EM1511H "Interstitial Pneumonia with Autoimmune Features (IPAF): A New Classification and a Call for Clarity" (IM GR-110615)

The field of interstitial lung disease is challenging in many regards. One of the most important distinctions is differentiating idiopathic interstitial pneumonia and interstitial lung disease associated with connective tissue disease. This distinction can be especially difficult as there are a group of patients that have several features of a connective tissue disease but don’t meet established diagnostic criteria. These patients occupy a gray zone between the two entities. The purpose of this presentation is to describe a new consensus criteria meant to classify the group of patients that occupy the gray zone between idiopathic interstitial pneumonia and interstitial pneumonia associated with well-defined connective tissue disease. It will start by reviewing the idiopathic interstitial pneumonias and then interstitial pneumonia secondary to connective tissue disease and discuss the importance of distinguishing the two. It will then review earlier attempts to codify the group of patients that don’t seem to fit well in either category including the clinical characteristics and available outcomes data. It will then review the recently proposed consensus definition for this group and the proposed criteria. Finally, this presentation will review the justification for the components of the criteria and review the available preliminary data.

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Target Audience

UT Southwestern faculty, fellows, residents and medical students, community physicians, nurse clinicians, physician assistants and nurses.

Learning Objectives

At the conclusion of this activity, the participant should be able to

  • Understand the importance of distinguishing idiopathic interstitial pneumonia from interstitial pneumonia secondary to connective tissue disease.
  • Describe a group of patients that have features of autoimmunity but do not meet established criteria for a connective tissue disease.
  • Review prior attempts at classifying the patients who have some features of autoimmunity plus interstitial disease.
  • Review the recent joint American Thoracic Society/European Respiratory Society consensus definition and diagnostic criteria for this group of patients.
  • Understand the justifications for the diagnostic criteria chosen.
Course summary
Available credit: 
  • 1.00 AMA
Course opens: 
Course expires: 

Photo: Craig Glazer, M.D.Craig Glazer, M.D.
Associate Professor of Internal Medicine
Division of Pulmonary and Critical Care Medicine
Medical Director, Advanced Lung Disease Clinic
Co-Director, Interstitial Lung Disease Program

Available Credit

  • 1.00 AMA


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