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Lymphoid interstitial pneumonia in adults

Karen A Fagan, MD
Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD


Lymphoid interstitial pneumonia (LIP) is an uncommon form of interstitial lung disease in adults that is characterized histopathologically by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells, and other lymphoreticular elements [1,2]. First described in 1966 by Carrington and Liebow [3], LIP is one entity within a spectrum of lymphoproliferative disorders that can involve the lung. Many of the cases previously diagnosed as LIP are now considered cellular nonspecific interstitial pneumonia (NSIP) [4].

An increased incidence of LIP has been seen in the pediatric population, especially in children with AIDS. (See "Approach to the HIV-infected patient with pulmonary symptoms" and "Lymphocytic interstitial pneumonia in children".)

The etiology, clinical manifestations, diagnosis, and treatment of LIP will be reviewed here. The pathologic classification of the idiopathic interstitial pneumonias and an approach to the evaluation and diagnosis of interstitial lung disease are discussed separately. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing".)

The American Thoracic Society (ATS) and European Respiratory Society (ERS) statement on the classification of idiopathic interstitial pneumonias, as well as other ATS guidelines, can be accessed through the ATS web site at www.thoracic.org/statements.


The lung is occasionally the primary organ of involvement in a variety of lymphoproliferative disorders [5]:

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Literature review current through: Nov 2017. | This topic last updated: Sep 20, 2017.
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