II. Epidemiology

  1. Prevalence
    1. Men: 29 per 100,000
    2. Women: 26 per 100,000
  2. Ages: 40-70 years

III. 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 (e.g. Nitrofurantoin when GFR<30 ml/min)
      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

IV. Symptoms

  1. Non-productive cough for 1 to 3 years
  2. Increasing breathlessness

V. Signs

  1. Bilateral basilar crackles
  2. Fingernail Clubbing

VI. Associated Conditions

  1. Associated autoimmune disease in 30%

VII. 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

VIII. 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

IX. Differential Diagnosis

  1. See also Interstitial Lung Disease
  2. Other causes of fibrosing alveolitis
  3. Occupational exposure etiologies

X. 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

XI. Prognosis

  1. Poor (recently improved on combination therapy)

XII. Resources

  1. Pulmonary Fibrosis Association
    1. http://www.pulmonaryfibrosisassn.com/
  2. Pulmonary Fibrosis Foundation
    1. http://www.pulmonaryfibrosis.org

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Ontology: Hamman-Rich syndrome (C0085786)

Concepts Disease or Syndrome (T047)
MSH D011658
ICD10 J84.112 , J84.114, J84.11
SnomedCT 196123009, 129459004, 155624004, 266410004, 196126001, 233721005, 45157009, 196125002, 236302005
English Alveolitides, Fibrosing, Fibrosing Alveolitides, Hamman Rich Syndrome, Hamman-Rich Syndrome, Syndrome, Hamman-Rich, ALVEOLITIS FIBROSING, Fibrosing Alveolitis, DIPF - Diff interstit pul fibr, Diff interstit pulm fibrosis, Idiopath.fibrosing alveol.NOS, Idiopathic fibrosing alveolitis NOS, Idiopath.fibrosing alveolitis, diffuse interstitial pulmonary fibrosis, 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 (diagnosis), Idiopathic interstitial pneumonia, Alveolitis fibrosing, Hamman - Rich syndrome, Hamman-Rich disease, Idiopath. fibrosing alveolitis (& Hamman-Rich syndrome), Idiopath. fibrosing alveolitis (& Hamman-Rich syndrome) (disorder), Idiopath. fibrosing alveolitis, Fibrosing alveolitis-idiopath., Idiopathic fibrosing alveolitis NOS (disorder), Alveolitis-idiopath. fibrosing, Idiopathic fibrosing alveolitis (disorder), Fibrosing alveolitis, Diffuse interstitial pulmonary fibrosis, DIPF - Diffuse interstitial pulmonary fibrosis, Diffuse interstitial pulmonary fibrosis (disorder), fibrosing; alveolitis, hamman-Rich syndrome, alveolitis; fibrosing, Diffuse idiopathic pulmonary fibrosis, Hamman-Rich syndrome, Alveolitis, Fibrosing, Idiopathic fibrosing alveolitis, 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, Alveolitis Fibrosante, Alveolitis fibrosante, enfermedad de Hamman - Rich, síndrome de Hamman - Rich, alveolitis fibrosante idiopática, SAI, alveolitis fibrosante idiopática, SAI (trastorno), alveolitis fibrosa idiopática (trastorno), alveolitis fibrosante idiopática, alveolitis fibrosante, fibrosis pulmonar intersticial difusa (trastorno), fibrosis pulmonar intersticial difusa, Síndrome de Hamman-Rich
Dutch fibroserende longblaasjesontsteking (idiopathisch), idiopathische fibroserende longblaasjesontsteking, syndroom van Hamman-Rich, fibroserende longblaasjesontsteking, alveolitis; fibroserend, fibroserend; alveolitis, 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 (idiopathisch), Hamman-Rich Syndrom, Alveolitis, fibrosierende, ALVEOLITIS FIBROSIERENDE, Alveoliltis fibrosierend, fibrosierende Alveolitis, Hamman-Rich-Syndrom
Italian Alveolite fibrosante idiopatica, Alveolite fibrosante (idiopatica), Sindrome di Hamman-Rich, Alveolite fibrosante, Sindrome di Hamman Rich
Japanese 特発性線維化性胞隔炎, 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グン, センイカセイホウカクエン, 線維化性胞隔炎
Czech Hamman-Richův syndrom, Idiopatická fibrotizující alveolitida, Fibrotizující alveolitida (idiopatická), fibrotizující alveolitida, fibrózní alveolitida, Hammanův-Richův syndrom, Fibrotizující alveolitida, Alveolitida fibrotizující
Hungarian Hamman-Rich-syndroma, Fibrotizáló alveolitis (idiopathiás), Idiopathiás fibrotizáló alveolitis, Fibrotizáló alveolitis, Fibrotisaló alveolitis

Ontology: Desquamative interstitial pneumonia (C0238378)

Definition (NCI) A rare idiopathic interstitial pneumonia characterized by accumulation of macrophages in alveolar spaces and interstitial inflammation. It usually occurs in smokers. Some patients develop progressive interstitial lung fibrosis.
Concepts Disease or Syndrome (T047)
MSH C562470
ICD9 516.34, 516.37
ICD10 J84.117 , J84.115
SnomedCT 8549006
English 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), Desquamatv interst pneu, Resp brncio interst lung, respiratory bronchiolitis interstitial lung disease, respiratory bronchiolitis interstitial lung disease (diagnosis), Respiratory bronchiolitis interstitial lung disease, Interstitial Pneumonitis, Desquamative, Familial, Pneumonia, Desquamative Interstitial, Familial, Pneumonitis, Desquamative Interstitial, Familial, Interstitial Lung Disease, Desquamative, 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

Ontology: Acute interstitial pneumonia (C1279945)

Definition (NCI) A rare, rapidly progressing interstitial lung disorder characterized by diffuse and bilateral alveolar damage. Patients present with sudden onset of dyspnea and rapid development of respiratory failure. Management is supportive and hospitalization and mechanical ventilation are usually required.
Concepts Disease or Syndrome (T047)
ICD9 516.33
ICD10 J84.114
SnomedCT 129459004, 236302005, 35037009, 45157009
Spanish Neumonitis intersticial aguda, fibrosis pulmonar idiopática, forma aguda mortal, neumonía intersticial aguda (concepto no activo), fibrosis pulmonar idiopática, forma fatal aguda, fibrosis pulmonar idiopática, neumonitis intersticial aguda, 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ゾウエン, 急性間質性肺臓炎
Czech Akutní mezižeberní pneumonitoda
English Acute interstitial pneum, pneumonitis acute interstitial, Acute interstitial pneumonitis (diagnosis), Acute interstitial pneumonia (disorder), Acute interstitial pneumonitis, Idiopathic pulmonary fibrosis, acute fatal form, Idiopathic fibrosing alveolitis, acute fatal form, Acute interstitial pneumonitis, NOS, Acute interstitial pneumonia [Ambiguous], Acute interstitial pneumonia, AIP, Acute Interstitial Pneumonia, Acute Interstitial Pneumonitis
Hungarian Interstitialis acut pneumonitis

Ontology: Idiopathic Pulmonary Fibrosis (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_NCI-GLOSS) 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 (NCI) Chronic and progressive fibrosis of the lung parenchyma of unknown cause.
Definition (NCI) Interstitial pneumonia characterized by the presence of fibrosis in the interstitial lung tissue. The pathologic diagnosis is based on the identification of fibrotic lesions at different stages of development within a lung biopsy specimen. Typically there are foci of normal lung parenchyma alternating with interstitial inflammation and honeycombing. The term usual interstitial pneumonia sometimes is used interchangeably with idiopathic interstitial fibrosis. The two terms are not entirely synonymous. Usual interstitial pneumonia may be associated with other conditions such as connective tissue disorders and asbestosis. The diagnosis of idiopathic interstitial fibrosis requires the exclusion of such conditions. Patients with usual interstitial pneumonia present with progressive dyspnea, cough, and restrictive pulmonary function abnormalities. The prognosis is usually poor.
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
ICD9 516.31
ICD10 J84.112
SnomedCT 426437004, 237121003, 233721005, 28168000, 196125002
English CFA - Crypt fibros alveolitis, CFA - Cryptogenic fibrosing alveolitis, Cryptogen fibrosing alveolitis, IPF - Idiopath pulm fibrosis, IPF - Idiopathic pulmonary fibrosis, UIP - Usual inter pneumonitis, FIBROSING ALVEOLITIS, CRYPTOGENIC, IDIOPATHIC PULMONARY FIBROSIS, FAMILIAL, PULMONARY FIBROSIS, IDIOPATHIC, UIP, FIBROCYSTIC PULMONARY DYSPLASIA, Familial idiopathic pulmonary fibrosis (disorder), Familial idiopathic pulmonary fibrosis, HAMMAN-RICH DISEASE, 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, Usual Interstitial Pneumonias, Cryptogenic Fibrosing Alveolitis, Cryptogenic Fibrosing Alveolitides, Idiopathic Pulmonary Fibroses, Pulmonary Fibroses, Idiopathic, Interstitial Pneumonia, Usual, Fibrosing Alveolitides, Cryptogenic, Interstitial Pneumonias, Usual, Pneumonia, usual interstitial, Idiopathic Pulmonary Fibrosis [Disease/Finding], cryptogenic fibrosing alveolitis, pulmonary fibrosis idiopathic, Idiopath pulmon fibrosis, cryptogenic fibrosing alveolitis (diagnosis), diopathic pulmonary fibrosis, Pulmonary Fibrosis, Idiopathic, Interstitial Pneumonitis, Usual, Idiopathic Pulmonary Fibrosis, Familial, Fibrosing Alveolitis, Cryptogenic, Idiopathic Pulmonary Fibrosis, IPF, Hamman-Rich Disease, Pneumonitis, Usual Interstitial, Pneumonitides, Usual Interstitial, Diseases, Hamman-Rich, Pulmonary Dysplasia, Fibrocystic, Pulmonary Dysplasias, Fibrocystic, Idiopathic Fibrosing Alveolitis, Chronic Form, Disease, Hamman-Rich, Fibrocystic Pulmonary Dysplasias, Dysplasias, Fibrocystic Pulmonary, Hamman-Rich Diseases, Dysplasia, Fibrocystic Pulmonary, Usual Interstitial Pneumonitis, Interstitial Pneumonitides, Usual, Familial Idiopathic Pulmonary Fibrosis, Fibrocystic Pulmonary Dysplasia, Hamman Rich Disease, Usual Interstitial Pneumonitides, Usual interstitial pneumonitis (disorder), INTERSTITIAL PNEUMONITIS, USUAL, pulmonary fibrosis idiopathic familial, Familial idiopathic pulmonary fibrosis (diagnosis), Idiopathic fibrosing alveolitis, chronic form (disorder), Usual interstitial pneumonia, fibrosis; lung, idiopathic, lung; fibrosis, idiopathic, Usual interstitial pneumonitis [Ambiguous], Usual interstitial pneumonitis, UIP - Usual interstitial pneumonitis, Cryptogenic fibrosing alveolitis, Idiopathic pulmonary fibrosis
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, fibrosis pulmonar familiar idiopática (trastorno), fibrosis pulmonar familiar idiopática, Fibrosis Pulmonar Idiopática, Alveolitis Fibrosante Criptogénica, alveolitis fibrosa idiopática, forma crónica, alveolitis fibrosa idiopática, forma crónica (trastorno), alveolitis fibrosante idiopática, forma crónica (trastorno), fibrosis pulmonar idiopática, neumonitis intersticial usual, neumonitis intersticial usual (trastorno), Alveolitis Fibrosante Criptogenética, Alveolitis Fibrosante Criptógena, 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
Norwegian Idiopatisk pulmonal fibrose, familiær interstitiell, Idiopatisk fibroserende alveolitt, Idiopatisk pulmonal fibrose, Fibroserende alveolitt, kryptogen, Idiopatisk lungefibrose, Idiopatisk lungefibrose, familiær interstitiell, Kryptogen fibroserende alveolitt