Difference between revisions of "Non-specific interstitial pneumonia"

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#redirect [[Diffuse_lung_diseases#Non-specific_interstitial_pneumonia]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Non-specfic_interstitial_pneumonia_(NSIP),_cellular_variant.jpg
| Width      =
| Caption    = NSIP. [[H&E stain]].
| Synonyms  =
| Micro      = diffuse fibrosis (uniform fibrosis (unlike [[UIP]]), +/-''linear fibrosis'' (fibrosis that follows alveolar walls + no architectural distortion), +/-lymphoid nodules (assoc. with collagen vascular disease), +/-focal [[organizing pneumonia]]
| Subtypes  = idiopathic NSIP, NSIP due to an underlying cause
| LMDDx      = [[organizing pneumonia]], [[collagen vascular disease]], drug reaction, [[hypersensitivity pneumonitis]], [[lymphocytic interstitial pneumonia]]
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[lung]] - see ''[[diffuse lung diseases]]''
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      = cough
| Symptoms  = dyspnea
| Prevalence = uncommon
| Bloodwork  =
| Rads      = lower lung zone fibrosis, patchy ground glass, no honeycombing
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    =
| Tx        = corticosteroids (?)
}}
'''Non-specific interstitial pneumonia''', abbreviated '''NSIP''', is an uncommon type of [[diffuse lung disease]].
 
==General==
*Better prognosis than [[UIP]].
*May respond to corticosteroids.<ref name=pmid22690098>{{cite journal |author=Lee JY, Jin SM, Lee BJ, ''et al.'' |title=Treatment response and long term follow-up results of nonspecific interstitial pneumonia |journal=J. Korean Med. Sci. |volume=27 |issue=6 |pages=661–7 |year=2012 |month=June |pmid=22690098 |pmc=3369453 |doi=10.3346/jkms.2012.27.6.661 |url=}}</ref>
*Some radiologists and pathologists don't believe in this entity.{{fact}}
 
Associations:<ref name=Ref_WMSP92>{{Ref WMSP|92}}</ref>
*[[Connective tissue disease]].<ref name=pmid21437858>{{cite journal |author=Hauber HP, Bittmann I, Kirsten D |title=[Non-specific interstitial pneumonia (NSIP)] |language=German |journal=Pneumologie |volume=65 |issue=8 |pages=477–83 |year=2011 |month=August |pmid=21437858 |doi=10.1055/s-0030-1256284 |url=}}</ref>
*[[Rheumatoid arthritis]].
 
Note:
*If no underlying cause is present it is known as ''idiopathic NSIP''.
 
Clinical features (typical) of the idiopathic form:<ref name=pmid20178304>{{cite journal |author=Romagnoli M, Poletti V |title=[Update on non-specific interstitial pneumonia: a pattern, a clinical entity or different phenotypes? Surgical or bronchoscopic diagnosis?] |language=Italian |journal=Recenti Prog Med |volume=100 |issue=12 |pages=531–4 |year=2009 |month=December |pmid=20178304 |doi= |url=}}</ref>
*Middle-aged, never-smoker women.
*[[Dyspnea]].
*Cough.
*Ground glass on HRCT.
*Very good prognosis.
 
==Gross/Radiology==
*No honeycombing.
*Fibrosis usually lower lung zone.
*Patchy ground glass.
 
==Microscopic==
Features:<ref name=Ref_WMSP92>{{Ref WMSP|92}}</ref>
*Diffuse fibrosis:
**Uniform fibrosis (unlike [[UIP]]).
**"Linear fibrosis" has a good prognosis - should be mentioned in the report.
***''Linear fibrosis'' = fibrosis that follows alveolar walls + no architectural distortion.
*+/-Lymphoid nodules - association with collagen vascular disease. (???)
*+/-Focal [[organizing pneumonia]].
 
Notes:
*Inflammation in NSIP usually more prominent than in UIP.
*No honeycombing - key difference between UIP and NSIP.
 
DDx:
*[[Collagen vascular disease]].
*[[Drug reaction]].
*[[Hypersensitivity pneumonitis]] (extrinic allergic alveolitis).
*[[Lymphocytic interstitial pneumonia]] (LIP) - much more inflammation.
*[[Organizing pneumonia]].
 
===Images===
<gallery>
Image: Non-specfic_interstitial_pneumonia_(NSIP),_cellular_variant.jpg | NSIP - cellular. (WC/Rosen)
Image: Non-specfic_interstitial_pneumonia_(NSIP),_cellular_variant_2.jpg | NSIP - cellular. (WC/Rosen)
Image: Non-specfic interstitial pneumonia (NSIP), fibrosing variant.jpg  | NSIP - fibrosing. (WC/Rosen)
Image: Non-specfic interstitial pneumonia (NSIP), fibrosing variant_2.jpg  | NSIP - fibrosing. (WC/Rosen)
</gallery>
 
==See also==
*[[Diffuse lung diseases]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Diffuse lung diseases]]

Latest revision as of 18:53, 17 March 2015

Non-specific interstitial pneumonia
Diagnosis in short

NSIP. H&E stain.
LM diffuse fibrosis (uniform fibrosis (unlike UIP), +/-linear fibrosis (fibrosis that follows alveolar walls + no architectural distortion), +/-lymphoid nodules (assoc. with collagen vascular disease), +/-focal organizing pneumonia
Subtypes idiopathic NSIP, NSIP due to an underlying cause
LM DDx organizing pneumonia, collagen vascular disease, drug reaction, hypersensitivity pneumonitis, lymphocytic interstitial pneumonia
Site lung - see diffuse lung diseases
Signs cough
Symptoms dyspnea
Prevalence uncommon
Radiology lower lung zone fibrosis, patchy ground glass, no honeycombing
Prognosis good
Treatment corticosteroids (?)

Non-specific interstitial pneumonia, abbreviated NSIP, is an uncommon type of diffuse lung disease.

General

  • Better prognosis than UIP.
  • May respond to corticosteroids.[1]
  • Some radiologists and pathologists don't believe in this entity.[citation needed]

Associations:[2]

Note:

  • If no underlying cause is present it is known as idiopathic NSIP.

Clinical features (typical) of the idiopathic form:[4]

  • Middle-aged, never-smoker women.
  • Dyspnea.
  • Cough.
  • Ground glass on HRCT.
  • Very good prognosis.

Gross/Radiology

  • No honeycombing.
  • Fibrosis usually lower lung zone.
  • Patchy ground glass.

Microscopic

Features:[2]

  • Diffuse fibrosis:
    • Uniform fibrosis (unlike UIP).
    • "Linear fibrosis" has a good prognosis - should be mentioned in the report.
      • Linear fibrosis = fibrosis that follows alveolar walls + no architectural distortion.
  • +/-Lymphoid nodules - association with collagen vascular disease. (???)
  • +/-Focal organizing pneumonia.

Notes:

  • Inflammation in NSIP usually more prominent than in UIP.
  • No honeycombing - key difference between UIP and NSIP.

DDx:

Images

  • NSIP - cellular. (WC/Rosen)

  • NSIP - cellular. (WC/Rosen)

  • NSIP - fibrosing. (WC/Rosen)

  • NSIP - fibrosing. (WC/Rosen)

See also

References

  1. ↑ Lee JY, Jin SM, Lee BJ, et al. (June 2012). "Treatment response and long term follow-up results of nonspecific interstitial pneumonia". J. Korean Med. Sci. 27 (6): 661–7. doi:10.3346/jkms.2012.27.6.661. PMC 3369453. PMID 22690098. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3369453/.
  2. ↑ 2.0 2.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 92. ISBN 978-0781765275.
  3. ↑ Hauber HP, Bittmann I, Kirsten D (August 2011). "[Non-specific interstitial pneumonia (NSIP)]" (in German). Pneumologie 65 (8): 477–83. doi:10.1055/s-0030-1256284. PMID 21437858.
  4. ↑ Romagnoli M, Poletti V (December 2009). "[Update on non-specific interstitial pneumonia: a pattern, a clinical entity or different phenotypes? Surgical or bronchoscopic diagnosis?]" (in Italian). Recenti Prog Med 100 (12): 531–4. PMID 20178304.
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