Non-specific interstitial pneumonia

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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

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. //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.