Nonspecific interstitial pneumonia associated with dermatomyositis with and without anti-jo-1 autoantibodies

Thomas A. Huebner*, Seth Kligerman, Rudy J. Castellani, Allen Burke

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review


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 languageEnglish (US)
Pages (from-to)127-131
Number of pages5
JournalPathology Case Reviews
Issue number3
StatePublished - May 2013


  • Anti-jo-1
  • Dermatomyositis
  • Interstitial pneumonia
  • Polymyositis
  • Pulmonary fibrosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


Dive into the research topics of 'Nonspecific interstitial pneumonia associated with dermatomyositis with and without anti-jo-1 autoantibodies'. Together they form a unique fingerprint.

Cite this