Interstitial lung diseasesJ84.9

Author:Dr. med. S. Leah Schröder-Bergmann

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Last updated on: 29.10.2020

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Synonym(s)

Cryptogenetically fibrosing alveolitis; Diffuse fibrosing alveolitis; Diffuse lung parenchyma diseases; DPLD; Idiopathically fibrosing alveolitis; Idiopathic interstitial pulmonary fibrosis; ILD

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HistoryThis section has been translated automatically.

Liebow and Carrington first summarized the group of idiopathic interstitial pneumonia (IIP) based on histological criteria in 1969.

DefinitionThis section has been translated automatically.

Interstitial lung diseases are chronic inflammations of the lung interstitium involving the alveolar capillary membranes. The consequence of chronic inflammation is an increase in connective tissue structural elements of the lung parenchyma with consecutive lung fibrosis (partly with honeycomb structure).

ClassificationThis section has been translated automatically.

The current classification of idiopathic interstitial pneumonia was jointly published in 2002 by the American Thoracic Society (ATS) and the European Respiratory Society (ERS), based on the summary prepared by Liebow and Carrington (see above). A distinction is now made:

Occurrence/EpidemiologyThis section has been translated automatically.

IIPs account for half of all interstitial lung diseases.

The prevalence is 67.5 in women and 80.9 in men per 100,000 inhabitants.

Mortality is high in certain subgroups (e.g. IPF 68%), but complete remissions are possible in others.

EtiopathogenesisThis section has been translated automatically.

Primarily a mostly unknown noxious agent leads to chronic epithelial damage, which in turn leads to irregular repair.

In all IIPs we find an activation of fibroblasts, an increase in the connective tissue matrix and a variably pronounced inflammation of the alveolar wall. Nowadays, alveolitis is seen as an accompanying phenomenon and no longer as a primary inflammation.

Addendum:

In patients with rheumatoid arthritis (RA) up to 60% have interstitial lung disease (ILD), which is detected in CT.

According to latest findings, the pathogenesis of RA-ILD and idiopathic pulmonary fibrosis have genetic similarities: The MUC5B promoter variant rs35705950 is associated with RA-ILD and is particularly specific for CT-diagnosed interstitial pneumonia.

This could lead to an earlier diagnosis of ILD in patients with RA and may also have an influence on drug therapy, since drugs that have been shown to be effective in idiopathic pulmonary fibrosis should also be tested in patients with RA-ILD.

literature:

- Juge P-A et al (2018) MUC5B-Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease. NEJM379: 2209-2219

ManifestationThis section has been translated automatically.

Common to all manifestations of interstitial pneumonia is shortness of breath, especially under stress and dry cough. Otherwise, the individual clinical pictures are more or less different.

For more details see the corresponding clinical picture

ImagingThis section has been translated automatically.

Nowadays, HRCT plays the primary role in the diagnosis and classification of the individual forms of disease and should always be performed in cases of idiopathic interstitial pneumonia.

The specificity of HRCT is quite high, but the sensitivity is low. It has been shown that if an experienced radiologist considers the diagnosis to be very likely, it is confirmed in about 90% of cases.

If the HRCT does not allow a clear diagnosis to be made, biopsies should be taken from different collection sites. This can be done transbronchially, by a VATS (video-assisted thoracoscopy) or by an open biopsy.

LaboratoryThis section has been translated automatically.

LDL slightly elevated, BSG accelerated, occasionally antinuclear AK or rheumatoid factors slightly elevated

DiagnosisThis section has been translated automatically.

There is a reasonable suspicion of interstitial pneumonia:

  • in the case of respiratory distress over a long period of time, especially on exertion, together with an unproductive cough
  • familial stress, especially fibrosis
  • inspiratory crackling above the base of the lung can be auscultated (in > 80 %) and in advanced fibrosis squeaking/creaking
  • FVC reduced in lung function
  • significantly limited CO diffusion (can precede the loss of FVC)
  • basally accentuated or pleura-edged reticular pattern in the X-ray thorax, but normal findings are also possible (at approx. 10 %)

On average, it takes about 21 months from the onset of the disease to diagnosis.

In the past, surgical lung biopsy was the gold standard for diagnosis. Nowadays, the preferred method of diagnosis is high-resolution computer tomography (HRCT).

Differential diagnosisThis section has been translated automatically.

Complication(s)This section has been translated automatically.

LiteratureThis section has been translated automatically.

  1. Baumann A (2015) On the Course of Idiopathic Pulmonary Fibrosis and the Influence of Clinical Exacerbations with Subsequent Inpatient Treatment on Disease Progression and Survival, Inaugural Dissertation. Justus-Liebig-University Giessen
  2. Gerok W et al (2007) Internal Medicine 1332-1333
  3. Günther A et al (2003) Dtsch Ärztebl 100 (24) A:1676/ B:1389/ C:1305
  4. Herold G et al (2018) Internal Medicine 374 and 392-394
  5. Kasper DL et al (2015) Harrison's Principles of Internal Medicine 1708-1713
  6. Kasper DL (2015) Harrisons Internal Medicine 2089-2096
  7. Köhler et al (2010) Pneumology 141-151
  8. Kreuter M et al (2016) Rare lung diseases 143-162
  9. Lorenz J et al (2016) Checklist Pneumology (Checklists XXL) S 323-330
  10. Müller HM (2003) The classification of interstitial pneumonia from a pathological-anatomical and clinical point of view. Inaugural dissertation. Ruhr University Bochum
  11. Travis WD (2013) An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Ideopathic Interstitial Pneumonias. AJRCCM 188 (6) 733-748

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Last updated on: 29.10.2020