumonitis, FIBROSING ALVEOLITIS, CRYPTOGENIC, IDIOPATHIC PULMONARY FIBROSIS, FAMILIAL, PULMONARY FIBROSIS, IDIOPATHIC, UIP, INTERSTITIAL PNEUMONITIS, USUAL, Usual interstitial pneumonitis, usual interstitial pneumonia (Carrington-Liebow), Carrington-Liebow syndrome, idiopathic pulmonary fibrosis (diagnosis), usual interstitial pneumonia, usual interstitial pneumonia (diagnosis), idiopathic pulmonary fibrosis, Usual Interstitial Pneumonia, Idiopathic Pulmonary Fibrosis, Fibrosing Alveolitis, Cryptogenic, Usual Interstitial Pneumonias, Cryptogenic Fibrosing Alveolitis, Cryptogenic Fibrosing Alveolitides, Idiopathic Pulmonary Fibroses, Pulmonary Fibroses, Idiopathic, Pulmonary Fibrosis, Idiopathic, Interstitial Pneumonia, Usual, Fibrosing Alveolitides, Cryptogenic, Interstitial Pneumonias, Usual, PULMONARY FIBROSIS IDIOPATHIC, Pneumonia, usual interstitial, Fibrosing alveolitis (cryptogenic), Idiopathic Pulmonary Fibrosis [Disease/Finding], cryptogenic fibrosing alveolitis, pulmonary fibrosis idiopathic, Idiopathic fibrosing alveolitis, chronic form, CFA - Cryptogenic fibrosing alveolitis, Cryptogenic fibrosing alveolitis, IPF - Idiopathic pulmonary fibrosis, Idiopathic pulmonary fibrosis, Idiopathic fibrosing alveolitis, chronic form (disorder), Usual interstitial pneumonia, fibrosis; lung, idiopathic, lung; fibrosis, idiopathic, Usual interstitial pneumonitis [Ambiguous], UIP - Usual interstitial pneumonitis, Usual interstitial pneumonitis (disorder), IPF
Dutch idiopathische pulmonale fibrose, cryptogene fibroserende longblaasjesontsteking, fibrose; long, idiopathisch, long; fibrose, idiopathisch
Portuguese Fibrose pulmonar idiopática, Alveolite fibrosante criptogenética, Alveolite Fibrosante Criptogênica, Fibrose Pulmonar Idiopática
Spanish Fibrosis pulmonar idiopática, Alveolitis fibrosante criptogenética, neumonitis intersticial usual, neumonitis intersticial usual (trastorno), Cryptogenic fibrosing alveolitis, Usual interstitial pneumonitis [Ambiguous], Usual interstitial pneumonitis, Alveolitis Fibrosante Criptogenética, Fibrosis Pulmonar Idiopática, Alveolitis Fibrosante Criptogena, Alveolitis Fibrosante Criptogenetica, Alveolitis Fibrosante Criptogenica, Alveolitis Fibrosante Criptogénica, Alveolitis Fibrosante Criptógena, Fibrosis Pulmonar Idiopatica, alveolitis fibrosa idiopática, forma crónica, alveolitis fibrosa idiopática, forma crónica (trastorno), alveolitis fibrosante idiopática, forma crónica (trastorno), fibrosis pulmonar idiopática, alveolitis fibrosante idiopática, forma crónica
German kryptogene fibrosierende Alveolitis, Gewöhnliche interstitielle Pneumonie, Idiopathische Lungenfibrose, Kryptogene fibrosierende Alveolitis, idiopathische Lungenfibrose
Italian Alveolite fibrosante criptogenetica, Alveolite criptogenica fibrosante, Polmonite interstiziale usuale, Fibrosi polmonare idiopatica
Japanese ﾄｸﾊﾂｾｲﾊｲｾﾝｲｼｮｳ, 特発性線維化性胞隔炎, ﾄｸﾊﾂｾｲｾﾝｲｶｾｲﾎｳｶｸｴﾝ, 肺胞炎-特発性線維化, 特発性肺線維症, 特発性線維化肺炎, 肺炎-特発性線維化, 肺線維症-特発性, 特発性線維化肺胞炎, 原因不明繊維化性肺胞隔炎
Czech Idiopatická plicní fibróza, Kryptogenní fibrotizující alveolitida, kryptogenní fibrózující alveolitida, obvyklá intersticiální pneumonie, idiopatická plicní fibróza, kryptogenní fibrotizující alveolitida
French Fibrose pulmonaire idiopathique, Alvéolite fibrosante cryptogénique, FPI (Fibrose Pulmonaire Idiopathique), Fibrose interstitielle diffuse idiopathique, Pneumonie interstitielle usuelle
Russian СИНДРОМ ХАММАНА-РИЧА, AL'VEOLIT KRIPTOGENNYI FIBROZIRUIUSHCHII, SINDROM KHAMMANA-RICHA, ЛЕГКИХ ФИБРОЗ ИДИОПАТИЧЕСКИЙ, АЛЬВЕОЛИТ КРИПТОГЕННЫЙ ФИБРОЗИРУЮЩИЙ, LEGKIKH FIBROZ IDIOPATICHESKII, ДИФФУЗНЫЙ ФИБРОЗИРУЮЩИЙ АЛЬВЕОЛИТ, DIFFUZNYI FIBROZIRUIUSHCHII AL'VEOLIT
Swedish Idiopatisk lungfibros
Polish Zapalenie płuc śródmiąższowe zwykłe, Zapalenie pęcherzyków płucnych włókniejące kryptogenne, Idiopatyczne włóknienie płuc, Zwłóknienie płuc samoistne, Idiopatyczne zwłóknienie płuc, Zwłóknienie płuc idiopatyczne
Hungarian Idiopathiás pulmonalis fibrosis, Cryptogen fibrotizáló alveolitis
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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