Demystifying idiopathic interstitial pneumonia.
Careful histopathological evaluation has shown the traditionally clinical diagnosis of idiopathic interstitial pneumonia to be more heterogeneous than once thought. Its subclassification, based on clinicopathological criteria, has important therapeutic and prognostic implications. The most important distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of idiopathic pulmonary fibrosis suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. Although the clinical and radiographic diagnosis of idiopathic pulmonary fibrosis can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathological pattern. A structured, clinicopathological approach to the diagnosis of idiopathic interstitial pneumonia, with particular attention to the identification of idiopathic pulmonary fibrosis, ensures proper therapy, enhances prognosis, and allows for further investigation of therapies aimed at the distinct pathophysiology.
Collard
Harold R
HR
Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, USA.
King
Talmadge E
TE
D000208:Acute Disease; D000367:Age Factors; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D001999:Bronchoscopy; D002908:Chronic Disease; D002986:Clinical Trials as Topic; D003937:Diagnosis, Differential; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology* / Q000601:surgery; D017563:Lung Diseases, Interstitial / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology; D016040:Lung Transplantation; D016239:MEDLINE; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000175:diagnosis; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D013256:Steroids; D015996:Survival Rate; D014057:Tomography, X-Ray Computed
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D013256:Steroids