Non-specific interstitial pneumonia

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Non-specific interstitial pneumonia
Classification and external resources
eMedicine article/2078612

Non-specific interstitial pneumonia (NSIP) is a form of idiopathic interstitial pneumonia.


Symptoms include cough, difficulty breathing, and fatigue.[1]


It has been suggested that idiopathic nonspecific interstitial pneumonia has an autoimmune mechanism, and is a possible complication of undifferentiated connective tissue disease.[2]



Lung biopsies performed on patients with NSIP reveal two different disease patterns - cellular and fibrosing - which are associated with different prognoses. The cellular pattern displays chronic inflammation with minimal fibrosis. The fibrosing pattern displays interstitial fibrosis with various inflammation levels. Both patterns are uniform and lack the prominent fibroblastic foci that are found in other types of idiopathic interstitial pneumonia.[3]


The fibrosing pattern of NSIP has a five year survival rate of 86% to 92%, while the cellular pattern of NSIP has a 100% five year survival rate. Patients with NSIP(whether cellular or fibrosing), have a better prognosis than those with usual interstitial pneumonia (UIP).[4]


  1. ^ Cottin, V; Donsbeck, AV; Revel, D; Loire, R; Cordier, JF (October 1998). "Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients". American Journal of Respiratory and Critical Care Medicine. 158 (4): 1286–93. doi:10.1164/ajrccm.158.4.9802119. PMID 9769293. 
  2. ^ Kinder, Brent W.; Collard, Harold R.; Koth, Laura; Daikh, David I.; Wolters, Paul J.; Elicker, Brett; Jones, Kirk D.; King, Talmadge E. (October 2007). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine. 176 (7): 691–697. doi:10.1164/rccm.200702-220OC. PMC 1994238Freely accessible. PMID 17556720. 
  3. ^ Travis, William D.; Hunninghake, Gary; King, Talmadge E.; Lynch, David A.; Colby, Thomas V.; Galvin, Jeffrey R.; Brown, Kevin K.; Chung, Man Pyo; Cordier, Jean-François; du Bois, Roland M.; Flaherty, Kevin R.; Franks, Teri J.; Hansell, David M.; Hartman, Thomas E.; Kazerooni, Ella A.; Kim, Dong Soon; Kitaichi, Masanori; Koyama, Takashi; Martinez, Fernando J.; Nagai, Sonoko; Midthun, David E.; Müller, Nestor L.; Nicholson, Andrew G.; Raghu, Ganesh; Selman, Moisés; Wells, Athol (15 June 2008). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine. 177 (12): 1338–1347. doi:10.1164/rccm.200611-1685OC. PMID 18388353. 
  4. ^ Travis, William D.; Matsui, Kazuhiro; Moss, Joel; Ferrans, Victor J. (January 2000). "Idiopathic Nonspecific Interstitial Pneumonia: Prognostic Significance of Cellular and Fibrosing Patterns". The American Journal of Surgical Pathology. 24 (1): 19. doi:10.1097/00000478-200001000-00003. PMID 10632484.