Non-specific interstitial pneumonia

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


Symptoms

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

Causes

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

Classification

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]

Prognosis

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]

References

  1. ↑ Cottin, Vincent, et al. "Nonspecific interstitial pneumonia: individualization of a clinicopathologic entity in a series of 12 patients." American journal of respiratory and critical care medicine 158.4 (1998): 1286-1293.
  2. ↑ Kinder, Brent W., et al. "Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?." American journal of respiratory and critical care medicine 176.7 (2007): 691-697.
  3. ↑ Travis, William D., et al. "Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project." American journal of respiratory and critical care medicine 177.12 (2008): 1338-1347.
  4. ↑ Travis, William D., et al. "Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia." The American journal of surgical pathology 24.1 (2000): 19-33.



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