Desquamative interstitial pneumonia

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

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

The term desquamative interstitial pneumonia is a misnomer. The airspace cells that characterize the condition were once thought to represent desquamated epithelial cells, but they are now know to represent macrophages.[1]

General

  • Rare.[2]
  • Strong association with smoking.[3][4]
    • 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.[5]

Diagnosis:

  • Requires - surgical biopsy.[5]

Treatment:

  • Stop smoking/remove or manage underlying cause.

Microscopic

Features:[2]

  • 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. 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.
  2. 2.0 2.1 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.
  3. 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.
  4. 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.
  5. 5.0 5.1 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.