Lung - nontumor
Other interstitial pneumonitis / fibrosis
Lymphoid interstitial pneumonia (LIP)

Author: Elliot Weisenberg, M.D. (see Authors page)

Revised: 1 March 2017, last major update September 2011

Copyright: (c) 2003-2017,, Inc.

PubMed search: lymphoid interstitial pneumonia

Cite this page: Lymphoid interstitial pneumonia (LIP). website. Accessed March 21st, 2018.
Definition / general
  • 1/3 associated with Sjögren's syndrome; also associated with AIDS, pulmonary drug reactions
  • May occur in children with AIDS
  • May be an IgG4 related disease
  • May be EBV related, particularly in non HIV related cases
  • Symptoms: shortness of breath, cough and polyclonal gammopathy
  • Often progressive, long standing cases may culminate in lymphoma
  • Poor response to steroids or chemotherapy
Microscopic (histologic) description
  • Lymphocytes with germinal centers, plasma cells, macrophages and epithelioid granulomas in lung interstitium
  • No effacement of alveolar architecture
  • No invasion of parietal pleura, although visceral pleura may have mild inflammation
  • Late - diffuse interstitial fibrosis
Molecular / cytogenetics description
  • Polyclonal
Differential diagnosis