Lymphocytic Interstitial Pneumonia

Clin Chest Med. 2016 Sep;37(3):463-74. doi: 10.1016/j.ccm.2016.04.009. Epub 2016 Jun 25.


Lymphocytic interstitial pneumonia (LIP) is a rare lung disease on the spectrum of benign pulmonary lymphoproliferative disorders. LIP is frequently associated with connective tissue diseases or infections. Idiopathic LIP is rare; every attempt must be made to diagnose underlying conditions when LIP is diagnosed. Computed tomography of the chest in patients with LIP may reveal ground-glass opacities, centrilobular and subpleural nodules, and randomly distributed thin-walled cysts. Demonstrating polyclonality with immunohistochemistry is the key to differentiating LIP from lymphoma. The 5-year mortality remains between 33% and 50% and is likely to vary based on the underlying disease process.

Keywords: Cystic lung disease; Interstitial lung disease; Interstitial lung disease in HIV-positive patients; Interstitial lung disease in Sjögren syndrome; Polyclonal lymphoid lung infiltrates; Pseudolymphoma; Pulmonary lymphoproliferative disorders.

Publication types

  • Review

MeSH terms

  • Bronchoalveolar Lavage
  • Comorbidity
  • Connective Tissue Diseases / immunology
  • Dysgammaglobulinemia / immunology*
  • Epstein-Barr Virus Infections / immunology*
  • HIV Infections / immunology*
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung Diseases / diagnosis
  • Lung Diseases / immunology
  • Lung Diseases / pathology
  • Lung Diseases, Interstitial / diagnosis
  • Lung Diseases, Interstitial / immunology*
  • Lung Diseases, Interstitial / pathology
  • Lymphoproliferative Disorders / diagnosis
  • Lymphoproliferative Disorders / immunology
  • Lymphoproliferative Disorders / pathology
  • Pseudolymphoma / diagnosis
  • Pseudolymphoma / immunology
  • Pseudolymphoma / pathology
  • Sjogren's Syndrome / immunology*
  • Tomography, X-Ray Computed

Supplementary concepts

  • Lymphoid Interstitial Pneumonia