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non-specific interstitial pneumonia

Wednesday 8 June 2011

nonspecific interstitial pneumonia, NSIP

Links

- Non-specific interstitial pneumonia (NSIP) at the Yale Rosen Collection.

- Non-specfic interstitial pneumonia (NSIP), cellular variant - Case 137.

- Non-specfic interstitial pneumonia (NSIP), fibrosing variant Case 138

Definition: Non-specific interstitial pneumonia (NSIP) is a diagnosis that is made when the lesion doesn’t meet the diagnostic criteria for the other recognized idiopathic interstitial pneumonias (UIP, LIP,COP, etc.)

It is not a specific disease but rather a pathological reaction pattern that may be associated with collagen vascular diseases, drug reactions, hypersensitivity reactions and others. It may be idiopathic.

The abnormality appears identical throughout the specimen, a feature described by the term "temporal uniformity".

It is important to distinguish NSIP from UIP because of the management and prognostic implications.

The prognosis of NSIP is usually significantly better than that of UIP and many patients with NSIP respond well to corticosteroids.

The presence of "temporal uniformity" and absence of fibroblastic foci are helpful in distinguishing NSIP from UIP.

NSIP occurs in two variants:
- 1) a cellular variant without fibroblastic proliferation and fibrosis. (NSIP cellular variant)
- 2) A fibrosing variant exhibiting fibroblastic proliferation and collagen deposition. (NSIP fibrosing variant)

The fibrosing variant has a worse prognosis than the celllular variant.

References

- Nonspecific interstitial pneumonia: a study of 6 patients with progressive disease. Schneider F, Hwang DM, Gibson K, Yousem SA. Am J Surg Pathol. 2012 Jan;36(1):89-93. PMID: 22173118

- Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia. Travis WD, Matsui K, Moss J, Ferrans VJ. Am J Surg Pathol. 2000 Jan;24(1):19-33. PMID: 10632484

- Nonspecific interstitial pneumonia and the other idiopathic interstitial pneumonias: classification and diagnostic criteria. Katzenstein AL, Myers JL. Am J Surg Pathol. 2000 Jan;24(1):1-3. PMID: 10632482