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Lymphoid Interstitial Pneumonia*: A Narrative Review

Jeffrey J. Swigris, DO; Gerald J. Berry, MD; Thomas A. Raffin, MD, FCCP; Ware G. Kuschner, MD, FCCP
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*From the Division of Pulmonary and Critical Care Medicine (Drs. Swigris and Raffin), Stanford University, Stanford, CA; the Department of Pathology (Dr. Berry), Stanford University, Stanford, CA; and Veterans Affairs Palo Alto Health Care System (Dr. Kuschner), Palo Alto, CA.

Correspondence to: Jeffrey J. Swigris, DO, Stanford University Medical Center, Division of Pulmonary and Critical Care Medicine, 300 Pasteur Dr, Stanford, CA 94305-5236, e-mail: jjswig@pol.net



Chest. 2002;122(6):2150-2164. doi:10.1378/chest.122.6.2150
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Lymphoid interstitial pneumonia (LIP) is regarded as both a disease and a nonneoplastic, inflammatory pulmonary reaction to various external stimuli or systemic diseases. It is an uncommon condition with incidence and prevalence rates that are largely unknown. Liebow and Carrington originally classified LIP as an idiopathic interstitial pneumonia in 1969. Although LIP had since been removed from that category, the most recent consensus classification sponsored by the American Thoracic Society and the European Respiratory Society recognizes that some cases remain idiopathic in origin, and its clinical, radiographic, and pathologic features warrant the return of LIP to its original classification among the idiopathic interstitial pneumonias. LIP also belongs within a spectrum of pulmonary lymphoproliferative disorders that range in severity from benign, small, airway-centered cellular aggregates to malignant lymphomas. It is characterized by diffuse hyperplasia of bronchus-associated lymphoid tissue. The dominant microscopic feature of LIP is a diffuse, polyclonal lymphoid cell infiltrate surrounding airways and expanding the lung interstitium. Classically, LIP occurs in association with autoimmune diseases, most often Sjögren syndrome. This has led to consideration of an autoimmune etiology for LIP, but its pathogenesis remains poorly understood. Persons who are seropositive for HIV, and children in particular, are at increased risk of acquiring LIP. Some studies suggest causal roles for both HIV and Epstein-Barr virus. The incidence of LIP is approximately twofold greater in women than men. The average age at diagnosis is between 52 years and 56 years. Symptoms of progressive cough and dyspnea predominate. There is great variability in the clinical course of LIP, from resolution without treatment to progressive respiratory failure and death. Although LIP is often regarded as a steroid-responsive condition, and oral corticosteroids continue to be the mainstay of therapy, response is unpredictable. Approximately 33 to 50% of patients die within 5 years of diagnosis, and approximately 5% of cases of LIP transform to lymphoma.

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