Nonspecific Interstitial Pneumonia Associated With Dermatomyositis With And Without Anti-Jo-1 Autoantibodies

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.