Abstract
Nonspecific interstitial pneumonia is a type of diffuse parenchymal lung disease characterized by interstitial inflammation and fibrosis, without the heterogeneous distribution and peripheral honeycombing, typical of usual interstitial pneumonia (UIP). It is seen in 2 settings as follows: as an idiopathic disease and associated with a variety of other, including rheumatologic, disorders. There is evidence that patients with idiopathic nonspecific interstitial pneumonia often have some autoimmune manifestations that do not fulfill criteria for a specific rheumatologic disease and therefore manifest as a primary autoimmune lung disease. Diffuse lung injury may occur in established cases as a superimposed process (acute exacerbation), similar to UIP, or be present at initial presentation. The distinction from UIP is important because the prognosis is generally far better.
Original language | English (US) |
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Pages (from-to) | 127-131 |
Number of pages | 5 |
Journal | Pathology Case Reviews |
Volume | 18 |
Issue number | 3 |
DOIs | |
State | Published - May 2013 |
Keywords
- Anti-jo-1
- Dermatomyositis
- Interstitial pneumonia
- Polymyositis
- Pulmonary fibrosis
ASJC Scopus subject areas
- Pathology and Forensic Medicine