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Obstructive Lung Diseases |

Longitudinal Evolution of Lymphocytic Interstitial Pneumonia (LIP) in an HIV-Positive Man

Leslie Jette, MD; Michael Perkins, MD
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Walter Reed National Military Medical Center, Bethesda, MD


Chest. 2013;144(4_MeetingAbstracts):673A. doi:10.1378/chest.1702640
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Abstract

SESSION TITLE: Interstitial Lung Disease Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Lymphocytic Interstitial Pneumonia (LIP) is an exceedingly rare disease of unknown incidence and etiology. Very little is known about the longitudinal course of LIP.

CASE PRESENTATION: A 44 year old man presents with six weeks of productive cough and fevers. These symptoms were initially accompanied by myalgias and nausea that resolved after six days; evaluation was negative for influenza and bacterial pathogens, though his cough and fevers persisted. Medical history includes infection with Human Immunodeficiency Virus (HIV), and Lymphocytic Interstitial Pneumonia (LIP), both diagnosed twenty years previously, and has remained clinically and radiographically stable since that time. Evaluation reveals a fever of 101.5F and faint crackles at the bilateral bases. His laboratory studies are without leukocytosis or neutrophilia, with a CD4 count of 480cells/mm3, and an undetectable viral load. Sputum cultures and interferon gamma assay are negative. Computed tomography (CT scan) revealed progression of bilateral infrahilar interstitial thickening, with stable perivascular cystic changes when compared to a CT scan three years previously. Bronchoalveolar lavage was neutrophil predominant with no organisms noted or evidence of lymphoma by flow cytometry, and transbronchial biopsy was nondiagnostic. After a course of levofloxacin, his fevers gradually improved, though he retained a chronic nonproductive cough. A repeat CT scan with subsequent open lung biopsy is planned to help clarify and differentiate between the progression of LIP and transformation to lymphoma.

DISCUSSION: This is an exceedingly rare disease of uncertain prevalence or etiology that affects patients with autoimmune diseases or acquired immune deficiencies, and causes polyclonal lymphocyte interstitial proliferation of Bronchus-Associated Lymphoid Tissue (BALT). Review of the literature reveals that some patients will die of this disease while others will remain stable or even improve; early evidence points toward the ability of this disease to transform into a lymphoma. New evidence suggests, however, that many early diagnoses of LIP were actually Non-Specific Interstitial Pneumonia (NSIP), calling into question the little we currently know about the behavior of this disease.

CONCLUSIONS: This case serves to highlight the longitudinal progression of LIP in a patient with many years of experience with the disease. It appears that his LIP remained clinically and radiographically stable for 22 years before either progressing in severity or transforming to lymphoma. Additional data is pending to help with this differentiation.

Reference #1: Tian X, ES Yi, JH Ryu. Lymphocytic Interstial Pneumonia and other benign lymphoid disorders. Semin Respir Crit Care Med 2012;33:450-61.

DISCLOSURE: The following authors have nothing to disclose: Leslie Jette, Michael Perkins

No Product/Research Disclosure Information


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