Desquamative interstitial pneumonia

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Desquamative interstitial pneumonia
Diagnosis in short

LM abundant brown pigmented airspace macrophages (smoker's macrophages), architecture preserved ("linear fibrosis")
LM DDx amiodarone toxicity, fibrotic NSIP, RBILD
Site lung - see diffuse lung diseases

Associated Dx +/-smoking
Prevalence rare
Treatment stop smoking/remove insult

Desquamative interstitial pneumonia, abbreviated DIP, is a diffuse lung disease that is strongly associated with smoking.


General

  • Rare.[1]
  • Strong association with smoking.[2][3]
    • Thought to be advanced RBILD.
  • May be seen in non-smokers (up to ~40% of cases) due to occupational exposures, drugs, viral illnesses and autoimmune diseases.[4]

Diagnosis:

  • Requires - surgical biopsy.[4]

Treatment:

  • Stop smoking/remove or manage underlying cause.

Note:

  • The term desquamative interstitial pneumonia is a misnomer.[5] **The airspace cells that characterize the condition were once thought to represent desquamated epithelial cells but are now know to represent macrophages.

Microscopic

Features:[1]

  • Abundant airspace macrophages - usually with brown pigment (so called smoker's macrophages) - key feature.
  • Interstitial inflammation or interstitial fibrosis with a preserved architecture - so called "linear fibrosis".

Notes:

  • Some fields of view may be indistinguishable from RBILD.

DDx:

Images

Stains

  • Macrophages PAS +ve.

See also

References

  1. 1.0 1.1 Tazelaar, HD.; Wright, JL.; Churg, A. (Mar 2011). "Desquamative interstitial pneumonia.". Histopathology 58 (4): 509-16. doi:10.1111/j.1365-2559.2010.03649.x. PMID 20854463.
  2. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 93. ISBN 978-0781765275.
  3. Margaritopoulos, GA.; Vasarmidi, E.; Jacob, J.; Wells, AU.; Antoniou, KM. (Sep 2015). "Smoking and interstitial lung diseases.". Eur Respir Rev 24 (137): 428-35. doi:10.1183/16000617.0050-2015. PMID 26324804.
  4. 4.0 4.1 Godbert, B.; Wissler, MP.; Vignaud, JM. (Jun 2013). "Desquamative interstitial pneumonia: an analytic review with an emphasis on aetiology.". Eur Respir Rev 22 (128): 117-23. doi:10.1183/09059180.00005812. PMID 23728865.
  5. Attili, AK.; Kazerooni, EA.; Gross, BH.; Flaherty, KR.; Myers, JL.; Martinez, FJ.. "Smoking-related interstitial lung disease: radiologic-clinical-pathologic correlation.". Radiographics 28 (5): 1383-96; discussion 1396-8. doi:10.1148/rg.285075223. PMID 18794314. http://pubs.rsna.org/doi/full/10.1148/rg.285075223.